Educational objective: Review features of Wegener’s granulomatosis.
Wegener’s granulomatosis is the systemic necrotizing, granulomatous vasculitis of the small veins and arteries. It primarily affects the upper and lower respiratory tract and kidneys. Respiratory tract involvement manifests as recurrent otitis media, sinusitis, inflammation of the tracheobronchial tree with mucosal erosions and pneumonitis with multiple cavitations. In addition, saddle nose deformity may occur as well as arthritis, neuropathies and eye inflammation.
Laboratory data are usually nonspecific but c-ANCA (antibodies against proteinase-3 (PR-3) and myeloperoxidase that are found in azurophilic granules of the PMN leucocytes) is very specific, although it still can’t substitute for tissue diagnosis. There are also reports about good correlation between c-ANCA titer and activity of the disease.
Double-stranded DNA is related to SLE, HLA-B27 to Ankylosing spondylitis, Reiter’s syndrome and similar diseases; Jo-1 is related to polymyositis and anticentromere antibody to CREST syndrome.
Educational objective: Review indications for fiberoptic bronchoscopy and high resolution CT scanning.
Fiberoptic bronchoscopy and high resolution CT (HRCT) are, in many ways, complementary to each other. Both of those procedures have advantages in certain clinical situations. In one study HRCT demonstrated all tumors seen on bronchoscopy as well as several which were beyond bronchoscopic range. On the other hand, HRCT could not detect bronchitis or subtle mucosal abnormalities that could be seen on bronchoscopy. In one study HRCT was particularly useful in diagnosing bronchiectasis and aspergillomas, while bronchoscopy was diagnostic of bronchitis and mucosal lesions such as Kaposi’s sarcoma.
The patient in question is at high risk for pulmonary carcinoma; as of today both procedures are considered complementary in this setting.
Educational objective: Review features of assist/control mode of mechanical ventilation.
Assist/control mode of mechanical ventilation is in fact a combination mode. One mode is active while patients have spontaneous breathing effort. In this case, the ventilator delivers breaths of predetermined tidal volume each time the patient initiates the breath (not to the airway pressure). This mechanism of action is called “assist” mode.
However, if the patient fails to initiate a breath for some predetermined period of time, the ventilator will deliver a mechanical breath and continue to deliver a predetermined minimal number of breaths until the patient resumes spontaneous breathing effort. This “backup” respiratory rate is called “controlled” ventilation.
To trigger the ventilator and start the assisted breath, patients must lower the airway pressure to the pre-set amount. This amount of pressure is called “trigger sensitivity”. This mode is used only in intubated patients.
Educational objective: Emphasize benefits of lower tidal volumes during mechanical ventilation of patients with acute lung injury.
Traditionally, a tidal volume for mechanical ventilation was set at 10 ml/kg of body weight, and this still remains the preferred setting for patients without acute lung injury. However, recent clinical trial demonstrated lower mortality in patients with ARDS who received tidal volume of 6 ml/kg than in patients who received 12 ml/kg. It appears (in animal models) that over distension of alveoli by large tidal volumes may induce lung injury (increased pulmonary microvascular permeability, pulmonary edema, and lung rupture).
This Gram stain demonstrates many polymorphonuclear leukocytes and gram-positive cocci in chains and pairs. This is indicative of streptococcus pneumoniae. To be adequate for diagnostic evaluation, a sputum sample must have ¡Ý25 leukocytes per low-power field. In addition, sample should not contain any epithelial cells, because organisms seen in those samples may represent contamination by the oral bacterial flora. Gram stain is a more sensitive diagnostic procedure for pneumococcus than sputum culture.
This organism causes infections of the middle ear, sinuses, trachea, bronchi, and lungs. Infections of the CNS, heart valves, bones, joints, and peritoneal cavity usually arise by hemathogenous spread. Pneumonia caused by pneumococcus is characterized by cough and sputum production, fever, and radiologic detection of infiltrate.
Most adults with this disease have underlying conditions that predispose to infection (outbreaks in military recruits may be exceptions). The most common predisposing condition is preceding upper respiratory viral illness followed by alcoholism, malnutrition, chronic pulmonary disease of any kind, HIV infection, diabetes mellitus, liver cirrhosis, renal insufficiency, and congestive heart failure.
This homeless man is presenting with a clinical picture of lower respiratory infection; sputum specimen stain reveals acid-fast bacilli (not gram-negative rods). In light of this presentation, his condition is most likely pulmonary tuberculosis. Close contact with patient is recognized as the most important risk factor for transmission of the disease. Thus, respiratory isolation is necessary for the period of time that patient is infective. It is general agreement that two weeks after start of the effective (multiple drug) therapy, the patient is no longer infective.
Isolation may include use of monitored rooms with negative air pressure and with minimum of 6 air exchanges per hour. Exhaust of air is to the outdoors or recirculation through HEPA (high-efficiency-particulate-air) filters before recirculation within the hospital. Also, use of masks (approved by the National Institute for Occupational Safety and Health) by caregivers entering isolation rooms is an important part of the isolation.
The classification of pulmonary involvement in sarcoidosis is based upon the radiographic stage of disease:
The radiograph in question exhibits marked interstitial pulmonary infiltrates and hilar and paratracheal adenopathy. This makes his radiographic finding fit into Stage II.
There is no stage V.
It is immediately clear that the right hemithorax is smaller than the left. This is caused by the loss of volume on the right side as evidenced by much steeper descent of the ribs on right side. Whole mediastinum is pulled somewhat to the right (trachea is not in the middle). Region of the right lower lobe is opacified signifying that volume loss occurred in this area. Around the densely opacified area there is also an area of patchy infiltrates indicating that the area is not just atelectatic.
Finally, although some pleural effusion might be present in this patient it is not the sole finding. Pleural effusions have no tendency to cause shrinking of the hemithorax space on the same side.
Hypercalcemia is a relatively common complication of malignancy, occurring in approximately 10-20% of cases. The most common malignancies associated with hypercalcemia are breast and lung cancer and multiple myeloma.
Hypercalcemia of malignancy is due to release of calcium from bone by one of three mechanisms--osteolytic metastases, secretion of PTH-related protein, or secretion of calcitriol by tumor. The most common cause of hypercalcemia with nonmetastatic solid tumors is the release of PTH related peptide (humoral hypercalcemia of malignancy). Patients usually have advanced disease and poor prognosis. Of the lung tumors squamous cell carcinoma has been the most common type of tumor associated with humoral hypercalcemia of malignancy. The small cell lung cancer is associated with many paraneoplastic syndromes (SIADH, Ectopic ACTH etc.), but rarely with hypercalcemia. Other malignancies offered as answers rarely cause paraneoplastic syndromes.
Educational objective: Review imaging modalities for diagnosis of PCP pneumonia.
Chest radiographs are initially normal in up to one fourth of patients with PCP. When positive, diffuse, bilateral interstitial and alveolar infiltrates are most commonly seen. Other features seen include pneumothorax, cysts, nodules, and pleural effusions.
Gallium-67 scintigraphy is a highly sensitive method and may be used to exclude disease, but it requires two days' delay to perform adequate scintigraphic imaging.
High resolution CT-scan (HRCT) of the chest is the method of choice establishing near 100% sensitivity in patients with normal or equivocal chest x-rays. Negative result on HRCT may allow exclusion of PCP in such patients.
MRI imaging has not been evaluated in this clinical situation.
Educational objective: Review the modalities for sputum sampling in patients with PCP pneumonia.
Specific diagnosis of PCP requires demonstration of the organism in respiratory specimens. Gram-stain, however, cannot be used to diagnose the organism. Stains that are used include toluidine blue O, Grocott’s methenamine silver, or Geimsa stain. In addition, an immunoflourescent stain using the monoclonal antibodies may be used and appears to be more sensitive than conventional stains.
Sputum induction by the inhalation of hypertonic saline is the most rapid and least invasive method of obtaining respiratory material. Specificity of this method approaches 100% but sensitivity ranges from 55-92%. PCP prophylaxis, particularly with aerosolized pentamidine, appears to lower diagnostic sensitivity.
Other methods used to obtain sputum samples are bronchioalveolar lavage (sensitivity also suffers if patient takes prophylaxis), transthoracic needle biopsy (rarely used because of high incidence of pneumothorax – up to 30%), and open lung biopsy.
A 74-year-old female patient with past medical history of hypertension, congestive heart failure, chronic renal insufficiency and coronary artery disease underwent triple bypass surgery. After the surgery she had successful recovery. On the fourth week after surgery she became gradually short of breath, started coughing, and developed mild fever. Arterial blood gases (ABG) were obtained and revealed the following: Ph-7.38; pCO2-31.4; PO2–53.2 on oxygen supplementation via nasal canula at 2 l/minute. Chest x-ray was done and showed bilateral pleural effusions, right larger than left, and infiltrate or atelectasis in the right lung base. Thoracocentesis was done on the right side. During the procedure 550 ml of fluid was withdrawn. It was clear and non-bloody. Fluid cultures were negative for bacteria and yeast. Gram stain was also negative.
Laboratory results were as follows: pleural LDH/serum LDH – 0.35 pleural cholesterol - 40 mg/dl pleural protein 2.7 serum protein 5.8
Which of the following is least likely to be the cause of this pleural effusion?
Educational objective: Review diagnostic criteria for differentiation between transudative and exudative pleural fluid.
- We have pleural fluid with characteristics of transudate. A number of chemical tests have been proposed to differentiate pleural transudate from exudates. Criteria proposed by Light are considered "gold standard". These criteria require simultaneous measurement of pleural and serum protein and lactate dehydrogenase. Ratio of pleura/serum LDH >0.6 is a marker of exudate and ratio of pleural/serum protein >0.5 is also marker of exudate. An alternative approach has a sensitivity and specificity comparable to Light’s criteria. The alternative diagnosis markers are: pleural LDH >0.45 of upper limit of normal serum values; pleural cholesterol greater than 45 mg/dl and pleural protein greater than 2.9 g/dl. Our patient's pleural effusion qualifies to be transudate. Of all five above-mentioned conditions the only one which causes exudative pleural effusion is post cardiac injury syndrome. Although the patient is at risk to develop post open-heart surgery pleural effusion the analysis of fluid makes this condition least likely.
The post-cardiac injury syndrome: postpericardiotomy syndrome Post-cardiac injury syndrome develops within days to months after cardiac, pericardial injury or both. It resembles the post-myocardial infarction syndrome, both appearing to be variants of a common immunopathic process. Unlike post-myocardial infarction syndrome, post-cardiac injury syndrome acutely provokes a greater antiheart antibody response (antisarcolemmal and antifibrillary), probably related to more extensive release of antigenic material. Pericardial effusion also occurs after orthotopic heart transplantation (21%). It is more frequent in patients receiving aminocaproic acid during the operation. Cardiac tamponade after open heart surgery is more common following valve surgery (73%) than coronary artery bypass grafting (CABG) alone (24%) and may be related to the preoperative use of anticoagulants. Constrictive pericarditis may also occur after cardiac surgery.
Warfarin administration in patients with early postoperative pericardial effusion imposes the greatest risk, particularly in those who did not undergo pericardiocentesis and drainage of the effusion. Symptomatic treatment is as in acute pericarditis (NSAIDs or colchicine for several weeks or months, even after disappearance of effusion). Long-term (3 to 6 months) oral corticoids or preferably pericardiocentesis and intrapericardial instillation of triamcinolone (300 mg/m2) are therapeutic options in refractory forms. Redo surgery and pericardiectomy are very rarely needed. Primary prevention of postpericardiotomy syndrome using short-term perioperative steroid treatment or colchicine is under investigation.
A 52-year-old woman presents with complaints of fever, cough productive of yellowish sputum, and dyspnea which have lasted for several weeks. She also has experienced fatigue, anorexia and generalized weakness for the same period of time. Her PPD is negative and she is immunocompetent. She has been treated with several antibiotics including ciprofloxacin, ceftriaxone and clarithromycin without success. Lung auscultation reveals bilateral wheezing; the rest of the exam is normal. Chest radiography demonstrates bilateral peripheral infiltrates with small bilateral pleural effusions. Complete blood count is WBC count–5,4 x 109/l, differential count–neutrophil 37%, lymphocytes 19% and eosinophils 42%.
Bronchoscopy was done–-stains and cultures for bacteria: fungi and tuberculosis were all negative. Stool tests for ova and parasites were repeatedly negative. Which one of the following is the most appropriate diagnosis?
Patients with chronic eosinophilic pneumonia are usually in their 50's. The onset of symptoms is usually over several weeks. Symptoms include cough, fever, dyspnea, and weight loss. Asthma is present in about 45% of cases. Peripheral eosinophilia is mandatory. Eosinophils can be found in the sputum of half of the patients. ESR is usually elevated. Chest radiography shows peripheral–based infiltrates located in the outer two thirds of the lung fields in about 65% of patients. Treatment of choice is corticosteroids. Churg–Strauss syndrome is a rare syndrome of asthma, eosinophilia and systemic vasculitis. This patient does not have any signs of vasculitis. Usually Churg–Strauss vasculitis involves the small arteries and veins. Fungal pneumonia is highly unlikely in this immunocompetent patient with negative cultures and status of bronchioalveolar lavage fluid. Acute eosinophilic pneumonia may present with acute febrile illness accompanied by pleuritic chest pain, myalgia and hypoxemic respiratory failure, often requiring mechanical ventilation. The percentage of eosinophils is usually normal in the peripheral blood. Simple pulmonary eosinophilia (Löffler syndrome) is characterized by transient pulmonary infiltrates with peripheral eosinophilia and minimal or pulmonary symptoms.
A 37-year-old African-American man with a two-month history of low-grade fever, dry cough, and fatigue had a chest radiography which revealed bilateral hilar adenopathy and diffuse bilateral reticular infiltrates. Bronchoscopy was performed and biopsy obtained. Histology demonstrated non-caseating granulomas consistent with diagnosis of sarcoidosis.
Which one of the following if presented in this patient would not be considered part of the clinical picture of the above-mentioned disease?
All of the above are clinical features of sarcoidosis except option E. Bronchioalveolar fluid is characterized by high CD4/CD8 ratio and an increase in lymphocytes. This feature is useful in following the activity of the disease in 20% of the patients. Angiotensin converting enzyme (ACE) levels are elevated in 40-80% of patients with active sarcoidosis, but this finding is neither specific nor sensitive enough to have any diagnostic significance. Skin test anergy is present in 70% of patients with active sarcoidosis. Erosive arthritis is another typical extrapulmonary involvement in patients with sarcoidosis.
A 26-year-old woman presents to your office with complaints of episodes of shortness of breath and wheezing. She has had this problem for the last three months. She wakes up during the night feeling severely short of breath; she coughs and wheezes. Her condition improves after several puffs of albuterol. On average she has had symptoms twice weekly--only during the night. She does not have any complaints during the day and has never used Albuterol during the day. She smokes 10-15 cigarettes daily.
Which one of the following is the most appropriate treatment for this patient?
Educational objective: Review treatment for moderate persistent asthma.
This patient presents with symptoms of moderate persistent asthma. Usually the patient’s clinical features before treatment are used to classify the patient. The presence of only one of several features is sufficient to place a patient in a particular category. Patients should be assigned to the most severe grade in which any features occur. For somebody to be classified as having moderate persistent asthma one of the following must be present: daily symptoms or daily use of inhaled short acting beta 2-agonist or nighttime symptoms >1 time a week. Treatment for moderate persistent asthma is inhaled steroid daily (medium dose) or inhaled corticosteroid plus long acting beta 2-agonist. When patient is symptomatic, short acting beta 2-agonist on as-needed basis is recommended.
A 28-year-old female patient was admitted to a local hospital after an automobile accident. During the accident she sustained multiple orthopedic and internal organ injuries. 48 hours after admission Pt became confused and had tachypnea
Lung exam didn’t reveal any abnormal findings. Arterial blood gases revealed pH - 7.46, pCO2 - 31 mm Hg, pO2 - 61 mm Hg. EKG revealed sinus tachycardia with 2 mm elevation of ST-T segment in lead II and aVF. Which one of the following is most likely to confirm the exact diagnosis of this patient’s condition?
Educational objective: Review risk factors for fat embolism.
In persons who have had recent orthopedic injuries there is a high risk of fat embolism syndrome. This disorder usually occurs within 12 hours of injury and may lead to acute respiratory failure and death. Pathognomonic for this condition is the triad of hypoxia, neurologic abnormalities and petechial rash. Petechiae are usually found in the axilla, over the neck and conjunctiva. These lesions are often evanescent, which makes thorough and repeated physical exams crucial. There are no laboratory tests specific for fat embolism syndrome.
A 31-year-old woman presents to her physician's office with complaints of dyspnea and substernal chest pain related to exertion. There is no radiation of the pain to the neck and arms. She has had these complaints for the last 5 months.
Physical exam is unremarkable. Arterial blood gases (ABG) reveal pH-7.46, pCO2 –32 mm/Hg, pO2-74 mm/Hg. EKG reveals right axis deviation. Chest radiography doesn’t show infiltrate, cardiomegaly or pleural effusion, but pulmonary arteries are enlarged. Ventilation perfusion scan is low-probability for pulmonary embolism. The most appropriate next step in the evaluation of this patient’s condition is:
Educational objective: Emphasize diagnostic modalities for primary pulmonary hypertension.
This patient presents with signs and symptoms of primary pulmonary hypertension, an uncommon disorder affecting young women. Early in the illness, patients present with complaints of dyspnea and chest pain on exertion but with normal results on chest x-rays and ABGs. With progression of the disease, signs of right-side heart failure are more evident. Chest x-ray typically shows enlarged pulmonary arteries with or without attenuation of peripheral markings.
Diagnosis is made by documentation of elevated pressures by right side catheterization and exclusion of other pathologic processes. The main consideration in differential diagnosis is pulmonary hypertension secondary to other diseases affecting the lung. Those diseases must be of sufficient severity to be made evident by the history and physical examination.
A 41-year-old man went to the emergency room with a 3-day history of fever and pleuritic chest pain which was localized in the middle of his chest and did not radiate to his arms and chin. He denied shortness of breath and cough. Two weeks earlier he had undergone esophagogastroduodenoscopy (EGD) in conjunction with esophageal and gastric biopsies for symptoms of gastroesophageal reflux.
Physical exam revealed a middle-aged man in acute distress with temperature of 38.7ºC, heart rate 116 beats/minute and respiratory rate of 18 per minute. Blood pressure was 130/85 mm/Hg. There was no evidence of pulsus paradoxus or a pericardial friction rub. Jugular venous pressure and pulsations were normal. Lung exam showed poor inspiratory effort, diminished respiratory excursions bilaterally, and vesicular breath sounds. The rest of the exam was unremarkable.
Laboratory investigation revealed Hb-13.3 g/dl, WBC-16.7x109/ l, and ESR-68 mm/hour. Admission chest radiograph showed widening of mediastinum. EKG was normal. CT scan showed infiltration and irregular densities in the anterior mediastinum.
Which one of the following is not a likely complication of this patient's condition?
Educational objective: Review clinical presentation of acute mediastinitis.
This patient presents with a picture of acute mediastinitis. A history of recent EGD should alert to the possibility of an esophageal perforation. All of the above mentioned are complications of acute mediastinitis. Sternal osteomyelitis is a common complication of mediastinitis after a cardiothoracic surgery but is an infrequent complication of nonsurgical causes of mediastinitis; therefore it is unlikely to occur in this patient.
A 35-year-old Caucasian man presents to the office with complaints of cough, yellowish sputum expectoration, sore throat, and fever for the last 3 days. Chest radiography is normal except for incidental finding of solitary nodule in right middle lobe. CT scan of the chest reveals nodule with diameter of 2.5 cm. Patient’s PPD is 4 mm. His past medical history is unremarkable. He works as a lawyer. He has smoked 1 pack/day for the last 17 years.
Which one of the following features will not be consistent with the eventual malignant nature of this lesion?
Educational objective: Review clinical characteristics of benign and malignant solitary lung nodules.
A lesion is almost certainly benign if the volume doubling time is less than 30 days or more than 500 days. Doubling time between 30 and 500 days is more typical of malignant lesions. Malignant pulmonary nodules are usually larger than 2 cm, often have indistinct margins, and are rarely calcified. Presence of satellite lesions is a factor which favors a benign diagnosis. It is recommended that all solitary nodules in patients older than 35 years be considered potentially malignant and be resected unless calcifications typical of benign lesions or stability on radiography for 2 years is documented.
Educational objective: review features of acute chest syndrome.
Acute chest syndrome is the most common form of acute pulmonary disease in patients with sickle cell disease (SCD), occurring in almost one-half of patients. The syndrome is characterized by chest pain, a new infiltrate on chest radiograph, and fever without bacteremia. It is most frequently reported as a cause of death in adult patients and is a risk factor for early mortality.
Educational objective: Review therapeutic modalities for acute chest syndrome.
Management of acute chest syndrome is primarily supportive and is based upon potential etiologic factors. These include the following.
Although most of the time microbiologic investigations are negative, antibiotics should be used and should cover for community-acquired and atypical organisms.
Supplemental oxygen should be used to maintain a PaO2 of 70-100 mmHg, which is critical in preventing further sickling.
Narcotic analgesics should be used judiciously to avoid potential respiratory depression.
Aggressive hydration is necessary because dehydration also promotes sickling.
Unless thromboembolic phenomena are documented, anticoagulation is not recommended because of the potential risk of intracranial and renal bleeding.
Incentive spirometry is used to prevent atelectasis after rib or sternal infarction.
Exchange transfusion is recommended in the setting of progressive infiltrates and hypoxemia refractory to conventional therapy. Reduction of the hemoglobin S to a level below 30% can lead to marked improvement in the majority of cases.
Flu vaccine is not part of the management of the acute complications of SCD.
In one big study, the following factors were isolated as risk factors for persistent wheezing and development of asthma in children:
Other studies revealed similar results. Current understanding is that there are at least two groups of children who have wheezing and asthma-like symptoms at an early age. One group tends to have intermittent symptoms at an early age, usually in relation to viral illnesses, and to outgrow the symptoms.
The other group tends to have later onset and more persistent symptoms; it is characterized by atopy, eczema, increased IgE level, positive family history of asthma, and is at increased risk for asthma later in life.
Sarcoidosis is a multisystem granulomatous disorder of unknown etiology which affects individuals worldwide. It is characterized pathologically by the presence of noncaseating granulomas in involved organs. It usually affects young adults, and usually presents with one or more of the following features:
Renal involvement in sarcoidosis is certainly not a typical feature of the disease.
Sarcoidosis appears to vary in incidence among geographical regions and can also aggregate in families and specific races. It is 3-4 times more common in blacks. It is estimated that the lifetime risk of sarcoidosis in blacks in the United States is 2.4% and for Caucasians 0.85%. African-Americans also tend to be affected more acutely and severely than whites, who tend to present with asymptomatic and chronic disease.
Despite an extensive research effort, the exact etiology and pathogenesis of sarcoidosis remain unknown.
In addition, radiographs may also show nodular opacities, endobronchial involvement, submucosal granulomas of the airways, and significant stenosis of the airways.
Abnormalities related to calcium metabolism are the most common renal and electrolyte abnormalities in patients with sarcoidosis. This defect in calcium metabolism is caused by extrarenal production of 1,25-dihydroxycholecalciferol (calcitriol) by the activated sarcoid macrophages. Manifestations include increased intestinal calcium absorption, hypercalciuria (50% of cases), hypercalcemia (10-20% of cases), and nephrocalcinosis. Renal calcium deposition may lead to chronic renal failure and end-stage renal disease.
The serum ACE level is elevated in 75% of untreated patients with sarcoidosis. Treatment usually causes decrease in the ACE level. False positive results are unusual and occur in less than 5% of cases (there is, however, a significant number of false negative results). Tissue levels of ACE are usually high in patients with sarcoidosis.
The value of monitoring the ACE level to assess the activity and course of the disease remains unclear at this time.
Biopsy of the affected tissue for confirmation of the diagnosis of sarcoidosis is recommended in all cases (however, some experts believe that biopsy may not be necessary in patients with typical clinical and x-ray findings, uveitis, and erythema nodosum). Biopsy should be performed on accessible lesions including any palpable lymph node, subcutaneous node, cutaneous lesion, enlarged parotid glands, or lacrimal gland.
If there is no easily accessible lesion, fiberoptic bronchoscopy with transbronchial lung biopsy is currently the preferred procedure to obtain pathologic samples for tissue diagnosis. Open lung biopsy or thoracoscopic lung biopsy or mediastinal lymph node biopsy may occasionally be required if less invasive tests prove unsatisfactory.
Bronchioalveolar lavage which demonstrates increased number and percent of activated T-cell, CD4 cells, immunoglobulins, IgG-secreting cells, reduced number of CD8 cells, and an elevated CD4 to CD8 ratio may be used as an adjunct to support the diagnosis, but is nevertheless neither specific nor sensitive enough to be diagnostic in sarcoidosis.
Establishing the diagnosis of sarcoidosis (since there is no pathognomonic laboratory finding) requires recognition of the characteristic clinical findings and /or X-ray findings and histologic confirmation of the presence of noncaseating granulomas.
Which one of the following is also necessary for diagnosis?
There are three essential steps in the diagnosis of sarcoidosis (because there is no single pathognomonic laboratory finding that would confirm the diagnosis).
Recognition of the typical clinical presentation (signs and symptoms) and/or characteristic findings on the x-ray of the chest.
Demonstration of the noncaseating granulomas in the tissue sample (this is an essential component of the diagnosis).
Ruling out infection, particularly tuberculosis (material obtained for histologic evaluation should be sent for acid-fast stain, and cultured for tuberculosis as well as for fungal culture).
The majority of cases of sarcoidosis create minimal diagnostic dilemmas. However, absence of pulmonary manifestations and HIV infection cases may delay the diagnosis.
Sarcoidosis is a multisystem disease characterized by noncaseating granulomas. The granulomas may occur in any organ, with the most commonly affected sites being the lungs, peripheral lymph nodes, skin, eyes, and liver.
Patients may present with a wide variety of signs and symptoms, but a patient with pulmonary sarcoidosis classically presents without symptoms but with an abnormal chest x-ray obtained for an unrelated purpose. When symptomatic, sarcoidosis most commonly presents with an acute or subacute picture (20-40% of cases). These individuals usually have constitutional symptoms such as fever, anorexia, malaise, fatigue, and weight loss associated with respiratory symptoms of dyspnea, dry cough, and vague retrosternal chest discomfort.
A symptom complex consisting of erythema nodosum, bilateral hilar adenopathy on chest x-ray and joint symptoms (arthritis of the ankles, knees, wrists or elbows) represents Löfgren’s syndrome (frequently seen in Scandinavian, Irish, and Puerto Rican females).
A complex of symptoms consisting of parotid enlargement, anterior uveitis, and facial nerve palsy is called Heerfordt-Waldenström syndrome.
The usual indications for the treatment of patients with pulmonary sarcoidosis are the following: o Worsening pulmonary symptoms (cough, shortness of breath, chest pain or discomfort, hemoptysis, etc.) o Deterioration of pulmonary function (assessed by pulmonary function testing at 3-6 month intervals and demonstrated as a fall in total lung capacity of 10% or more, a fall in forced vital capacity of 15% or more, a decrease in diffusing capacity of 20% or more, and decreased gas exchange at rest or with exercise). o Progressive radiographic changes (worsening interstitial opacities, development of cavities, progressive fibrosis – honeycombing or development of pulmonary hypertension)
Treatment is not indicated in following groups of patients: o Asymptomatic patients with Stage I disease. o Asymptomatic patients with Stage II disease and normal or mildly abnormal lung function (mild restrictive or obstructive findings with normal gas exchange). These patients should be followed for 3-6 months to document any progression of the disease before instituting therapy. o Asymptomatic patients with Stage III and normal or mildly abnormal lung function. These patients should be followed for 3-6 months, too, to demonstrate progression (although the majority of these patients will eventually require therapy).
A 45-year-old Caucasian female was recently diagnosed with sarcoidosis after having an abnormal chest x-ray obtained for employment purposes. Radiographic findings corresponded to Stage II. She has been entirely asymptomatic. Follow up chest x-ray obtained 6 months after diagnosis was unchanged. Decision was made not to start treatment. Patient wants to know the prognosis of her condition.
Which one of the following statements about her prognosis is correct?
Most cases of pulmonary sarcoidosis have spontaneous remission. Asymptomatic patients with Stage I disease have a high rate of spontaneous remission (about 70%). Asymptomatic patients with Stage II disease have approximately a 50% chance of spontaneous remission within the first 3 years of the diagnosis. Asymptomatic patients with Stage III disease have only approximately a 33% remission rate within 5 years of diagnosis.
Patients presenting with acute or subacute clinical pictures are usually left without significant sequelae. In approximately 15-20% of cases the disease remains active or recurs intermittently.
This patient fulfills the criteria for starting therapy. Therapy is initiated with relatively high doses of oral prednisone (optimal dose is not known). Slow taper follows this initial phase to the lowest effective dose for a total duration of therapy of 6-12 months. There are three phases in the treatment protocol:
Alternate day therapy with oral prednisone has been also recommended, but there is not enough data to suggest that this kind of management is effective.
There are certain features that confer good or poor prognosis in patients suffering from sarcoidosis.
Good prognostic factors are:
Poor prognostic features are:
In patients unwilling to take steroids, those with intolerable side effects on steroid therapy, those for whom there is progression of the disease despite adequate corticosteroid therapy, or those who require long-term corticosteroid therapy but have been unable to taper below 15 mg/day and have at least one documented problem with corticosteroid toxicity, alternative therapeutic options must be considered.
Several alternative approaches have been proposed, such as the use of immunosuppressive, cytotoxic, and antimalarial drugs. Specific agents include methotrexate, azathioprine, cyclophosphamide, colchicines, Chlorambucil, cyclosporine, chloroquine, hydroxychloroquine, NSAID-s, and pentoxifylline. In addition, radiation has been used for cases of neurosarcoidosis, and organ transplantation has been performed successfully for end stage hepatic, renal, cardiac, or respiratory failure of advanced sarcoidosis.
Busulfan is an alkylating agent that is almost exclusively used in the treatment of chronic granulocytic leukemia. Busulfan was the first cytotoxic drug reportedly associated with pulmonary toxicity (about 5% patients get symptomatic lung injury and a considerably higher number may develop subclinical injury). This drug is not used in treatment of sarcoidosis.
The number of cases of tuberculosis decreased dramatically in the U.S. from 1953 to 1985. After that time there has been resurgence in the number of tuberculosis patients. In large measure this is related to the HIV epidemic. The HIV infection is the greatest known risk factor for reactivating latent tuberculosis infection. The estimated risk for a tuberculin positive individual who acquires HIV infection to develop active tuberculosis is 7-10% per year. As a consequence, TB is particularly prevalent in populations likely to have high HIV infection prevalence (inner city minority populations and intravenous drug abusers). In some populations up to 40% of patients with tuberculosis are HIV positive.
Tuberculosis occurring in foreign-born individuals also has a great role in the resurgence of tuberculosis. In 1994 31% of all tuberculosis patients were foreign-born. People from Mexico, the Philippines, and Vietnam accounted for one-half of all foreign-born cases.
About 50% of the foreign-born cases occur in the first 5 years after arrival in the U.S. Age of more than 5 years upon immigration confers a 2-6 times higher risk for tuberculosis than in individuals of similar age who immigrated before their fifth birthday.
Tuberculosis is a disease that appears with higher incidence in persons of low socioeconomic status. Low income, crowded living conditions, unemployment, and lower educational attainment account for most of the elevated risk for tuberculosis that has been noted in minority populations in the U.S. (African-Americans, Hispanic-Americans, Asian-Americans, and Native-Americans). It is important to recognize that low socioeconomic status confers higher risk in the Caucasian population, too.
Decreased funding of public health services in the U.S. is also a major contributor to the increased incidence of tuberculosis due to substantial deterioration of the public health infrastructure in some large U.S. cities.
The infectious agent of tuberculosis, mycobacterium tuberculosis, is carried on airborne droplets. Droplets are produced when patients with pulmonary tuberculosis cough, sneeze, speak, or sing. The manipulation of lesions or processing of tissue secretions in the hospital or laboratory also may produce them. Infectious droplets are small (1-5 ìm) and air current normally present in any indoor space can keep them airborne for long periods of time. Inhalation of those particles by other persons brings microbes to the alveolar spaces of the lungs, which leads to infection.
Patients with tuberculosis also produce large droplets containing numerous microbes, but these particles are not effective vehicles for transmission. They cannot stay airborne for significant amounts of time and even when inhaled do not reach alveoli. Organisms deposited to the intact skin or mucosa does not invade tissue. Inhaled and deposited to the mucosa of the oral cavity or trachea, these large droplets are carried to the oropharynx and swallowed or expectorated.
There are several techniques of reducing the number of infectious droplets in the air of a room in which a patient is held, thereby preventing the transmission of tuberculosis. Ventilation of the room with fresh air is especially important. It is desirable to exchange the air in the room at least 6 times every hour. Ultraviolet irradiation of the air in the upper parts of the room is also shown to reduce the number of viable airborne tubercle bacilli. Effective antituberculous chemotherapy reduces the number of bacilli released into the air by reducing the number of organisms in the patient’s sputum and also reducing the frequency of coughing. Covering the mouth and nose while coughing or sneezing, with tissues or a mask, reduces the number of microbes released into the air. If a mask is to be used, it should be fabricated to filter out small infectious droplets and to fit tightly around the nose and mouth.
Methods such as disposal of the patient’s personal items (clothes, bedding), and sterilizing fomites, which were once considered important, are unnecessary because they do not prevent airborne transmission. The same is the case with boiling the dishes and washing the walls of the room.
During the primary infection specific immunity is elicited, and this is usually adequate to limit further multiplication of the bacilli. The infected host remains asymptomatic and the lesion heals. In about 5% of all infected persons inflammatory response is inadequate to control the replicating microbe and active disease occurs within one year of infection. In another 5% of patients control and containment of the microorganism fails sometime later in life and active disease results. Thus, about 10% of individuals infected with tuberculosis will develop active disease during their lifetime.
Certain diseases such as silicosis, diabetes mellitus, HIV infection, gastrectomy and use of immunosuppressive drugs increase the likelihood of developing active tuberculosis. Also, susceptibility may be greater during the first 2 years of life and during adolescence.
Indications for yearly tuberculin skin testing in asymptomatic individuals with negative skin tests include the following:
A single tuberculin skin test is appropriate for the following groups:
Often the mistake made by inexperienced readers is to measure the transverse diameter of the erythema on the place of inoculation. The transverse diameter of subcutaneous indurations should be measured and recorded in millimeters. Erythema is present in most patients, but it should be ignored.
There are two methods of measurement. First, use a ballpoint pen to trace a line toward the induration; the reader should stop when he feels resistance of induration. By repeating the procedure on the opposite side, it is easy to get the size of the induration.
The second method is simple palpation of the borders of the induration (this is shown to be little less accurate than the ballpoint pen method).
Taking all newly diagnosed cases of pulmonary tuberculosis and testing them by tuberculin skin test will result in 15-25% of them having negative tests. This may be caused by several different factors. Inadequate nutrition is one of them. Specific cytokines, which inhibit delayed hypersensitivity, are liberated in active disease. The specific form of clinical tuberculosis: more than 50% of patients with disseminated tuberculosis and TB meningitis have negative tuberculin tests; the presence of an immunosuppressive disorder (more than 50% of AIDS patients have negative tuberculin tests).
As a result, a negative tuberculin test cannot be relied on for diagnosis in cases of active tuberculosis.
The American Thoracic Society and the Centers for Disease Control have made the following recommendations for interpretation of the tuberculin skin test:
--5 mm – is considered positive in persons who are very likely to be infected with M. tuberculosis (recent close contacts with active patients) and patients with HIV infection.
--10 mm – is considered positive in persons with moderate likelihood of tuberculosis infection (persons born in high prevalence countries, residents of long term care facilities, medical personnel who might periodically be exposed to active tuberculosis).
--15 mm – is considered positive in individuals with no specific risk factors for tuberculosis (those individuals should not be routinely screened to avoid problems with false positives results).
In certain cases an initial falsely negative tuberculin test can be boosted to a true positive by the initial skin test itself. This usually occurs among individuals who had initial tuberculosis infection many years ago and whose delayed hypersensitivity reaction has markedly diminished with time. Also, it happens in individuals with BCG history and in those who have weak cross-reactions to antigens found in other mycobacteria.
The booster phenomenon is particularly common in populations with a large number of small to intermediate reactions to skin testing. It is frequently useful to detect this phenomenon by placing the second test 1 week following the initial negative test in patients with BCG vaccination or possible remote tuberculosis exposure (two-step test). The detection of these booster reactions decreases the number of false skin test “converters” which may happen in cases of serial skin tests.
Children who receive BCG vaccine tuberculin skin tests usually demonstrate reactions between 3-19 mm several months after inoculation. The size of the reaction does not correlate with the degree of protection conferred by vaccination. Most of those reactions wane in intensity significantly over the ten-year period. Tuberculin skin tests may be used in individuals with a BCG vaccine history, and patients with large reactions are likely to be infected by tuberculosis. Also, individuals who have received BCG vaccine are more likely to have the booster phenomenon and should have two-step tests prior to enrollment into the serial testing program.
Isoniazid (INH) is the most common drug employed in prevention of active tuberculosis in patients with positive tuberculin tests. It has been shown to be effective in this setting. The protective effect of INH prophylaxis may be as high as 90% if all the medication is taken. In a large population when the drug is given by self-administration, observed protective rates are in the 60-70% range. The protective effect lasts at least 20 years and probably for life.
Using isoniazid for tuberculosis prevention requires certain precautions. Patients younger than 35 should be monitored for adverse effects of isoniazid by monthly symptom review (nausea, vomiting, stomach pain, weakness, peripheral neuropathy, dizziness, slurred speech, lethargy, etc). For persons 35 years of age or older, hepatic enzymes should be measured prior to start of therapy and then monthly throughout the treatment in addition to monthly symptom reviews.
Factors associated with increased risk of hepatitis include daily use of alcohol, chronic liver disease, and injection drug use (there are suggestions that postpubertal Hispanic and black women may be at greater risk).
Race does not change risk or interpretation of PPD induration. Believe it or not, even coming from Cleveland doesn't raise his risk!
HIV infection caries such a high risk for progression to active disease that preventive therapy is recommended for all PPD positive, HIV infected patients (positive PPD test in this group of patients means reaction of 5mm or more after 5 tuberculin units intradermal inoculation). In addition to those patients, preventive therapy is recommended in the following circumstances:
Recent contact with a patient with infectious TB, regardless of PPD status or history of previous preventive treatment
A history of prior untreated or inadequately treated TB, regardless of PPD status
HIV positive and PPD negative individuals with unavoidable high risk of TB exposure (residents of long term facilities, or homeless shelters) should receive prophylaxis for the duration of exposure (ie: the duration of their stay at the homeless shelter).
In the immunocompetent host preventive therapy should be treated with isoniazid, rifampin and ethambutol, therapy is continued for 9 months. Breastfeeding in not contraindicated while receiving antituberculous therapy. In HIV-infected pregnant patients, the preventive course of medication should be started immediately (one should not wait even for the end of the first trimester). The only regimen recommended in these circumstances is a 9-month course of isoniazid. No other medications should be used.
Pyrazinamide in combination with rifampin for 2 months is an alternative preventive regimen for HIV positive.
Before initiation of prophylactic therapy in PPD positive patients, the physician should exclude active tuberculosis, obtain any history of prior treatment or prophylaxis, and assess the patient’s risk for toxicity from prophylaxis.
The minimum evaluation for exclusion of active tuberculosis consists of a chest radiograph. If the patient’s chest x-ray (or patient's history) is consistent with active tuberculosis, further studies are warranted. Sputum tuberculosis culture is imperative. Evaluation may also include bronchoscopy. These patients should be treated for active tuberculosis pending the results of cultures. Asymptomatic patients with normal chest x-rays should be started on preventive therapy. However, HIV positive patients with a CD4 count of less than 200 mm3 have a 20% incidence of normal chest x-rays during active tuberculosis, and these patients should have sputum sent for culture regardless of the symptoms or chest x-ray results.
In cases of prior adequate preventive therapy, patients with positive PPD tests should not be treated again unless there has been close contact with active disease in the meantime. A history of previous isoniazid-associated complications (hepatic injury, drug fever, rash or arthritis) is a contraindication for further INH therapy (asymptomatic raise of hepatic transaminases levels to less than 5 times normal does not preclude the treatment).
Gallium 67 lung scintigraphy is of limited value as a means of evaluating patients with interstitial lung disease (ILD). It was used in cases of sarcoidosis but is now being abandoned.
Abnormal chest radiography is often the initial finding in patients with ILD. It is also used to assess the rate of change in disease activity (it is, however, normal in as many as 10% of patients with some forms of ILD – especially hypersensitivity pneumonitis).
High resolution computed tomography (HRCT) is an increasingly important method for the evaluation of diffuse pulmonary parenchymal disease. The accuracy of HRCT in determining specific etiologies of ILD has been inconsistent but is expected to improve with establishment of uniform scanning protocols at different centers.
Complete lung function testing (spirometry, lung volumes and diffusing capacity) should be obtained in all patients with ILD. Pattern of lung injury (restrictive vs. obstructive) helps in narrowing the number of possible diagnoses.
The resting arterial blood gases (ABG) may be normal or may reveal hypoxemia and respiratory alkalosis. Carbon dioxide retention is rare, as is manifestation of end stage disease. Normal resting oxygen saturation does not rule out significant hypoxemia during exercise or sleep. Hence, it is important to perform exercise testing with serial measurements of ABG-s.
Lung biopsy is not required for diagnosis in all patients with suspected ILD. However, following the initial evaluation, it is often not possible to establish a definitive diagnosis or to stage the disease process accurately without a tissue sample. Indications for lung biopsy include the following:
Establishment of specific diagnosis (especially in a patient with atypical or progressive symptoms and signs or rapid clinical deterioration)
Assessment of disease activity
Exclusion of infectious or neoplastic processes that may mimic ILD
Identification of more treatable processes than originally suspected
Establishment of definitive diagnosis and prognosis before institution of aggressive therapies that may have serious side effects
Dosing of steroid therapy is not based on the histological findings but on clinical activity of the disease.
Fiberoptic bronchoscopy with transbronchial lung biopsy is often the initial procedure of choice for tissue sampling. This is especially true if lymphangitic carcinomatosis, eosinophilic pneumonia, Good pasture’s syndrome, pulmonary histiocytosis X, sarcoidosis or infection is suspected. Normal tissue is sometime obtained because of sampling error. In those cases, a larger tissue sample should be obtained by surgical biopsy.
The preferred method for surgical biopsy is video-assisted thoracoscopic lung biopsy (especially if multiple lung tissue samples are required).
A 24-year-old Caucasian male presents in the winter with a one-week history of shortness of breath, dry cough and wheezing. He noticed those symptoms in the morning after a birthday party he attended one week ago and at which he had more drinks than usual.
On physical examination patient is afebrile and in no acute distress while sitting. He talks in complete sentences. Auscultation of the lungs reveals wheezing that can be heard during the first half of every expiration. Wheezing does not change over time and sounds the same regardless of place of auscultation. The patient has a history of asthma as young child, but he does not remember the symptoms. The most likely diagnosis in this patient is:
Widespread, high-pitched, musical wheezes are characteristic of asthma (but not specific). Wheezes are of multiple different pitches, starting and stopping at various points in the respiratory cycle and varying in tone and timing over time.
This is opposed to and needs to be distinguished from the monophasic wheezing of a local bronchial narrowing due to an aspirated foreign body or bronchogenic cancer. This wheeze has a single pitch and repeatedly begins and ends at the same point in each respiratory cycle. Description in this patient matches for local bronchial narrowing. He is young and is unlikely to have developed bronchogenic cancer. He also has possible risk factors for aspiration (drinking at a party). This makes a foreign body in the airway the most likely explanation for his symptoms.
The peak expiratory flow rate (PEFR) is measured during maximal exhalation after a maximal inhalation. The technique requires simple and inexpensive equipment and patients can be taught to measure their performance at home. The results are highly dependent upon patients’ technique and effort. Thus, it is important that the physician assess the patient’s use of the monitor and correct any mistakes.
Forced expiratory volume in one second and forced vital capacity are parts of the spirometric testing, which is a readily available and useful test. It takes 10-15 minutes, uses $2000 instrumentation, costs approximately $40, and carries no risk. The forced expiratory volume in one second is the most important spirometric variable for assessment of airflow obstruction (but is not as practical for every day management of asthma as a PEFR).
Pulse oximetry and arterial blood gases have a role in the management of acute asthma exacerbation but not in the daily management of asthma.
Administration of bronchodilators by MDI is indicated during the spirometry if one suspects an obstructive lung problem. Any bronchodilator may be used, but if patient is known to have angina or episodes of cardiac arrhythmias it may be safer to use a beta-2 selective drug (albuterol). Spirometry should be repeated 15 minutes after inhalation of albuterol. The technique for using the MDI is very important to avoid false negative results.
In a patient with mild to moderate obstruction, an increase in FEV1 of more than 12% and greater than 0.2 L suggests acute bronchodilator responsiveness. However, the lack of response should not preclude a 6-8 week therapeutic trial of bronchodilators and/or corticosteroids.
Patients with symptoms suggestive of asthma but having an entirely normal lung exam and lung function testing may be diagnosed consistently by utilizing serial PEFR measurements. Asthma is characterized by a variable expiratory flow. Patients experiencing asthmatic symptoms can be expected to have significant obstruction and a decrease in their peak expiratory flow compared with when they feel entirely well. On the other hand, individuals without asthma experience little variability in their PEFR over time (less than 20% - even during the respiratory infections).
A reliable series of recordings that documents a more than 20% variability in PEFR over time (especially when PEFR reductions are associated with symptoms) confirms a diagnosis of asthma.
Diagnosis of asthma in patients with normal lung function and normal chest exams may be established by provocation of airflow obstruction using a stimulus known to elicit airway narrowing (bronchoprovocation testing). This procedure is available in most pulmonary function testing laboratories. The provocative stimulus most widely used to evaluate for asthma is inhaled methacholine, although exercise, hyperventilation, inhalation of cold air or histamine may also be used.
Inhalation or intravenous administration of any substance to which patient is allergic should never be attempted.
Asthma is generally classified as mild, moderate and severe based upon history, medication use, and studies of lung function.
Subjective measures of asthma severity:
Objective measures of asthma severity:
Mild intermittent asthma:
Mild persistent asthma:
Moderate persistent asthma:
Severe persistent asthma:
Adenocarcinoma is the one lung cancer most commonly associated with hypertrophic osteoarthropathy (clubbed fingers, etc.) and bronchioalveolar carcinoma is often characterized by the production of a large amount of watery sputum.
Squamous cell cancer often causes hypercalcemia and hypophosphatemia due to production of PTH or PTH-related peptides.
Small cell lung cancer is associated with SIADH, secretion of the atrial natriuretic hormone and ACTH.
Large cell lung cancer is associated with gynecomastia and galactorrhea.
Adenocarcinoma is the one lung cancer most commonly associated with Hypertrophic osteoarthropathy (clubbed fingers, etc.) and bronchioalveolar carcinoma is often characterized by the production of a large amount of watery sputum.
Educational objective: Review the different muscle types of the esophagus.
This patient has lung cancer associated with dermatomyositis as a paraneoplastic syndrome. Because dermatomyositis and polymyositis are diseases of skeletal muscle, his dysphagia is due to weakness of the pharynx, upper esophageal sphincter, and upper 1/3 of the esophagus. This predisposes him to aspiration pneumonia. Other diseases of skeletal muscle function that can cause dysphagia are stroke, multiple sclerosis, amyotrophic lateral sclerosis, and muscular dystrophy. Removal of the cancer often causes resolution of the dermatomyositis.
Educational objective: Review the most effective way of diagnosing smoking.
Smoking remains an important preventable cause of morbidity and mortality in elderly persons. Even individuals older than 70 years of age are shown to have a decrease in the mortality risk after quitting. The majority of smokers who quit relapse, but many succeed, particularly after more than one attempt.
Counseling by the physician and provision of a nicotine patch or gum for assistance have proven to be effective methods of improving the abstinence rate.
Asking patients if they have smoked in the preceding 3 months should be an established routine in medical practice and is more sensitive than asking if the individual smokes now.
Educational objective: Review the strategies for influenza A prevention and therapy.
Infections caused by influenza virus cause significant morbidity and mortality among the elderly population. Vaccination is at least moderately effective in preventing infections among the community- dwelling elderly persons. Serious adverse reactions are rare.
The data for nursing homes is less clear but suggests that decreased infection rates and decreased illness severity occur in highly vaccinated populations. An interesting recent study suggests that vaccination of the staff was the most effective way to prevent influenza morbidity and mortality among nursing home residents.
Health care workers, including physicians, should be immunized. In the event of influenza A outbreak in the community, prophylactic amantadine or rimantadine for nursing home residents is appropriate. Doses of amantadine should be adjusted for age and renal function (100 mg/day is the usual dose in elderly nursing-home residents).
Annual influenza vaccination for individuals over 65 years of age is widely recommended.
Educational objective: Review pulmonary manifestation of SLE.
Systemic lupus erythematosus (SLE) is a disease that affects virtually all organ systems. Several different patterns of pulmonary involvement are recognized. Most common is pleuritis, which may be seen in up to 45% of patients with SLE. Pleural effusion is seen in concert with pleuritis, but only in about 20% of patients. Cough is often mentioned as a feature of pulmonary involvement of SLE, but it appears that cough is most often caused by upper respiratory infections.
Full-blown pneumonitis is relatively rare (1-12%), presents with a clinical picture of pneumonia (even with pulmonary infiltrates), but shows no apparent infection, and pathogens cannot be isolated.
Chronic pneumonitis (fibrosing) appears to follow acute pneumonitis in some patients (usually those with longstanding lupus, and those with anti-Ro antibodies appear to be more prone to develop chronic pneumonitis).
Pulmonary hypertension is a rare complication in SLE and is more commonly associated with Scleroderma, mixed connective tissue disease, and rheumatoid arthritis.
Educational objective: Emphasize clinical features of patients whose asthma is associated with gastroesophageal reflux disease.
Gastroesophageal reflux disease (GERD) should be considered as a potentially important contributing factor in any patient with poorly controlled asthma. In a majority of these patients reflux can be diagnosed by measurement of esophageal pH testing. However, only a minority of patients will have significant GERD symptoms.
Patients who are most likely to benefit from antireflux therapy are those who have significant nocturnal asthma and symptomatic GERD. These patients should be started on either an H2-blocker or a proton pump inhibitor and instructed in conservative management of GERD. Patients who are currently on theophylline may benefit from lowering the dose to diminish blood level and thus diminish the detrimental effect on lower esophageal sphincter function.
The odds are that a upper lobe cavitary lesion is TB until proven otherwise. Always go with your first guess!!
