A 60-year-old man has had a chronic cough with clear sputum production for over 5 years. He has smoked one pack of cigarettes per day for 40 years and continues to do so. X-ray of the chest shows hyperinflation without infiltrates. Arterial blood gases show pH of 7.38, P CO2 of 40 mm Hg, PO2 of 65 mm Hg, O2 saturation of 93%. Spirometry shows an FEV1 /FVC of 45% without bronchodilator response. Which of the following is the most important treatment modality for this patient?
This patient’s chronic cough, hyperinflated lungs, abnormal pulmonary function tests, and smoking history are all consistent with chronic bronchitis. A smoking cessation program can decrease the rate of lung deterioration and is successful in as many as 40% of patients, particularly when the physician gives a strong antismoking message and uses both counseling and nicotine replacement. Continuous low-flow oxygen becomes beneficial when resting arterial oxygen saturation falls below 88%. Inhaled beta agonists or anticholinergics such as ipratropium or tiotropium are the cornerstones of symptomatic therapy but do not prevent progression of airways obstruction if the patient continues to smoke. Antibiotics are indicated only for acute exacerbations of chronic lung disease, which present with fever, change in sputum color, and increasing shortness of breath. Oral corticosteroids are helpful in acute exacerbations, but their side-effect profile precludes chronic use. Theophylline is a fourth-line treatment in COPD.
A 57-year-old man presents with hemoptysis and generalized weakness. His symptoms began with small-volume hemoptysis 4 weeks ago. Over the past 2 weeks, he has become weak and feels “out of it.” His appetite has diminished, and he has lost 10 lb of weight. He has a 45-pack year history of cigarette smoking. Physical examination is unremarkable. Laboratory studies reveal a mild anemia and a serum sodium value of 118 mEq/L. Chest x-ray shows a 5-cm left mid-lung field mass with widening of the mediastinum suggesting mediastinal lymphadenopathy. MR scan of the brain is unremarkable. What is the most likely cause of his symptoms?
Hyponatremia in association with a lung mass usually indicates small cell lung cancer (SCLC) with inappropriate ADH production by the tumor. About 10% of lung cancers present with a paraneoplastic syndrome. Tumors producing ADH or ACTH are overwhelmingly SCLCs, which arise from hormonally active neuroendocrine cells. SCLC is a rapidly growing neoplasm; early mediastinal involvement, as in this case, is common. Tumor staging for SCLC differs from non–small cell cancers. SCLCs are simply classified as limited (confined to one hemithorax) or extensive. Limited tumors are usually managed with combination radiation and chemotherapy, with approximately 20% cure rate. Extensive tumors are treated with palliative chemotherapy alone; durable remissions are rare. Surgery is not curative in SCLC. Bronchial carcinoids are usually benign. Although they can produce ACTH, mediastinal involvement and hyponatremia would not be expected. Adenocarcinoma of the lung, although common, rarely causes a paraneoplastic syndrome. Localized benign lung infections (especially lung abscess) can cause SIADH, but would not account for this patient’s mediastinal adenopathy. Lung abscess usually causes fever and fetid sputum. Pulmonary aspergilloma (a fungus ball growing in an old cavitary lesion) can cause hemoptysis but not this patient’s hyponatremia or mediastinal lymphadenopathy.
A 42-year-old woman presents with gradually worsening dyspnea over the preceding 6 months. She has a mild nonproductive cough. She previously had been diagnosed with systemic sclerosis (scleroderma) but her skin thickening has been stable. She controls her Raynaud syndrome with amlodipine and her esophageal reflux with daily omeprazole. She has no renal disease or hypertension. On physical examination, her RR is 22/minute and resting O2 saturation is 92%. She has thickened, hide-bound skin on the face, torso, and abdomen. Lung examination shows mild “Velcro” rales in the bases bilaterally. Neck veins are flat. Cardiac examination is normal with normal P 2 and no lift or heave. Chest x-ray shows increased interstitial lung markings and a normal heart size. What is the most important next step in evaluating this patient’s dyspnea?
This patient has interstitial lung disease (ILD) due to her systemic sclerosis. Over 75% of patients will have CT evidence of ILD. Now that scleroderma renal crisis can be managed with ACE inhibitors, ILD is the most common disease related cause of death in scleroderma. The two most important studies for the diagnosis of ILD are spirometry with measurement of diffusion capacity of carbon monoxide (DLCO) and high-resolution CT scan. The latter will show reticular interstitial thickening and subpleural microblebs. Advanced cases will show thickened fibrotic bands with parenchymal destruction known as honeycombing. Treatment of connective tissue disease associated ILD is unsatisfactory, but cyclophosphamide will slow progression in some patients. In addition to systemic sclerosis, ILD is an important potential complication of polymyositis/dermatomyositis, rheumatoid arthritis, and occasionally systemic lupus. An arterial blood gas study would probably show alveolar hyperventilation (ie, low PCO2 ) with a widened alveolar-arterial oxygen gradient but would not give specific information as to cause and prognosis. In most cases, finger oxygen saturation measurements provide sufficient information. Pulmonary hypertension can be detected with 2D echocardiogram according to the degree of tricuspid regurgitation. Pulmonary hypertension can be an important complication of connective tissue disease (especially limited scleroderma but sometimes diffuse scleroderma as well). You would expect, however, a loud P 2 and evidence of central pulmonary artery enlargement on CXR. In addition, Velcro rales and increased interstitial markings are not seen in uncomplicated pulmonary hypertension. Autoantibodies are usually found in systemic sclerosis, but the diagnosis is already established. The titer of anti-Scl antibodies does not correlate with disease activity; so once the diagnosis is established, serial measurement of autoantibodies is not necessary. Aspiration due to esophageal dysmotility can complicate scleroderma, but usually causes intermittent exacerbations associated with sputum production, fever, and alveolar infiltrates, none of which has characterized this patient’s course.
A 30-year-old quadriplegic man presents to the emergency room with fever, dyspnea, and a cough. He has a chronic indwelling Foley catheter. Recurrent urinary tract infections have been a problem for a number of years. He has been on therapy to suppress the urinary tract infections. On examination, the patient has a temperature of 38°C (100.4°F), HR 88, and BP 126/76. Mild wheezing is audible over both lungs. A diffuse erythematous rash is noted. The chest x-ray shows diffuse alveolar infiltrates. The CBC reveals a WBC of 13,500, with 50% segmented cells, 30% lymphocytes, and 20% eosinophils. Which of the following is the most likely diagnosis?
Clues to this diagnosis are recurrent urinary tract infections and the use of suppressive therapy to control these infections. Nitrofurantoin is commonly used for this purpose. Nitrofurantoin can cause an acute hypersensitivity pneumonitis. This condition can progress to a chronic alveolitis with pulmonary fibrosis. The presenting symptoms are fever, chills, cough, and bronchospasm. In addition, the patient may experience arthralgias, myalgias, and an erythematous rash. The chest x-ray will show interstitial or alveolar infiltrates. CBC often shows leukocytosis with a high percentage of eosinophils. The treatment is to discontinue the nitrofurantoin, and to begin corticosteroids. Sepsis secondary to a urinary tract infection and healthcare-related pneumonia might be considered. However, these would not present with a diffuse erythroderma or eosinophilia. Acute bacterial infections cause a neutrophilic leukocytosis; eosinophils are usually undetectable owing to the stress effect of catecholamines and cortisol. COPD rarely presents in a 30-year-old. Lymphocytic interstitial pneumonia is a rare disease and would cause interstitial rather than alveolar infiltrates. Lung biopsy to establish the diagnosis of an interstitial lung disease would be considered only after the potentially offending drug had been discontinued.
A 35-year-old woman complains of slowly progressive dyspnea. Her past history is negative, and there is no cough, sputum production, pleuritic chest pain, or thrombophlebitis. She has taken appetite suppressants at different times. Physical examination reveals jugular venous distention, a palpable right ventricular lift, and a loud P2 heart sound. Chest x-ray shows clear lung fields. Oxygen saturation is 94%. ECG shows right axis deviation. A perfusion lung scan is normal, with no segmental deficits. Which of the following is the most likely diagnosis?
Although a difficult diagnosis to make, primary pulmonary hypertension is the most likely diagnosis in this young woman who has used appetite suppressants. Primary pulmonary hypertension in the United States has been associated with fenfluramines. The predominant symptom is dyspnea, which is usually not apparent until the disease has advanced. When physical findings, chest x-ray, or echocardiography suggest pulmonary hypertension, recurrent pulmonary emboli must be ruled out. In this case, a normal perfusion lung scan makes pulmonary angiography unnecessary. Right-to-left cardiac shunts cause hypoxia (oxygen desaturation) that characteristically does not improve with oxygen supplementation. Restrictive lung disease should be ruled out with pulmonary function testing but is unlikely with a normal chest x-ray. An echocardiogram will show right ventricular enlargement and a reduction in the left ventricle size consistent with right ventricular pressure overload. Left ventricular diastolic dysfunction can cause pulmonary edema but not pulmonary hypertension.
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