Chest
Pulmonary and Critical Care PearlsDyspnea and Muscle Weakness in a 65-Year-Old Woman
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Physical Examination
Vital signs: normal except for respiratory rate of 24 breaths/min. Chest: anterior and posterior bibasilar mid-late inspiratory crackles. Cardiac: grade 2/6 systolic murmur at left sternal border. Extremities: no cyanosis or clubbing; peripheral edema of ankles and lower legs. Neurologic: marked and diffuse muscle weakness, particularly involving the proximal muscles. Skin: normal.
Laboratory Findings
Hematocrit, 40.5 percent; WBC, 7,800 µl with normal differential; creatine phosphokinase, 3,959 IU/L; lactate dehydrogenase, 2,287 IU/L. Rheumatoid factor: negative. Thyroid function: normal. Chest radiograph: bilateral lower lobe infiltrates with small left pleural effusion (Fig 1). Pulmonary function tests: forced vital capacity (FVC), −1.67 L (55 percent predicted); total lung capacity, 3.35 L (66 percent predicted); FEV1, 1.34 L (60 percent predicted); FEV1 FVC ratio, 80; diffusing capacity
Diagnosis: Polymyositis with bronchiolitis obliterans with organizing pneumonitis
Since its original description in 1956, the association between parenchymal lung disease and polymyositis has become well established. Open lung biopsy specimens from patients with polymyositis/dermatomyositis have demonstrated three major histologic patterns: bronchiolitis obliterans with organizing pneumonitis (BOOP); usual interstitial pneumonia; and diffuse alveolar damage. The histologic categorization of the lung biopsy specimen in a patient with polymyositis/dermatomyositis may better
Clinical Pearls
- 1.
Pulmonary manifestations of polymyositis include respiratory muscle weakness, various interstitial lung diseases, aspiration pneumonia secondary to dysphagia, pleural effusions, and pulmonary alveolar proteinosis. The presence of BOOP in polymyositis has a more favorable prognosis than usual interstitial pneumonitis or diffuse alveolar damage.
- 2.
Measurement of inspiratory and expiratory mouth pressures is important in patients with neuromuscular disease because the severity of respiratory muscle
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