Restrictive Lung Disease USMLE Step 1 Practice Question
A 52-year-old woman with a 15-year history of polymyositis presents to clinic with a 3-month history of progressive exertional dyspnea and nonproductive cough. She reports mild arthralgia but denies chest pain, orthopnea, or peripheral edema. Vital signs: BP 128/76 mmHg, HR 102 bpm, RR 22/min, SpO2 92% on room air, temperature 37.2°C. Physical examination reveals fine bibasilar crackles and 4/5 hip flexor strength bilaterally. Pulmonary function tests show FVC 58% predicted, FEV1 61% predicted, FEV1/FVC ratio 0.82. DLCO is markedly reduced at 35% predicted. High-resolution CT of the chest demonstrates basilar-predominant reticular opacities with traction bronchiectasis. Transthoracic echocardiogram shows normal left ventricular function and estimated RVSP of 35 mmHg. Which of the following best explains this clinical presentation?
Answer choices
- APolymyositis-associated interstitial lung disease with usual interstitial pneumonia (UIP) patternCorrect answer
- BAcute decompensated heart failure with pulmonary edema secondary to diastolic dysfunction
- CPolymyositis-associated pulmonary hypertension with right ventricular dysfunction
- DAspergillus fumigatus infection with chronic cavitary lung disease
- EDrug-induced interstitial pneumonitis from methotrexate therapy
- FDermatomyositis with skin-predominant disease and incidental pulmonary nodules
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