A physical examination revealed a pale, chronically ill-appearing individual without adenopathy, clubbing, rash, oral lesions, or joint abnormalities. His chest was clear to percussion and auscultation, and there was no hepatosplenomegaly. Chest radiograph showed diffuse interstitial disease without lymphadenopathy. WBC count and differential count were normal. Hematocrit was 19.6%, with microcytic and hypochromic RBC indexes. Serum lactate dehydrogenase levels and arterial blood gas levels obtained with the patient breathing room air were normal. A chest CT scan revealed multiple faint bilateral nodules (Fig 1
top left). Three sputum smears were negative for acid-fast bacilli, but one sputum culture grew Neisseria meningitidis. A sinus CT scan revealed diffuse sinusitis, for which an additional course of ampicillin therapy was administered. HIV serology was negative, the antinuclear antibodies result was positive at 1:80 (speckled pattern), and the results of other rheumatologic and serologic testing, including for antineutrophil cytoplasmic antibodies, were negative. There was no pulmonary hypertension found by transthoracic echocardiogram. A lung biopsy was performed using video-assisted thoracoscopic surgery. While the results were pending, therapy with prednisone (60 mg daily), trimethoprim-sulfamethoxazole for Pneumocystis prophylaxis, and a fluticasone metered-dose inhaler was initiated.