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Subclinical Pulmonary Granulomatosis With Polyangiitis With Dramatic Response to Treatment.

A 17-year-old male adolescent presented to the emergency center because of frothy urine. On review of systems, he endorsed recent onset of fatigue, arthralgias, and a mild dry cough. Physical examination showed small clusters of palpable purpura on the extensor surfaces of the forearms that he first noticed 1 week prior to presentation. His chest auscultation did not reveal any abnormalities or adventitious sounds. The rest of his examination was unremarkable. Vital signs were within reference ranges, including respiratory rate and oxygen saturation. Laboratory evaluation revealed hematuria and proteinuria (1.4 g/24 h), and a renal biopsy showed pauci-immune crescentic glomerulonephritis with segmental fibrinoid necrosis (8% crescents). Subsequently, PR3 ANCA resulted elevated (851 AU/mL), confirming a diagnosis of granulomatosis with polyangiitis (GPA). As part of the evaluation ofGPA, a computed tomography (CT) of the chest was done. It revealed a 4 3.7-cm large left cavitary lung lesion with bilateral ground-glass opacities consistent with pulmonary hemorrhage (Fig. A), with additional scattered pulmonary nodules. He responded to induction with corticosteroids and rituximab (peak creatinine 2 mg/dL, new baseline 1 mg/dL). Azathioprine was added as maintenance therapy. After 8 months, repeat imaging of the chest revealed resolution of the ground-glass opacities and healing of the cavitation into a scar (Fig. B). He is in remission, off corticosteroids, more than 15 months from diagnosis.