CTICasesIsolated Pauci-Immune Pulmonary Capillaritis
Imaging findings
Chest radiograph and CT show bilateral ground-glass opacities and crazy paving with superimposed reticulation, representing diffuse alveolar hemorrhage. The opacities resolve and then recur over several months. Surgical lung biopsy confirms pulmonary capillaritis. Systemic serologies, renal function, and skin biopsies are normal.
Key takeaways
Isolated pauci-immune pulmonary capillaritis is a rare, lung-limited vasculitis that causes recurrent episodes of diffuse alveolar hemorrhage. It is characterized histologically by neutrophilic infiltration of the alveolar septa and capillaritis without systemic vasculitis or autoantibody markers (pauci-immune). It typically responds to high-dose corticosteroids and immunosuppressive therapy (e.g., rituximab).
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