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Year : 2023  |  Volume : 23  |  Issue : 1  |  Page : 52-56

A young boy with severe pulmonary-renal syndrome: Will you suspect IgA nephropathy?

1 Department of Internal Medicine, Post Graduate Institute of Medical Education and Research, Chandigarh, India
2 Department of Histopathology, Post Graduate Institute of Medical Education and Research, Chandigarh, India

Correspondence Address:
Ashok Kumar Pannu
4th Floor, F Block, Nehru Hospital, Post Graduate Institute of Medical Education and Research, Chandigarh - 160 012
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2452-2473.357334

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IgA nephropathy is a renal-limited form of systemic vasculitis, and pulmonary manifestations are uncommon. An initial presentation with severe diffuse alveolar hemorrhage (DAH) or pulmonary-renal syndrome is rare and only confined to a few case reports. Herein, we present a young male admitted with acute-onset dyspnea, hemoptysis, and rapidly progressive renal failure. With an initial diagnosis of an immune-mediated pulmonary-renal syndrome, he was treated with high-dose corticosteroids and therapeutic plasmapheresis along with intensive organ support (including hemodialysis, red cell transfusion, and high-flow oxygen). After a detailed laboratory evaluation and kidney biopsy, IgA nephropathy was diagnosed. The patient continued to worsen with persistent DAH and died. IgA nephropathy-associated severe DAH or pulmonary-renal syndrome is rare but increasingly recognized. The condition is difficult to diagnose early and has no proven disease-targeted therapy.

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