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A YOUNG BOY WITH SEVERE PULMONARY-RENAL SYNDROME: WILL YOU SUSPECT IGA NEPHROPATHY?

TEJİNDERPAL SİNGH GREWAL, DİPESH SONİ, RİTAMBHRA NADA, NAVNEET SHARMA, ASHOK KUMAR PANNU

Türkiye Acil Tıp Dergisi - 2023;23(1):52-56

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

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.