A young boy with severe pulmonary-renal syndrome: Will you suspect IgA nephropathy?
1Department of Internal Medicine, Post Graduate Institute of Medical Education and Research, Chandigarh, India
2Department of Histopathology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
Keywords: Berger’s disease, diffuse alveolar hemorrhage, glomerulonephritis, IgA nephropathy, pulmonary‑renal syndrome
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.
How to cite this article: Grewal TS, Soni D, Nada R, Sharma N, Pannu AK. A young boy with severe pulmonary-renal syndrome: Will you suspect IgA nephropathy?. Turk J Emerg Med 2023;23:52-6.
Written informed patient consent/next of kin are present. The patient’s brother was explained about the confidentiality, and the case information will be used for education purposes only.
1 TSG: collected patient data, drafted the manuscript.
2DS, 3 RN: collected patient data.
4 NS: revised the manuscript.
5 AKP: drafted and revised the manuscript.
The corresponding author is responsible for ensuring that the descriptions are accurate and agreed by all authors.