Granulomatosis With Polyangiitis: A Pauci-Immune Rapidly Progressive Glomerulonephritis With Isolated Renal Involvement in an Elderly Male.

Autor: Mir WAY; Internal Medicine, Mount Sinai Hospital, Chicago, USA., Shrestha DB; Medicine, Mount Sinai Hospital, Chicago, USA., Reddy VK; Internal Medicine, Mount Sinai Hospital, Chicago, USA., Adhikari A; Intensive Care Unit, Nepal Korea Friendship Municipality Hospital, Madhyapur Thimi, NPL., Verda L; Internal Medicine, Mount Sinai Hospital, Chicago, USA.
Jazyk: angličtina
Zdroj: Cureus [Cureus] 2021 Aug 11; Vol. 13 (8), pp. e17098. Date of Electronic Publication: 2021 Aug 11 (Print Publication: 2021).
DOI: 10.7759/cureus.17098
Abstrakt: Granulomatosis with polyangiitis (GPA) is a necrotizing vasculitis with upper and lower respiratory tract and renal system involvement. We present a case of a 59-year-old male presenting with complaints of abdominal pain with deranged renal function and acute increase in creatinine level. On investigation, the antineutrophil cytoplasmic autoantibody, cytoplasmic (c-ANCA) was found to be significantly elevated in association with pauci-immune crescentic glomerulonephritis on biopsy. This was diagnostic of Wegener's granulomatosis. He was treated with intravenous cyclophosphamide 10 mg/kg/pulse along with steroids at 1 mg/kg/day for induction and trimethoprim/sulfamethoxazole (TMP-SMX) 80/400 mg for pneumocystis carinii pneumonia (PCP) prophylaxis after a negative tuberculosis QuantiFERON® assay (Qiagen, Netherlands). On discharge, he was on TMP-SMX prophylaxis for PCP, prednisone 60 mg daily, and cyclophosphamide on pulse dosing every 14 days with instructions to follow up. The patient showed improvement in therapy.
Competing Interests: The authors have declared that no competing interests exist.
(Copyright © 2021, Mir et al.)
Databáze: MEDLINE