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

被引:0
作者
Mir, Wasey Ali Yadullahi [1 ]
Shrestha, Dhan B. [2 ]
Reddy, Vijay K. [1 ]
Adhikari, Anurag [3 ]
Verda, Larissa [1 ]
机构
[1] Mt Sinai Hosp, Internal Med, Chicago, IL USA
[2] Mt Sinai Hosp, Med, Chicago, IL 60608 USA
[3] Nepal Korea Friendship Municipal Hosp, Intens Care Unit, Madhyapur Thimi, Nepal
关键词
wegener's granulomatosis; antineutrophil cytoplasmic antibodies; granulomatosis with polyangiitis; glomerulonephritis; abdominal pain; WEGENERS-GRANULOMATOSIS;
D O I
10.7759/cureus.17098
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
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 (R) 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.
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