A RARE CASE OF ANCA-NEGATIVE PAUCI-IMMUNE CRESCENTIC GLOMERULONEPHRITIS IN AN ADOLESCENT MALE

Introduction:

              Pauci-immune crescentic glomerulonephritis (PICG) is a potentially life-threatening condition leading to renal failure within days or weeks. Only 10% of PICG are Antineutrophil cytoplasmic antibodies (ANCA) negative. Here we present a case of ANCA negative PICG in an Adolescent male.

Methods:

A 13 year old male with no comorbidities presented to the emergency department with fever for 1wk, body swelling for 1wk, cola-coloured urine for 3days, decrease urine output for 2 days.

On evaluation patient was found to have peri orbital puffiness, pallor, ascites, bilateral pedal edema with normal blood pressure. Urine dipstick showed protein 3+++, blood 3+++. On further urine microscopy showed presence of RBC 20-25/hpf. On blood examination Hb 10.4 gm/dl, urea 139 mg/dl, creatinine3.4 mg/dl, which rapidly increased to urea 210 mg/dl ,creatinine 6.8 mg/dl over the next 3days.

Ultrasound showed preserved kidney size with raised cortical echogenicity with maintained CMD. On further workup ANA, ANCA, Anti-GBM and complement panel all were negative. In view of rapidly declining renal function with volume overload related symptoms due to oliguria patient was initiated hemodialysis. After few sessions of hemodialysis patient underwent renal biopsy.

Results:

Kidney biopsy: Pauci-immune crescentic GN

                           Patient was started on IV Methylprednisolone 500mg daily for 3 days, followed by prednisone 1mg/kg. He also received inj. Cyclophosphamide as per EUVAS Protocol. Patients urine output showed increasing trend after 3 weeks of therapy. His hemodialysis session was subsequently spaced and patient became dialysis independent on 5th week of therapy.

Conclusions:

                       ANCA negative Pauci-immune crescentic glomerulonephritis are very rare. we conclude that early diagnosis and early initiation of IV methylprednisolone  and cyclophosphamide with renal replacement therapy (RRT) support is crucial in complete recovery of renal function.

I have no potential conflict of interest to disclose.

I did not use generative AI and AI-assisted technologies in the writing process.