Introduction:
IgA nephropathy (IgAN) is the most common primary glomerulonephritis worldwide. The role of ANCAs in the prognosis of IgA nephropathy is currently unknown.
Methods:
A 55-year-old woman with a history of well-controlled type 2 diabetes mellitus and normal baseline renal function 6 months prior presented with two months of abdominal distension and nausea, unresponsive to treatment, accompanied by three weeks of macroscopic hematuria. Laboratory tests revealed progressive deterioration of renal function, leading to her referral to our service with a serum creatinine of 3.2 mg/dl, hypertension, oliguria, and active urinary sediment. She was managed as rapidly progressive glomerulonephritis and treated with three doses of 500 mg methylprednisolone, followed by a kidney biopsy. Further workup showed decreased C3, P-ANCA 1:320, ANAs AC-4 1:320, AC-19 1:640, AC-26 1:320, Anti-MPO > 200, and negative anti-GBM antibodies. The biopsy revealed IgA nephropathy (M1, E1, S1, T1, C2) with 41% cellular crescents and fibrinoid necrosis. Due to the presence of ANCAs, the number and type of crescents, intravenous cyclophosphamide was initiated using a CYCLOPS regimen adjusted for creatinine (2.5 mg/kg), along with 30 mg/day prednisone, enalapril, atorvastatin, and dapagliflozin. The patient had a serum creatinine of 4.3 mg/dl at the time of the first cyclophosphamide dose, with progressive improvement over the following weeks. A year later the patient has GFR of 65 ml/min and proteinuria is not present.
Results:
Patients with diabetes should be evaluated for other causes of kidney disease, as in this case, where IgAN was identified. Crescents are found in less than 10% of IgAN cases, while positive ANCAs represent only 0.2–2%.
Conclusions:
Early treatment in crescentic glomerulonephritis has a positive impact on renal prognosis. Hematuria may be a manifestation of small vessel vasculitis in the presence of IgAN.
I have no potential conflict of interest to disclose.
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