IgA Nephropathy and IgA Vasculitis, Two Sides of the Same Coin: A Rare Case of Rapidly Progressive IgA Nephropathy with IgA Vasculitis of the Skin
- Glomerular Diseases: Case Reports
November 02, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Trials
- Grivas, Christopher, Georgetown University School of Medicine, Washington, District of Columbia, United States
- Abdalla, Marwa, MedStar Georgetown University Hospital, Washington, District of Columbia, United States
- Kwon, Donghyang, MedStar Georgetown University Hospital, Washington, District of Columbia, United States
- Nilubol, Chanigan, MedStar Georgetown University Hospital, Washington, District of Columbia, United States
IgA Nephropathy (IgAN) is the most common type of primary glomerular disease worldwide. About 25-30% of IgAN patients develop end-stage kidney disease within 20-25 years. IgA vasculitis (IgAV) is a small-vessel vasculitis that affects multiple organs including the skin and kidneys. Renal involvement occurs in 45 to 85% of adults with IgAV. Despite the similarities between IgAN and IgAV, most IgAN trials excluded those with vasculitis. Here we report a case of rapidly progressive glomerulonephritis (RPGN) secondary to IgAN in a patient presenting with IgAV involving the skin.
A 44-year-old Asian man with hypertension, gout, anemia, hypothyroidism and alcoholic cirrhosis presented with profuse epistaxis and pruritic skin rash. The physical examination was notable for palpable purpuric rash on all extremities. His blood urea nitrogen and serum creatinine were 90 and 4.8 mg/dL. His serology tests were negative. C3 was low at 50 mg/dL. He had microscopic hematuria and proteinuria. A skin biopsy showed superficial perivascular lymphohistiocytic infiltrate with perivascular fibrin and 2+ IgA staining. The patient received methylprednisolone and was initiated on hemodialysis. A kidney biopsy showed focal proliferative glomerulonephritis with approximately 18% active cellular crescents in conjunction with ubiquitous IgA mesangial deposits.
The pathophysiology of both IgAN and IgAV is based on the multi-hit hypothesis involving galactosidase deficient IgA and complement pathways. The histologic differentiation between the two diseases remains impossible. Despite the possibility of secondary IgAN from cirrhosis, the evidence of IgAV in the skin of our patient suggested a primary systemic disease. The therapy for IgAN with RPGN is methylprednisolone followed by a combination of oral or intravenous cyclophosphamide and corticosteroids. Rituximab has been reported previously in treatment of IgAV. Due to concerns for cyclophosphamide hepatotoxicity and non-adherence, we recommended rituximab for our patient. He remained dialysis dependent at the date of this report. This report calls for the inclusion of IgAN with IgAV on future clinical trials and a more specific therapeutic guideline for this subgroup of patients.