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논문분류	춘계학술대회 초록집
제목	Henoch-Schonlein Nephritis in an Adult Woman Presenting as ANCA-Negative Pauci-Immune Crescentic Glomerulonephritis
저자	Diane Tabucanon
출판정보	2024; 2024(1):
키워드
초록	Henoch-Schonlein Purpura nephritis (HSPN) has received considerable attention in pediatric research, yet its progression in adults remains relatively understudied. Pauci-immune crescentic glomerulonephritis (CrGN) is the leading cause of RPGN and in most instances are associated with detectable ANCAs. We report a rare but important association of ANCA-negative pauci-immune glomerulonephritis and HSPN in a 54-year-old female with diabetes and hypertension who, presented with abdominal pain, purpuric rashes, edema and oliguria. Diagnostics revealed normal platelets but with significantly elevated creatinine along with the presence of hematuria and proteinuria consistent with HSPN. C3 and C4 levels were normal, and the absence of C-ANCA and P-ANCA ruled out autoimmune conditions like lupus and vasculitides. Skin biopsy revealed small vessel capillary vasculitis consistent with HSP (Figure1). Kidney biopsy showed crescentic glomerulonephritis with scanty granular immune deposits of IgA, IgG, IgM and C3, indicating a pauci-immune necrotizing CrGN (Figure 2). The patient underwent hemodialysis and received methylprednisolone pulse therapy followed by treatment with Prednisone and Mycophenolate mofetil. A gradual improvement in kidney function was observed, and became dialysis-free after six weeks. This case presents a highly uncommon manifestation of renal complications in HSP, alongside an ANCA-negative pauci-immune glomerulonephritis. It highlights the critical importance of thorough clinical evaluation, serologic testing, and a high degree of suspicion to uncover less common and occasionally overlooked diagnoses. While no standardized treatment protocol exists, therapeutic decisions were based on careful assessment of organ involvement and disease severity. In this case, effective disease management was achieved through temporary hemodialysis, corticosteroids and immunosuppression.
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