A CASE OF ADULT ONSET CRESCENTIC HENOCH-SCHÖNLEIN PURPURA NEPHRITIS (HSPN) WITH MASSIVE PROTEINURIA AND SEVERE RENAL INSUFFICIENCY

K ABDUL RAZAK1,3, T HAN1,2,Z THET1,2
1Department Of Nephrology, Rockhampton Base Hospital, Rockhampton, Australia, 2Rural Clinical School, University of Queensland, Rockhampton, Australia, 3School of Medicine, Griffith University, Gold Coast, Australia

Background: The clinical presentation of HSPN in adults is severe with relatively poor outcome. As HSPN primarily affects children, evidence on the treatment of HSPN in adults is quite limited. We report a case of severe HSPN with acute kidney injury and proteinuria that was treated successfully.
Case report: 53 year old obese female, with a history of type 2 diabetes mellitus and hypertension presented with nonblanching purpuric vasculitic rash on anterior abdominal wall and posterior surface of both arms. Vasculitis was associated with nephrotic range proteinuria (30g/day), microscopic hematuria and acute kidney injury with eGFR 43ml/min/1.73m2, creatinine 123µmol/L, urea 8.0 mmol/L and albumin 30g/L on admission that later declined to eGFR 20ml/min/1.73m2 and creatinine 231µmol/L during the hospital stay. There was no arthralgia, gastrointestinal symptoms or macroscopic hematuria. She was treated with oral prednisolone after skin biopsy report of florid small vessel or leukocytoclastic vasculitis, negative for immunofluorescence staining. Vasculitis screenings were negative for ANA, anti-dsDNA, anti-GBM, ANCA, hepatitis B, hepatitis C and serum C3 and C4 levels. Renal biopsy reported crescentic glomerular nephritis containing 93% cellular crescents and IgA deposits following which oral cyclophosphamide was initiated. Skin rashes subsided and renal disease improved to eGFR 61ml/min/1.73m2 and creatinine 93µmol/L but proteinuria persisted at 11g/day. Cyclophosphamide was switched to cyclosporin after 6 months of treatment and oral steroid tapered. A few months after initiation of cyclosporine, proteinuria improved persistently at <1g/day.
Conclusion: This case highlights the severity of renal involvement in adult-onset HSPN. Aggressive treatment is required when severe crescentic nephritis and nephrotic syndrome are observed simultaneously at presentation in adult HSPN and the treatment can alter the course of renal disease.


Biography:
Specialist Physician, Rockhampton Base Hospital, Queensland, Senior Lecturer, Rural Clinical School, Rockhampton, Queensland

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