Severe IgA Vasculitis Nephritis in Adults: Two Challenging Cases

Introduction: Immunoglobulin A vasculitis (IgAV) in adults is characterized by the combination of skin, gastrointestinal, joint and renal involvement. Yearly incidence is 1-1.8 per 1 million. IgAV nephritis occurs in 45-85% of adults with IgAV, manifesting as acute kidney injury (AKI), active urine sediment and proteinuria. Treatment protocols for severe cases of IgAV nephritis presenting with AKI, nephrotic range proteinuria and crescentic glomerulonephritis (GN) are not validated. We present two cases of severe IgAV nephritis over the span of 2 months. Case Description: Case I – A previously healthy 54-year-old female presented with a lower limb purpuric rash. A skin biopsy was consistent with IgA vasculitis. Severe terminal ileitis developed, and prednisone 40 mg/d led to symptomatic improvement. Shortly after, proteinuria was noted and increased up to protein/creatinine ratio (UPCR) 6.7 g/g and serum creatinine rose from 0.68 mg/dL to 1.78 mg/dL. Kidney biopsy demonstrated proliferative necrotizing GN with >50% crescents and dominant IgA immune deposits (Figure 1). Methylprednisolone 500 mg/d for 3 days followed by prednisone 60 mg daily and IV cyclophosphamide were started; Proteinuria peaked at a UPCR of 14 g/g Therefore, Rituximab was added, total dose of 2 grams, leading to significant improvement (UPCR 0.5 g/g, creatinine 1.3 mg/dL). Case II – A 58-year-old male, with past medical history of diabetes, hypertension and peripheral vascular disease presented with a lower limb purpuric rash. Skin biopsy was consistent with IgAV. Nephrotic syndrome with proteinuria of 6 g/D, leg edema, albumin of 2.6 g/dL and a rise in serum creatinine 0.6 mg/dL to 1.5 mg/dL were observed. Kidney biopsy demonstrated proliferative necrotizing GN with >25% crescents and dominant IgA immune deposits.3 grams IV solumedrol followed by 60 mg/d prednisone, and a total of 6 grams of IV cyclophosphamide were given, with improvement (creatinine to 1.2 mg/dL and UACR below 500 mg/g). Discussion: Evidence is lacking regarding the optimal treatment of severe IgAV nephritis. The above-described cases presented with severe findings clinically and histologically and responded well to aggressive immunosuppression. Case 1 highlights that Rituximab could be added to our 2nd line treatment options and emphasizes the need for studies in IgAV nephritis treatment.