Abstract: PUB393
"A" Is for Adult: Nonclassic Presentation of IgA Vasculitis
Session Information
Category: Glomerular Diseases
- 1402 Glomerular Diseases: Clinical, Outcomes, and Therapeutics
Authors
- Freibert, Hannah, University of Kentucky College of Medicine, Lexington, Kentucky, United States
- Sims, Tyler, University of Kentucky, Lexington, Kentucky, United States
Introduction
IgA vasculitis (IgAV), formerly Henoch-Schönlein purpura, is an immune-mediated disease which most commonly affects children but also occurs in adults. Here we discuss the diagnosis and treatment of an IgAV patient with a history of multiple chronic immune-mediated disorders.
Case Description
A 46-year-old woman was evaluated by nephrology due to proteinuria in the setting of newly diagnosed leukocytoclastic vasculitis (LCV) on skin biopsy. Patient with history of T2DM, autoimmune hemolytic anemia, eosinophilic asthma on mepolizumab, and chronic sinusitis. The patient presented with one month of pruritic rash with blistering on all extremities and polyarthralgia. Skin punch biopsy revealed small-vessel LCV. Steroid taper initially helped, but symptom regression occurred on decreased steroid dose. She was readmitted and noted to have leukocytosis, eosinopenia, 24-hour urine protein 1528 mg/day. Workup including complement levels, ANA, Anti Beta 2 Glycoprotein, cryoglobulins, ANCA and kappa lambda light chain ratio were all negative. A kidney biopsy showed significant 2-3+ IgA granular staining, minimal IgG staining, minimal c3, normal cellularity, minimal tubular atrophy consistent with IgA vasculitis. A 2nd skin biopsy with immunofluorescence also confirmed the diagnosis. She was started on high dose steroid taper and mycophenolate, alongside lisinopril and empagliflozin in the setting of her proteinuria.
Discussion
IgA vasculitis (IgAV) is an immune-mediated disease which most commonly affects children. It is characterized by IgA and complement deposition along with subsequent leukocyte inflammation, forming immune complexes within affected organs. IgAV may result from an immune reaction to infectious triggers. Patients often have a tetrad of findings: palpable purpura without underlying thrombocytopenia or coagulopathy, arthralgias, abdominal pain, and kidney disease. Biopsy of the dermis or kidney is indicated in unusual presentations or with concern for renal involvement. Pursuing a tissue biopsy to make the diagnosis of IgAV in adults is of notable consequence as adults are at increased risk of recurrent disease and IgAV-associated end stage kidney disease.