Hypocomplementemic Urticarial Vasculitis Syndrome With Crescentic Glomerulonephritis
| Autor: | Mohit Agarwal, Tauqeer Yousuf, Sohail Abdul Salim, Tibor Fülöp, Asha Patel |
|---|---|
| Rok vydání: | 2017 |
| Předmět: |
Adult
Vasculitis medicine.medical_specialty Pathology Abdominal pain Urticaria Glomerulonephritis Membranoproliferative medicine.medical_treatment 030232 urology & nephrology Renal function urologic and male genital diseases Gastroenterology 03 medical and health sciences 0302 clinical medicine Internal medicine Medicine Humans 030212 general & internal medicine Cyclophosphamide Dialysis Hematuria medicine.diagnostic_test business.industry Complement C1q Glomerulonephritis General Medicine Syndrome Mycophenolic Acid medicine.disease Proteinuria Kidney Failure Chronic Rituximab Female Renal biopsy medicine.symptom business medicine.drug Rare disease Kidney disease |
| Zdroj: | The American journal of the medical sciences. 355(2) |
| ISSN: | 1538-2990 |
| Popis: | Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare autoimmune disease characterized by multiple organ system involvement, including renal disease, with low complement levels. We report the case of a 31-year-old woman who presented with nonspecific symptoms including fatigue, diarrhea, macular rash and abdominal pain with acute renal failure leading to end-stage kidney disease. Laboratory results showed hematuria, nephrotic range proteinuria, worsening creatinine and low C1q levels. Left kidney biopsy showed proliferative glomerulonephritis with crescent formation. She was treated with 6 months of intravenous cyclophosphamide, followed by 2 doses of intravenous rituximab (1g each), thereafter maintained on mycophenolate mofetil and glucocorticoid-based therapy. She experienced a full recovery of renal function after 12 months of dialysis dependence. Hypocomplementemic urticarial vasculitis syndrome with crescentic glomerulonephritis is a rare disease with only 5 other reported cases in literature. In our case, we document a delayed but excellent renal recovery during a 2-year follow-up. |
| Databáze: | OpenAIRE |
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