Gabexate Mesylate as Treatment in the Course of ANCA-Negative Microscopic Polyangiitis
| Autor: | Antonietta Gigante, Liborio Sardo, Tamara Jovanovic, Antonio Amoroso, Biagio Barbano, Rosario Cianci, Marta Liberatori, Konstantinos Giannakakis, Maria Ludovica Gasperini |
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| Rok vydání: | 2013 |
| Předmět: |
Male
Pathology medicine.medical_specialty Gabexate medicine.medical_treatment Microscopic Polyangiitis Critical Care and Intensive Care Medicine Gastroenterology chemistry.chemical_compound Internal medicine medicine Humans Rapidly progressive glomerulonephritis cardiovascular diseases immunosuppressive treatment disseminated intravascular coagulation microscopic polyangiitis anca gabexate mesylate Aged Anti-neutrophil cytoplasmic antibody Disseminated intravascular coagulation medicine.diagnostic_test business.industry Anticoagulants General Medicine medicine.disease chemistry Nephrology Plasmapheresis Renal biopsy Vasculitis business Microscopic polyangiitis |
| Zdroj: | Renal Failure. 35:721-724 |
| ISSN: | 1525-6049 0886-022X |
| DOI: | 10.3109/0886022x.2013.780620 |
| Popis: | Patients with small vessel vasculitis present fluctuating antineutrophil cytoplasmic antibodies (ANCA) levels to the point that positive ANCA may be missed even if only up to 10% of patients with microscopic polyangiitis (MPA) are ANCA-negative. The first-line treatment of MPA is the association of steroids and cyclophosphamide, especially in the presence of a rapidly progressive glomerulonephritis. Plasmapheresis, intravenous immunoglobulins, and tumor necrosis factor inhibitors have been proposed as alternative to standard therapy. Disseminated intravascular coagulation (DIC) is a possible event in the course of small vessel vasculitis. Gabexate mesylate is a protease inhibitor able to suppress endothelial cell injury, and it may be administered to treat DIC related to different diseases. In ANCA-associated vasculitis, cytokines play a key role in promoting endothelial damage. DIC-related thrombocytopenia may be misinterpreted as drug-induced because of the immunosuppressive properties of cyclophosphamide. Two cases of ANCA-positive MPA associated with DIC and treated with gabexate are reported in the literature with improvement of both hematological disorder and renal function. Our patient presented a rapidly progressive glomerulonephritis, and the renal biopsy showed MPA, in the absence of ANCA. After two weeks of steroid treatment, our patient developed a DIC. This case represents the first report of ANCA-negative MPA managed with gabexate, which showed improvement of coagulation disorders and kidney function. In conclusion, the anti-inflammatory properties of gabexate could be helpful in MPA at increased bleeding risk when immunosuppressive treatment is contraindicated, even in ANCA-negative vasculitis. |
| Databáze: | OpenAIRE |
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