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Microscopic polyangiitis associated with marked systemic bleeding tendency caused by disseminated intravascular
Takatoshi Saito1, Masahito Tsuchiya, Chihiro Shikata
1Division of Respiratory and Infectious Diseases, the Department of Internal Medicine, Aoto Hospital, Jikei University School of Medicine, Tokyo.
Internal Medicine (Tokyo, Japan)
|October 2, 2003
Summary
Microscopic polyangiitis (MPA) can lead to disseminated intravascular coagulation (DIC), causing severe bleeding. Gabexate mesilate showed effectiveness in managing bleeding complications in this case of ANCA-associated vasculitis.
Area of Science:
- Nephrology
- Rheumatology
- Hematology
Background:
- Microscopic polyangiitis (MPA) is a systemic vasculitis characterized by inflammation of small blood vessels.
- ANCA-associated vasculitis (AAV) can present with diverse clinical manifestations, including renal and pulmonary involvement.
Observation:
- A 57-year-old female presented with severe dyspnea, pulmonary hemorrhage, and rapidly progressive renal failure.
- The patient tested positive for perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA) and exhibited gastrointestinal and brain hemorrhage.
- Laboratory findings revealed thrombocytopenia, prolonged prothrombin time, elevated D-dimer, and markers of disseminated intravascular coagulation (DIC).
Findings:
- The patient was diagnosed with microscopic polyangiitis (MPA) complicated by disseminated intravascular coagulation (DIC).
- Treatment included plasma exchange, hemodialysis, corticosteroids, cyclophosphamide, and gabexate mesilate.
- Continuous infusion of gabexate mesilate demonstrated efficacy in improving the patient's systemic bleeding tendency.
Implications:
- This case highlights the potential co-occurrence of ANCA-associated vasculitis and DIC.
- Gabexate mesilate may represent a valuable therapeutic option for managing bleeding complications in patients with MPA and DIC.
- Further research is warranted to explore the role of gabexate mesilate in AAV-associated coagulopathy.