Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) syndrome and pulmonary thromboembolism: an overlooked concomitance

• Main Authors: Vilma Takayasu, Aloisio Felipe-Silva
• Format: Article
• Language: English
• Published: University of São Paulo 2013-06-01
• Series: Autopsy and Case Reports
• Subjects: Churg–Strauss Syndrome; Granulomatous Allergic Angiitis; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Asthma; Pulmonary Embolism; Autopsy.
• Online Access: http://www.revistas.usp.br/autopsy/article/view/58910

The Eosinophilic Granulomatosis with Polyangiitis (formerly Churg- Strauss Syndrome) (EGPA) is a systemic inflammatory disease characterized by the presence of rhinitis, asthma, peripheral eosinophilia, and vasculitis—the latter being characteristic of the late stage of the disease. After several years from the onset of the disease, small- and medium-sized vessel vasculitis ensues, undertaking various organs and systems. Upper and lower airways, skin, nervous system, gastrointestinal tract, heart, and kidneys are the most commonly involved organs. It is believed that tissue injury is the result of processes mediated by antineutrophil cytoplasmic antibody (ANCA), or toxic mediators released by eosinophils. Although it is classified as ANCA-associated vasculitis, these autoantibodies are present in only 40% of cases. The authors report the case of a patient with EGPA, who had a history of asthma, peripheral and central neuropathy, palpable purpura, gastrointestinal micro perforation, peripheral eosinophilia, and the presence of myeloperoxidase-antineutrophil cytoplasmic antibody. Inflammatory parameters improved after the initiation of treatment, but 1 month after hospital discharge the patient developed symptoms compatible with pulmonary embolism and died. Thrombophilia that occurs in EGPA is due to the interaction between the inflammatory response and eosinophilia with the clotting system resulting in a pro-thrombotic state. Although not yet well-determined, the authors call attention to the possibility of the impact of thromboembolic events on the prognosis of patients with EGPA. In addition to the adequate immunosuppressive treatment, prophylaxis and treatment for thrombosis should never be overlooked.