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

Ano: 2013 | Volume: 3 | Número: 2

Autores: V. Takayasu, A. Felipe-Silva

Palavras-chave: churg–strauss syndrome, granulomatous allergic angiitis, anti-neutrophil cytoplasmic antibody-associated vasculitis, asthma, pulmonary embolism, autopsy

Resumo Inglês:

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.