Updates for the treatment of EGPA.

Eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss syndrome) is the least frequent antineutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV). Major advances of our knowledge on its pathophysiology have revealed features of both AAV and eosinophilic disorders. The development of targeted biotherapies for both diseases opened new possibilities for EGPA management. In this review, we highlight the rationale underlying the routine treatment strategy, which relies mainly on corticosteroids, with immunosuppressant adjunction for severe disease. However, novel therapies are still needed for refractory/relapsing disease and to alleviate the corticosteroid-dependence of asthma and chronic rhinosinusitis. At present, the most promising biotherapies target either eosinophil biology, like mepolizumab, an anti-interleukin-5, or the B-cell compartment, with rituximab. Recent clinical data on new treatment options are discussed and therapeutic strategies are proposed.

Antineutrophil cytoplasmic antibody-associated vasculitis in Taiwan: A hospital-based study with reference to the population-based National Health Insurance database.

BACKGROUND: Antineutrophil cytoplasmic antibody-associated vasculitis (AAV), including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and Churg-Strauss syndrome (CSS), comprises a group of diseases with significant morbidity and mortality. The incidence and relative frequency of GPA/MPA/CSS are different all over the world. The epidemiology of AAV in Taiwan is still not clear. METHODS: The current study aimed to provide a population-based estimate of the annual incidence of GPA using the Taiwan National Health Insurance (NHI) research database and a single hospital-based estimate of the relative frequency of AAV in Taiwan. RESULTS: The annual incidence of GPA in Taiwan was 0.37 per million patient-years (95% Poisson rate confidence interval: 0.30-0.45) from 1997 to 2008, according to the NHI database. In our hospital, 24 patients were newly diagnosed with AAV between 2003 and 2011, including eight patients with GPA, 14 with MPA, and two with CSS. The ratio of the number of patients with GPA to that of MPA was 0.57. CONCLUSION: The current results provide an estimate of the annual incidence of GPA and the relative frequency of AAV in the Chinese Han community in Taiwan. Such geoepidemiology information may help illuminate the interaction between ethnic background and environment in these autoimmune diseases.

[Churg-Strauss syndrome with rectal localization and exudative enteropathy]. / Syndrome de Churg et Strauss avec localisation rectale et entéropathie exsudative.

The authors report the case of a 61-year old patient with a Churg-Strauss syndrome revealed by abdominal pain. Investigations showed a diffuse inflammatory digestive tract involvement, documented by endoscopy associated with protein-losing enteropathy. Deep rectal biopsy revealed vasculitis in an otherwise macroscopically normal rectum. Faced with acute and life-threatening course of disease, emergency medical treatment with steroids and cyclophosphamide led to rapid regression of clinical, biological and radiological abnormalities.