両側舌下部無痛性腫脹が先行発症した好酸球性多発血管炎性肉芽腫症の1例

抄録

Eosinophilic granulomatosis with polyangiitis （EGPA） is classified as anti-neutrophil cytoplasmic antibody associated vasculitis, which is a necrotizing vasculitis with mainly lesions in small blood vessels such as capillaries and venules. It is a rare systemic vasculitis disease. The number of people affected in Japan is currently around 2000, and it is estimated that there are 100 new patients each year.
The disease had been conventionally called Churg-Strauss syndrome or allergic granulomatous vasculitis, but in 2012 it was renamed EGPA. In the head and neck region, bilaterally symmetrical salivary gland swelling has been shown. Treatment is mainly based on steroid therapy and has a good prognosis, but occasionally there are reports of severe complications such as intestinal perforation and heart failure.
We report a case of EGPA showing IgG4-related disease. The symptoms such as salivary gland swelling disappeared with steroid therapy after we consulted the department of rheumatology and connective tissue disease, but four months after the initial treatment, systemic symptoms such as fatigue, facial edema, skin redness in the upper and lower limbs and re-elevation of peripheral blood IgE, IgG4 and eosinophil were observed. Accordingly, we consulted the department again and diagnosed EGPA.