Gastrointestinal perforation due to vasculitis at primary diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA) despite a high dose glucocorticosteroids treatment

BACKGROUND: Eosinophilic granulomatosis with polyangiitis (EGPA) belongs to the systemic ANCA-associated vasculitides which may develop life-threatening major organ involvement, such as eosinophilic pulmonary infiltration, neuropathy, acute nephritis, myocarditis, and gastrointestinal (GI) tract involvement. Here, two cases of EGPA are presented developing perforation of the bowel at primary diagnosis after 7 respectively 10 days of initiation of high-dose glucocorticosteroides (GC) therapy. FINDINGS: Two male patients, aged 32 (case 1) and 36 years (case 2), were admitted to the hospital with the symptoms of dyspnea, fatigue, fever, and chest pain. The patients completed the previously published revised international Chapel Hill nomenclature of EGPA. The two patients (case 1 and 2) developed acute abdominal pain after 7 (case 1) and ten days (case 2) on GC treatment. Computed tomography followed by surgery detected acute perforation of the small intestine (middle part of jejunum, case 1) and colon transversum (case 2). The resected specimens disclosed bowel perforation with severe transmural inflammation, edema, hemorrhage and vasculitis typically of EGPA. On the first post-operative day, therapy with cyclophosphamide (according to the Austin protocol) with a dosage of 750 mg/qm every 3 weeks (for 8 cycles) was initiated with good response within three months of treatment. CONCLUSION: The course of disease of the two presented EGPA patients suggest that very early initiation of intensified immunosuppressive treatment, preferentially with cyclophophomide or B cell depletion strategies, needs to be considered to avoid life-threatening complications of GI involvement.