[Vasculitis associated with Churg-Strauss syndrome]

Area of Science:

• Internal Medicine
• Rheumatology
• Gastroenterology

Background:

• Churg-Strauss syndrome (CSS), also known as eosinophilic granulomatosis with polyangiitis (EGPA), is a systemic vasculitis characterized by asthma, eosinophilia, and granulomatous inflammation.
• Gastrointestinal involvement in CSS is uncommon but can present with diverse and severe manifestations.

Observation:

• A 52-year-old male presented with abdominal pain, erythema, and eosinophilia, later developing mononeuropathy and positive anti-neutrophil cytoplasmic antibodies (ANCA) against myeloperoxidase, leading to a CSS diagnosis.
• Despite high-dose corticosteroid therapy, the patient experienced worsening abdominal symptoms, including a caecal soft tissue mass, ulcerative lesions, and colonic ischemia on imaging and colonoscopy.

Findings:

• Diagnosis of Churg-Strauss syndrome was confirmed by clinical presentation, laboratory findings (eosinophilia, ANCA positivity), and characteristic symptoms.
• Initial treatment with high-dose corticosteroids was insufficient to resolve severe gastrointestinal manifestations.
• Intensified immunosuppressive therapy with cyclophosphamide led to the resolution of abdominal complaints and healing of colonic lesions, with negative cytomegalovirus PCR.

Implications:

• This case underscores the importance of considering gastrointestinal vasculitis in patients with refractory abdominal symptoms and eosinophilia.
• Cyclophosphamide can be an effective treatment option for severe, steroid-refractory gastrointestinal manifestations of Churg-Strauss syndrome.
• Early recognition and aggressive management are crucial for improving outcomes in patients with severe CSS-related gastrointestinal disease.