TREATMENT RESISTANT EOSINOPHILIC GRANULOMATOSIS WITH POLYANGIITIS (CHURG-STRAUSS SYNDROME) WITH NEUROLOGICAL COMPLICATIONS: A CASE REPORT

Abstract

Background: Eosinophilic Granulomatosis with Polyangiitis (EGPA), formerly known as Churg-Strauss Syndrome, is a rare autoimmune vasculitis affecting small- and medium-sized blood vessels. It is characterized by multisystem involvement, including respiratory, dermatological, and neurological manifestations. Neurological complications, such as mononeuritis multiplex and peripheral neuropathy, are reported in up to 70% of cases and can result in significant functional impairment. Standard treatments, including glucocorticoids and cyclophosphamide, may fail in refractory cases, necessitating alternative therapeutic approaches.

Case: A 23-year-old female with a known history of asthma presented with progressive shortness of breath, generalized weakness, and neurological symptoms, including seizures, bilateral leg rashes, and limb paralysis, leading to bed confinement. Initial misdiagnosis and antituberculous therapy delayed appropriate management. Imaging studies, clinical findings, and laboratory investigations confirmed a diagnosis of EGPA with significant peripheral neuropathy and mononeuritis multiplex. Despite six cycles of cyclophosphamide and glucocorticoids, the patient exhibited no improvement, indicating treatment resistance. Monoclonal antibody therapy with rituximab was initiated, resulting in marked improvement in both respiratory and neurological symptoms. The patient remains under regular follow-up, with sustained symptomatic relief and improved functional status.

Conclusion: This case highlights the challenges in diagnosing and managing refractory EGPA, particularly in young patients with atypical presentations and severe neurological complications. The successful response to rituximab underscores its potential as an effective therapeutic option in treatment-resistant cases. It further emphasizes the importance of early diagnosis, multidisciplinary care, and the need for personalized treatment strategies to optimize outcomes for EGPA patients.