P037 Exploring eosinophilic fasciitis: a case series and insights into the treatment modalities

Eosinophilic fasciitis (EF) is a rare autoimmune disorder characterised by inflammation and fibrosis of the fascia, often with peripheral eosinophilia. First described by Shulman in 1974, it manifests as skin thickening, joint contractures, and induration, resembling scleroderma-like conditions. Despite distinct features, EF’s pathogenesis remains poorly understood, complicating diagnosis and treatment. Therapeutic approaches primarily involve corticosteroids and immunosuppressive agents, though responses vary widely. This article presents a case series of EF patients, shedding light on the efficacy and challenges of treatment.

Case 1: A 60-year-old male with hypertension developed cough, breathlessness, and swelling in November 2022 after starting Atorvastatin and contracting COVID-19. His blood showed normal inflammatory markers but raised eosinophil which initially responded to steroids, but skin thickening and restricted movement led to an EF diagnosis. Treatment involved IV steroids, Cyclophosphamide, and later Methotrexate, MMF, and Abatacept. By March 2024, skin condition improved, allowing prednisolone tapering. Case 2: A 68-year-old male presented in 2019 with widespread rash, stiffness, and swelling. Diagnosed initially with morphoea and eosinophilia, but later confirmed as EF with the skin biopsy. He initially responded to IV methylprednisolone but relapsed during tapering. Methotrexate, MMF, and hydroxychloroquine were ineffective, but abatacept, started in November 2021, significantly improved symptoms and maintained remission. Case 3: An 82-year-old male with bilateral hand swelling, forearm and foot pain, and vasculopathy was diagnosed with EF after a skin biopsy. High-dose steroids provided partial relief, but relapses occurred. Methotrexate was effective but caused fatigue, and other immunosuppressants were discontinued due to side effects. Abatacept provided mild improvement, and neuropathic pain was managed with gabapentin and carbamazepine.

Managing EF is challenging due to its rarity, variable presentation, and unpredictable treatment response. Corticosteroids remain the primary treatment but carry long-term risks, prompting the use of steroid-sparing agents. In this series, abatacept, a T-cell co-stimulation modulator, showed variable outcomes. Its effectiveness may depend on the balance of inflammation and fibrosis in different disease stages. Cyclophosphamide, used in one patient, showed limited benefit, highlighting the difficulty in reversing established fibrosis. The variability in treatment outcomes underscores the need for individualized approaches, as a universal treatment strategy for EF is ineffective. Moreover, the lack of standardized guidelines and robust clinical trials further complicates decision-making, often leading to reliance on case reports and small studies to guide therapy.

EF poses diagnostic and therapeutic challenges due to its rarity and variable treatment responses. This case series emphasizes the need for early, tailored treatment strategies using immunosuppressive agents. Further research into EF’s pathophysiology and targeted therapies is crucial for improving patient outcomes.

T. Nasrin: None. S. Bahrani: None. T. Guruparan: None. S. Young Min: None.