Introduction <p>Eosinophilic fasciitis (EF) is a rare condition characterised by sclerosis of the skin, fascia and subcutaneous tissues. We aimed to review cases of EF managed in a multidisciplinary clinic from an Australian tertiary hospital, highlighting clinical characteristics, treatment approaches including success with rituximab and the benefits of a multidisciplinary approach.</p> Method <p>Patients with EF (Pinal-Fernandez and/or Jinnin criteria) managed in the inflammatory skin clinic between 01/22 and 07/25 at Flinders Medical Centre, South Australia, were identified. Clinical characteristics (timeline/extent of skin involvement, extracutaneous symptoms, time-to-diagnosis), laboratory investigations, imaging, histology and treatment exposures were reviewed.</p> Results <p>Five patients with EF were identified; 4/5 were female, and the mean age was 60&#xa0;years. Time-to-diagnosis ranged from 3 to 18&#xa0;months. Four patients had upper and lower limb involvement; one had upper limb involvement only. Skin tightness universally impaired the range of motion of affected joints. Peripheral eosinophilia was present in 4/5 patients. Of the three patients who underwent MRI, two showed myoedema with fascial thickening. Eosinophils were present on incisional skin biopsy in 4/5 patients. Although methotrexate and mycophenolate were frequently trialled, 4/5 patients required escalation to rituximab. Over 11–36&#xa0;months of follow-up, all patients demonstrated significant improvement in skin thickening, symptoms and function.</p> Conclusions <p>We have reported the first Australian single-centre cohort of patients with EF. Although not universal, peripheral eosinophilia and eosinophils on skin biopsy frequently occurred. Muscle involvement, constituting myofasciitis, was common. Rituximab appears to be an efficacious treatment with a potentially durable response to induction without requiring maintenance dosing. At our centre, a multidisciplinary approach has assisted in accurately diagnosing EF and appropriately escalating treatment.</p>

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The utility of rituximab in patients with eosinophilic fasciitis: case series from an Australian tertiary multidisciplinary clinic

  • Syed B. Ali,
  • Thomas Khoo,
  • Gillian Marshman,
  • Kate Newland,
  • Fiona Goldblatt

摘要

Introduction

Eosinophilic fasciitis (EF) is a rare condition characterised by sclerosis of the skin, fascia and subcutaneous tissues. We aimed to review cases of EF managed in a multidisciplinary clinic from an Australian tertiary hospital, highlighting clinical characteristics, treatment approaches including success with rituximab and the benefits of a multidisciplinary approach.

Method

Patients with EF (Pinal-Fernandez and/or Jinnin criteria) managed in the inflammatory skin clinic between 01/22 and 07/25 at Flinders Medical Centre, South Australia, were identified. Clinical characteristics (timeline/extent of skin involvement, extracutaneous symptoms, time-to-diagnosis), laboratory investigations, imaging, histology and treatment exposures were reviewed.

Results

Five patients with EF were identified; 4/5 were female, and the mean age was 60 years. Time-to-diagnosis ranged from 3 to 18 months. Four patients had upper and lower limb involvement; one had upper limb involvement only. Skin tightness universally impaired the range of motion of affected joints. Peripheral eosinophilia was present in 4/5 patients. Of the three patients who underwent MRI, two showed myoedema with fascial thickening. Eosinophils were present on incisional skin biopsy in 4/5 patients. Although methotrexate and mycophenolate were frequently trialled, 4/5 patients required escalation to rituximab. Over 11–36 months of follow-up, all patients demonstrated significant improvement in skin thickening, symptoms and function.

Conclusions

We have reported the first Australian single-centre cohort of patients with EF. Although not universal, peripheral eosinophilia and eosinophils on skin biopsy frequently occurred. Muscle involvement, constituting myofasciitis, was common. Rituximab appears to be an efficacious treatment with a potentially durable response to induction without requiring maintenance dosing. At our centre, a multidisciplinary approach has assisted in accurately diagnosing EF and appropriately escalating treatment.