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AB0619 Effectiveness of D-Penicillamine plus Corticosteroids versus Corticosteroids Alone in the Treatment Severe Eosinophilic Fasciitis
  1. F.A. Mendoza1,
  2. R. Bai2,
  3. A. Kebede3,
  4. S. Jimenez4
  1. 1Department of Medicine, Division of Rheumatology. Jefferson Institute of Molecular Medicine and Thomas Jefferson Scleroderma Center
  2. 2Jefferson Medical College
  3. 3Department of Medicine, Division of Rheumatology
  4. 4Jefferson Institute of Molecular Medicine and Thomas Jefferson Scleroderma Center, Thomas Jefferson University, Philadelphia, United States

Abstract

Background Eosinophilic fasciitis is an uncommon cutaneous fibrosing disorder characterized by fascial fibrosis often accompanied by tissue eosinophilic inflammation and peripheral eosinophilia. Corticosteroids (CS) are used as a first line treatment. Despite of its high relapse rate, there is no consensus on the use and choice of additional treatment.

Objectives To evaluate the efectiveness of D-Penicillamine (D-Pen) plus CS versus CS alone for the treatment of severe eosinophilic fasciitis.

Methods A 20 year (1993-2013) retrospective chart review identified 16 patients with clinical and histopathological diagnosis of severe eosinophilic fasciitis. This was defined as clinically apparent fibrotic involvement of more than 15% of the body suface area and/or the presence of fibrotic lesions crossing joints in patients with confirmed histopathology who had been treated with either D-Pen plus CS or CS alone. Analysis of disease characteristics, comorbidities, efficacy, adverse events (AE) and relapse was performed in this cohort.

Results Eosinophilic fasciitis lesions involved an average of 25% of BSA and 68.8% of the subjects developed a joint contractures during the course of the disease. 10/16 (62.5%) patients were treated with D-Pen plus CS and 6/16 (37.5%) with CS alone. Both groups were comparable in terms of involved body surface area, time from the first symptoms, age and co-morbidites. All of the patients initially treated with D-Pen plus CS, responded to the treatment. In contrast, only 33.3% of patients treated with CS alone had a favorable response (p=0.008). Rates of AEs were not statistically significant among the groups (80% vs 66.67% in the group treated with D-Pen plus CS vs CS alone). The most common AE reported in the D-Pen plus CS group was proteinuria which occurred in 33.3% of the subjects. However 16.6% of the patients on CS alone also developed proteinuria.

Figure 1.

Clinical outcomes of patients treated with CS alone and D-Pen plus CS.

Conclusions In this cohort with severe eosinophilic fasciitis, a very high rate of subsequent joint contractions was observed. The treatment with D-Pen plus CS was associated with a significantly greater improvement as compared to CS alone. Proteinuria was unexpectedly common in both groups although it was more frequent in the D-Pen plus CS group. A high frequency of relapse was seen in both treatment groups.

References

  1. Shulman LE. Diffuse Fasciitis with Hypergammaglobulinemia and eosinophilia: A new syndrome. J Rheumatol 1974; (suppl 1) 1:46

  2. Barnes L, Rodnan GP, Medsger TA, Short D. Eosinophilic fasciitis. A pathologic study of twenty cases. Am J Pathol. 1979 Aug;96(2):493-518.

  3. Lebeaux D, Sène D. Eosinophilic fasciitis (Shulman disease). Best Pract Res Clin Rheumatol. 2012 Aug;26(4):449-58

Disclosure of Interest None declared

DOI 10.1136/annrheumdis-2014-eular.4513

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