The electronic format lowered both false at-risk screens and false not-at-risk screens and had higher parental satisfaction.
Objectives To compare the therapeutic effectiveness of corticosteroids (CS) alone versus CS plus D-penicillamine (D-Pen) in severe eosinophilic fasciitis. Methods Long term prospective non-randomized trial of D-Pen plus CS vs. CS alone in patients with severe eosinophilic fasciitis, defined as clinically apparent cutaneous fibrotic involvement affecting greater than 15% body surface area (BSA), or greater than 10% BSA with joint flexion contractures. Results Sixteen patients with severe eosinophilic fasciitis entered the study. Ten patients received D-Pen plus CS and 6 CS alone. Affected BSA decreased from an average of 29% to 8.9% in the D-Pen plus CS group compared to a decrease in affected BSA from 28% to 22.83% in the CS alone group. The reduction in affected BSA in the D-Pen plus CS group was significantly greater than in the CS alone group (p= 0.038). Clinical improvement occurred in all D-Pen plus CS patients, compared to only 33.3% of CS alone patients (p=0.008). There was no difference in overall adverse events frequency between the groups (p=0.60). The most common adverse event in the D-Pen plus CS group was proteinuria (33.3%). However, proteinuria also occurred in 16.6% in the CS-alone group. Conclusions Treatment with CS alone failed to induce clinical improvement in the majority of the severe eosinophilic fasciitis patients. In contrast, D-Pen plus CS resulted in significantly greater clinical improvement. These results suggest that initial treatment of severe eosinophilic fasciitis with CS alone is not sufficient for optimal therapeutic response and that addition of an antifibrotic agent results in an improved outcome.
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 Shulman LE. Diffuse Fasciitis with Hypergammaglobulinemia and eosinophilia: A new syndrome. J Rheumatol 1974; (suppl 1) 1:46 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. 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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