FRI0244 EOSINOPHILIC FASCIITIS-LIKE SECONDARY TO CHRONIC GRAFT-VERSUS-HOST DISEASE: CLINICAL DESCRIPTION OF 28 PATIENTS
Authors: C. Hidalgo, C. Román, M. E. Acosta, L. Gómez-Lechón, M. D. Sánchez, L. Pantoja, O. Compán, S. Pastor, C. A. Montilla-Morales, L. López Corral
Journal: Annals of the Rheumatic Diseases (2020)

Background: Eosinophilic fasciitis (EF) is an uncommon chronic inflammatory disease characterized by myalgia, soft tissue hardening, peripheral eosinophilia and increased acute phase reactants, often triggered after strenuous physical exercise. Its appearance has been described as a rare complication after allogeneic transplantation of hematopoietic progenitors in the context of chronic graft-versus-host disease (cGVHD). Its etiopathogenesis is not well known and usually the treatment with systemic corticosteroids is effective Objectives: Describe the clinical and transplant-related characteristics of a cohort of patients with eosinophilic fasciitis-like in the context of cGVHD Methods: Observational, retrospective and descriptive study of the clinical characteristics of 28 patients affected by EF-like followed in a multidisciplinary consultation of cGVHD, started in March, 2014. Regular demographic variables, clinical characteristics related to the transplant and with the cGVHD, laboratory parameters, rescue therapies and their response were collected. The statistical analysis was done with Microsoft Excel 2007. Results: Seventeen (60.7%) patients were male and 11 (39.35%) were women with a mean age of 48.75 years (range from 10 to 74). Acute myeloid leukemia was the most frequent cause of the transplant in 11 patients (39.3%). Transplant related characteristics are reflected in Table 1 and the clinical manifestations, therapies received and their response in Table 2. Four (14.2%) patients died during their follow-up, being the cause of death in 2 cases due to sepsis, and in 1 case attributable to GVHD. Table 1. Baseline and transplant related characteristics (N = 28). . Variables N (%) Donor Type (related / not related) 13(46.4%)/15(53.6%) Type of conditioning (reduced intensity / myeloablative) 18(64.25%)/10(35.7%) Source of cells (Peripheral blood / bone marrow) 27(96.4%/1(3.6%) cGVHD type quiescent / de novo / progressive 11(39.3%)/13(46.4%)/4(14.3%) Other affected organs (cGVHD score) -Mouth 6(21.4%) -Eyes 10(35.7%) -Lung 2(7.1%) -Liver 3(10.7%) -Gastrointestinal tract 0(0%) -Genital 2(7.1%) -Cutaneous 16(57.14%) Global Score NIH 1 (mild / moderate / severe) 4(14.2%)/14(50%)/10(35.7%) 1 NIH: National Institute of Health. Table 2. Clinical manifestations and therapies (N = 28). . Variables N (%)/Median (range) Prodromic symptoms: yes / no 20(71%)/8(29%) - Stiffness 2(7.1%) - Artromyalgia 17(60.7%) - Edema 3(10.7%) Time until first visit 31.3 months (range 9-73) Contracture Yes / No 18(64.3%)/10(35.7%) Mobility limitation (mild / moderate) 13(46.4%)/9(32.1%) ECOG 1 affected 11(39.2%) Eosinophilia 17(60.7%) Positive autoantibodies 8(28.5%) First line therapies (corticosteroids) 28(100%) Extracorporeal photoapheresis 19(67.9%) Therapies of 2nd line/ 3rd or more 6(21.4%)/12 (42.8%) Physiotherapy 14 (50%) Response: complete / sequels 10(35.7%)/18(64.2%) 1 ECOG: Eastern Cooperative Oncology Group scale to assess the quality of life Conclusion: Nonspecific joint symptoms such as stiffness, edema or arthromyalgia in patients undergoing allogeneic transplantation of hematopoietic progenitors may be factors that predict the development of sclerotic GVHD type eosinophilic fasciitis-like and should be closely monitored in order to be able to perform early stage diagnoses of the disease. It is necessary to deepen the pathogenesis of this entity and the multidisciplinary approach to improve the prognosis of patients with GVHD References: [1]Inamoto Y. Arthritis Rheumatol. 2014;66(4):1044–52. Disclosure of Interests: None declared