THU0347 CLINICAL DESCRIPTION OF A COHORT OF PATIENTS WITH SCLEROTIC-TYPE CHRONIC GRAFT-VERSUS-HOST DISEASE TREATED IN A MULTIDISCIPLINARY PRACTICE
Authors: C. Hidalgo, C. Román, M. E. Acosta, L. Gómez-Lechón, L. Pantoja, M. D. Sánchez, O. Compán, S. Pastor, C. A. Montilla-Morales, L. López
Journal: Annals of the Rheumatic Diseases (2020)

Background: Graft versus host disease is the most frequent complication after allogeneic transplantation of hematopoietic progenitors. Its chronic form usually involves a multisystemic syndrome that reflects a complex immune response with varying degrees of inflammation, immune dysregulation and fibrosis, responsible for the characteristic clinical manifestations of the disease. Joint, muscular and fascial involvement represents one of the areas, often unnoticed or poorly evaluated, that negatively impacts the physical function and quality of life of these patients. Objectives: Describe the presence of musculoskeletal manifestations and their clinical characteristics in patients with chronic GVHD (cGVHD) evaluated in a multidisciplinary consultation Methods: Descriptive and retrospective observational study to detail the initial presence and during the follow-up of diagnostic and nonspecific musculoskeletal manifestations of cGVHD in a cohort of 103 patients included in the database. The clinical characteristics of 68 patients with a defined diagnosis of sclerotic cGVHD are described. Demographic variables are collected along with clinical conditions about transplant and in a systematic way the assessment according to diagnostic criteria of the American National Institute of Health 2015 highlighting: range of motion scale and photographic range of motion (P-ROM) scale in shoulders, elbows, hands and ankles; and laboratory data: presence of eosinophilia and autoantibodies. Descriptive and frequency statistical analysis was done using Microsoft Office Excel 2007. Results: Sixty-eight(66%)patients meet diagnostic criteria for sclerotic cGVHD during follow-up. Forty-five(66.2%) women and 23(33.8%) men, with a mean age of 54.5 years (range 10-78), Acute myeloid leukemia was the reason for transplant in 20(29, 4%) followed by non-Hodgkin lymphoma in 15 (16.2%). In 40(58.7%) patients it was performed from a related donor and with reduced intensity conditioning in 43(63.2%). Only in one patient the source of hematopoietic progenitors was bone marrow (rest peripheral blood). The average time from transplant to the first visit was 29.5 months (range 4 -168). Twelve(17.64%) patients presented isolated joint/fascial involvement without objective skin involvement (Table 1) Table 1. Clinical features of the sclerotic joint/fascial chronic GVHD cohort (N= 68) Unspecific musculoskeletal symptoms: 46 (67.6%) -Artromyalgia 39(57.3%) -Edema 13(19%) -Stiffness 4(5.8%) Fasciitis/contractures 28(41.2%)/43(63,2%) Restricted ROM 1 -Mild/Moderate/Severe 34(50%)/14(20.2%)/4(5.8%) Impaired mobility -Shoulders 26(38.2%) -Elbows 21(30.8%) -Wrist/fingers 29(42.6%) -Ankles 21(30.6%) Sclerodermatous involvement -Superficial /deep 6(8.8%)/7(10.3%) -Mixed (sclerodermiform + lichenoid) 20(29.4%) -Overlapping skin sclerosis 13(19.2) NIH 1 Global Score -Mild/Moderate/Severe 25(36.7%)/7(10.3%)/1(1.5%) Eosinophilia 21 (30.8%) Autoantibodies 14 (20.6%) 1 ROM (range of motion) 2 NIH: National Institute of Health Conclusion: Joint involvement secondary to sclerosis is very common in our cohort, mainly of the dorsal wrist flexion with deleterious repercussion on physical function. It needs to be recognized and evaluated early with validated scales. The search for new biomarkers associated with fibrosis, the use of advanced imaging techniques and the multidisciplinary approach can help improve the prognosis of patients with cGVHD. References: [1]Jagasia MH,Blood Marrow Transplant. 2015;21(3):389–40 Disclosure of Interests: None declared