AB0543 CLINICAL CHARACTERISTICS OF A GROUP OF CHRONIC GRAFT-VERSUS-HOST DISEASE PATIENTS WITH POSITIVE AUTOIMMUNITY.
Authors: M. E. Acosta, L. Gómez-Lechón, O. Compán, S. Pastor, C. A. Montilla-Morales, O. Martínez González, A. I. Turrión, J. Del Pino, C. Hidalgo
Journal: Annals of the Rheumatic Diseases (2020)

Background: Graft-versus-host disease (GVHD) is a commonly severe multiorgan complication in patients undergoing allogeneic transplantation of hematopoietic progenitors. Its chronic form reflects a complex immune response with different degrees of inflammation, immune dysregulation and fibrosis. In some chronic graft-versus-host disease (cGVHD) patients, positive antibodies have been detected, which represent the presence of immune activity and suggest the possible involvement of B lymphocytes in the disease etiopathogenesis, but their clinical utility is controversial. Objectives: To describe the clinical characteristics of a group of cGVHD patients with positive autoimmunity treated in a multidisciplinary consultation of Rheumatology-Dermatology- Hematology of GVHD. Methods: Observational and retrospective study to describe the clinical characteristics of the patients with positive autoimmunity collected in the database of the multidisciplinary consultation of GVHD. The variables reviewed for this study, in addition to the demographic ones, were type of antibody, disease causing the transplant, presentation, severity and type of involvement. The statistical analysis was done with Epi-info 7.2.2.6. Results: Only 16 (16%) of the 100 patients included in the database had positive autoimmunity. Twelve (75%) tested positive to ANA, although 5 (31.25%) in a lower titer (1/80). The most common immunofluorescence pattern was the nucleolar in 88.89% (66.67% nucleolar and 22.22% nucleolar + cytoplasmic). Other antibodies detected were: 6 anti-Ro52, 2 anti-dsDNA, 1 anti-RP155, 1 anti-Fibrillarin, 1 anti-SAE1, 1 p-ANCA and 1 anti-NOR-90. The mean of age was 51.31±14.03 years. As for sex 4 (25%) were female and 12 (75%) were men. The most frequent disease that caused the transplant was acute myeloid leukemia (58.3%). Ten (62.5%) patients presented de novo cGVHD, 1 (6.25%) progressive and 5 (31.25%) quiescent. The time since receiving the transplant until the first visit was 14 to 79 months. Ten (62.5%) patients had nonspecific symptoms (arthralgia and myalgia), 2 (12.5%) edema, 8 (50%) contractures, 8 (50%) fasciitis and 6 (37.5%) eosinophilia. Eight (50%) patients had ocular involvement and 6 (37.5%) of the oral mucosa in the form of dry syndrome (Sjögren-like syndrome). Ten (62.5%) patients had limitation of joint mobility detected by the range of motion scale (ROM), of which 6 were mild and 4 moderate. Only 5 (31.25%) patients had general condition impairment. As for the skin involvement 10 (62.5%) patients had sclerodermiform involvement (8 of them being eosinophilic fasciitis- like), 2 (12.5%) lichenoid, and 3 (18.5%) mixed (sclerodermiform + lichenoid). Only 1 patient didn´t meet diagnostic criteria for GVHD. The sclerodermiform was the most common type of involvement in the positive ANA patients. Regarding the severity according to the of the American National Institute of Health (NIH) classification: 8 (50%) had serious affectation, 5 (31.25%) moderate and 2 (12.5%) mild, with 4 (25%) exitus. Conclusion: In our cohort of patients with cGVHD, serum detection of autoantibodies is uncommon, being the ANA with nucleolar pattern the most frequent. Although the small sample size does not allow correlations with the clinical variables it´s worth highlighting a greater positivity of autoantibodies in the sclerodermiform skin forms. References: [1]Kuzmina Z et al. Clinical significance of autoantibodies in a large cohort of patients with chronic graft-versus-host disease defined by NIH criteria. Am J Hematol. 2015 February; 90(2): 114–119. [2]Rhoades R, Gaballa S. The Role of B Cell Targeting in Chronic Graft-Versus-Host Disease, Biomedicines 2017, 5, 61: 2-10 Disclosure of Interests: Maria Elisa Acosta: None declared, Luis Gómez-Lechón: None declared, Olga Compán: None declared, Sonia Pastor: None declared, Carlos A. Montilla-Morales: None declared, Olga Martínez González: None declared, Ana Isabel Turrión: None declared, Javier del Pino Grant/research support from: Roche, Bristol, Consultant of: Gedeon, Cristina Hidalgo: None declared