TAFRO syndrome: A severe manifestation of Sjogren's syndrome? A systematic review |
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| Institution: | 1. Department of Internal Medicine, Centre Hospitalier Universitaire de Saint-Etienne, Saint-Etienne, France;2. Department of Hematology and Cell Therapy, Centre Hospitalier Universitaire de Saint-Etienne, Saint-Etienne, France;3. Inserm U1059-SAINBIOSE, dysfonction vasculaire et hémostase, Université de Lyon, Saint-Etienne, France;4. Clinical Immunology Department, Hôpital Saint Louis, Université Paris Cité, Paris, France;5. National Reference Center for Castleman disease, Hôpital Saint Louis, Université Paris Cité, Paris, France;6. U976 HIPI, INSIGHT Team, Hôpital Saint Louis, Université Paris Cité, Paris, France;7. Department of Immunology, Centre Hospitalier Universitaire de Saint-Etienne, Saint-Etienne, France;8. CIRI – Centre International de Recherche en Infectiologie, Team GIMAP, Université Jean Monnet, Unversité Claude Bernard Lyon 1, Inserm, U1111, CNRS, UMR530, F42023 Saint-Etienne, France;9. CIC 1408 Inserm, Centre Hospitalier Universitaire de Saint-Etienne, Saint-Etienne, France;1. Università degli Studi di Milano, Dipartimento di Fisiopatologia medico-chirurgica e dei Trapianti, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico Milano, Medicina Interna – Emostasi e Trombosi, Milano, Italy;2. Experimental Laboratory of Immunological and Rheumatologic Researches, Istituto Auxologico Italiano, Istituto di Ricovero e Cura a Carattere Scientifico, Milano, Italy;3. Department of Rheumatology and Clinical Immunology, Faculty of Medicine, Alexandria University, Alexandria, Egypt;4. Department of Experimental and Clinical Medicine, University of Firenze, Firenze, Italy;5. Department of Pathology and Immunology, School of Medicine, University of Geneva, Switzerland;6. Clinical Immunology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy;7. Department of Microbiology, Immunology and Transplantation, KU Leuven, Leuven, Belgium;8. Department of Immunology, Genetics and Pathology, Uppsala University, Uppsala, Sweden;9. Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy;10. Department of Biomedical Sciences, Humanitas University, Milan, Italy;11. Lupus Clinic, Careggi University Hospital, Firenze, Italy;12. Department of Laboratory Medicine, University Hospitals Leuven, Belgium;1. Department of Internal Medicine, Caen University Hospital, Avenue de la Côte de Nacre, 14000 Caen, France;2. Normandie Univ, UNICAEN, UR4650 PSIR, CHU de Caen Normandie, 14000 Caen, France;3. Department of Pharmacology, CHU de Caen Normandie, 14000 Caen, France;4. Caen University-Normandy, UNICAEN, INSERM U1086 ANTICIPE, Avenue du Général Harris, CHU de Caen-Normandie, Caen F-14000, France;1. Centre Hospitalier Universitaire, Medecine Interne, Toulouse, France;2. Centre Hospitalier Universitaire, Laboratoire d‘Immunologie, Toulouse, France;3. Clinique Saint-Exupery, Medecine Interne, Toulouse, France;4. Centre Hospitalier Universitaire, Néphrologie, Toulouse, France;5. Centre Hospitalier Universitaire, Cardiologie, Toulouse, France;6. Clinique Ambroise-Paré, Medecine Interne, Toulouse, France;7. Centre Hospitalier Universitaire, Pneumologie, Toulouse, France;8. Hopital Joseph Ducuing, Medecine Interne, Toulouse, France;9. Centre D''investigation Clinique (CIC), 1436 PEPSS Team, Toulouse, France;1. Department of Biotechnological and Applied Clinical Sciences, University of L''Aquila, L''Aquila, Italy;2. Rheumatology Unit, IRCCS Istituto Ortopedico Rizzoli, Bologna, Italy;3. Department of Biomedical and Neuromotor Sciences, Alma Mater Studiorum University of Bologna, Bologna, Italy;4. Rheumatology and Immunology Unit, Department of Medicine, University of Rome Campus Biomedico, Rome, Italy;1. Service de Néphrologie-Dialyse-Transplantation, CHU d''Angers, Angers, France;2. Université d''Angers, Inserm, CNRS, Nantes Université, CRCI2NA, Angers, France;3. Service de Pathologie, Université d''Angers, CHU d''Angers, Angers, France;4. Service de Néphrologie-Dialyse, CH, Le Mans, 72000 Le Mans, France |
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| Abstract: | BackgroundSjögren's syndrome (SjS) is a systemic autoimmune disease characterized by lymphocytic infiltration of the salivary and lacrimal glands associated with sicca syndrome. TAFRO syndrome is a systemic inflammatory disease of unknown cause, characterized by Thrombocytopenia, Anasarca, Fever, Reticulin fibrosis, Renal dysfunction and Organomegaly, first reported in 2010 in Japanese patients. Despite their rarity, both conditions have been concurrently reported in several patients during the recent years, hence questioning the existence of shared or related features.MethodsA systematic review of the literature regarding SjS associated with TAFRO syndrome (SjS-TAFRO) was performed. The 2019 updated Masaki diagnostic criteria were used for TAFRO syndrome and SjS was considered when the diagnosis was mentioned by the authors, necessarily with either anti-Sjogren's Syndrome A (SSA) ± anti-Sjogren's Syndrome B (SSB) antibodies and/or histological evidence of focal lymphocytic sialadenitis.ResultsTen cases of SjS-TAFRO have been reported in the literature to date. Compared to SjS patients without TAFRO syndrome, these 10 SjS-TAFRO had a lower female predominance (2.3:1 vs 9:1 women to man ratio) and a higher frequency of anti-SSA antibodies (90% vs 70%). All fulfilled the three major Masaki criteria i.e., anasarca, thrombocytopenia, and systemic inflammation. Seven of them (70%) had megakaryocyte hyperplasia or reticulin fibrosis in the bone marrow. Lymph node biopsy was performed in 8 out of 10 cases (80%) and results were consistent with Castleman disease in 6 (75%). Eight of them had developed renal failure (80%) within six months. Nine of them (90%) had organomegaly, with hepatosplenomegaly in 8 cases and splenomegaly alone in 1.ConclusionThis review brings new insights regarding TAFRO syndrome and suggests it could be a severe manifestation of SjS. The identification of shared abnormal signaling pathways could help in the therapeutic management of both diseases, which face an unmet therapeutic need. |
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