Etienne Ghrénassia1, Jérome Avouac, Dinesh Khanna, Chris T Derk, Oliver Distler, Yossra Atef Suliman, Paolo Airo, Patricia E Carreira, Rosario Foti, Brigitte Granel, Alice Berezne, Jean Cabane, Francesca Ingegnoli, Edoardo Rosato, Paola Caramaschi, Roger Hesselstrand, Ulrich A Walker, Juan Jose Alegre-Sancho, Virginie Zarrouk, Christian Agard, Valeria Riccieri, Elena Schiopu, Heather Gladue, Virginia D Steen, Yannick Allanore. 1. From the Department of Rheumatology A, Paris Descartes University, Cochin Hospital, AP-HP, Paris, France; University of Michigan Scleroderma Program, Ann Arbor, Michigan, USA; Division of Rheumatology, University of Pennsylvania, Philadelphia, USA; Department of Rheumatology, University Hospital Zurich, Switzerland; Rheumatology and Clinical Immunology Unit, Spedali Civili and University of Brescia, Brescia, Italy; Rheumatology Department, Hospital Universitario 12 de Octubre, Madrid, Spain; Rheumatology Unit, Azienda Ospedaliera Universitaria V. Emanuele, Ferrarotto, S. Bambino, Catania, Italy; Service de Médecine Interne, Centre Hospitalier Nord, Marseille, France; Service de Médecine Interne, University Hospital Centre (CHU) Cochin; Service de Médecine Interne, CHU Saint-Antoine, Paris, France; Division of Rheumatology, Istituto Gaetano Pini, University of Milan, Italy; Department of Clinical Medicine, Clinical Immunology Unit, Sapienza University of Rome, Rome, Italy; Rheumatology Unit, Azienda Ospedaliera Universitaria Integrata, Verona, Italia; Department of Rheumatology, Lund University, Lund, Sweden; Department of Rheumatology, Basel University, Basel, Switzerland; Sección de Reumatología, Hospital Dr Peset, Valencia, Spain; Médecine interne, Hôpital Beaujon, Clichy, France; Internal Medicine, Hôtel-Dieu Hospital, CHU Nantes, Nantes, France; Rheumatologic Unit, Department of Internal Medicine and Clinical Specialties, Sapienza University of Rome, Rome, Italy; and Department of Medicine, Georgetown University Medical Center, Washington DC, USA.
Abstract
OBJECTIVE: To estimate the prevalence, determine the subgroups at risk, and the outcomes of patients with systemic sclerosis (SSc) and gastric antral vascular ectasia (GAVE). METHODS: We queried the European League Against Rheumatism Scleroderma Trials and Research (EUSTAR) network for the recruitment of patients with SSc-GAVE. Each case was matched for cutaneous subset and disease duration with 2 controls with SSc recruited from the same center, evaluated at the time the index case made the diagnosis of GAVE. SSc characteristics were recorded at the time GAVE occurred and the last observation was collected to define the outcomes. RESULTS: Forty-nine patients with SSc and GAVE were included (24 with diffuse cutaneous SSc) and compared to 93 controls with SSc. The prevalence of GAVE was estimated at about 1% of patients with SSc. By multivariate analysis, patients with SSc-GAVE more frequently exhibited a diminished (< 75%) DLCO value (OR 12.8; 95% CI 1.9-82.8) despite less frequent pulmonary fibrosis (OR 0.2; 95% CI 0.1-0.6). GAVE was also associated with the presence of anti-RNA-polymerase III antibodies (OR 4.6; 95% CI 1.2-21.1). SSc-GAVE was associated with anemia (82%) requiring blood transfusion (45%). Therapeutic endoscopic procedures were performed in 45% of patients with GAVE. After a median followup of 30 months (range 1-113 months), survival was similar in patients with SSc-GAVE compared to controls, but a higher number of scleroderma renal crisis cases occurred (12% vs 2%; p = 0.01). CONCLUSION: GAVE is rare and associated with a vascular phenotype, including anti-RNA-polymerase III antibodies, and a high risk of renal crisis. Anemia, usually requiring blood transfusions, is a common complication.
OBJECTIVE: To estimate the prevalence, determine the subgroups at risk, and the outcomes of patients with systemic sclerosis (SSc) and gastric antral vascular ectasia (GAVE). METHODS: We queried the European League Against Rheumatism Scleroderma Trials and Research (EUSTAR) network for the recruitment of patients with SSc-GAVE. Each case was matched for cutaneous subset and disease duration with 2 controls with SSc recruited from the same center, evaluated at the time the index case made the diagnosis of GAVE. SSc characteristics were recorded at the time GAVE occurred and the last observation was collected to define the outcomes. RESULTS: Forty-nine patients with SSc and GAVE were included (24 with diffuse cutaneous SSc) and compared to 93 controls with SSc. The prevalence of GAVE was estimated at about 1% of patients with SSc. By multivariate analysis, patients with SSc-GAVE more frequently exhibited a diminished (< 75%) DLCO value (OR 12.8; 95% CI 1.9-82.8) despite less frequent pulmonary fibrosis (OR 0.2; 95% CI 0.1-0.6). GAVE was also associated with the presence of anti-RNA-polymerase III antibodies (OR 4.6; 95% CI 1.2-21.1). SSc-GAVE was associated with anemia (82%) requiring blood transfusion (45%). Therapeutic endoscopic procedures were performed in 45% of patients with GAVE. After a median followup of 30 months (range 1-113 months), survival was similar in patients with SSc-GAVE compared to controls, but a higher number of scleroderma renal crisis cases occurred (12% vs 2%; p = 0.01). CONCLUSION: GAVE is rare and associated with a vascular phenotype, including anti-RNA-polymerase III antibodies, and a high risk of renal crisis. Anemia, usually requiring blood transfusions, is a common complication.
Authors: Vivek Nagaraja; Marco Matucci-Cerinic; Daniel E Furst; Masataka Kuwana; Yannick Allanore; Christopher P Denton; Ganesh Raghu; Vallerie Mclaughlin; Panduranga S Rao; James R Seibold; John D Pauling; Michael L Whitfield; Dinesh Khanna Journal: Arthritis Rheumatol Date: 2020-05-18 Impact factor: 10.995