AB1271 DESCRIPTION OF A COHORT OF PATIENTS WITH SYSTEMIC SCLEROSIS AND ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES (ANCA)

Abstract

Background: The presence of antineutrophil cytoplasmic antibodies (ANCA) in systemic sclerosis (SSc) has been described rarely. The series published in the literature are scarce1 Its role in the evolution on the disease is unknown Objectives: To describe the epidemiological and clinical characteristics of ANCA positive patients from a cohort of patients with SSc Methods: Retrospective observational descriptive study in a cohort of 312 SSc. We analyzed those patients who were positive for ANCA, collecting variables: gender, race, age, age at diagnosis of SSc, at onset of symptoms, type and immunological profile, clinical manifestations of SSc, association with other systemic autoimmune diseases (SAD), capillaroscopic findings, and treatment. Regarding the ANCA profile, pattern, specificity and titration, as the time of evolution of SSc until the appearance of ANCA Results: Four of 312 patients with SSc had ANCA (prevalence = 1.28%). All were women and 2 had perinuclear pattern. The reason to request ANCA was kidney failure with proteinuria in 2 cases. One patient developed anti-myeloperoxidase (MPO) renal vasculitis and biopsy showed segmental glomerular necrosis and extracapillary proliferation. Other had proteinuria with hematuria without renal failure and biopsy with glomeruli in wafer. Regarding clinical manifestations all ANCA had some degree pulmonary disease 3 interstitial lung disease (ILD)1 pulmonary arterial hypertension (PAH) confirmed by right heart catheterization (KT). Three had SAD associated Characteristics of the sample in table 1 Conclusion: In our cohort the presence of positive ANCA was associated with renal vasculitis in one case. All patients with ANCA had vascular or interstitial pulmonary involvement, as well as other associated SADs. Further studies are needed to characterize the clinical associations of the presence of ANCA in SSc References [1] J Rheumatol21:864–870 [2] Rheumatol Int (2006) 26: 369–375 Disclosure of Interests: Sycille Jeria: None declared, HyeSang Park: None declared, Andrea Garcia-Guillen: None declared, DAVID LOBO: None declared, Ana Laiz Consultant for: Lilly, Novartis, AbbVvie, MSD, UCB and Janssen, Speakers bureau: Lilly, Novartis, Abvvie, MSD, UCB and Janssen, Berta Magallares: None declared, Patricia Moya: None declared, Ana Milena Millan Arciniegas: None declared, Cesar Diaz-Torne: None declared, Susana P. Fernandez-Sanchez: None declared, Hector Corominas: None declared, Ivan Castellvi Consultant for: I received fees less than 5000USD as a consultant for Kern and Actelion, Paid instructor for: I received fees less than 2000USD as a instructor for Boehringer -Ingelheim, Novartis and Gebro, Speakers bureau: ND