Objective Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are rare small to medium-size vessel systemic diseases. As their clinical picture, organ involvement, and factors influencing outcome may differ between countries and geographical areas, we decided to describe a large cohort of Polish AAV patients coming from several referral centers-members of the Scientific Consortium of the Polish Vasculitis Registry (POLVAS). Methods We conducted a systematic multicenter retrospective study of adult patients diagnosed with AAV between Jan 1990 and Dec 2016 to analyze their clinical picture, organ involvement, and factors influencing outcome. Patients were enrolled to the study by nine centers (14 clinical wards) from seven Voivodeships populated by 22.3 mln inhabitants (58.2% of the Polish population). Results Participating centers included 625 AAV patients into the registry. Their distribution was as follows: 417 patients (66.7%) with GPA, 106 (17.0%) with MPA, and 102 (16.3%) with EGPA. Male-to-female ratios were almost 1:1 for GPA (210/207) and MPA (54/52), but EGPA was twice more frequent among women (34/68). Clinical manifestations and organ involvement were analyzed by clinical phenotype. Their clinical manifestations seem very similar to other European countries, but interestingly, men with GPA appeared to follow a more severe course than the women. Fifty five patients died. In GPA, two variables were significantly associated with death: permanent renal replacement therapy (PRRT) and respiratory involvement (univariate analysis). In multivariate analysis, PRRT (OR = 5.3; 95% confidence Electronic supplementary material The online version of this article (
We report the case of coexistence of five autoimmune diseases in a 36-year-old woman, who initially developed psoriasis. Several years later, the patient was diagnosed with a mixed connective tissue disease and primary biliary cirrhosis (PBC). On admission to the Department of Rheumatology and Connective Tissue Diseases, the patient fulfilled classification criteria of an overlap syndrome systemic lupus erythematosus (SLE) with secondary antiphospholipid syndrome/systemic sclerosis (SSc)/Sjogren's syndrome (SS) with coexisting PBC and psoriasis. The SLE symptoms included discoid lupus erythematosus, arthritis, pancytopenia, antinuclear antibodies and anticardiolipin antibodies. Moreover, the patient met the criteria of antiphospholipid syndrome diagnosed based on preterm delivery before week 34, and high values of anticardiolipin antibodies were found at repeated determinations. The SSc symptoms included sclerodactyly, pulmonary fibrosis with pulmonary hypertension and esophageal dysfunction. The SS syndrome involved xerostomia, xerophthalmia, the positive Schirmer's test and presence of anti-SS antibodies. The literature reports overlap syndromes in various combinations; however, the coexistence of five autoimmune diseases is extremely rare.
IntroductIon It is still unclear how important the presence of antiphospholipid antibodies (aPL) is in patients with rheumatoid arthritis (RA). objectIves The aim of the study was to assess the prevalence of selected aPL in RA patients and their correlation with the presence of markers for RA antibodies and with disease activity. PAtIents And methods The study group consisted of 97 patients with RA who had never been treated with biological agents. In all patients, serum anticardiolipin antibodies (aCL), anti-β 2-glycoprotein I antibodies (a-β 2-GPI), lupus anticoagulant (LAC), immunoglobulin M (IgM) rheumatoid factor (RF), and anticyclic citrullinated peptide antibodies (anti-CCP) were measured and disease activity was assessed. results The presence of aPL was observed in 27 patients (27.8%): aCL in 20 patients (20.6%), a-β 2-GPI in 12 patients (12.4%), and LAC in 1 patient (1%). Positive aCL of low or medium levels were detected in the IgM class in 11 patients (11.3%) and in the IgG class in 12 patients (12.5%). Positive a-β 2-GPI of low and medium levels were found only in the IgM class. The presence of LAC was associated with aCL-IgM and a-β 2-GPI-IgM. A significant correlation was observed between the presence of anti-CCP and different types of aPL. There was no correlation between aPL and IgM-RF or disease activity markers. conclusIons The prevalence of aPL in patients with RA is relatively high. There is a relationship between the prevalence of aPL and anti-CCP-serological marker of RA, but there are no significant correlations between disease activity and the presence of aPL.
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