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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 (

Objective. ANCA-associated vasculitides (AAV) are a heterogeneous group of rare diseases with unknown aetiology and the clinical spectrum ranging from life-threatening systemic disease, through single organ involvement to minor isolated skin changes. Thus, there is an unmet need for phenotype identification, especially among patients with granulomatosis with polyangiitis (GPA). Patients with microscopic polyangiitis (MPA) seem to be clinically much more uniform. Recently, three subcategories of AAV have been proposed and described as non-severe AAV, severe PR3-AAV, and severe MPO-AAV. Methods. In line with these attempts, we decided to use an unbiased approach offered by latent class analysis (LCA) to subcategorise GPA and MPA in a large cohort of Polish AAV patients included in a multicentre POLVAS registry. Results. LCA of our AAV group identified a four-class model of AAV, including previously proposed three subphenotypes and revealing a fourth (previously not described) clinically relevant subphenotype. This new subphenotype includes only GPA patients, usually diagnosed at a younger age as compared to other groups, and characterised by multiorgan involvement, high relapse rate, relatively high risk of death, but no end-stage kidney disease. Conclusion. Based on multiple clinical and serological variables, LCA methodology identified 4-class model of AAV. This newly described fourth class of AAV may be of clinical relevance and may require prompt diagnosis and aggressive treatment due to the multio-rgan involvement, high risk of relapse and marked mortality among these relatively young GPA subjects.

Objectives The aim of the study was to determine risk factors of interstitial lung disease (ILD) in patients with rheumatoid arthritis (RA). Methods 111 unselected patients (94 women and 17 men) with RA, as defined by the 1987 ARA criteria, were included in the study. The diagnosis of ILD was based on high resolution computed tomography (HRCT) of the chest. The following risk factors of RA related ILD (RA-ILD) were evaluated: demographic: age and gender, smoking status: current, former and cigarette pack-year, RA related: disease duration, Steinbrocker radiological stage, presence of atlanto-axial subluxation; presence of extra-articular manifestations (vasculitis, Sjögren syndrome, amyloidosis and subcutaneous rheumatoid nodules); activity indices assessed by both patient and rheumatologist (morning stiffness, tender and swollen joint counts, DAS28, VAS-pain, VAS-activity, HAQ, Steinbrocker functional class); laboratory test results (ESR, CRP, Hgb, PLT), serological markers (RF, ACPA, ANA, ENA) treatment related: methotrexate (MTX) use, duration of MTX use, MTX dose (current, cumulative and yearly; yearly MTX dose was defined as cumulative MTX dose/year of RA duration), prednisone use, cumulative dose of prednisone, other synthetic and biological DMARDs use. Results The mean age was 60.7 years. Median duration of RA was 7 years. Abnormalities consistent with ILD were found in 53 patients (47,8%). Univariate analysis revealed significantly increased risk ofRA-ILD in patients with older age (per 5 years: OR 1,41; 95% CI 1,16-1,73; p- 0,001), presence of atlanto-axial subluxation (OR 4,39; 95% CI 1,33-14,46; p- 0,02), higher Steinbrocker functional class (OR 2,96; 95% CI 1,39-6,32; p- 0,01) and higher serum RF level (per 100 IU/ml: OR 1,22; 95% CI 1,05-1,41; p- 0,01). Cyclosporine A use (OR 0,31; 95% CI 0,12-0,79; p- 0,01), infliximab use (OR 0,12; 95% CI 0,01-0,99; p- 0,05) and treatment with higher current (per 5mg/week: OR 0,7; 95% CI 0,57-0,88; p- 0,002), cumulative (per 1000 mg: OR 0,74; 95% CI 0,58-0,95; p- 0,02) and yearly MTX dose (per 100 mg/1 year of RA: OR 0,74; 95% CI 0,62-0,88; p- 0,001) were associated with a significantly lower ILD risk. Multivariate analysis showed following risk factors ofRA-ILD: age, serum RF level and yearly MTX dose. The risk of RA-ILD significantly increased with older age (per 5 years: OR 1,35; 95% CI 1,001-1,68; p- 0,007) and higher serum RF level (per 100 IU/ml: OR 1,16; 95% CI 1,001-1,36; p- 0,05), while higher yearly MTX dose significantly decreased the risk of RA-ILD (per 100 mg/1 year of RA: OR 0,74; 95% CI 0,61-0,9; p- 0,003). Conclusions The independent risk factors of ILD in patients with RA were older age, higher serum RF level and treatment with lower MTX dose. Disclosure of Interest None Declared

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