Although we only included histologically proven cases of IgAV, the annual incidence rate of 5·1 per 100,000 adults is 3-6-times higher than previously reported.

Idiopathic Inflammatory myopathies (IIM) are rare disorders. The aim of our study was to determine the incidence of IIM in a well-defined Slovenian region. This retrospective study was conducted at the Department of Rheumatology, University Medical Centre Ljubljana, the only secondary/tertiary rheumatology center in a region with a population of 704,342 adults. We identified potential IIM cases by searching the electronic patient records for ICD-10 codes M33, M35.1, M35.8, M60, G72, G73, and J84. We included incipient IIM cases between January 2010 and December 2017, who were at the time of the diagnosis, residents of the inspected region. To avoid under-reporting due to miscoded cases, we obtained a list of the patients who had histological patterns consistent with IIM on muscle biopsy from the Institute of Pathology. The annual incidence rate for IIM was calculated. During the eight-year observation period, we identified 65 IIM cases (72.3% female, median (IQR) patient age 64.8 (54.8-73.2) years). The estimated annual incidence of IIM in the studied population was 11.5 (95% CI 9.0-14.6) per 10 adults, in females 16.2 (95% CI 12.1-21.4), and in males 6.6 (95% CI 4.0-10.2) per 10 adults. The incidence rate of IIM in Slovenia is consistent with data from the literature.

We evaluated the occurrence of antiphospholipid antibodies (aPLs) in acute adult IgA vasculitis (IgAV), and potential correlations with IgAV clinical presentation. We determined lupus anticoagulants (LAs) and IgG, IgM, and IgA isotypes of anticardiolipin antibodies (aCL), antibodies against β2-glycoprotein I (aβ2GPI) and against the phosphatidylserine-prothrombin complex (aPS/PT) in prospectively collected, histologically proven IgAV, diagnosed for the first time between January 2013 and February 2018 at our secondary/tertiary rheumatology center. During the 62 months, we determined aPLs in 125 IgAV patients (56.8% male; median (IQR) age 64.7 (48.6-78.2) years). Sixty-four (51.2%) patients had aPLs. We found LAs, aPS/PT, aβ2GPI, and aCL in 24.8%, 21.6%, 13.6%, and 11.2% of cases, respectively. With 17.6%, the IgA aPS/PT was the most common aPL subtype. aPL-positive and aPL-negative patients did not differ in the clinical presentation of acute IgAV or in the frequency of thrombotic events. aPL-positive IgAV patients had significantly higher erythrocyte sedimentation rate (p < 0.001), and C-reactive protein (p < 0.001). The subset of IgA aPS/PT-positive patients more commonly had renal involvement in acute disease (RR 2.4 (95% CI 1.6-3.7)). aPLs are commonly detected during acute IgAV episodes. Patients with aPLs have similar clinical presentation, but higher markers of inflammation at than those without them. The subset of IgAV patients with IgA aPS/PT more commonly had renal involvement.

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BackgroundFollow-up data on IgA vasculitis (IgAV) in adults are scarce. We aimed to investigate the outcome of adult IgAV in a well-defined cohort.MethodsData from histologically proven patients diagnosed between January 2010 and July 2022 with at least a 3-month follow-up were analyzed. The frequency and type of relapses and information on kidney function were extracted. Risk factors for IgAV relapse and decline in renal function were studied using the Cox hazards regression analysis. Mortality in IgAV was assessed using the Kaplan–Meier analysis and the standardized mortality ratio (SMR).ResultsIn total, 265 patients were followed for a median of 24 months. At baseline, 38.9, 29.8, and 44.5% had articular, gastrointestinal, and renal involvement, respectively. Initially, 189 (71.3%) patients received systemic glucocorticoids, and 32 (12.1%) patients received an additional immunomodulator. During follow-up, 42 (15.8%) patients relapsed. Relapses were more common in younger patients (HR 1.03 [95%CI 1.01–1.05]) and those without baseline glucocorticoid treatment (HR 3.70 [95%CI 2.0–6.67]). Furthermore, 74 (27.9%) patients had persistent abnormal urinalysis and a substantial (≥20%) decline in glomerular filtration rate (eGFR) was recorded in 41 (15.5%) patients. The factors associated with persistent abnormal urinalysis were an absence of IgAV joint involvement and baseline immunomodulatory treatment. Pre-existent chronic kidney disease and heart failure were associated with eGFR decline. The overall SMR was 1.4 (95%CI 1.14–1.71) compared to the Slovenian general population.ConclusionIgAV relapses occurred in 15% of patients, with younger patients with symptomatically managed IgAV experiencing it more frequently. Heart failure emerged as a predictor of persistent abnormal urinalysis and a decline in eGFR. Adults with IgAV had increased mortality compared to the general population.

BackgroundThere is no consensus or recommendations for the treatment of idiopathic inflammatory myopathies (IIM).ObjectivesWe explored how we treated incipient IIM patients in our secondary/tertiary rheumatology centre.MethodsWe retrospectively included cases with IIM diagnosed between January 2010 and June 2018, who were followed at least 6 months. The remission inducing treatment and the treatment at the last follow-up were recorded.ResultsDuring the 102-month period we identified 102 IIM cases (71.6% female, median (IQR) age 62.7 (52.2–72.1) years): 27.5% dermatomyositis, 22.5% anti-synthetase syndrome, 14.7% myositis in an overlap syndrome, 13.7% immune mediated necrotizing myopathy, 11.5% polymyositis, 6.9% cancer associated myositis, 2.9% inclusion body myositis (IBM), 1% unspecified myositis. 94 (92.2%) patients received glucocorticoids (GC), 18/94 (17.6%) as monotherapy. The remaining 81 (79.4%) were treated with additional immunomodulatory agents (Table 1), most commonly methotrexate. In addition to therapy presented in Table 1, 13 (12.7%) also received human immunoglobulins (IVIG), one (1%) received plasma exchange therapy.Of the 8 (7.8%) cases who were not treated with GC 3 received methotrexate, 1 rituximab, and 4 no additional treatment (1 paraneoplastic polymyositis received chemotherapy, 2 IBM, 1 mild polymyositis in elderly, multimorbid patient).In the first 6 months following diagnosis, 11 patients died (3 due to infection, 2 as a consequence of IIM, 1 due to haemorrhagic shock, 5 of unknown causes), and 5 were lost to follow-up. The remaining 86 patients were followed for a median (IQR) 37.7 (20.2–62.6) months. At last follow-up 49 (57%) patients were still receiving GC, 8 (9.3%) in monotherapy. 9 (10.5%) cases were off GC and other maintenance treatment. Maintenance treatment at last follow-up is presented in Table 2. Treatment was changed in 13 (15.1%) patients (due to progression of pulmonary involvement in 3, active myositis in 4, pulmonary involvement progression and active myositis in 1 and due to intolerance to medication in 6 cases). In addition to therapy presented in Table 2, 2 (2.3%) patients received IVIG (1 concurrently with rituximab and mycophenolic acid due to recalcitrant anti-synthetase syndrome and 1 due to IBM complicated with dysphagia).Table 1Induction treatment of idiopathic inflammatory myopathy Type of treatment N (%) Cyclophosphamide; median dose (range)23 (22.5); 6 (2–12) gMethotrexate43 (42.2)monotherapy30 (69.8)+ cyclosporine a8 (18.6)+ cyclosporine a and rituximab4 (9.3)+ chloroquine1 (2.3)Rituximab monotherapy4 (3.9)Azathioprine3 (2.9)Mycophenolic acid3 (2.9)Cyclosporine a monotherapy1 (1.0)Table 2Maintenance treatment of idiopathic inflammatory myopathy Type of treatment N (%) Methotrexate35 (40.7)monotherapy20 (57.1)+ cyclosporine a11 (18.6)+ rituximab2 (5.7)+ chloroquine2 (5.7)Azathioprine monotherapy13 (15.1)Rituximab monotherapy3 (3.5)Mycophenolic acid15 (17.4) monotherapy13 (86.7) + tacrolimus1 (6.7) + rituximab1 (6.7)Cyclosporine a1 (1.2)Tocilizumab*1 (1.2...

BackgroundInternational Consensus Criteria for diagnosing Raynaud’s Phenomenon (RP) have been proposed recently (1). These criteria are based on the three-step conditional approach (i.e. a screening question, assessment of colour changes and an item of disease score calculation). It is unclear whether differences exist between the self-administered and interview-based way of questionnaire fulfilment.ObjectivesWe aimed in our study to evaluate the two approaches in patients with suspected RP.MethodsThis cross-sectional study was conducted at our secondary/tertiary rheumatology centre between 1 October 2018 and 31 December 2018. Each patient referred for the video capillaroscopy was asked to complete the RP questionnaire on the day of the referral. The same questionnaire was applied as an interview by the rheumatologist before the video capillaroscopy. Differences in answers and level of individual agreement on individual three items, as well as the result of the questionnaire, between the two ways of questionnaire completion were assessed with Fisher exact test and Kappa coefficient, respectively.ResultsDuring the 3-month period we included 84 consecutive patients (88.1% female, median age (IQR) 49.5 (41.9–56.5) years). Results of answers to each item, final outcome of the questionnaire and the level of individual agreement for each item are presented in Table 1.Table 1 Answers to Raynaud’s phenomenon International Consensus Criteria questionnaire according to mode of administrationSelf-administeredInterview basedp-value**Measure of individual agreement– Kappa coefficient (95% CI)Yes, N (%)Yes, N (%)Item 1. Are your fingers unusually sensitive to cold?79 (94.0%)78 (92.9%)10.514 (0.101–0.927)Item 2. Occurrence of biphasic colour change during the vasospastic episodes (white and blue)40 (47.6%)42 (50.0%)0.8770.762 (0.623–0.900)Item 3. RP disease score53(63.1%)67 (79.8%)0.0260.549 (0.330–0.747)Presence of RP according to RPICq31 (36.9%)36 (42.9%)0.5290.777 (0.640–0.915)*RP – Raynaud’s phenomenon; RPICq - International Consensus Criteria for the Diagnosis of Raynaud’s Phenomenon questionnaire, ** Fisher-exact testConclusionOverall, there was a substantial level of agreement between self-administered and interview-based application of International Consensus Criteria for the Diagnosis of Raynaud’s Phenomenon questionnaire. However, we observed that the patients were inclined to score the severity of their RP lower than the physicians.Reference[1] Maverakis E, Patel F, Kronenberg DG, Chung L, Fiorentino D, Allanore Y, et al. International consensus criteria for the diagnosis of Raynaud’s phenomenon. J Autoimmun2014;48–49:60–5.Disclosure of InterestsNone declared