The objective of the study is to compare the longitudinal performance of different classification criteria for primary Sjögren's syndrome (SS) in a cohort of patients previously diagnosed with primary SS. In each patient, we repeated diagnostic tests as required by the Copenhagen, European, Californian, and American-European Consensus Group (AECG) or the new American College of Rheumatology (ACR) classification criteria. Sixty-three out of 90 eligible patients (70 %) consented to participate. During the follow-up (mean (standard deviation, SD) 7.6 years (0.5)), we observed evolution from primary SS to SS with another systemic autoimmune disease (SAD) in 9/63 (14 %) patients, on average after 4.0 years (SD 0.9). The evolution from primary SS to SS-SADs was significantly more common if the diagnosis of primary SS was initially made using AECG (17 %, p = 0.008) or ACR (16 %, p = 0.016) criteria. In the 34 patients who underwent a full diagnostic reassessment, the diagnosis retention rate was statistically significant for all the criteria, except the European criteria. At reassessment, 3/32 (9 %) patients initially diagnosed as having primary SS using the European criteria could not be classified as having primary SS by any of the criteria. The differences in classification when using the AECG and the new ACR criteria were not statistically significant. The longitudinal diagnosis retention rate was highest for the Californian and AECG criteria. Regardless of the classification criteria, some patients eventually develop another SAD.
Background:Adult Onset Still’s disease (AOSD) is an uncommon systemic inflammatory disease.Objectives:To determine for the first time the incidence rate of AOSD in our population.Methods:We retrospectively collected AOSD cases diagnosed between 1 January 2010 and 31 December 2019 at our secondary/tertiary rheumatology centre, which is the only referral centre for an average population of 704,000 adults. AOSD cases were identified by searching the electronic medical database both for ICD-10 code M06.1 and a full text search for »AOSD«. Patients’ records were analyzed and descriptive statistics was used to describe our study group. The adult population was obtained from the national statistics institute database. The annual incidence rate for AOSD was calculated.Results:During the 10-year observation period we identified 22 incipient AOSD cases. All 22 cases fulfilled Yamaguchi classification criteria for AOSD1. Five cases were excluded from analyses since they were referred to our department from regions served by other secondary/tertiary centres. Hence, we finally analyzed 17 AOSD cases (11 females; median (IQR) age 38.9 (29.9; 56.5) years, range 20-71 years), resulting in the average annual incidence rate of 2.4 (95%CI 1.5-3.8) cases per 106adults. Age specific incidence rate of AOSD is presented in Figure 1. Clinical characteristics of AOSD cases at presentation are shown in Table 1. AOSD was complicated with macrophage activating syndrome in 4/17 (23.5%) cases, and with pulmonary hypertension in one case. Patients were followed for a median (IQR) 31 (20; 58) months. Twelve (70.5%), 2 (11.8%), and 3 (17.6) patients had monophasic, relapsing, and chronic disease course, respectively.Table 1.Clinical characteristics of AOSD at presentationCharacteristicAOSD (%)CharacteristicAOSD (%)Female gender64.7Lung infiltrates23.5Age*38.9 (29.9;56.5)Pericardial effusion23.5Fever94.1Abdominal pain17.6Weight loss64.7Lymphadenopathy52.9Skin rash76.5Splenomegaly41.2Throat pain88.2Hepatomegaly17.6Arthralgia/Arthritis88.2/47.1Leukocytosis88.2Myalgia29.4Elevated AST/ALT88.2Pleural effusion23.5Ferritin >1000ng/ml94.1Legend: *median (IQR);Figure 1.Age specific incidence rate of AOSDConclusion:AOSD is rare in our population, with an average annual incidence rate of 2.4 cases per 106adults.References:[1]Yamaguchi M, Ohta A, Tsunematsu T, Kasukawa R, Mizushima Y, Kashiwagi H, et al. Preliminary criteria for classification of adult Still’s disease. J Rheumatol. 1992;19(3):424-30.Acknowledgments -Disclosure of Interests:ALOJZIJA HOCEVAR: None declared, Ziga Rotar Consultant of: Speaker and consulting fees from Abbvie, Amgen, Biogen, Eli Lilly, Medis, MSD, Novartis, Pfizer, Roche, Sanofi., Speakers bureau: Speaker and consulting fees from Abbvie, Amgen, Biogen, Eli Lilly, Medis, MSD, Novartis, Pfizer, Roche, Sanofi., Monika Krosel: None declared, Martina Plešivčnik Novljan: None declared, Sonja Praprotnik: None declared, Matija Tomsic: None declared
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
BackgroundIn early rheumatoid arthritis (RA), first assessment by a rheumatologist and/or initiation of disease-modifying anti-rheumatic drugs (DMARD) within 12 weeks of symptom onset are associated with a significant benefit in long-term disease outcome.ObjectivesOur objective was to determine the proportion of patients with newly diagnosed RA in whom first rheumatology assessment and/or initiation of DMARD therapy was within the desired time frame.MethodsA retrospective chart review of adult patients diagnosed with RA during years 2014 and 2015 was performed at the rheumatology department of an integrated secondary/tertiary teaching hospital that provides rheumatology services for a population of more than 500.000 residents. Potential cases were identified by searching the electronic medical records for ICD-10 codes M05.* and M06.* Electronic and paper records of patients were then thoroughly reviewed. In addition to demographic and clinical data, dates were recorded for onset of inflammatory joint symptoms, referral to rheumatologist, initial assessment by a rheumatologist and initiation of DMARD therapy. The percentage of patients assessed by a rheumatologist and/or treated with a DMARD within 12 weeks of symptom onset and the median times for delay were then calculated.ResultsBetween January 1st 2014 and December 31st 2015, 243 new cases of RA were identified at our Department of Rheumatology. Of those, 197 (81.1%) were referred to our early interventional clinic. Within 12 weeks of symptom onset, 111 (45.7%) new RA patients were examined by a rheumatologist and 87 (35.8%) were started on DMARD therapy; the median time from symptom onset to consultation was 13.0 (IQR 4.6–27.8) weeks, median time from referral to consultation was 1 (IQR 1–3) days and median DMARD treatment delay was 15.7 (IQR 8.7–31.9) weeks.Table 1.Demographic data, clinical history, and delaysGender (female/male) (%)183/60 (75/25)Age, years (median)64.2 (IQR, 52.1–75.9)Patients fulfilling 2010 ACR/EULAR classification criteria for RA, N (%)228 (93.8)DAS28 3v (mean ± SD)5.3±1.3Erosive disease (plain radiographs) at first rheumatologist assessment, N (%)67 (31.5)Time from symptom onset to first rheumatologist assessment, weeks (median)13.0 (IQR, 4.6–27.8)Time from referral to first rheumatologist assessment, weeks (median)0.14 (IQR, 0.14–0.43)Time from symptom onset to glucocorticoid initiation, weeks (median)13.1 (IQR, 5.6–26.9)Time from symptom onset to DMARD initiation, weeks (median)15.7 (IQR, 8.7–31.9)Conclusions46% of new RA patients were assessed by a rheumatologist and 36% were treated with a DMARD within the recommended time frame of 12 weeks. Most of the treatment delay was due to the time elapsed between symptom onset and referral to a rheumatologist. These results substantiate the efficacy of our early interventional clinic in diagnosing and treating patients with early RA: despite the heavily protracted nationwide waiting times for first rheumatologist assessment and significantly (40%) lower number of rheumatologists per capita c...
BackgroundIn early rheumatoid arthritis (RA), first assessment by a rheumatologist and/or initiation of disease-modifying anti-rheumatic drugs (DMARD) within 12 weeks of symptom onset are associated with a significant benefit in long-term disease outcome.1,2ObjectivesTo determine the proportion of patients with newly diagnosed RA in whom first rheumatology assessment and/or initiation of DMARD therapy was within the desired time frame.MethodsA retrospective chart review of adult patients diagnosed with RA during year 2014 and the first half of year 2015 was performed at our rheumatology department, which is a part of an integrated secondary/tertiary teaching hospital that provides rheumatology services for a population of more than 500.000 residents. Potential cases were identified by searching the electronic medical records for ICD-10 codes M05.* and M06.* Electronic and paper records of patients were then thoroughly reviewed. Dates were recorded for onset of inflammatory joint symptoms, referral to rheumatologist, initial assessment by a rheumatologist and initiation of DMARD therapy. The percentage of patients assessed by a rheumatologist and/or treated with a DMARD within 12 weeks of symptom onset and the median times for delay were then calculated.ResultsBetween 01.01.2014 and 30.06.2015, 188 new cases of RA were identified at our Department of Rheumatology. Of those, 153 (81.4%) were referred to our early interventional clinic. Within 12 weeks of symptom onset, 89 (47.3%) new RA patients were examined by a rheumatologist and 68 (36.2%) were started on DMARD therapy; the median time from symptom onset to consultation was 12.8 (IQR 4.9–27.7) weeks, median time from referral to consultation was 1 (IQR 1–3) day and median DMARD treatment delay was 16.1 (IQR 8.6–32.8) weeks.Table 1.Demographic data, clinical history, and delaysGender (female/male) (%)143/45 (76/24)Age, years (mean ± SD)62.4±15.4DAS28 3v (mean ± SD)5.3±1.3Patients fulfilling 2010 ACR/EULAR classification criteria for RA, # (%)177 (94.1)Time from symptom onset to first rheumatologist assessment, weeks (median)12.8 (IQR, 4.9–27.7)Time from referral to first rheumatologist assessment, weeks (median)0.14 (IQR, 0.14–0.43)Time from symptom onset to glucocorticoid initiation, weeks (median)13.0 (IQR, 5.8–27.2)Time from symptom onset to DMARD initiation, weeks (median)16.1 (IQR, 8.6–32.8)Legend: SD: standard deviation, IQR: interquartile range.Conclusions47% of new RA patients were assessed by a rheumatologist and 36% were treated with a DMARD within the recommended time frame of 12 weeks. Most of the treatment delay was due to the time elapsed between symptom onset and referral to a rheumatologist. These results substantiate the efficacy of our early interventional clinic in diagnosing and treating patients with early RA: despite the heavily protracted nationwide waiting times for first rheumatologist assessment and significantly (40%) lower number of rheumatologists per capita compared to European Union average, the percentage of timely treated patients...
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