Background:Scleroderma is a connective tissue disease that is characterized by fibrosis of the skin.The modified Rodnan Skin Score (mRSS) is a measure generally used to assess the skin thickness in patients with scleroderma.Data on the effectiveness of the mRSS training courses differ in the literature.Objectives:The objective of our study was to evaluate the effectiveness of the mRSS training course in rheumatology fellows in the rheumatology departments.Methods:The study included 6 fellows from the departments of rheumatology.Participants were given a 1-hour-long theoretical training, including dermal involvement, and mRSS assessment by 3 rheumatology experts experienced in scleroderma, which was followed by an applied training on 4 patients for one-hour.Participants scored two patients before and after training on a form, which included 17 domains with a total score-range between “0” and “51”. Then using the SPSS15 software program, inter-rater reliability was assessed with intraclass correlation(ICC) analysis for both pre- and post-training mRSS.Fleiss’ kappa was used to measure the degree of agreement according to 12 Rodnan score areas before and after the training.Results:The ICC value for pre-training and post-training total Rodnan scores was 0.867 (95% CI-0.625-1.00, P=0.05), and 0.905(95% CI 0.045-1.00, P=0.02), respectively. Individual analysis of score areas showed that after the training there was an increase in degree of agreement in some of these areas, while there was no difference in one area, and it decreased in others (Table 1).Conclusion:Several studies have demonstrated the applicability, reliability, and validity of mRSS, a measure of dermal involvement, and that evaluation of the score requires experience, and an attentive learning process.In the literature, the inter-rater ICC values during previous training courses are reported to range between 0.378 and 0.92(1). These studies show differences in terms of the number and experience of participants, number of patients, course-length, and repeated courses. Similar to the observation by Ionescu et al(2), the pre-training inter-observer ICC value was very high(0.867), and it increased to 0.905 after the training. Presence of an increase in some areas, and reduction in some others in individual analysis of areas after the training may suggests the need for repeated training. Our limitations were low number of participants and patients. We obtained very good inter-rater ICC values in the mRSS training course given to rheumatology fellows; however we may benefit from new studies for optimization of these conclusions by increasing the numbers of trainees, patients, and the lenght of courses.References[1] Czirjak, L., et al., the EUSTAR model for teaching and implementing the modified Rodnan skin score in systemic sclerosis. Ann Rheum Dis, 2007.[2] Ionescu, R., et al., Repeated teaching courses of the modified Rodnan skin score in systemic sclerosis. Clin Exp Rheumatol, 2010.Disclosure of interests:None declared
Propylthiouracil is a drug commonly used in patients with hyperthyroidism. Some patients with Graves using this drug may have a positive perinuclear antineutrophil cytoplasmic antibody (p-ANCA) associated with different forms of vasculitis and neutrophilic dermatoses. However, skin lesions such as maculopapular purpura, erythema, sensitive nodules, ulceration, vesiculobullous lesions, and livedo can be induced by antineutrophil cytoplasmic antibody (ANCA) -positive vasculitis. In these cases, the development of pyoderma gangrenosum (PG) is very rare. We aimed to present a concurrent development of pyoderma gangrenosum, p-ANCA positivity and inraalveolar hemorrhage in a patient who had been using PTU for a long time and to discuss the current literature with this presentation. We think that the baseline ANCA levels should be measured in all graves patients because of severe ANCA-related vasculitis in some patients during PTU therapy
Background:Limited joint mobility (LJM) is a musculoskeletal disorder caused by flexion contractures of hand is a common complication in systemic sclerosis (SSc) patients. The distal parts of the upper limb (hands and fingers) is the most involved site in SSc.Objectives:In this study, we aimed to evaluate LJM in SSc patients and to determine the relationship between the clinical features of the disease.Methods:A total of 113 patients (>18 years old) diagnosed with diffuse cutaneous systemic sclerosis (DcSSc) and limited cutaneous systemic sclerosis (LcSSc) and 104 healthy controls were included in this study. LJM was evaluated with “prayer sign” and “table top sign” tests. LJM staging was done by Rosenbloom classification method(1, 2). LJM (+) and LJM (-) patients were compared in terms of demographic findings (gender, age and duration of disease), laboratory results (ESR, CRP, ANA, anti-topoisomerase I and anti-centromere) and modified Rodnan Skin Score (mRSS) results.Results:In our study, a total of 113 patients diagnosed with SSc and 104 healthy controls with similar age and gender distribution were included. While LJM (+) was detected in 75 (66.4%) (LcSSc = 38, DcSSc = 37) of the patients diagnosed with SSc, LJM (mild) (+) was detected in 3 (2.8%) of the control group. One of these people had right 2nd DIF joint contracture due to osteoarthritis, and 1 patient was found to have simple contractures due to minor hand injury previously (Table 1). A statistically significant difference was observed in between LcSSc and DcSSc patients according to the presence of LJM (p<0.001) (Table 2). There was a moderate positivity relationship between LJM and mRSS (LcSSc: r=0.449 ve p<0.001, DcSSc: r= 0.565 ve p<0.001) (Figure 1).Table 1.Comparison of demographic findings between SSc and Control groupSSc Group (n=113)Control Group (n=104)p valueAge, year57.02 ± 11.5858.47 ± 11.260.061Gender (F / M)98 (86.7) / 15 (13.3)65 (62.5) / 39 (37.5)0.064CRP (mg/L)5.45 ± 5.392.14 ± 1.12<0.001ESR (mm/hr)25.19 ± 18.914.46 ± 10.090.024Smoking, n (%)Smoker89 (78.8)70 (67.3)0.464Non-Smoker24 (21.2)34 (32.7)LJM (Absent / Present)Present75 (66.4)3 (2.8)<0.001Absent38 (33.6)101 (97.2)Rosenbloom classificationLcSSc (n=71) (%)DcSSc (n=42) (%)Total (n=113) (%)Normal46.511.933.6Mild22.514.319.5Moderate23.933.327.4Severe7.140.519.5Table 2.Comparison of demographic and clinical findings LJM(-) and LJM(+) in SScLJM (-) (n=38)LJM (+) (n=75)p valueAge, year54.16 ± 11.8258.47 ± 11.260.061SSc Typen (%)n (%)LcSSc, n (%)DcSSc, n (%)33 (56.8)38 (50.7)<0.0015 (13.2)37 (49.3)Gender,F/M (%)37 (97.3) / 1 (2.7)61 (81.3) / 14 (18.7)0.018Raynaud’s (symptom duration), month148 (44-456)150 (35-588)0.990Non-raynaud (symptom duration), month108 (28-458)138 (38-447)0.132mRSS, median2 (0-14)8 (0-36)<0.001CRP (mg/L)4.21 ± 4.486.08 ± 5.710.069ESR (mm/hr)19.74 ± 1027.95 ± 21.60.270Renal crisis, n (%)1 (2.6)4 (5.3)0.662PAH, n (%)8 (21.1)14 (18.7)0.762ANA positivity, n (%)36 (94.7)70 (93.3)1Anti-centromere positivity, n (%)18 (47.4)19 (25.3)0.01Anti-topoisomerase-1, n (%)8 (21)34(45.3)0.01Smoking, n (%)n (%)n (%)Non-smoker30(78.9)59 (78.7)0.970Smoker8 (21.1)16 (21.3)Figure 1.Conclusion:In our study, it was found that LJM staging positively correlated with mRSS and DcSSc patients had more severe LJM findings than LcSSc. We conclude that “prayer sign” and “table top sign” tests used in hand evaluation in SSc patients have similar clinical results with mRSS and can be easily performed in daily practice in about 3 minutes.References:[1]Rosenbloom AL. Limitation of finger joint mobility in diabetes mellitus. The Journal of diabetic complications 1989; 3: 77-87.[2]Nashel J, Steen V. Scleroderma mimics. Current rheumatology reports 2012; 14: 39-46.Disclosure of Interests:None declared
BackgroundIgG4-related disease is a recently recognised inflammatory disease of unkown etiology, often seen in men over the age of 50 and may affect many organs and systems with elevated serum IgG4 levels and typical histopathological features.ObjectivesThe aim of this study is to determine the demographic and clinical characteristics of patients with IgG4-related disease.MethodsPatients diagnosed as having Ig-G4-related disease by their typical histopathological findings and imaging features and/or increased serum IgG4 concentrations (> 135 mg/dl) from two university hospital in Izmir were included in the study.ResultsThere were 53 patients with a mean age of 51.49 yrs (69.8% male). The most common involvement was retroperitoneal fibrosis (54.7%), followed by the cardiovascular system (CVS) involvement (45.3%) (Table 1). While 22 patients had at least two organ involvement, the most common association was retroperitoneal fibrosis and CVS involvement (15 patients). Serum IgG4 levels were studied in 36 patients (67.9%) and found to be higher levels in 20 patients. (55.5%) (Table 2). In 44 patients (83%), acute phase reactants (APRs) were increased at the time of the diagnosis. There was no correlation between the extent of the disease and serum IgG4 levels and initial erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP) values. 28 patients (52.8%) were diagnosed by imaging, 9 (17%) by imaging and IgG4 elevation, 5 (9.4%) by imaging and histopathology, 10 (18.9%) by imaging, histopathology and IgG4 elevation and 1 patient (1.9%) by only histopathology. The most commonly (58.5%) used imaging method for diagnosis was computed tomography (CT). All the patients used initial glucocorticoid treatment.4 patients (7.5%) recieved only glucocorticoid,others were underwent the following treatments combined with glucocorticoid: azathioprine (AZA) (60.4%); methotrexate (mtx) (11.3%), rituximab (RTX) + AZA (9.4%), mtx + AZA (5.7%), RTX (3.8%) and infliximab (1.9%).In the follow-up, a significant decrease in acute phase reactants was found in 62% of the patients at their last visits. While 27.3% of the patients had complete remission, 36.4% had partial remission, 20.5% had stable course, 13.6% had progression in the disease and 2.3% had recurrence. In 18 patients (64.3%) out of 28 patients who were in partial or complete remission, remission was achieved by using glucocorticoid and AZA combination treatment.ConclusionIn conclusion, we have described a considerably large serie of patients with IgG4-related disease from Turkey. The results of the study suggested that AZA and glucocorticoid combination treatment was commonly used in Turkish patients with IgG4-related disease and it might be a good treatment option to achieve remission.References[1] Terumi Kamisawaet al; IgG4-related disease, Lancet2015; 385: 1460–71Disclosure of InterestsAydan Köken Avşar: None declared, Önay Gerçik: None declared, Gerçek Can: None declared, Berrin Zengin: None declared, Sinem Burcu Kocaer: None declared, Atilla Okan Kılıç: None declared,...
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