Erosive arthritis in a patient with primary sjogren's syndrome: a case report

Primer Sjögren sendromu, sıklıkla ağız ve göz kuruluğu ile seyreden bir kronik otoimmun ekzokrinopatidir. Ağız içi bezleri ve göz yası bezleri dışında, nadir de olsa diğer ekzokrin bezler de etkilenebilir. En sık kas-iskelet sistem tutulusu ile karsımıza çıkmaktadır. Artralji, sabah tutukluğu ve romatoid artrite benzer kronik inflamatuvar poliartrit, eklem bulgularını oluşturmaktadır. Romatoid artrit'ten farklı olarak, Sjögren sendromunda sabah tutukluğu ve hareket kısıtlığı daha hafif olup, el ve el bilek deformasyonları görülmektedir. Romatoid artrit'ten ayıran en önemli özellik ise, direk grafi ve/veya magnetik rezonans görüntülerde, eklemlerde eroziv değişikliklerin olmamasıdır. Bu bildiride, primer Sjögren sendromu tanısı almış hastada, eroziv artrit rapor edilmiştir.Primary Sjogren's syndrome (SS) is an autoimmune exocrinopathy characterized by dry eyes and dry mouth. Exocrine glands other than salivary and lacrimal glands may be affected less frequently. The most common mode of presentation is musculoskeletal system involvement. Articular signs and symptoms include arthralgias, morning stiffness, and chronic polyarthritis that resemble those seen in rheumatoid arthritis (RA). Compared with RA, the arthritis tends to be more relapsing and remitting, and stiffness is less marked. The distinction fromRAis that, there is not any erosive changes neither on direct radiography nor magnetic resonance imaging (MRI).We report a patient of primary SS presented with erosive arthritis

Magnetic resonance ımaging of the sacroiliac joints in ankylosing spondilitis before and after therapy with anti-tumor necrosis factor alpha

AMAÇ: Çalışmanın amacı, dirençli AS'li hastalarda, anti-TNF-alfa ilaçların etkinliğini ve güvenirliğini yanısıra, manyetik rezonans (MR) görüntüleme ile tedavi öncesi ve sonrası sakroiliak eklem değişiklerini tespit etmektir. GEREÇ ve YÖNTEM: Modifiye New York tanı kriterlerine göre AS tanısı almış, 27 hasta çalışmaya dahil edildi. Sakroiilitis bulguları, anti-TNF-alfa tedavi öncesi ve sonrası, Gd-MR ile tespit edildi. Sekiz hastaya, 4 haftada bir İnfliximab 4 mg/kg i.v. infüzıon verildi. Diğer 19 hastaya ise Etanercept 2x25 mg/hafta s.c. verildi. Değerlendirilen klinik ve laboratuvar parametreler; BASDAı, BASFı, ağrı (VAS skoru), Schöber testi, göğüs ekspansiyonu, C-reaktif protein (CRP), eritrosit sedimentasyon hızı (ESH). BULGULAR: Hastaların çoğu, anti-TNF-alfa tedavilerine iyi yanıt verdi. 24. haftanın sonunda, takip edilen tüm parametrelerde iyileşme gözlendi. MR görüntüleme çalışmalarında, anti-TNF-alfa tedavi sonrası sadece 3 hastanın sakroiliak eklem inflamasyonunda gerileme gözlendi. SONUÇ: Aktif AS'li hastalarda, 24. hafta sonunda anti-TNF-alfa ilaçları güvenilir ve etkin bulundu. BASDAı, BASFı, ağrı skorlarında belirgin düşüş gözlendi. Fakat, sakroiliak eklemin akut inflamatuvar bulgularında, MR görüntüleme ile herhangi bir gerileme tespit edilmedi.OBJECTIVE: The goal of this study is to assess the changes in the sacroiliac joints (Sİ) by magnetic resonance imaging (MRI) in a 24-week follow-up period and to determine the efficacy and safety of anti-TNF-α therapies for refractory AS. MATERIALS and METHODS: Twenty-seven patients who met the modified New York criteria for AS were enrolled in this study. Activity of sacroiliitis was determined by Gd-MRI scan before and after anti-TNF-α treatment. Eight patients received infliximab at a dose of 4mg/kg by intravenous infusion over 2 hours at every 4 week. Other 19 patients were treated with 25mg subcutaneous etanercept twice weekly. Total observational period was 24 weeks. The clinical and laboratory variables included: Bath AS Disease Activity Index (BASDAI), Bath AS Functional Index (BASFI), pain on a visual analog scale, Schober's index, chest expansion, C-reactive protein(CRP), erythrocyte sedimentation rate (ESR). RESULTS: Most patients responded well to treatment of anti-TNF-α antagonists. At 24 weeks, there was an improvement in all of the following measures. Imaging studies showed decreased inflammation of the SI joints after 24 weeks of treatment with anti-TNF-α therapies in only 3 patients. CONCLUSION: The anti-TNF-α therapy was safe and effective in treating patients with active AS during 24-week study period. The BASDAI, BASFI, VAS of pain were decreased well. But we could not determine any regression of acute inflammatory changes of the SI joints as depicted by MR

Progressive skin fibrosis is associated with a decline in lung function and worse survival in patients with diffuse cutaneous systemic sclerosis in the European Scleroderma Trials and Research (EUSTAR) cohort.

Objectives To determine whether progressive skin fibrosis is associated with visceral organ progression and mortality during follow-up in patients with diffuse cutaneous systemic sclerosis (dcSSc). Methods We evaluated patients from the European Scleroderma Trials and Research database with dcSSc, baseline modified Rodnan skin score (mRSS) ≥7, valid mRSS at 12±3 months after baseline and ≥1 annual follow-up visit. Progressive skin fibrosis was defined as an increase in mRSS >5 and ≥25% from baseline to 12±3 months. Outcomes were pulmonary, cardiovascular and renal progression, and all-cause death. Associations between skin progression and outcomes were evaluated by Kaplan-Meier survival analysis and multivariable Cox regression. Results Of 1021 included patients, 78 (7.6%) had progressive skin fibrosis (skin progressors). Median follow-up was 3.4 years. Survival analyses indicated that skin progressors had a significantly higher probability of FVC decline ≥10% (53.6% vs 34.4%; p<0.001) and all-cause death (15.4% vs 7.3%; p=0.003) than non-progressors. These significant associations were also found in subgroup analyses of patients with either low baseline mRSS (≤22/51) or short disease duration (≤15 months). In multivariable analyses, skin progression within 1 year was independently associated with FVC decline ≥10% (HR 1.79, 95% CI 1.20 to 2.65) and all-cause death (HR 2.58, 95% CI 1.31 to 5.09). Conclusions Progressive skin fibrosis within 1 year is associated with decline in lung function and worse survival in dcSSc during follow-up. These results confirm mRSS as a surrogate marker in dcSSc, which will be helpful for cohort enrichment in future trials and risk stratification in clinical practice

Stratification in systemic sclerosis according to autoantibody status versus skin involvement: a study of the prospective EUSTAR cohort

Background: The current subclassification of systemic sclerosis into cutaneous subtypes does not fully capture the heterogeneity of the disease. We aimed to compare the performances of stratification into LeRoy's cutaneous subtypes versus stratification by autoantibody status in systemic sclerosis. Methods: For this cohort study, we assessed people with systemic sclerosis in the multicentre international European Scleroderma Trials and Research (EUSTAR) database. Individuals positive for systemic-sclerosis autoantibodies of two specificities were excluded, and remaining individuals were classified by cutaneous subtype, according to their systemic sclerosis-specific autoantibodies, or both. We assessed the performance of each model to predict overall survival, progression-free survival, disease progression, and different organ involvement. The three models were compared by use of the area under the curve (AUC) of the receiver operating characteristic and the net reclassification improvement (NRI). Missing data were imputed. Findings: We assessed the database on July 26, 2019. Of 16 939 patients assessed for eligibility, 10 711 patients were included: 1647 (15·4%) of 10 709 were male, 9062 (84·6%) were female, mean age was 54·4 (SD 13·8) years, and mean disease duration was 7·9 (SD 8·2) years. Information regarding cutaneous subtype was available for 10 176 participants and antibody data were available for 9643 participants. In the prognostic analysis, there was no difference in AUC for overall survival (0·82, 95% CI 0·81-0·84 for cutaneous only vs 0·84, 0·82-0·85 for antibody only vs 0·84, 0·83-0·86 for combined) or for progression-free survival (0·70, 0·69-0·71 vs 0·71, 0·70-0·72 vs 0·71, 0·70-0·72). However, at 4 years the NRI showed substantial improvement for the antibody-only model compared with the cutaneous-only model in prediction of overall survival (0·57, 0·46-0·71 for antibody only vs 0·29, 0·19-0·39 for cutaneous only) and disease progression (0·36, 0·29-0·46 vs 0·21, 0·14-0·28). The antibody-only model did better than the cutaneous-only model in predicting renal crisis (AUC 0·72, 0·70-0·74 for antibody only vs 0·66, 0·64-0·69 for cutaneous only) and lung fibrosis leading to restrictive lung function (AUC 0·76, 0·75-0·77 vs 0·71, 0·70-0·72). The combined model improved the prediction of digital ulcers and elevated systolic pulmonary artery pressure, but did poorly for cardiac involvement. Interpretation: The autoantibody-only model outperforms cutaneous-only subsetting for risk stratifying people with systemic sclerosis in the EUSTAR cohort. Physicians should be aware of these findings at the time of decision making for patient management. Funding: World Scleroderma Foundation

Racial differences in systemic sclerosis disease presentation: a European Scleroderma Trials and Research group study

Objectives. Racial factors play a significant role in SSc. We evaluated differences in SSc presentations between white patients (WP), Asian patients (AP) and black patients (BP) and analysed the effects of geographical locations.Methods. SSc characteristics of patients from the EUSTAR cohort were cross-sectionally compared across racial groups using survival and multiple logistic regression analyses.Results. The study included 9162 WP, 341 AP and 181 BP. AP developed the first non-RP feature faster than WP but slower than BP. AP were less frequently anti-centromere (ACA; odds ratio (OR) = 0.4, P < 0.001) and more frequently anti-topoisomerase-I autoantibodies (ATA) positive (OR = 1.2, P = 0.068), while BP were less likely to be ACA and ATA positive than were WP [OR(ACA) = 0.3, P < 0.001; OR(ATA) = 0.5, P = 0.020]. AP had less often (OR = 0.7, P = 0.06) and BP more often (OR = 2.7, P < 0.001) diffuse skin involvement than had WP.AP and BP were more likely to have pulmonary hypertension [OR(AP) = 2.6, P < 0.001; OR(BP) = 2.7, P = 0.03 vs WP] and a reduced forced vital capacity [OR(AP) = 2.5, P < 0.001; OR(BP) = 2.4, P < 0.004] than were WP. AP more often had an impaired diffusing capacity of the lung than had BP and WP [OR(AP vs BP) = 1.9, P = 0.038; OR(AP vs WP) = 2.4, P < 0.001]. After RP onset, AP and BP had a higher hazard to die than had WP [hazard ratio (HR) (AP) = 1.6, P = 0.011; HR(BP) = 2.1, P < 0.001].Conclusion. Compared with WP, and mostly independent of geographical location, AP have a faster and earlier disease onset with high prevalences of ATA, pulmonary hypertension and forced vital capacity impairment and higher mortality. BP had the fastest disease onset, a high prevalence of diffuse skin involvement and nominally the highest mortality

Behçet hastalığı olan olgularımızda, serum nitrat değerlerinin klinik ve laboratuar bulguları eşliğinde değerlendirilmesi

Bu tezin, veri tabanı üzerinden yayınlanma izni bulunmamaktadır. Yayınlanma izni olmayan tezlerin basılı kopyalarına Üniversite kütüphaneniz aracılığıyla (TÜBESS üzerinden) erişebilirsiniz.9- ÖZETBu çalışmada, Behçet Hastalığı ve normallerde, damar tortusu, trombosit adhezyonu ve agregasyonunda ve înflamatuar olayların gelişiminde efektör bir molekül olan NO etkisini araştırmak için, serum nitrit ve nitrat düzeyleri ölçülmüştür. Hasta kontrol grupları olarak, idyopatik eritema nodosum, idyopatik tekrarlayıcı oral aftöz ülserleri olan olgular ile, romatoid artrit, sistemik iupus eritematosus ve ankilozan spondiiitli olgular alınmıştır. Behçet Hastalığı, RA ve idyopatik eritema nodosumlu olgularda, normallere göre düşük, SLE, AS ve idyopatik oral aftöz ülserli olgularda ise, normallerden farksız serum nitrit+nitrat düzeyleri saptanmıştır. Behçet Hastalığı ile hasta kontrol grupları arasında ve hasta kontrol gruplarının kendi aralarında, serum nitrit+nitrat düzeyleri açısından anlamlı bir farklılık saptanmamıştır. RA'li grupta literatürden farklı olarak, düşük serum nitrit+nitrat düzeyleri saptamamızda, RA'li olgularımızın sağlıklı kontrol grubuna göre, daha yaşlı (sonuçlarımıza göre, normallerde yaş azaldıkça NO yapımı da azalmaktadır) ve remisyonda olan hastalardan oluşması ile, NO sentezini azalttığı bilinen methotreksat başta olmak üzere çeşitli temel etkili ilaçlar kullanıyor olmalarının etkili olduğu düşünülmüştür. Literatürde, SLE ve AS'li olgularda NO yapımı ile ilgili çalışma yoktur. SLE ve AS' li kontrol gruplarımız, sınırlı olmaları nedeniyle, gerçek hasta populasyonunu tam olarak yansıtmayabilir ancak, bu olgularda saptadığımız sağlıklı kontrol grubumuzdan farksız serum nitrit+nitrat düzeyleri, Behçet'ti olgulanmızdaki düşük serum nitrit+nitrat düzeylerini daha anlamlı kılmaktadır, idyopatik eritema nodosumlu olgu grubumuzda da, Behçet Hastalığı grubumuzda olduğu gibi, normallerden düşük serum nitrit+nitrat düzeyleri saptanmıştır. Bu düşüklüğün nedenleri veya Behçet Hastalığında sorumlu olan mekanizma(lar)ın burada da geçerli olup olmadığı belli değildir. Ancak idyopatik eritema nodosum ile eritema nodosum geliştirmiş Behçet olguları arasında, serum nitrit+nitrat düzeyleri açısından fark olmaması, bu ölçümlerin, Behçet Hastalığına bağlı eritema nodosum olgularını ayırmada kullanılamayacağını göstermektedir. Aynı şekilde, idyopatik ora! aftöz ülserli olgularımızda da, Behçefli olgularımızdan farksız serum nitrit+nitrat düzeyleri saptamamız, bu ölçümlerin oral aft ayırıcı tanısında da yararlı olmayacağını düşündürmektedir. Sağlıklı kontrol grubunda, yaş arttıkça serum nitrit+nitrat düzeyleri azalmaktadır (p0.01). Hasta kontrol gruplarında, yaş ile serum nitrit ve nitrat düzeyleri arasında korelasyon yoktur. Buna karşılık Behçefli olgularda, normallerde görülenin tersine, yaş arttıkça serum nitrit+nitrat düzeyleri artmaktadır (p0.05). Ayrıca Behçet Hastalığında serum nitrit ve nitrat düzeyleri, hastalık yaşı ile de pozitif bir korelasyon göstermektedir (p0.05). 38Klinik olarak inaktif Behçet olgularında, serum nitrit ve nitrat düzeyleri, normallerdeki düzeylerden farksızdır. Bundan başka, en az bir klinik bulgusu olan Behçefli olgularda ise, klinik olarak hiçbir aktif bulgusu olmayanlara göre, daha düşük serum nitrit ve nitrat düzeyleri saptanmıştır (p0.05). Başka bir deyişle, klinik aktivite ile serum nitrit ve nitrat düzeyleri düşmektedir. Behçet Hastalığında hasta yaşı ve hastalık yaşı arttıkça, klinik aktivite azalmaktadır. Bu nedenle, daha önce bahsettiğimiz, Behçefli olgularda hasta yaşı ve hastalık yaşı ile birlikte serum nitrit ve nitrat düzeylerinin artarak normallerdeki düzeylere yaklaşması da, Behçet Hastalığında serum nitrit ve nitrat düzeyleri ile klinik aktivite arasında paralellik olduğunu düşündüren bulgulardır. Behçet Hastalığında görülen semptomların, serum nitrit ve nitrat düzeyleri ile ilgisini araştırmak için olgular; kontrol sırasında bir semptoma klinik olarak aktif bir şekilde sahip olanlar ile olmayanlar olarak ayrıldıklarında, grupların serum nitrit+nitrat düzeyleri arasında, bazı semptomlar için istatistik olarak anlam kazanmayan göreli farklılıklar dışında, belirgin bir fark görülmemiştir. Aynı şekilde, olgular; bir semptomu hiç geliştirmemişler ile kontrol sırasında aktif olup olmadığına bakılmaksızın, o semptomu daha önce geliştirmişler olarak iki gruba ayrıldıklarında da, gruplar arasında, serum nitrit+nitrat düzeyleri bakımından istatistik olarak anlamlı bir fark saptanmamıştır. Behçefli olgularımızda saptadığımız düşük serum nitrit+nitrat düzeyleri nedeniyle, gelişen lezyonlannın çoğu vaskülitik kökenli olan Behçet Hastalığında ortaya çıkan hasarda, NOS enzimlerinin indüklenmesine bağlı olarak patolojik miktarlarda yapılan NO'in rolünün ön planda olmadığı düşünülebilir. Ancak, azalmış NO yapımı da, vasokonstriksiyona neden olarak ve trombosit adhezyonu ile agregasyonunu arttırarak, gelişen vaskülitik hasan arttırıcı bir rol oynayabilir. Çalışmamızda, Behçet Hastalığının klinik aktivite durumu ile serum nitrit ve nitrat düzeyleri arasında anlamlı bir ilişki saptanmıştır. Daha aktif olgulanmızdaki daha düşük serum nitrit+nitrat değerleri, literatürde, Behçet Hastalığında görüldüğü bildirilen belli başlı immünolojik bozukluklardan olan, klinik olarak aktifleşen olgularda, T4.T8 oranının ve NOS enzimini indükleyen önemli sitokinlerden biri olan ve başlıca Th1 hücrelerinde yapılan IFN^y düzeylerinin düşmesi ile de uyumludur. Yani, klinik aktivite gelişen Behçet' li olgularda, görüldüğü bildirilen bu immünolojik bozukluklar nedeniyle, NO yapımının daha az indüklendiği söylenebilir. Azalmış NO yapımı da, vaskülitik hasarın gelişmesine katkıda bulunabilir. 3

The Efficacy and Safety of Rituximab in a Patient with Rheumatoid Spondylitis

Rheumatoid arthritis (RA) is considered as a connective tissue disease while ankylosing spondylitis (AS) is a prototype of spondyloarthritis. These diseases are seen concomitantly only very rarely. Also, rituximab has proven efficacy in the treatment of RA while its role in the treatment of AS is unclear. In this presentation, the concomitant presence of RA and AS in a 43-year-old male patient as well as the efficacy and safety of rituximab is discussed. Rituximab was given due to lack of response to treatment with anti-TNF-alpha. Evaluations made at the 6th and 12th months of treatment showed complete response for RA and partial response for AS

Quality of life and diet: A paired match study on Behcet's disease

BACKGROUND: It has been reported that the quality of life and diet quality in individuals with rheumatological diseases are poor and may adversely affect the course of the disease. OBJECTIVE: This study aims to compare the quality of life and diet of individuals with Behcet's Disease (BD) compared to healthy controls. METHODS: This study was planned as a case-control study, and 60 adult patients with BD were compared with age (+/- 1) and sex paired match healthy controls concerning the quality of life and diet. Diet quality was assessed using nutrient adequacy ratio (NAR) and the mean adequacy ratio (MAR) values calculated from 24 h dietary food recall and obesity was also evaluated by various anthropometric measurements. The Short Form-36 Health Survey (SF-36) was used to evaluate the quality of life (QoL). In addition, the 24-hour physical activities were recorded to calculate physical activity levels (PAL). Data were analyzed by SPSS 25.0 via paired sample t-test and McNemar test. p 0.05 was deemed significant. RESULTS: The findings showed that cases were more obese (p = 0.005), less physically active (p 0.001), had lower QoL (p 0.01 for all subscales) and had higher Beck depression scores (p = 0.001). Controls had higher means of energy (p 0.001), CHO% (p = 0.025), fat% (p = 0.004), and fiber (p = 0.007) intake and mean MAR value (p 0.001). CONCLUSIONS: Compared to healthy controls, patients with BD were more obese, had lower QoL and lower diet quality. Therefore, people with BD should be evaluated for comorbid diseases and be supported by health professionals, such as dietitians and psychologists

Retrospective review of the clinical and laboratory data in silent lupus nephritis

Purpose To determine the ratio of renal disease necessitating immunosuppressive treatment in lupus patients who are clinically asymptomatic by means of renal disease. It was also examined whether silent lupus nephritis is associated with any of the non-renal clinical findings. Methods All kidney biopsies performed in lupus patients between 1990 and 2009 at the Rheumatology Department of Ege University Faculty of Medicine were retrospectively screened. Among the 258 kidney biopsies screened, 54 had no clinical renal findings but had active disease together with anti-dsDNA positivity and/or hypocomplementemia. Patients were classified into two groups who require and do not require immunosuppressive therapy according to their final pathological results at biopsy. The frequency of serious renal involvement in the sample was calculated. Then subgroups were compared with each other in terms of the clinical and laboratory features using Statistical Package for Social Sciences version 13 software. Results Thirteen of the 54 patients (24%) had severe renal involvement requiring immunosuppressant therapy. When the groups were compared to each other, it was found that serositis and hematologic involvement were significantly more frequent in patients who needed immunosuppressive treatment (42.9% versus 10.0%; p = 0.003 and 64.3% versus 37.5; p = 0.039). Conclusion Even in the absence of clinical renal manifestations, active patients at high risk of renal disease such as hypocomplementemia, anti-ds DNA positivity may have severe renal disease requiring immunosuppressive treatment. Thus, renal biopsy indications in lupus patients should better be revaluated