Survival in patients with systemic sclerosis: A single center cohort

Sistemik sklerozis (SSk), oto-antikor pozitifliği, vaskülopati, deride ve iç organlarda progresif fibrozis ile karakterize olan kronik bir hastalıktır. Hastalığın tutulumuna bağlı olan veya hastalıkla ilişkili olmayan sebeplerle SSk’de yaşam beklentisi azalmıştır. SSk’a bağlı ölüm oranları yıllar içerisinde azalma göstermiş olsa da, genel popülasyona oranla hala yüksek seyretmektedir. Çalışmamızda, merkezimizde takip ettiğimiz SSk hastalarının başlıca ölüm sebeplerini ve ilişkili klinik, laboratuvar ve demografik özelliklerini bildirmeyi amaçladık. Kocaeli Üniversitesi Tıp Fakültesi Romatoloji Polikliniği’nde 2007-2022 arasında SSk tanısı ile takip edilen 168 hasta arasından 2013 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) sınıflama kriterlerini dolduran 157 hasta dahil edildi. Hastaların çoğu (%66,4) sınırlı cilt tutulumlu, hastalık süresi 10,3 (±6,3) yıl olan kadınlardan (%88) oluşmaktaydı. 15 yıllık takip sırasında 23’ü (%14,6) hayatını kaybedildi. Ölen hastaların tanı sırasındaki yaşları daha ileri, dijital ülserleri (aktif ve inaktif) ve malignite sıklığı daha fazla saptandı. İki hasta grubu arasında oto-antikor ve major organ tutulumu açısından fark görülmedi. Kohortumuzdaki en sık ölüm sebebi enfeksiyon (n=5, %21,7) olup bu hastaların 3’ü (%13) COVID19 ve 2’si (%8,7) başka enfeksiyöz etkenlere bağlı pnömoni sebebiyle kaybedildi. Enfeksiyondan sonra en sık ikinci ölüm sebebi malignite (n=4, %17,4) olarak saptandı. Cox regresyon analizi sonucunda tanı sırasındaki yaşta yaklaşık 1 yıl artış ölüm riskinde 10 kat artış (hazard oranı (HR)= 10,93; %95 GA=10,51-11,37) ile ilişkili bulundu. Sonuç olarak, çalışmamız ülkemizde dikkatli takip edilen SSk kohortlarından bildirilmiş az sayıdaki sağ kalım verilerine katkı sağlamaktadır. Sonuçlarımız yorumlanırken SSk hastalarının klinik özelliklerinin coğrafi ve etnik farklılıklar gösterebileceği göz önünde bulundurulmalıdır.Systemic sclerosis (SSc) is a chronic disease characterized by auto-antibody positivity, vasculopathy, and progressive fibrosis in the skin and internal organs. Life expectancy in SSc is decreased due to reasons both related to the involvement of the disease or not related to the disease. Although mortality rate due to SSc have decreased over the years, it still remains increased compared to the general population. In our study, we aimed to report the main causes of death and associated clinical, laboratory and demographic characteristics of SSc patients followed in our center. Among 168 patients followed up with the diagnosis of SSc in Kocaeli University Faculty of Medicine Rheumatology Polyclinic between 2007 and 2022, 157 patients who fulfilled the 2013 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) classification criteria were included. Most of the patients (66.4%) were women (88%) with limited skin involvement and the disease duration was 10.3 (±6.3) years. During the 15-year follow-up, 23 (14.6%) patients died. The patients who died were older at the time of diagnosis with more digital ulcers (active and inactive) and malignancy. There was no difference between the two patient groups in terms of auto-antibody and major organ involvements. The most common cause of death in our cohort was infection (n=5, 21.7%), and 3 (13%) of these patients died due to COVID19 and 2 (8.7%) due to pneumonia due to other infectious agents. The second most common cause of death after infection was found to be malignancy (n=4, 17.4%). As a result of Cox regression analysis, an increase of approximately 1 year in age at diagnosis was associated with a 10-fold increase in the risk of death (hazard ratio (HR) = 10.93; 95% CI = 10.51-11.37). In conclusion, our study contributes to the few survival data reported to date from SSc cohorts in our country that are carefully followed. While interpreting our results, it should be considered that the clinical features of SSc patients may show geographical and ethnic differences

New computed tomography-based parameters for monitoring treatment response in systemic sclerosis-associated interstitial lung disease

Yüksek çözünürlüklü bilgisayarlı tomografi (YÇBT) ve solunum fonksiyon testi (SFT) günümüzde Sistemik Skleroza bağlı interstisyel akciğer hastalığının (SSk-İAH) tanısında, tedavi kararında ve tedavinin takibinde kullanılan başlıca yöntemdir. Çalışmamızın amacı, YÇBT görüntülerini analiz ederek hastaların takibinde kullanılabilecek yeni kantitatif parametreleri araştırmaktır. Çalışmaya SSk-İAH nedeniyle tedavi gören, başlangıç ve 12.ay takipleri sırasında YÇBT ve SFT sonuçlarına ulaşılabilen 35 hasta dahil edildi. Hastaların YÇBT görüntüleri Vitrea® Advanced Visualization yazılımı ile analiz edildi. Akciğer atenuasyon eğrisi kullanılarak sağ ve sol akciğerler için ortalama akciğer atenuasyonu (OAA), düşük-, orta- ve yüksek-yoğunluklu akciğer hacimleri, düşük dansite (DD) indeksi ve yüzde dansite 15 (YD15) elde edildi. Elde edilen parametrelerin zaman içerisindeki değişimi ve SSk-İAH hastalarında tedaviye yanıtı değerlendirmek için kullanılan FVC ve DLCO testleri ile arasındaki ilişki incelendi. Çalışmadaki hastaların %82,9’u kadın, %63’ü diffüz cilt tutulumlu, hastalık süresi 8,13±5,96 yıl ve ortalama yaş 53,8±9,96’tı. Takip FVC, DLCO ve tüm BT parametrelerinde başlangıca göre bir değişim saptanmadı. Başlangıç ve takipte DLCO’nun BT parametrelerinden hiçbiri ile korelasyon göstermediği, ancak FVC’nin hem başlangıçta hem de takipte sağ/sol orta- yoğunluklu akciğer volümü ve sağ/sol total akciğer hacmi ile ilişkili olduğu saptandı. Ayrıca fibrozis skoru ile hem başlangıç hem de takipteki sağ ve sol akciğer ortalama atenuasyonu, orta dansiteli volüm, yüksek dansiteli volüm ve akciğer volümü ile orta-kuvvetli ilişki saptandı. Çalışmamızın sonucunda, SSk-İAH hastalarında hem başlangıç hem de takip FVC ve fibrozis skoru ile anlamlı korelasyon gösteren kantitatif YÇBT parametreleri saptanmıştır. Sonuçlarımız, SFT gibi hasta kooperasyonundan ve semi-kantitatif fibrozis yaygınlık skoru gibi radyologların değerlendirmesinden etkilenmeyen parametrelerin geliştirilmesine katkı sağlayacaktır.High-resolution computed tomography (HRCT) and pulmonary function test are currently the main methods used in the diagnosis, treat ment decision, and follow-up of systemic sclerosis-associated interstitial lung disease (SSc-ILD). We aimed to analyze new quantitative parameters that can be used in the follow-up of patients by analyzing HRCT images. 35 patients whose HRCT and PFT results were available at baseline and 12th-month follow-up were included in the study. HRCT images of the patients were analyzed with Vitrea® Advanced Visualization software. Using the lung attenuation curve, mean lung attenuation, low-, medium-, and high-density lung volumes, low-density index, and percent density 15 were obtained. Of the patients in the study, 82.9% were female, 63% had diffuse skin involvement, the disease duration was 8.13±5.96 years, and the mean age was 53.8±9.96 years. There was no change in follow-up FVC, DLCO, and CT parameters from baseline. DLCO did not correlate with any of the CT parameters at bas eline and follow-up, but FVC was associated with right/left medium-density lung volume and right/left total lung volume at baseline and follow-up. In addition, a moderate-strong relationship was found between the fibrosis score and the mean attenuation, medium-density volume, high-density volume, and lung volume both at baseline and at follow-up. Quantitative HRCT parameters were found to be significantly correlated with both baseline and follow -up FVC and fibrosis scores in SSc-ILD patients. Our results will contribute to the development of quantitative parameters that are not affected by patient cooperation, or the evaluation of radiologists' experience

Predictive factors for work-day loss in Behçet's syndrome: A multi-center study

Objective: The aim of this multi-center study was to assess predictive factors for work-day loss as an indirect cost element in Behçet's syndrome (BS). Methods: In this cross-sectional, multi-center study, 834 BS patients (F/M: 441/393, age mean: 38.4 ± 10.9 years) were included. Data were collected by a questionnaire regarding treatment protocols, disease duration, smoking pattern, frequency of medical visits during the previous year and self-reported work-day loss during the previous year. Results: Work-day loss was observed in 16.2% of patients (M/F: 103/32). The percentages of being a smoker (81.8%), using immunosuppressive (IS) medications (82%), and having disease duration <5 years (74%) were higher in male patients with work-day loss (P < .05). The majority of males (90.9%) had more than four clinic visits during the previous year. Moreover, the mean work-day loss (30.8 ± 57.7 days) was higher in patients with vascular involvement (56.1 ± 85.9) than those without (26.4 ± 50.6 days) (P = .046). In addition, increased frequency of ocular involvement (25.9%) was also observed in patients with work-day loss compared to others (8.6%) (P = .059). Conclusion: Work-day loss was associated with both vascular and ocular involvement. Close associations were observed among male gender, early period of the disease, frequent medical visits, being a smoker and treatment with IS medications in patients with work-day loss.PubMe

Extended autoantibody panel in Turkish patients with early‐stage systemic sclerosis: Coexpressions and their influences on clinical phenotypes

Abstract Background/Aim To investigate the frequency and clinical relevance of an extended autoantibody profile in patients with systemic sclerosis (SSc). Materials and Methods In this cross‐sectional study, serum from 100 consecutive patients was subjected to indirect immunofluorescence (IIF) (HEp‐20‐10/primate liver mosaic) and Systemic Sclerosis Profile by EUROIMMUN to evaluate anti‐nuclear antibodies (ANA) and autoantibodies against 13 different autoantibodies in patients with SSc less than 3 years. Results Ninety‐three of 100 patients were positive for ANA by IIF. Fifty‐three patients showed single positivity, 26 anti‐topoisomerase antibodies (anti‐Scl70 ab), 16 anticentromere antibodies (ACAs), six anti‐RNA polymerase III antibodies (anti‐RNAPIII ab), one anti‐Ku antibody, one anti‐PM/Scl100 antibody, two anti‐PM/Scl75 antibodies, one anti‐Ro52 antibody, whereas 32 patients had multiple autoantibody positivities. Among classic SSc‐specific autoantibodies, anti‐Scl70 and anti‐RNAPIII abs showed the highest cooccurrence (n = 4). One patient was simultaneously positive for anti‐RNAPIII ab and ACA, and one was positive for ACA and anti‐Scl70 ab. The clinical features were not statistically different between single and multiple autoantibody‐positivity for classic SSc‐specific autoantibodies (ACA, anti‐Scl70 ab, and anti‐RNAPIII ab), except for digital ulcer in the multiantibody positive ACA group (p = .019). Conclusion Based on our results, coexpression of autoantibodies is not uncommon in SSc patients. Although autoantibodies specific to SSc in early disease show generally known clinical features, it remains to be investigated how the coexpression of autoantibodies will affect clinical presentation

Oral ulcer activity assessment with the composite index according to different treatment modalities in Behçet's syndrome: a multicentre study

Objectives: The aim of this multicentre study was to understand patients' needs and to evaluate the oral ulcer activity with the Composite Index (CI), according to different treatment modalities in Behçet's syndrome (BS). Methods: BS patients (n=834) from 12 centres participated in this cross-sectional study. Oral ulcer activity (active vs. inactive) and the CI (0: inactive vs. 1-10 points: active) were evaluated during the previous month. The effects of treatment protocols [non-immunosuppressive: non-IS vs. immunosuppressive: (ISs)], severity (mild vs. severe), disease duration (<5 years vs. ≥5 years) and smoking pattern (non-smoker vs. current smoker) were analysed for oral ulcer activity. Results: Oral ulcer activity was observed in 65.1% of the group (n=543). In both genders, the activity was higher in mild disease course with non-IS treatment group compared to severe course with ISs (p<0.05). As a resistant group, patients with mild disease course whose mucocutaneous symptoms were unresponsive to non-IS medications were treated with ISs in a limited period and achieved the highest CI scores in females. Oral ulcer activity and poor CI score were associated with disease duration less than 5 years compared to others in male patients (p<0.05). Conclusions: Oral ulcer activity pattern is affected by both the combination of disease course, treatment protocols and disease duration. CI scores reflected the oral clinical activity and CI might be a candidate scale to evaluate the efficacy of treatments during the follow-up of oral ulcer activity in BS.PubMe