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

The effect of parental consanguinity on the clinical and laboratory findings of rheumatoid arthritis

Aims: We aimed to evaluate the frequency of consanguinity among the parents of patients with rheumatoid arthritis (RA) and the influence of parental consanguinity on several clinical and laboratory parameters which reflect the severity of the disease. Methods and patients: The study population consisted of 265 patients with RA which were divided into two groups with respect to the presence or absence of consanguinity between their parents. The frequency of parental consanguinity was compared with the general population. The two groups were compared with respect to family history of RA, the age of onset, the age at which RA was diagnosed, duration of the disease, the presence of rheumatoid nodules, vasculitis, serositis and the need for orthopaedic surgery, amyloidosis, the presence and level of rheumatoid factor and anti-cyclic citrullinated peptide antibodies, erosive changes on radiographs, and the need for anti-tumour necrosis factor therapy. Results: Twenty-one patients (8%) had parents who were consanguineous, which was not more frequent compared with the general population (14%). The mean age of disease onset and the mean age at which RA was diagnosed were lower in patients with parental consanguinity, although the difference was not statistically significant. The other clinical and laboratory parameters were also not different between the two groups. Conclusion: The present data suggests that parental consanguinity has no effect on disease severity, and the frequency of consanguinity is not increased among the parents of patients with RA. A possible exception is the earlier disease onset and age at diagnosis which needs to be confirmed by larger studies

IL-22-secreting Th22 and IFN-gamma-secreting Th17 cells in Behcet's disease

Objective. Behcet's disease (BD) is a systemic inflammatory disease with unknown etiology. Studies have shown that some T helper (Th) 1-associated cytokines have role in the inflammation of BD. The CD4(+) Th cells can be differentiated into Th1, Th2, Th17 and Th22 secrete different cytokines to regulate immune system. In this study, cytokine secretion of Th subsets in BD was investigated

The HLA-B27 frequency, and ıts relationship with demographic, clinical, radiological findings in patients with psoriatic arthritis

Psoriatik artritte (PsA) klinik bulgular ile HLA-B27 arasındaki ilişkiyi araştıran çalışmalar başta ülkemiz olmak üzere literatürde oldukça sınırlıdır. Bu çalışmada PsA’lı hastalarda HLA-B27 sıklığının tespit edilmesi ve hastaların demografik, klinik ve radyolojik bulguları ile karşılaştırılması amaçlanmıştır. PsA tanısı konulan 51 olgu çalışmaya alındı. Hastaların poliklinik takip dosyaları taranarak demografik özellikleri, klinik ve radyolojik bulguları, tedavi şekilleri ve HLA-B27 gen analizi sonuçları kaydedildi. Hastaların HLA-B27 analizleri Kocaeli Üniversitesi Tıp Fakültesi Tıbbi Genetik Anabilim Dalı’nda real-time PCR yöntemiyle yapıldı. PsA’lı 51 hastanın 8’inde (%15,69) HLA-B27’nin pozitif olduğu saptandı. HLA-B27 pozitiflik oranı aksiyel tutulum ve periferik tutulum olan grupta sırası ile %37,5, %11,6 idi (p=0,099). Diğer klinik bulgularla HLA-B27 varlığı arasında anlamlı ilişki saptanmadı. Çalışmamızda HLA-B27 pozitiflik oranı son yayınlardaki değerlere benzer şekilde bulundu. Sonuçlarımız HLA-B27 pozitifliği olan PsA’lılarda artmış aksiyel tutulum riskini desteklemektedir.The studies which are investigating the relationship between HLA-B27 and the clinical findings in psoriatic arthritis (PsA) are too limited in the literature and also in our country. In this study, we aimed to find out the frequency of HLA-B27 and compare the demographic, clinical and radiological findings in patients with PsA. Fifty one patients who have been diagnosed with PsA enrolled in the study. The demographic characteristics, the clinical and radiological findings, the treatment modalities, and the HLA-B27 gene analysis results of the patients were recorded by scanning the follow-up charts of all patients. HLA-B27 analysis of patients were performed by using real-time PCR in Kocaeli University, in Faculty of Medicine, in Department of Medical Genetics. HLA-B27 was positive in 8 of 51 patients (15.69%). In patients with axial involvement and peripheral involvement, the rate of HLA-B27 pozitivity was 37.5%, 11.6%, respectively (p=0.099). There was no relationship between the presence of HLA-B27 and with other clinical findings. In our study, HLA-B27 positivity rate was similar to the values found in recent publications. The results of HLA-B27 supports the increased risk of axial involvement in PsA patients