Survival and causes of death in systemic sclerosis patients: a single center registry report from Iran

Poormoghim, Hadi; Amini, Elham; Jalali, Arash; Ghaderi, Afshin; Ghorbannia, Ali; Mojtabavi, Nazanin

doi:10.1007/s00296-016-3475-6

Cited by 13 publications

(19 citation statements)

References 45 publications

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“…With regards to the correlation of these autoantibodies with disease prognosis, it seems that more Iranian SSc patients have a poor prognosis. However, a study has reported that survival rates in Iranian SSc patients are 93% and 83% at 5 and 10 years from diagnosis, respectively [36], which is not different from other countries [37][38][39][40][41].…”

Section: Discussion_____________________________mentioning

confidence: 82%

Evaluation of Autoantibodies in patients with Systemic Sclerosis

et al. 2020

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Systemic sclerosis (SSc, scleroderma) is a multisystemic chronic autoimmune connective tissue disorder characterized by extensive fibrosis, vascular abnormalities, and immune dysfunction [1]. The exact etiopathogenesis of SSc remains unknown, although in genetically susceptible individuals, environamental triggers and dysregulated epigenetic changes contribute to the development of SSc [2]. Systemic sclerosis is more prevalent in black people; however, it is found in all racial groups and all geographic regions [3]. A serological hallmark of systemic sclerosis is the presence of serum autoantibodies against various intracellular antigens. Autoantibodies have been observed at first diagnosis in more than 95% of patients affected with systemic sclerosis [4]. Each of the autoantibodies is beneficial in the diagnosis of affected patients. Different autoantibodies have been associated with different disease subtypes and with differences in disease severity, including the extent of skin involvement, internal organ manifestations, as well as determining the prognosis [5]. All of these antibodies are directed against structures within the nucleus of the cell and, thus, are ANAs. Some ANAs, including ACA, ATA/anti-Scl-70 antibody, antifibrillarin/anti-U3-ribonucleoprotein (AFA), anti-PM/Scl antibody, anti-Th/To antibody, and anti-RNAP III are found in the sera of systemic sclerosis patients [4]. Original Article Open Access Systemic sclerosis is an autoimmune disease clinically characterized by vascular and immune dysfunction, leading to fibrosis that can damage multiple organs. The presence of non-overlapping SSc-associated autoantibodies best presents the autoimmune nature of systemic sclerosis. The primary purpose of this study was to investigate the autoantibody profile in Iranian patients with systemic sclerosis. Sera from 481 patients with systemic sclerosis were collected from 2013 to 2016. Levels of anti-nuclear antibodies (ANA) were quantitatively detected using the indirect immunofluorescence (IIF) method, and levels of specific autoantibodies including anti-topoisomerase I antibody (ATA), anti-centromere antibody (ACA) and anti RNA polymerase III antibody (anti-RNAP III) were determined qualitatively using the enzyme-linked immunosorbent assay (ELISA) technique. Among all patients evaluated, a predominance of females (86.7%) was found, and 434 (90.2%) patients showed positive ANA results by IIF. ANA was detected in 87.3% and 92.0% of limited cutaneous systemic sclerosis (lcSSc) and diffuse cutaneous systemic sclerosis (dcSSc) patients, respectively, which was not significantly different. The frequency of anti-RNAP III, ACA, and ATA was 5.19%, 6.09%, and 72.3%, respectively. Furthermore, anti-RNAP III, ATA, and ANA levels were correlated with dcSSc, whereas ACA levels were correlated with lcSSc. It was confirmed that ATA expression is significantly higher in dcSSc patients. This study had a lower frequency of ACA (6.09%) than most previous cohorts. The results demonstrated that the clinical subtype of systemic s...

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Section: Discussion_____________________________mentioning

confidence: 82%

Evaluation of Autoantibodies in patients with Systemic Sclerosis

et al. 2020

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“…Telangiectasia was slightly associated with a higher mortality in our study population. In contrast, Poormoghim et al [51] reported a non-significant, yet elevated HR of 1.44 in a smaller cohort of Iranian patients. An increased number of telangiectasia has been suggested to be a clinical marker of microvascular disease in SSc and is associated with an increased risk of PAH [59].…”

Section: Discussionmentioning

confidence: 84%

Survival and prognosis factors in systemic sclerosis: data of a French multicenter cohort, systematic review, and meta-analysis of the literature

et al. 2019

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Background Data on survival and prognosis factors in incident cohorts are scarce in systemic sclerosis (SStc). To describe survival, standardized mortality ratio (SMR), and prognosis factors in systemic sclerosis (SSc), we analyzed a multicenter French cohort of incident patients and performed a systematic review of the literature and meta-analysis. Methods A multicenter, French cohort study was conducted between January 1, 2000, and December 31, 2013. Patients were followed-up until July 1, 2016. A systematic review of the literature was carried out in MEDLINE and EMBASE up to July 2017. Meta-analysis was performed using all available data on SMR and hazard ratios of prognosis factors. Results A total of 625 patients (493 females, 446 lcSSc) were included. During the study period, 104 deaths (16.6%) were recorded and 133 patients were lost to follow-up. Overall survival rates at 1, 3, 5, and 10 years from diagnosis were 98.0%, 92.5%, 85.9%, and 71.7% respectively in the French cohort. Overall SMR was 5.73 (95% CI 4.68–6.94). Age at diagnosis > 60 years, diffuse cutaneous SSc, scleroderma renal crisis, dyspnea, 6-min walking distance (6MWD), forced vital capacity < 70%, diffusing capacity of the lungs for carbon monoxide < 70%, pulmonary hypertension (PH), telangiectasia, valvular disease, malignancy, anemia, and CRP > 8 mg/l were associated with a poorer survival after adjustment. Eighteen studies (11,719 patients) were included in the SMR meta-analysis and 36 studies (26,187 patients) in the prognosis factor analysis. Pooled SMR was 3.45 (95%CI 3.03–3.94). Age at disease onset, male sex, African origin, diffuse cutaneous SSc, anti-Scl70 antibodies, cardiac and renal involvement, interstitial lung disease, PH, and malignancy were significantly associated with a worse prognosis. Anti-centromere antibodies were associated with a better survival. Conclusions Overall, our study highlights a high mortality rate in SSc patients and confirms previously described prognosis factors related to skin extension and organ involvement while identifying additional prognosis factors such as autoantibody status, telangiectasia, 6MWD, and valvular disease. Electronic supplementary material The online version of this article (10.1186/s13075-019-1867-1) contains supplementary material, which is available to authorized users.

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“…The number of articles included in this meta‐analysis is low, few studies focused on the association between DUs and mortality, and data extraction depended on the quality of the studies. Two studies included were published more than twenty years ago, and four studies had a short follow‐up of <5 years, suggesting a potential bias. Despite these limitations, however, this meta‐analysis based on two exhaustive research strategies reveals for the first time an increased risk of mortality in SSc patients with DUs.…”

Section: Discussionmentioning

confidence: 99%