Sine scleroderma, limited cutaneous, and diffused cutaneous systemic sclerosis survival and predictors of mortality

Abstract

Background Systemic sclerosis (SSc) is associated with a variability of mortality rates in the literature. Objective To determine the mortality and its predictors in a long-term follow-up of a bi-centric cohort of SSc patients. Methods A retrospective observational study by systematically analyzing the medical records of patients diagnosed with SSc in Toulouse University Hospital and Ducuing Hospital. Standardized Mortality Ratio (SMR), mortality at 1, 3, 5, 10, and 15 years of disease and causes of death were described. Predictors of mortality using Cox regression were assessed. Results Three hundred seventy-five patients were included: 63 with diffuse cutaneous SSc, 279 with limited cutaneous SSc, and 33 with sine scleroderma. The SMR ratio was 1.88 (95% CI 1.46–1.97). The overall survival rates were 97.6% at 1 year, 93.4% at 3 years, 87.1% at 5 years, 77.9% at 10 years, and 61.3% at 15 years. Sixty-nine deaths were recorded. 46.4% were SSc related deaths secondary to interstitial lung disease (ILD) (34.4%), pulmonary hypertension (31.2%), and digestive tract involvement (18.8%). 53.6% were non-related to SSc: cardiovascular disorders (37.8%) and various infections (35.1%) largely distanced those from cancer (13.5%). Four significant independent predictive factors were identified: carbon monoxide diffusing capacity (DLCO) < 70% (HR=3.01; p=0.0053), C-reactive protein (CRP) >5 mg/l (HR=2.13; p=0.0174), cardiac involvement (HR=2.86; p=0.0012), and the fact of being male (HR=3.25; p=0.0004). Conclusion Long-term data confirmed high mortality of SSc. Male sex, DLCO <70%, cardiac involvement, and CRP> 5mg/l were identified as independent predictors of mortality.

Systemic sclerosis sine scleroderma: clinical and serological features and relationship with other cutaneous subsets in a large series of patients from the national registry ‘SPRING’ of the Italian Society for Rheumatology

“…Possibly, long-term follow-up studies may verify the natural course and outcome of ssSSc patients in comparison to the other cutaneous subsets. 14 33 …”

“…Demographic and clinical hallmarks of the present ssSSc series and those previously published are summarised in table 3. The papers are characterised by a significant heterogeneity for what concerns the number, the modalities of patients’ recruitment (mono/multicentre), and the classification criteria adopted 8–14. This condition may account for the variability in the ssSSc prevalence (from 1.4% to 8.9%), as well as in the clinical phenotype, namely peripheral vascular, heart, lung, renal, oesophageal and musculoskeletal involvement.…”

“…Its clinical presentation can be misleading, generating diagnostic uncertainties because of the lack of skin involvement, although it may have the involvement of lung, heart and gastrointestinal (GI) system 6 7. Currently, the literature is conflicting concerning the real prevalence of ssSSc, the female/male ratio, and the presence/severity of both visceral organ and peripheral vascular involvement, mostly depending on the characteristics of the studied population 8–14…”

Changes on chest HRCT in systemic sclerosis-related interstitial lung disease after autologous haematopoietic stem cell transplantation

“…Our study is the first to document WOD and oesophageal volume over a 5-year post-aHSCT follow-up. This is of major clinical importance, because oesophageal dilatation is associated with SSc severity and mortality in SSc [ 13 , 52 ] as well as SSc-related ILD severity and progression. The Canadian Scleroderma Research Group confirmed the link between oesophageal involvement and SSc-associated ILD.…”

Impact of Innovative Treatment Using Biological Drugs for the Modulation of Diffuse Cutaneous Systemic Sclerosis: A Systematic Review

“…Currently, the annual incidence is 1 to 20 cases per million per year [ 10 ], with variations found depending on the geographical area, which may suggest that the rate of occurrence of new cases in the susceptible, at-risk population is conditioned by a genetic predisposition, individual hormonal behavioral determinants, and exposure to environmental factors [ 10 , 11 , 12 ]. The incidence is higher in the United States than in Europe, with a higher prevalence in African Americans than Caucasians [ 5 ].…”