Survival prognostic factors and markers of morbidity in Spanish patients with systemic sclerosis

Abstract

Objective-To identify survival prognostic factors and markers of morbidity among patients with systemic sclerosis (SSc).Patients and Methods-The study included 72 patients diagnosed with SSc. According to the extent of skin involvement, three groups of patients were established: group 1, without sclerosis and with sclerosis of fingers and neck; group 2, with sclerosis of face and distal to elbows and knees; group 3, with generalised sclerosis including the trunk. All patients were included in a study protocol to determine visceral involvement. Cumulative survival after first symptom has been estimated according to the Kaplan-Meier method. The association between a hypothetical prognostic factor and cumulative survival after first symptom was assessed by log rank test. The association between a hypothetical risk factor and the prevalence of severe morbity was assessed by the odds ratio. Multiple logistic regression models were used to identify the main predictors of severe morbidity. Results-Survival was estimated to be 85% 10 years after first SSc symptom. Survival was higher among SSc patients with skin involvement distal to elbows and knees than among the rest of patients; a forced vital capacity (FVC) on spirometry lower than 70% of expected value was associated with a shorter survival, even after adjustment for diVuse SSc. Skin involvement proximal to elbows or knees was associated with a higher prevalence of severe morbidity (OR = 46.57; p<0.001). According to a multiple logistic regression, severe morbidity was higher among patients with skin involvement proximal to knees or elbows (OR = 40.92; p<0.001) or among patients with pulmonary hypertension detected by Doppler echocardiography (OR = 23.66 p<0.001). Conclusions-In patients with SSc the extent of skin sclerosis was found to be a determining factor on the prognosis. According to skin sclerosis extent two main subsets of SSc patients with diVerent survival incidence and degree of morbidity could be clearly established: limited SSc, formed by patients with no skin sclerosis or with sclerosis distal to elbows and knees and diVuse SSc, formed by patients with skin sclerosis distal and proximal to elbows and knees. Moreover, lung involvement (FVC<70% on survival study and pulmonary hypertension on morbidity study) was an important and independent prognostic factor.

Association of C‐reactive protein with high disease activity in systemic sclerosis: Results from the Canadian Scleroderma Research Group

“…There are 2 subtypes of SSc, i.e., diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc (lcSSc), according to the extent of cutaneous involvement (2); dcSSc has more extensive skin involvement, including the trunk or proximal extremities, and is associated with more internal organ involvement such as significant interstitial lung disease (ILD), cardiomyopathy, and scleroderma renal crisis (SRC) and with increased mortality (3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18).…”

Prediction of five-year survival following presentation with scleroderma: Development of a simple model using three disease factors at first visit

“…Perhaps not surprisingly, individuals who already present with evidence of internal organ involvement, particularly elevated blood urea levels (6)(7)(8), reduction in lung capacity (4,6,7,(10)(11)(12), and evidence of cardiac involvement (4,7,9,10,14), have increased risks of mortality. In addition, more generalized aspects of the disease, such as an elevated erythrocyte sedimentation rate (ESR) (11)(12)(13) and widespread skin disease (6,7,12,14,15), are also linked to poorer prognosis.…”

Induced Sputum in Systemic Sclerosis Interstitial Lung Disease: Comparison to Healthy Controls and Bronchoalveolar Lavage

“…Lung involvement at autopsy is detectable in approximately 70% of patients with systemic sclerosis (SSc) [1], and interstitial lung disease (ILD) is the most common cause of death [2,3,4]. ILD is characterized by overproduction and deposition of collagen within the alveolar wall and the peribronchial tissue.…”