Clinical features and prognostic factors of systemic sclerosis in Guangxi, China: Retrospective, single‐center study of long‐term survival in 470 patients

Abstract

Objective Systemic sclerosis (SSc) is an autoimmune disease the prevalence of which varies among populations. We analyzed SSc patients from Guangxi to improve the clinical understanding of this disease. Methods Data of 470 SSc patients admitted to our institution from October 1,2012 to January 1,2019 were examined. The characteristics of these patients were analyzed using Kaplan‐Meier survival analysis. Cox proportional‐hazard regression was used to identify prognostic factors. Results The average age was 50.44 ± 12.31 years, 285 patients (60.6%) were women, 2.1% had pneumoconiosis, 58.2% had pulmonary interstitial disease (ILD), 18.7% had pulmonary hypertension (PH), and 3.6% had renal crisis. These patients had diffuse cutaneous systemic sclerosis (dcSSc, 70.2%) or limited cutaneous systemic sclerosis (29.7%), and PH and renal crisis were more common in the dcSSc group. Patients 50 years old or more had greater prevalences of ILD, PH, and musculoskeletal damage, greater positivity of laboratory biomarkers, and increased mortality (all P < .05). Seventy‐four patients (15.7%) died. The non‐survivors were older, had longer disease duration, had higher prevalences of ILD, restrictive ventilation dysfunction, PH, and renal crisis, and had higher levels of creatine kinase myocardial band (CK‐MB), C‐reactive protein, and immunoglobin A (all P < .05). Renal crisis, PH, and high CK‐MB were independent risk factors for death. Conclusions Pneumoconiosis was more common in SSc patients than the general population from this region. Our patients had a 10‐year cumulative survival rate of 74.9%, higher than reported for patients from the US. Renal crisis, PH, and high CK‐MB level were independent risk factors for death.

Comparing clinical characteristics of systemic sclerosis with or without interstitial lung disease: A cross-sectional study from a single center of the Chinese Rheumatism Data Center

“…However, ultrasonic cardiogram examinations were used to estimate pulmonary pressure instead of right heart catheterization, and the thresholds defined by the studies differed, making direct comparisons among studies difficult. In addition, digestive system involvement, mainly GERD, was common in SSc patients, with an incidence of 17.6% in our study, similar to that reported in other Chinese cohorts ( 29 , 31 ). Moreover, a previous study reported an association between gastroesophageal reflux and ILD in SSc ( 14 , 15 ), yet these findings were not confirmed in our study.…”

“…In addition, the proportion of localized scleroderma in SSc patients was high, accounting for 65.6%, indicating that lcSSc was more common than dcSSc, which is consistent with domestic and international reports (31,32,(35)(36)(37)(38)(39)(40)(41)(42). The differences in clinical patterns and prognosis between patients with dcSSc and lcSSc have been well reported in many previous studies (29,32,33). Our study revealed markedly more dcSSc than lcSSc in SSc patients with ILD, which is supported by similar findings from the European Scleroderma Trials and Research (EUSTAR) group and other studies, proving the significance of LeRoy's criterion (22) in clinical implications.…”

“…Vascular complications dominate the clinical picture of scleroderma, of which Raynaud phenomenon was the most common initial clinical symptom, with an incidence of 88.5% in this study. In addition, other typical manifestations such as proximal skin sclerosis, puffy finger, sclerodactyly, telangiectasia, fingertip ulcer, and loss of finger pad also prevalently occur in patients with SSc, and this frequency was largely similar in previous studies (29)(30)(31)(32)(33). In this study, sclerodactyly and loss of the finger pad occurred more frequently in SSc patients with ILD than in others.…”

Tree shrews as a new animal model for systemic sclerosis research

“…The prevalence of SSc is estimated to be approximately 1 in 10,000 individuals (2). Despite its relatively low incidence, SSc stands as one of the rheumatic diseases associated with the highest mortality rates, primarily due to the involvement of vital organs such as the lung, heart, digestive tract, and kidney (3). Notably, the 5-year survival rate for SSc patients is reported to be 74.9%, which significantly declines to 40% in cases where visceral organ damage occurs (4,5).…”

Scleroderma pulmonary arterial hypertension: the same as idiopathic pulmonary arterial hypertension?

“…Pulmonary hypertension in SSc may arise from a primary vasculopathy of the pulmonary arteries called pulmonary arterial hypertension (SSc-PAH) or as a complication of SSc-related interstitial lung disease (ILD), left heart disease, or chronic thromboemboli [2]. In total, 3–19% of SSc patients are affected by pulmonary hypertension with SSc-PAH accounting for 63–78% of pulmonary hypertension in SSc [1–4,5 ▪ ,6–9]. Pulmonary hypertension confers significant morbidity to SSc patients, increases their healthcare utilization and costs, and accounts for 30% of SSc-related mortality [1,6].…”