Different clinical features in patients with limited and diffuse cutaneous systemic sclerosis

Abstract

This study aims to analyze differences among established disease damage indicators in patients with limited cutaneous systemic sclerosis (lcSSc) and diffuse cutaneous systemic sclerosis (dcSSc). Fifty patients with lcSSc and 55 patients with dcSSc were included in this study. Difference in mean disease duration between the two subgroups of patients was not statistically significant (z=-0.88, p=0.38). Patients with lcSSc and dcSSc were compared, and differences in vascular, esophageal, lung, heart, renal, and musculoskeletal involvement were statistically assessed using chi (2), Mann-Whitney, and Kruskal-Wallis tests. Using the technique of nailfold capillaroscopy, we found normal capillaries or nonspecific capillary change in 10.0% of the patients with lcSSc and only in 3.6% of the patients with dcSSc. Dilated capillaries without loss of capillaries were found in 42% of the patients with lcSSc and in 10.9% of the patients with dcSSc (p=0.05). However, severe capillary damage (loss of capillaries) was noticed more frequently in patients with dcSSc (dcSSc/lcSSc: 85.5%/48.0%, p=0.002). Pitting scars or digital ulcers were found in 46.0% of the patients with lcSSc and in 67.3% of the patients with dcSSc (p=0.04). We did not notice a significant difference in frequency of fingertip osteolysis and telangiectasia. Esophageal hypomotility was found in 64% of the patients with lcSSc and in 85.5% of the patients with dcSSc (p<0.01). We found interstitial lung fibrosis more frequently in patients with dcSSc (lcSSc/dcSSc: 16.0%/72.7%, p<0.001). Reduced forced vital capacity (FVC) was found in 6.0% of the of patients with lcSSc and in 41.8% of the patients with dcSSc (p<0.001). A decreased value of the transfer factor for carbon monoxide (DLCO) was also observed more frequently in patients with dcSSc. Heart involvement was found in 29.1% of the patients with dcSSc and less frequently (p<0.001) in patients with lcSSc (8%). Similarly, we found renal involvement more frequently in patients with dcSSc (lcSSc/dcSSc: 2.0%/16.3%). Tendon friction rubs were noticed in 23.6% of the patients with dcSSc and only in 6% of the patients with lcSSc (p<0.01). Joint contractures were observed in 70.9% of the patients with dcSSc and in 26.0% of the patients with lcSSc (p<0.001). Muscle weakness was noticed more frequently in patients with dcSSc (lcSSc/dcSSc: 22.0%/40.0%, p<0.05). Arthralgia was found more frequently in patients with dcSSc, but arthritis became apparent, without significant difference in frequency, in 16% of the patients with lcSSc and in 16.4% of the patients with dcSSc. Loss of capillaries (detected by nailfold capillaroscopy), digital ulcers, interstitial lung fibrosis, decreased FVC and DLCO, esophageal hypomotility, musculoskeletal impairment, and heart and renal involvement are more common in patients with dcSSc. Fingertip osteolysis, telangiectasia, and arthritis are equally frequent in both forms of the disease.