Systemic sclerosis sine scleroderma - Demographic, clinical, and serologicfeatures and survival in forty-eight patients

Objective. To describe the demographic, clinical, and laboratory features a nd natural history of patients with systemic sclerosis sine scleroderma (ss SSc), and to compare them with those of patients with SSc and limited cutan eous involvement (IcSSc), Methods, The University of Pittsburgh Scleroderma Databank served as the da ta source, Patients were divided into those who had no skin thickening (ssS Sc) and those who had skin thickening only distal to elbows or knees and/or of the face (IcSSc) during their disease course. These two groups were com pared with regard to demographic characteristics, clinical, laboratory, and serologic features, and survival rates. Chisquare and Student's t-test ana lyses were performed, and Fisher's exact test was used as appropriate. Results. Of 555 consecutive patients without diffuse cutaneous SSc, 48 (9%) had ssSSc and 507 (91%) had IcSSc, The ssSSc patients had a mean total dis ease duration of 18.6 years (15.1years before study entry and 3.5 years of followup after study entry), and had not developed IcSSc or another connect ive tissue disease. Other than the absence of skin thickening, the ssSSc gr oup had no significant differences in individual internal organ involvement s, laboratory features, serum autoantibody type, or survival rate compared with patients with IcSSc, Within the category of lung involvement, patients with ssSSc had a significantly greater frequency of dyspnea with mild exer tion or at rest, and a tendency toward reduced carbon monoxide diffusing ca pacity (<70% of predicted normal) and primary pulmonary arterial hypertensi on. Patients with IcSSc had significantly more frequent individual manifest ations of digital pitting scars, digital-tip ulcers, telangiectasia, and ca lcinosis than those with ssSSc, in part related to increased time of observ ation. Puffy fingers and finger joint contractures were detected significan tly more often in IcSSc patients, Conclusion. Systemic sclerosis sine scleroderma should be included in the s pectrum of SSc with limited cutaneous involvement and should not be conside red a distinct or separate disorder.