ABSTRACT
Objective To investigate the clinical and serologic features of scleroderma overlap syndromes.Methods Scleroderma overlap syndrome was diagnosed in 67 patients referred to our department from January 2003 to August 2013.Clinical and laboratory date were retrospectively analyzed.Comparisons between groups were tested by t test and chi-square test and Fisher exact method.Results ① Sixty-seven patients satisfied the criteria for scleroderma overlap syndrome,and 66 were female.The incidence of additional connective tissue diseases in the overlap syndrome group was systemic lupus erythematosus (SLE) in 27％,Sj(o)gren's syndrome (SS) in 24％,rheumatoid arthritis in 16％,dermatomyositis or polymyositis in 10％,two or more connective tissue diseases in 22％ respectively.② Systemic sclerosis preceded the development of additional connective tissue diseases was frequent (58％).The median onset age of SSc in SSc-SLE was younger,in contrast,SSc-SS was older.③ Antinuclear antibody (ANA) was positive in 87％ of patients,rheumatoid factor (RF) was in 42％.Anticentromere antibody (ACA) was more common in SSc-SS,RF and anti-cyclic citrullinated peptide (CCP) antibody were more frequent in SSc-RA,anti ds-DNA antibody was more common in SSc-SLE.Conclusion SSc-SLE is more common in scleroderma overlap syndrome in particular,and the median onset age of SSc in SSc-SLE is younger than others.There are distinct clinical and serological features that may predict the possibility of SSc patients with overlap syndrome.
ABSTRACT
Systemic sclerosis is often associated with other autoimmune diseases such as autoimmune thyroiditis, primary Sjogren syndrome, dermatomyositis and autoimmune hepatitis, as the so-called scleroderma overlap syndrome. However, coexistence of scleroderma and discoid lupus erythematosus (DLE) is extremely rare. Association of scleroderma and DLE has not been described definitely, but it is suggested that various factors such as genetic factor, sex hormone, environmental factor, immunologic factor, viral infection and HLA typing may contribute. A 40-year-old woman presented with an 18-year history of Raynaud's phenomenon and generalized hypopigmented sclerotic patches over the entire body. Skin biopsy and other examinations showed characteristics of scleroderma. The diagnosis was diffuse systemic sclerosis. During treatment, erythematous and erosive patches and plaques on face and arms appeared. The clinical features, histopathologic presentation, and laboratory findings were consistent with DLE.