ABSTRACT
Scleroderma, or systemic sclerosis (SSc), is a collagen vascular disorder characterized by immune dysregulation, widespread tissue fibrosis and sclerosis of skin. The frequency of scleroderma overlap syndrome ranges from 10% to 38%, as recorded in multiple cohort studies. Scleroderma may develop in long-standing rheumatoid arthritis (RA), and conversely, established diffuse cutaneous patients may develop rheumatoid factor positive RA; this is especially in scleroderma associated with interstitial lung disease and scl-70 antibodies. Management of scleroderma overlap syndrome is not standardized, mainly due to the absence of controlled trials. The natural course and prognosis of overlap syndromes is variable and depends upon the individual patient, response to therapy, as well as the underlying pathology. Most patients with scleroderma overlap syndrome are younger in age, as compared to individual collagen vascular disorders, particularly the SSc/systemic lupus erythematosus overlap syndrome. Scleroderma/myositis overlap syndrome is considered a severe form with reported mortality rate as high as 21%.
