Systemic Sclerosis for General Practitioner

Abstract

Abstract

          Systemic sclerosis (SSc) is one of connective tissue diseases which skin tightness and internal organ fibrosis are characteristic features of the disease. The diagnosis is not a problem when the skin tightness is obviously observed. However, clinical presentations at onset are various and difficult to make an early SSc diagnosis such as Raynaud’s phenomenon, puffy hands, sclerodactyly etc. Capillaroscopy and serology test could be helpful for solving this problem. In case of the clinical presentations are compatible with SSc, the next step should be looking for internal organ invovlement such as gastrointestinal involvement, pulmonary fibrosis, alveolitis, and pulmonary arterial hypertension (PAH) because they cause high mortality in SSc. Recently, there are specific therapies in an early internal organ involvement particular alveolitis and PAH.  Therefore, the early evaluation of the internal organ involvement and the regular disease monitoring are very important in SSc in order to early detect disease complication and give them an early treatment.