Skin fibrosis, internal organ involvement and auto antibodies in systemic sclerosis: longitudinal development and impact on survival

Sammanfattning: Systemic sclerosis (SSc) is a connective tissue disease with a highly variable prognosis. Since new treatment strategies are emerging, there is a need for prognostic markers in order to help allocate patients to the optimal treatment. When comparison is made to the general population, there is an increased mortality rate in this study, both in the series as a whole and in the subgroups of men and women, and of the diffuse and limited skin involvement variations of the disease. Five- and 10-year survival rates are 86% and 69% respectively. There is a need for early detection, since pulmonary complications, related either to interstitial or vascular lung disease, account for half of the deaths. Treating patients suffering interstitial lung disease with cyclophosphamide did not increase the risk of developing malignancies. Auto antibodies serve as valuable indicators in predicting organ involvement and death. A nucleolar immunofluorescence pattern, anti histone antibodies, male gender, diffuse skin involvement, and the involvement of the kidneys, the heart or the interstitial or vascular involvement of the lungs, are all associated with an increased risk of death. High fibroblast production of versican and biglycan in cell culture are associated with increasing skin echogenicity, when measured by high-frequency (20 MHz) ultrasound, and may predict a more progressive skin involvement. Biglycan production was greater in the earlier stages of the disease and may be involved in the initial steps of the fibrotic process. Pulmonary arterial hypertension, when measured by Doppler echocardiography, is common and progressive in SSc. Age and the presence of interstitial lung disease increase the risk of developing pulmonary arterial hypertension.