Metrics details. Systemic sclerosis SSc is a generalized disease of the connective tissue, arterioles, and microvessels, characterized by the appearance of fibrosis and vascular obliteration. This is called SSc sine scleroderma.
The prognosis depends essentially on the occurrence of visceral damage and more particularly interstitial lung disease which is sometimes severe , pulmonary arterial hypertension, or primary cardiac damage, which represent the three commonest causes of mortality in SSc. Cutaneous extension is also an important parameter, with the diffuse cutaneous forms having less favorable prognosis. The severity of visceral damage justifies systematic and repeated assessment by directed interview and clinical and additional examinations, even in the absence of suggestive symptoms, as early treatment is a determining factor for patient survival Fig.
Recommendations for initial evaluation and follow-up of patients with systemic sclerosis. To date, no antifibrotic or immunosuppressant DMARD monotherapy has been able to achieve an improvement in survival in a prospective randomized study.
Use of cyclophosphamide at very high doses during intensive conditioning procedures for myelosuppressive or myeloablative purposes depending on the type of conditioning with or without antilymphocytic serum followed by autologous hematopoietic stem cell HSC transplantation has been proven efficacious in terms of event-free and overall survival in three randomized trials ASSIT, ASTIS, and SCOTT.
These therapeutic procedures are reserved for severe, rapidly progressive forms, after rigorous selection of patients, and must be carried out in special centers accredited for these procedures. Nonpharmacological therapies such as functional rehabilitation are in all cases recommended to combat disability.
