Vasculitis associated with connective tissue disease

Vasculitis, one of the clinical features shared by connective tissue diseases, should be considered when signs and symptoms are observed that may result from tissue ischaemia due to damaged vessels. The lesions seem to result from specific and non-specific immunopathogenic mechanisms targeted at the vascular endothelium. Because of the therapeutic implications it is the physician's responsibility to document its presence and the extent of organ involvement. Prompt institution of immunosuppressive drugs may be lifesaving. On the other hand there are some forms of vasculitis accompanying connective tissue disease which are entirely benign. Patients with infarctions of extremities and progressive functional disturbances of the central nervous system or internal organs because of vasculitis should be treated with high dosages of corticosteroids in combination with cytostatic drugs. Remissions are frequently obtained within three to six months of initiation of treatment and can be maintained with a less aggressive treatment regimen.