Vasculitis in connective tissue diseases

Vasculitis is a recurrent complication of connective tissue diseases. It is often an under-recognized manifestation that can lead to significant morbidity and mortality due to vital organ damage. Cutaneous lesions, representing small-vessel vasculitis, dominate clinical presentation, although widespread necrotizing visceral medium- and large-vessel involvement, mimicking primary vasculitic syndromes, may also occur. The pathogenesis of vascular inflammation is not completely understood, but immune complexes are believed to play a crucial role. The diagnosis is usually based on the combination of clinical findings, laboratory testing, tissue biopsy and imaging of the involved blood vessels. Timely and aggressive pulse treatment with high dose of glucocorticosteroids and immunosuppressive agents, followed by gradual taper, can significantly improve patients’ survival.