Chapter 1.3 - Vasculitis Secondary to Systemic Disease

Summary

SLE is an autoimmune chronic progressive disease which affects the central and peripheral nervous system and causes different neuropsychiatric symptoms. Neuropsychiatric Syndromes of Systemic Lupus Erythematosus (NPSLE) can be observed in 10-80% of SLE patients. NPLSE syndromes have been defined by America College of Rheumatology. Vascular and neuronal damage caused by inflammatory cytokines, autoantibodies and immune complexes are involved in pathogenesis of NPLSE. Stroke is the most severe complication of SLE and the incidence rate is approximately 3-20% especially in the first five years of the disease. Many factors such as high level of aPL, hyperhomocysteinemia, lupus disease activity, cerebral vasculitis, emboli from Libman-Sacks endocarditis, accelerated atherosclerosis cause stroke in SLE. Stroke related to vasculitis is a rare manifestation of NPSLE. Immune complex deposition in vascular endothelium, intrathecal immune complexes and other inflammatory mediators are involved in the pathogenesis of vasculopathy and vasculitis. Here, we present a 41-year-old Syrian woman with diagnosis of SLE and antiphospholipid antibody syndrome (APS). Her medical history showed several SLE attacks, ischemic cerebrovascular accidents (CVA) and seizure. Neurological examination revealed confusion, poor cooperation/orientation, left-sided hemiparesia. Cranial MRI, MRA and DSA findings were suggestive of vasculitis related to lupus. For the treatment, acetylsalicylic acid, clopidogrel, warfarin, cyclophosphamid 1g IV and uromitexan were applied. On the basis of this case, diagnostic and therapeutic approaches of NPSLE were discussed