30 What is the most common way that vasculitis affects the nervous system? Mononeuritis multiplex Define mononeuritis multiplex. Damage to one or more peripheral nerves Which systemic vasculitides have neurological complications? Temporal arteritis, granulomatous arteritis, aortic branch arteritis, polyarteritis nodosa (PAN), Churg-Strauss, Wegener granulomatosis, SLE, Behçet disease, postzoster arteritis, AIDS-related arteritis1 What is the most common vasculitis? Temporal arteritis What is the presentation of temporal arteritis? >50-year-old (usually late 60s) woman with onset of a new type of headache and jaw claudication; other symptoms are similar to those of polymyalgia rheumatica; high ESR What is the most feared complication in temporal arteritis and what is its incidence? Blindness (ischemic optic neuropathy), which occurs in approximately 25%1 What is the initial treatment of temporal arteritis? Prednisone 50 to 75 mg/d STAT. Do not wait for biopsy results from temporal artery! How does intracranial granulomatous arteritis differ from temporal arteritis? ESR is usually normal, and time course and presentation can be variable in intracranial granulomatous arteritis. How is granulomatous arteritis diagnosed? 50% can be diagnosed by angiography, but many require a brain biopsy, which often reveals a different diagnosis. What angiogram finding indicates vasculitis? Irregular narrowing or blunt termination of cerebral arteries. CT and MRI will show corresponding lesions. What are the pathological findings in granulomatous arteritis? Vessels 100-mm to 500-mm infiltrated by lymphocytes, plasma, mononuclear cells, and giant cells, which are distributed throughout the media, adventitia, and perivascular connective tissue. What is the most common aortic branch arteritis? Takayasu disease What are the clinical features of Takayasu disease? Fever, malaise, weight loss, night sweats, and occlusion of the proximal branches of the aorta that leads to pulselessness. Neurological symptoms can be postural and include blurred vision, dizziness, hemiparetic and hemisensory syndromes, along with TIA-like syndromes. What organs are involved in polyarteritis nodosa (PAN)? • Kidneys, heart (90%) • Liver (50%) • Skin (40%) • GI, testes, muscle, nervous (25%) Discuss the most common presentation in PAN. A 30-year-old man presents with low-grade fever, malaise, and other symptoms similar to a viral infection. Can also have peripheral neuropathy, mononeuritis, weight loss, or dyspnea on exertion. Physical exam may reveal severe HTN or livedo reticularis. The disease spares the lungs (unlike in Churg-Strauss). What is the frequency of brain involvement in PAN? <5%. Presents with microinfarcts and symptoms such as headache, convulsions, hemiplegia, and delirium. What is the typical presentation of Wegener granulomatosis (WG)? Triad in white men 50 to 60 years of age: • Granulomatous inflammation of respiratory tract • Necrotizing vasculitis of small-to-medium arteries • Glomerulonephritis What is the nervous system involvement in WG? 20% peripheral and central involvement; typically either mononeuritis multiplex or extension of respiratory tract granulomas into the cranium (from sinuses) What is the treatment of WG? Cyclophosphamide and high-dose steroids What are the neurological symptoms of SLE? Headache, mood disorder, seizures, cognitive impairment, cerebrovascular disease2 What is the incidence of neurological symptoms in SLE? As high as 60 to 75%3 What pathological finding accounts for most of the neurological symptoms of SLE? The proposed mechanism of injury involves widespread microinfarcts of the cerebral cortex and brainstem with subtle attachment of immune complexes to the endothelium.1 What other ways can SLE contribute indirectly to neurological pathology? • Hypertension from glomerulonephritis • Embolisms from Libman-Sacks endocarditis • Thrombotic thrombocytopenic purpura (TTP) • Injury related to antiphospholipid antibody syndrome What type of vasculitis can AIDS and drug abuse mimic? Polyarteritis nodosa Is Behçet disease an acute or chronic process? Chronic What is the most common geographical location for Behçet syndrome? Turkey What is its classical presentation? Intermittent arthritis, recurrent oral and genital ulcers, eye inflammation due to retinal vasculitis
Vasculitis and Paraneoplastic Syndromes
30.1 Vasculitis
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