Information about vasculitides

Published on April 21, 2009

Author: Dr.Ellen



The Vasculitides : Available on: The Vasculitides Definition : Available on: Definition Heterogeneous group of syndromes Presence of leukocytes in the vessel wall with reactive damage / necrosis Impairment of blood flow Clinical syndromes as a result of ischemia to tissues and systemic inflammation Presentation depends on the size of vessels involved and the extent of the disease Pathology : Available on: Pathology Inflammation Increased permeability Bleeding Ischemia & necrosis of tissue/organs Aneurysm/stricture formation Thrombus Clinical Clues to Systemic Vasculitis : Available on: Clinical Clues to Systemic Vasculitis Constitutional symptoms Unexplained ischemia, such as claudication, limb ischemia, angina, transient ischemic attack, stroke, mesenteric ischemia and cutaneous ischemia particularly in a young patient or a patient without risk factors for atherosclerosis Multiple organ dysfunction in a systemically ill patient, especially in the presence of other suggestive clinical features Differential Diagnosis : Available on: Differential Diagnosis Fibromuscular dysplasia Cholesterol emboli Atrial myxoma with emboli Infective endocarditis Some malignancies Mycotic aneurysm with embolization Bacteremia Rickettsial infection Ergotism Thrombocytopenia and other processes associated with purpura Radiation fibrosis Neurofibromatosis Coarctation of the aorta Amyloidosis Livedoid vasculopathy Malignant atrophic papulosis (Degos disease or syndrome) Necrotic arachnidism (Loxosceles species spider bites) Categorizing Vasculitides : Available on: Categorizing Vasculitides Clinical Manifestations (e.g., cutaneous, urticarial) Size of vessel involved Pathologic features (e.g., leukocytoclastic, granulomatous, etc.) Serologic (e.g., hypocomplementemic, cryoglobulinemia, etc.) Pathogenesis (e.g., Immune complex mediated, ANCA related, Cell mediated) Problems with Vasculitis : Available on: Problems with Vasculitis Often a complication of other diseases: RA, SLE, Behcets More helpful to label by disease, not as “vasculitis” Often used interchangeably with vasculopathy” (eg, Beurger’s, Raynaud’s etc.) Not vasculitis unless vessels are inflamed Classification by Vessel Size : Available on: Classification by Vessel Size Small capillaries and postcapillary venules Medium muscular arteries and arterioles Large the aorta and its major branches Slide 9: Available on: Clinical: Large Vessel : Available on: Clinical: Large Vessel Limb claudication Asymmetric blood pressures Absence of pulses Bruits Aortic dilation Renovascular hypertension Large Vessel Vasculitides : Available on: Large Vessel Vasculitides Clinical: Medium Vessel : Available on: Clinical: Medium Vessel Cutaneous nodules Ulcers Livedo reticularis Digital gangrene Mononeuritis multiplex Microaneurysms Renovascular hypertension Small & Medium Vessel Vasculitides : Available on: Small & Medium Vessel Vasculitides Clinical: Small Vessel : Available on: Clinical: Small Vessel Purpura Urticaria Glomerulonephritis Alveolar hemorrhage Cutaneous extravascular necrotizing granulomas Splinter hemorrhages Uveitis/episcleritis/scleritis Small Vessel Vasculitides : Available on: Small Vessel Vasculitides Demographic Associations of the Vasculitides : Available on: Demographic Associations of the Vasculitides Specific Laboratory Tests : Available on: Specific Laboratory Tests ANA — If positive suggests the presence of an underlying connective tissue disorder. Systemic lupus erythematosus. Sjögren's syndrome Complement— Low levels in mixed cryoglobulinemia and SLE. Consider HCV if associated with cryoglobulins ANCA — Not diagnostic, but suggestive Wegener's granulomatosis, Microscopic polyarteritis, Drug-induced vasculitis and Churg-Strauss vasculitis Selected Bibliography : Available on: Selected Bibliography ROANE DW, et al, An Approach to Diagnosis and Initial Management of Systemic Vasculitis, AFP, 1999; 1421 UpToDate, Classification of and approach to the vasculitides in adults

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