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SYSTEMIC VASCULITIS

الكلية كلية الطب القسم الباطنية المرحلة 5
أستاذ المادة علي محمد حسين خلف القزاز 6/6/2011 9:54:20 PM

SYSTEMIC VASCULITIS

Def :destruction and inflammation of the blood vessel s may be localized or involved many organs and may be

Primary or secondary vasculitis-induced injury to blood vessels may lead to increased vascular permeability, vessel weakening that causes aneurysm formation or hemorrhage, and intimal proliferation and thrombosis that result in obstruction and local ischemia.

Classification of Vasculitis

Classification of vasculitis has received much attention over the past several decades, but no universally accepted classification system has emerged. Vasculitis may be classified by the size and type of vessel involvement, by the histopathologic features (leukocytoclastic, granulomatous vasculitis, etc.) or by the pattern of clinical features

since certain types may be self-limited, whereas others may require corticosteroid therapy, with or without a cytotoxic agent, or other modalities such as plasmapheresis. Initially in the work-up, however, determining the extent of visceral organ involvement is more important than identifying the type of vasculitis, so that organs at risk of damage are not jeopardized if treatment is delayed or inadequate.

TABLE 1
Clinical Features and Primary Treatment of the Major Systemic Vasculitis Syndromes

Systemic vasculitis syndrome	Common presenting features	Primary treatment
Vasculitis of small vessels
Hypersensitivity vasculitis	Palpable purpura	Often self-limited if offending agent is removed. If isolated to skin, may not require therapy. In more severe cases, moderate- to high-dose corticosteroid therapy may be needed.
Henoch-Sch?nlein purpura	Palpable purpura, arthritis, glomerulonephritis, intestinal ischemia	Often self-limited and requires no treatment. Steroid therapy for some cases of gastrointestinal or renal involvement.
Cryoglobulinemia	Arthritis, Raynaud s phenomenon, glomerulonephritis, palpable purpura	Corticosteroids; plasmapheresis for severe involvement. Antiviral therapy required if associated with hepatitis C.
Vasculitis of small and medium-sized vessels
Polyarteritis nodosa	Peripheral neuropathy, mononeuritis multiplex, intestinal ischemia, renal ischemia, testicular pain, livedo reticularis	High-dose corticosteroids, often with cytotoxic agents (e.g., cyclophosphamide [Cytoxan])
Microscopic polyangiitis	Pulmonary hemorrhage, glomerulonephritis	High-dose corticosteroids, often with cytotoxic agents (e.g., cyclophosphamide)
Churg-Strauss vasculitis	Allergic rhinitis, asthma, eosinophilia, pulmonary infiltrates, coronary arteritis, intestinal ischemia	High-dose corticosteroids, often with cytotoxic agents (e.g., cyclophosphamide)
Wegener s granulomatosis	Recurrent epistaxis or sinusitis, pulmonary infiltrates and/or nodules, glomerulonephritis, ocular involvement	High-dose corticosteroids and cyclophosphamide. Corticosteroids and methotrexate may be used for less severe involvement.
Kawasaki disease	Fever, conjunctivitis, lymphadenopathy, desquamating rash, mucositis, arthritis, coronary artery aneurysms	High-dose aspirin and intravenous immune globulin
Vasculitis of large vessels
Giant cell, or temporal, arteritis	Headache, polymyalgia rheumatica, jaw or tongue claudication, scalp tenderness, fever, vision disturbances	High-dose corticosteroids
Takayasu s arteritis	Extremity claudication, athralgias, constitutional symptoms, renal ischemia	High-dose corticosteroids

General Approach to Diagnosis

1. Attempt to exclude other processes, particularly infection, thrombosis and neoplasia,

2. Consider the patient s age, sex and ethnic origin, since certain vasculitis syndromes occur more commonly in different groups

3. Determine which organs are involved and estimate the size of vessel involvement. The type and extent of organ involvement in vasculitis can be helpful in determining the specific type of vasculitis and the degree of urgency in initiating treatment

Laboratory Tests to Consider in the Evaluation of Systemic Vasculitis

Laboratory test	Purpose or interpretation
Routine tests (including complete blood cell count, liver enzymes, creatinine, urinalysis)	Evaluate for hematologic, renal and other organ involvement
Blood cultures	Rule out infection
Erythrocyte sedimentation rate	High value suggests inflammatory disease
C-reactive protein	High value suggests inflammatory disease
Rheumatoid factor	Very high titers in rheumatoid arthritis, Sj?gren s syndrome and cryoglobulinemia-associated vasculitis
Antinuclear antibody	Screen for SLE and Sj?gren s syndrome
Complements (C3, C4, CH50)	Low complement levels suggest consumption by immune complexes, which are commonly found in SLE and cryoglobulinemia
Cryoglobulins	Must be present to diagnose mixed essential cryoglobulinemia but can be found in any primary or secondary vasculitis
ANCA	Cytoplasmic ANCA pattern specific for Wegener s granulomatosis; perinuclear ANCA pattern may occur in other vasculitides
Creatine phosphokinase	Elevation suggests myositis, which can occur in many vasculitis syndromes
RPR/VDRL	Rule out syphilis
Serum protein electrophoresis	Evaluate for plasma cell dyscrasias
Hepatitis B and C serology	Rule out hepatitis B or hepatitis C infection
HIV	Rule out HIV infection
Anti-glomerular basement membrane	Rule out Goodpasture s syndrome, which can mimic vasculitis and cause pulmonary hemorrhage and glomerulonephritis

Arteriography or Biopsy?
Confirmation of a clinical suspicion of vasculitis usually requires arteriography, biopsy, or both. Evaluation should be directed toward establishing a tissue diagnosis, if possible. In general, because "blind" biopsy of asymptomatic sites or organs generally has a low yield,2 it is best to "go where the money is." For example, if a patient is over age 50 and presents with a new, unexplained headache and elevated ESR, with or without a tender or abnormal temporal artery, a temporal artery biopsy would be indicated. Similarly, in a patient who presents with a multisystem illness and testicular pain and swelling, a testicular biopsy should be considered. Sural nerve biopsy may be indicated in a patient with numbness and tingling in a lower extremity. If the urine sediment is abnormal, a renal biopsy might be obtained. If a biopsy is impractical, an angiogram may be diagnostic

Prognosis and Therapy

Although systemic vasculitis is a potentially life-threatening disorder, morbidity and mortality can be prevented if this disorder is recognized and treated early in its course. Initial therapy is primarily determined by the type and severity of organ involvement and by the rate of disease progression, although the specific type of vasculitis may further influence therapy.

The use of high-dose corticosteroids (i.e., prednisone in a dosage of 1 mg per kg per day), occasionally in divided doses, is standard initial therapy for most of the systemic vasculitis syndromes. Corticosteroid therapy is often administered as intravenous "pulse" steroids (e.g., methylprednisolone in a dosage of 1 g per day for three days) in patients who have severe or life-threatening organ involvement. Immunosuppressive therapy, particularly cyclophosphamide (Cytoxan), azathioprine (Imuran) or methotrexate (Rheumatrex), in combination with corticosteroids, is widely used,

The use now of biological agent in the treatment of under line disease help in management of vasculitis or in treatment of primary vasculitis .

المادة المعروضة اعلاه هي مدخل الى المحاضرة المرفوعة بواسطة استاذ(ة) المادة . وقد تبدو لك غير متكاملة . حيث يضع استاذ المادة في بعض الاحيان فقط الجزء الاول من المحاضرة من اجل الاطلاع على ما ستقوم بتحميله لاحقا . في نظام التعليم الالكتروني نوفر هذه الخدمة لكي نبقيك على اطلاع حول محتوى الملف الذي ستقوم بتحميله .