Vasculitis 2013

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Vasculitis Vasculitis 魏魏魏 魏魏魏 魏魏魏魏魏魏魏魏魏魏魏魏魏魏魏魏魏魏魏 魏魏魏魏魏魏魏魏魏魏魏魏魏魏魏魏魏魏魏 Tel: 04 24729595 ext 34314; Email: [email protected] 魏魏魏 魏魏魏魏魏魏 魏魏魏 魏魏魏魏魏魏 V V 魏魏 魏魏

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  • V Vasculitis Tel: 04 24729595 ext 34314; Email: [email protected]
  • References eMedicine : Microscopic Polyangiitis Polyarteritis Nodosa Wegener Granulomatosis UpToDate :vasculitis
  • Vasculitis Spectrum Pathophysiology Clinical manifestations Diagnosis Treatment Prognosis
  • Nomenclature of systemic vasculitis 1. Large vessel vasculitis Giant cell arteritis Takayasu arteritis 2. Medium-sized vessel vasculitis Polyarteritis nodosa Kawasaki disease 3. Small vessel vasculitis Granulomatous polyangiitis (GPA), Wegener granulomatosis Eosinophilic granulomatous angiitis, Churg-Strauss syndrome Microscopic polyangiitis (MPA) IgA vasculitis, Henoch-Schnlein purpura Cryoglobulinemic vasculitis Cutaneous leukocytoclastic angiitis
  • Pathogenesis Direct attack by an agent. A process directed specifically at components in vascular tissue (e.g. ANCA, anti-basement membrane disease). Secondary to an inflammatory process (e.g. immune complex disease).
  • Antineutrophil cytoplasmic antibodies, ANCA Cause of ANCA-associated vasculitis (AAV) C-antineutrophil cytoplasmic antibodies (CANCA) against proteinase 3 (PR3) Associated with GPA and MPA Disease activity is parallel to C-ANCA titer. P-ANCA against myeloperoxidase (MPO) is non-specific marker for vascultiis syndrome and other autoimmune diseases
  • Palpable purpura
  • Livido reticularis
  • mononeuritis multiplesx
  • MONONEURITIS MULTIPLEX Peripheral neuropathy of more than one nerve roots. Pathogenesis: nerve infarction resulting from widespread destruction of epineural arterioles. Causes: vasculitis (esp PAN), diabetes or other autoimmune conditions (eg. SLE, RA).
  • Vasculitis syndrome (Vasculitides) 1. 2. 3. Diagnostic triad: Palpable purpura Glomerulonephritis More than three organs involved Fever, fatigue, weight loss, granuloma Nerve Airway (ENT, Lung) Allergy GI Musculoskeletal
  • Laboratory of vasculitis Screening CBC, ESR, CRP, Urine analysis, ANA, Rheumatoid factor HBsAg, HCVAb, IgE, Eosonophil, VDRL Confirmatory IgA, C3, C4 Anti-cardiolipin Ab (ACA), anti-neutrophil cytoplasmic Ab (ANCA) Cryoglobulinemia
  • Giant Cell Arteritis (Temporal arteritis) Occurring primarily over the age of 50 years. Frequent features include fatigue, temporal headaches, jaw claudication, loss of vision, scalp tenderness, polymyalgia rheumatica and aortic arch syndrome. Rarely involves the skin, kidneys and lungs. The ESR is usually highly elevated
  • Temporal arteritis
  • Temporal arteritis
  • Polymyalgia Rheumatica Myalgia, low-grade fever, fatigue, weight loss and an elevated ESR. May combined with temporal arteritis Morning stiffness is usually the predominant feature Muscular pain is often diffuse and is accentuated by movement; pain at night is common. Muscle strength is unimpaired although the pain makes interpretation of muscle testing difficult. Rx: Corticosteroid treatment is for at least 2 years.
  • TAKAYASUS ARTERITIS Affecting aorta and its major branches. most commonly in females under 40 years of age. Clinical features Systemic phase: malaise, fever, night sweats and fatigue. Occlusive phase: upper limb claudication, headaches, postural dizziness and visual disturbances. Reduced or absent upper limb pulses. Arterial bruits over the carotid, abdominal and subclavian vessels.
  • At least 3 of 6 criteria
  • Polyarteritis nodosa (PAN) Medium-sized artery inflammation involving the skin, kidney, peripheral nerves, muscle and gut.
  • Polyarteritis nodosa
  • Polyarteritis nodosa
  • Kawasaki disease An acute febrile disease occuring most commonly in infants and children under 5 years of age. Sore red eyes , red lips tongue or mouth. Redness or swelling of the hands and feet. Rash all over the body. Vasculitis, especially of the coronary arteries, is the most serious and life-threatening complication of the disease.
  • Kawasaki disease
  • Granulomatosis with polyangiitis, GPA 2011-4 WEGENER'S GRANULOMATOSIS (WG) Young and middle age adults Small and occasionally medium-sized vessel vasculitis Midline granuloma Necrosis, granuloma formation and vasculitis of the upper and lower respiratory tracts. Glomerulonephritis in 75% of patients. Fever, malaise, weight loss, arthralgia, , sinusitis, nasal or oral ulceration, purpura C-ANCA related.
  • Eosinophilic granulomatous angiitis (CHURGSTRAUSS SYNDROME) Small sized artery granulomatous inflammation. Clinical features History of atopy. Constitutional symptoms fever, anorexia, weight loss. Asthma. Peripheral neuropathy. Skin involvement nodular lesions over pressure areas. Peripheral eosinophilia.
  • At least 4 of 6 criteria
  • Microscopic polyangiitis, MPA Pathogenesis is presumed to be due to circulating immune complexes. Cutaneous involvement palpable purpura or urticaria-like lesions are most common, less common are livedo reticularis, ulcerations or necrosis. Systemic involvement arthritis, glomerulonephritis, gastrointestinal hemorrhage or colic are most common. Skin biopsy: leukocytoclastic vasculitis Kidney biopsy: pauci-immune segmental necrotizing glomerulonephritis
  • Microscopic polyangiitis
  • Microscopic polyangiitis (Leukocytoclastic vasculitis)
  • IgA Vasculitis (HenochSchnlein purpura) A specific vasculitic syndrome which is due to IgA immune complexes and IgA deposition within the vessels and the kidney. mainly in children and young adults The full spectrum includes palpable purpura, nephritis, arthritis and gastrointestinal involvement.
  • Hypersensitivity vasculitis
  • Urticarial vasculitis
  • Antiphospholipid Antibodies Syndrome (APS) 1 clinical + 1 lab criteria Clinical criteria 1. Vascular thrombosis arterial, venous, or small-vessel thrombosis 1. Pregnancy morbidity One or more late-term (>10 weeks gestation) spontaneous abortions One or more premature births at or before 34 weeks gestation Three or more spontaneous abortions before 10 weeks gestation Laboratory criteria: on at least 2 occasions at least 12 weeks apart (1) anticardiolipin (aCL), (2) antib2 glycoprotein I (anti-b2 GPI), (3) Lupus anti-coagulant
  • Behcet disease 1. 2. 3. 1937: Turkish dermatologist Hulusi Behcet first described. A type of vasculitis and pannivulitis Symptom-complex of recurrent oral aphthous ulcers, genital ulcers, and uveitis.
  • Recurrent oral ulceration Minor or major aphthous, or herpetiform ulceration observed by physician or patient that recurred at least 3 times in one 12-month period Plus 2 of the following criteria: Recurrent genital ulceration Eye lesions Aphthous ulceration or scarring observed by physician or patient Skin lesions Positive pathergy test Anterior uveitis, posterior uveitis, or cells in vitreous on slit lamp exam.; or retinal vasculitis observed by ophthalmologist Erythema nodosum observed by physician or patient, pseudofolliculitis or papulo- pustular lesions; or acneiform nodules observed by physician in postadolescent patients not receiving corticosteroid Tx Read by physician at 24-48 hr International Study Group for Behcet's Disease: Criteria for the diagnosis of Behcet's disease. Lancet 335:1078-1080, 1990.
  • Therapy of Vasculitis 1. Corticosteroid with or without 2. Cytotoxic drugs
  • Steroid Therapy Glucocorticoids initially: 1 mg/kg/day prednisone tapering of the dosis: 5-10 mg every week, from 15 mg/day only 1 mg every several week, treatment at least for 2 years pulsus steroid: 1 g/day for 3 days 0.5 mg/kg/day after megadose pulse therapy
  • Cytotoxic drugs Cyclophosphamide (Endoxan) oral 2mg/kg/day pulse, monthly, keep WBC 3000~5000 Methotrexate: 0.3 mg/kg/week orally 25mg/kg/week Azathioprine 50-100 mg/day orally Mycophenolate 1000-1500 mg bid mofetil
  • Indication for addition of cytotoxic drug to prednisone on initial evaluation, rapidly progressive vasculitis with significant visceral involvement prednisone in high daily divided dosis is not controlling the activity and progression of vasculitis prednisone dose cannot be tapered to tolerable level and still controll the disease
  • Other Treatment of Vasculitis 1. 2. 3. 4. 5. Aspirin Anticoagulants Plasmapheresis Intravenous immunoglobulin, IVIG Biological agents 1. Anti-TNF: () 2. Anti-CD20, Rituximab for ANCA-associated vasculitis
  • Conclusion Vasculitis is a syndrome and disease spectrum Classification depends on vascular size and clinical manifestations ANCA, anti-cardiolipin antibody are key autoantibodies Treated by steroid and cytotoxic drugs