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Vasculitis 2013

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Vasculitis 2013

  1. 1. 醫學系「免疫與感染學 V 」模組  Vasculitis 魏正宗   中山醫學大學附設醫院過敏免疫風濕科主任 Tel: 04 24729595 ext 34314; Email: wei3228@gmail.com
  2. 2. References  eMedicine : – Microscopic Polyangiitis http://www.emedicine.com/med/topic2931.htm – Polyarteritis Nodosa http://www.emedicine.com/neuro/topic314.htm – Wegener Granulomatosis http://www.emedicine.com/ped/topic2430.htm  UpToDate :vasculitis http://www.lib.csmu.edu.tw/overlib/2305.php
  3. 3. 學習目標  Vasculitis – Spectrum – Pathophysiology – Clinical manifestations – Diagnosis – Treatment – Prognosis
  4. 4. 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-Schönlein purpura Cryoglobulinemic vasculitis Cutaneous leukocytoclastic angiitis
  5. 5. 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).
  6. 6. 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
  7. 7. 血管炎的臨床表現        皮膚:紫斑、蕁麻疹、水泡、壞死、潰瘍、網 狀斑等 骨骼肌肉:關節痛、關節炎、肌炎等 腎臟:血尿、蛋白尿、腎衰竭、高血壓等 腸胃:腹痛、消化性潰瘍、出血及穿孔等 肺臟:浸潤性病變、肋膜炎、出血、氣喘等 神經:運動或感覺神經病變、頭痛、複性單發 神經炎、腦血管病變 其它:胰臟炎、心包膜炎、心肌炎
  8. 8. Palpable purpura
  9. 9. Livido reticularis
  10. 10. 複性單發神經炎 mononeuritis multiplesx
  11. 11. 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). 
  12. 12. 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
  13. 13. 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
  14. 14. 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
  15. 15. Temporal arteritis
  16. 16. Temporal arteritis
  17. 17. 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.
  18. 18. TAKAYASU’S 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.
  19. 19. At least 3 of 6 criteria
  20. 20. Polyarteritis nodosa (PAN) • Medium-sized artery inflammation involving the skin, kidney, peripheral nerves, muscle and gut.
  21. 21. Polyarteritis nodosa
  22. 22. Polyarteritis nodosa
  23. 23. 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.
  24. 24. Kawasaki disease
  25. 25. Granulomatosis with polyangiitis, GPA  2011-4 前舊名 WEGENER'S GRANULOMATOSIS 韋格納氏肉芽腫 (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. 
  26. 26. Eosinophilic granulomatous angiitis (CHURG–STRAUSS 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.
  27. 27. At least 4 of 6 criteria
  28. 28. 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
  29. 29. Microscopic polyangiitis
  30. 30. Microscopic polyangiitis (Leukocytoclastic vasculitis)
  31. 31. IgA Vasculitis (Henoch–Schönlein 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.
  32. 32. Hypersensitivity vasculitis
  33. 33. Urticarial vasculitis
  34. 34. 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) anti–b2 glycoprotein I (anti-b2 GPI), (3) Lupus anti-coagulant
  35. 35. 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.
  36. 36. 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.
  37. 37. Therapy of Vasculitis 1. Corticosteroid with or without 2. Cytotoxic drugs
  38. 38. 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
  39. 39. 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
  40. 40. 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 
  41. 41. Other Treatment of Vasculitis 1. 2. 3. 4. 5. Aspirin Anticoagulants Plasmapheresis Intravenous immunoglobulin, IVIG Biological agents 1. Anti-TNFα: (−) 2. Anti-CD20, Rituximab for ANCA-associated vasculitis
  42. 42. 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 

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