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Vasculitis

Vasculitis. Dr. Müge Bıçakçıgil Kalaycı. Vasculitis. A heterogenous group of clinical syndromes characterized by inflammation of blood vessels The clinical picture is essentially dependent on the size and extent of vessel involvement. Vasculitis. Ambiguity of clinical presentations

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Vasculitis

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  1. Vasculitis Dr. Müge Bıçakçıgil Kalaycı

  2. Vasculitis • A heterogenous group of clinical syndromes characterized by inflammation of blood vessels • The clinical picture is essentiallydependent on the size and extent of vessel involvement

  3. Vasculitis • Ambiguity of clinical presentations • Limited diagnostic tests • Difficulty in obtaining diagnostic tissue • Therefore, difficult to diagnose • AND classify

  4. Incidence of Vasculitis • Variable because of definitions • Kawasaki seen almost exclusively in pediatric population • Most other vasculitides in the fifth decade of life

  5. All blood vessels can be affected from the largest (Aorta) to the smallest blood vessels in the skin (capillaries) • Blood Vessel Injury • Increased permeability • Weakening (Aneurysm +/-Hemorrhage) • Intimal proliferation and thrombosis, obstruction and local ischemia

  6. What is Vasculitis?

  7. Classification of Vasculitis • Vasculitis may be classified by: • The size and type of vessel involvement • The histopathologic features (leukocytoclastic, granulomatous vasculitis, etc.) • The pattern of clinical features

  8. Classification of Vasculitis • Important to classify the vasculitis since some types may be self-limited while others may be chronic • However, initially it is important to determine the amount and extent of organ system involvement

  9. Classification of Vasculitis

  10. Classification of Vasculitis

  11. CLASSIFICATION TREE Vasculitis Small Blood Vessel • Medium Blood Vessel • Polyarteritis Nodosa • Kawasaki’s Disease • Large Blood Vessel • Temporal Arteritis • Takayasu Arteritis Non-ANCA Associated • ANCA Associated • Wegener’s Granulomatosis • Churg-Strauss Vasculitis • Microscopic Polyangiitis • Drug Induced • Immune Complex • Hypersensitivity Vasculitis • Cryoglobulinemic Vasculitis • CTD related Vasculitis • Henoch Schonlein Purpura • Behcet’s • Miscellaneous • Paraneoplastic Vasculitis • Inflammatory Bowel Disease

  12. LARGE VESSEL VASCULITIS • Giant Cell Arteritis (Temporal Arteritis) • Takayasu’s Arteritis

  13. Temporal (Giant-Cell) Arteritis • Chronic granulomatousvasculitis affecting large arteries in older people • Inflammation of the walls of large arteries • Cranial arteritis(most common): • Temporal, occipital, ophthalmic • Subclavian, iliac/femoral • Aorta

  14. Temporal (Giant-Cell) Arteritis • Most are >50 years of age (average 72) • Women>men • Females 70% • Gradual onset 64% • Prevalence high in Scandinavian countries • VERY rare in Blacks and Hispanics

  15. Fever/wastingsyndrome • Feverandchills • Can sometimes present with Fever of Unknown Origin (FUO) • Anorexia, weightloss • Nightsweats • Weakness • Depression • Pain & Stiffness: • Around shoulders and hips (polymyalgiarheumatica) – 15%

  16. CranialArteries – mostcommon • Headaches…….severe • Scalptenderness +/- thickenedvessels • Ischemicopticneuropathy • Loss of vision-diplopia • Jawclaudication in 50% • Tongueclaudication • CNS ischemia • Strokes

  17. Large-vessel GCA/aortitis 10-15% • Arm claudication…femoral is rare • Pulselessness • Raynaud’sphenomenon • Aorticaneurysm • Aorticinsufficiency • PMR • Often lack cranial involvement

  18. Scalp necrosis • Tongue gangrene • Cranial and peripheral neuropathies • Rare, isolated organ involvement

  19. Temporal (Giant-Cell) Arteritis • Physical Examination • Very tender over temples • Swollen, rope like temporal artery • Optic disc swelling due to ischemia

  20. Temporal (Giant-Cell) Arteritis

  21. Temporal (Giant-Cell) Arteritis • Investigations • Complete Blood Count (CBC) • Normochromic, normocytic anemia • Reactive thrombocytosis • WBC is usually normal • Erythrocyte Sedimentation Rate (ESR) • Significantly elevated • C-Reactive Protein (CRP) • Significantly elevated

  22. Diagnosis • Biopsy in Temporal arteritis • Biopsy abnormal site • 4-6 cm. if not obviously abnormal • If strong suspicion and normal biopsy, then biopsy opposite side. • Doppler guidance?

  23. Temporal Artery Biopsy

  24. Temporal Artery Biopsy Inflammation Multi-Nucleated Giant Cell

  25. Three of thefollowingfivecriteriamust be met todiagnosis GCA. • 1-Age greater than 50 years • 2-new headaches • 3-abnormal temporal artery • 4-ESR≥50mm/h and • 5- positive temporal artery biopsy results for vasculitis

  26. Therapy • Relative EMERGENCY as patient can lose vision • Urgent temporal artery biopsy (get the tissue) to confirm the diagnosis • Initiate high-dose corticosteroids (40-60 mg per day) • Relief DRAMATIC • Taper by 10% per 2 weeks • Duration – 9-12 months • Steoridsparingdrugs- MTX 10 mg/wk - maybe • Low dose ASA can be considered

  27. Polymyalgia Rheumatica

  28. PolymyalgiaRheumatica • A clinical syndrome of the middle aged and elderly characterized by pain and stiffness in the neck, shoulder and pelvic girdles,often accompanied by constitutional symptoms. • The clinical response to small doses of corticosteroids can be dramatic.

  29. PolymyalgiaRheumatica: • Clinical features • The musculoskeletal symptoms are usually bilateral and symmetrical. • Morning Stiffness is the predominant feature • Muscular pain is often diffuse and is accentuated by movement; pain at night is common.

  30. PolymyalgiaRheumatica: • Clinical features • Systemic features include low-grade fever,fatigue, weight loss and an elevated ESR. • Corticosteroid treatment is usually required for at least 2 years. • .

  31. Increased ESR AND CRP • NO pathognomonic test • No myopathy

  32. All of thefollowingcriteriamust be met todiagnose PMR: • 1- Agegreaterthan 50 years • 2-achingandstiffnessfor at least 1 month,affecting at leasttwo of thethreeabovementionedareas(ie,shoulders,neck, andpelvicgirdle) • 3-morningstiffnesslasting at least 1 hour • 4-ESR>40 mm/h • 5-exclusion of otherdiseasesexcept GCA and • 6-rapidresponsetoprednisone(<20 mgd)

  33. Treatment of PMR • Prednisone 15 mg • Slow taper over 12 to 18 months • Possible mtx use as 2nd line agent • Look for temporal arteritis • Concept of benign outcome

  34. TAKAYASU’S ARTERITIS

  35. Takayasu’s arteritis • Takayasu’s arteritis is a chronicinflammatory disorder of unknown etiologyprimarily affecting the aorta and its major branches. • Occurs most commonly in females under 40 years of age.

  36. Takayasu’s Arteritis • Pathophysiology • Giant cells are seen invading the media & adventitia of affected vessels • Bw52 or DR12 • Immunogenetics of TA are less well defined • Exposure to an unknown antigen precipitates an uncontrolled,inflammatory immune response that primarily targets large vessels

  37. Takayasu’s Arteritis • Clinical Picture • TA had a triphasic course: • 1. Early systemic complaints • 2. Followed by symptoms related to vascular inflammation • 3. Finally sequelae of vascular stenosis

  38. 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

  39. 80% of patients had bruits most in the carotid arteries • Elevated blood pressure occurred in 33% • Diminished or absent extremity pulse occur over half of the patients with TA • Nervous system symptoms occurred , in over half experienced dizziness • Visual disturbance affected 1/3 of the patients, always bilateral

  40. Takayasu’s Arteritis • Evaluation • ESR > 20mm/hr • Ultrasonography and MRI have been used • Angiography is considered the diagnostic goldstandard • Long stenotic lesions were seen in 98% of patients. • Aneurysms were seen in 27%

  41. Diagnosis - 3 of 6 criteria 1. onset age < 40 years 2. Limb claudication 3. decreased brachial artery pulse 4.unequal arm BP(>10 mmHg) 5.subclavian and artic bruit 6.Angiographic evidence of narrowing or occlusion of aorta

  42. Takayasu’s Arteritis • Management • Glucocorticoids are the mainstay of therapy • Other immunosuppressive therapies are used for patients who fail to respond to steroid therapy • Azathioprine and Methotrexate are frequently used & cyclophosphamide has been used with some

  43. Beta-adrenergic blockers and angiotensin-converting enzyme (ACE) inhibitors can be used to treat hypertension • Percutaneous coronary transluminal angioplasty (PCTA) successfullyrestores patency in as many as 80% of these patients • Bypass surgery can be done.

  44. Takayasu’s Arteritis • Prognosis • 5-10 years survival rates are in the range of 80-97% • Myocardial infarction, stroke, cardiac failure, aneurismal rupture are causes of death

  45. Medium-sized Vessels • Polyarteritis Nodosa • Kawasaki’s Disease

  46. Polyarteritis Nodosa (PAN) • Necrotizing arteritis of mediumsized muscular arteries • Pathology: “fibrinoid necrosis”

  47. Hepatitis B Virus Association • Usually occurs during the first 6 months after infection • Usually positive for HBAgs and e antigen

  48. Epidemiology of Polyarteritis Nodosa • Age: 20-70 years-old • No racial or ethnic predilection • Incidence • 2-4/1,000,000 annual incidence • 70-80/1,000,000/ in regions which • are endemic for Hepatitis B

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