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Vasculitis syndromes

Vasculitis syndromes. Salehi I., M.D. Rheumatologist. Salehi I. Vasculitis syndrome. Definition :. Clinicopathologic process Inflammation & Damage of vessels Vessel lumen is compromised Ischemia of tissues. Salehi I. Vasculitis syndrome. Heterogeneous syndromes

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Vasculitis syndromes

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  1. Vasculitis syndromes Salehi I., M.D. Rheumatologist

  2. Salehi I Vasculitis syndrome Definition : • Clinicopathologic process • Inflammation & Damage of vessels • Vessel lumen is compromised • Ischemia of tissues

  3. Salehi I Vasculitis syndrome • Heterogeneous syndromes • Any vessel type, size & location • Primary & Secondary • Single to multiple organ

  4. Salehi I Vasculitis syndrome Classification of Vasculitis • Primary • Secondary : • Connective tissue disorders • Inflammatory bowel disease • Neoplasm • Infection • Drug

  5. Salehi I Vasculitis syndrome Classification of Vasculitis • Vessel size : • Large-vessel V. • Medium-vessel V. • Small-vessel V. • Pathology : • Granulomatous V. • Immune complex V. • Antibodies : • ANCA positive V. • Anti-GBM V.

  6. Salehi I Vasculitis syndrome Large-vessel V. • Takayasu’s arteritis • Temporal arteritis(GCA) • Aortitis (SPA, RP) • Cogan’s syndrome • Behcet’s disease

  7. Salehi I Vasculitis syndrome Medium-vessel V. • Polyarteritis nodosa(PAN) • Cutaneous PAN • Buerger’s disease • Kawasaki’s disease • Primary angiitis of CNS

  8. Salehi I Vasculitis syndrome Small-vessel V. • Hypersensitivity vasculitis • Microscopic PAN • Churg-Strauss syndrome • Wegener’s granulomatosis • Henoch-Schonlein purpura • Hypocomplementemic urticarial V. • Goodpasture’s disease • Essential cryoglobulinemia

  9. Salehi I Vasculitis syndrome Granulomatous V. • Temporal arteritis • Takayasu’s arteritis • Wegener’s granulomatosis • Churg-Strauss syndrome • Primary angiitis of CNS • Cogan’s syndrome

  10. Salehi I Vasculitis syndrome Immune complex V. • Hypersensitivity vasculitis • Henoch-Schonlein purpura • Hypo C. urticarial v. • Essential cryoglobulinemia(HC) • Hepatitis B associated PAN

  11. Salehi I Vasculitis syndrome Classic PAN : • Periarteritis nodosa • Panarteritis nodosa • Polyarteritis nodosa • Definition : • Necrotizing vasculitis • Multisystem disease • Medium & small vessel V. • Renal & visceral involvement

  12. Salehi I Vasculitis syndrome Classic PAN : • Epidemiology : • Uncommon • M/F ratio : 1.6 – 2 / 1 • All race • Mid-40s to mid60s • Mean age : 48 yrs

  13. Salehi I Vasculitis syndrome Pathophysiology • Unknown • Trigger factor: • HBV, HCV, HIV, CMV, HTLV1,… • Underlying disorder: • SLE, RA, DM, Hairy CL,… • Immune complex deposition • Leukocyte infiltration: • Acute: PMN, Chronic: MNC PAN

  14. Salehi I Vasculitis syndrome Clinical manifestation: • Constitutional: • Fever, weight loss, malaise • Multisystem involvement: • Peripheral nerve • Kidney • Skin • Joint & muscle • GI • Heart • CNS • Eye • Genitourinary • Others PAN

  15. Salehi I Vasculitis syndrome Clinical manifestation: • Peripheral nerve: • Mononeuritis multiplex • peripheral neuropathy • Kidney: • Renal failure • Hypertension • Hematuria • Proteinuria • Skin: • Palpable purpura • Cutaneous infarcts • Digital infarction • Livedo reticularis • Raynaud’s ph. PAN

  16. Salehi I Vasculitis syndrome Clinical manifestation: • Joint & muscle: • Arthralgia • Arthritis • Myalgia • Gastrointestinal: • Abdominal angina • Nausea, vomiting • Bowel infarction • Bleeding • Perforation • Cholecystitis • Hepatic infarction • Pancreatic infarction PAN

  17. Salehi I Vasculitis syndrome Clinical manifestation: • Heart: • Congestive heart failure • Myocardial infarction • Pericarditis • CNS: • Cerebral vascular accident • Seizure • Altered mental status • Eye: • Retinal hemorrhage • Retinal vasculitis • Genitourinary: • Testicular pain • Ovarian pain • Epididymal pain PAN

  18. Salehi I Vasculitis syndrome Typical case of PAN • Middle age man • Fever, weight loss, malaise • Palpable purpura, livedo reticularis, digital infarction • Wrist drop, Foot drop • Azotemia, HTN, CVA, MI • Abdominal angina • Arthralgia, Arthritis, Myalgia PAN

  19. Salehi I Vasculitis syndrome Lab. Data : • Nonspecific • CBC: • Leukocytosis • Anemia • Thrombocytosis • Elevated ESR • HBsAg(+): 30% • p-ANCA, c-ANCA • BUN/Cr, U/A • ECG PAN

  20. Salehi I Vasculitis syndrome Pathology : • Focal segmental necrotizing v. • Medium & smallarteries • PMN infiltrate all layers • Fibrinoid necrosis • Intimal proliferation • Disrupted elastic lamina • Intraluminal thrombosis • Oblitrated lumen • Aneurysmal dilatation • No pulmonary & splenic arteries • No venules • No granuloma, no eosinophil PAN

  21. Salehi I Vasculitis syndrome Diagnosis : • Typical clinico-lab. Feature • Biopsy: • Nodular skin lesion • G. muscle & sural nerve • Painful testes • Renal Bx. Is not diagnostic • Angiography: • Abdominal visceral & renal arteries • Aneurysms & stenosis PAN

  22. Salehi I Vasculitis syndrome • Prognosis: Poor • 5-year survival: • Without treatment: 10-20% • With Glucocorticoid: >40% • Death: • GI: Bowel infarct & perforation • Heart: CHF, MI • Kidney: Renal Failure, HTN PAN

  23. Salehi I Vasculitis syndrome • Treatment: • HBsAg(-): • Glucocorticoids: Pulse or HDS + • Cyclophosphamide: Pulse or PO • HBsAg(+): • IFN-a or Vidarabine + • Glucocorticoids & Plasma exchange • Relapse: • With treatment: 10% PAN

  24. Salehi I Vasculitis syndrome Microscopic polyangiitis(MPA) • Definition: • Systemic necrotizing vasculitis • Small vessel: capillary, venule, arteriol • Multisystem involvement: • Glomerulonephritis • Pulmonary capillaritis • Others • Epidemiology: • Mean age: 57 yrs • M/F ratio > 1 MPA

  25. Salehi I Vasculitis syndrome • Pathology: • Similar to PAN but • Predilection to capillary & venule • No Immune complex • Clinical feature: • Similar to PAN but • Predilection to Kidney & Lung • Glumerulonephritis: 80% • Hemoptysis: 12% • No HBsAg(+) • DD with Wegener’ G.: • Both are small vessel v. • Renal lesion is similar • ANCA(+) in both • Pulmonary involvement in both, but in MPA • No upper airway involvement • No pulmonary nodule • No granuloma MPA

  26. Salehi I Vasculitis syndrome • Treatment: • Similar to Wegener’s G. • CYC + HDS • 5-year survival: • 75% with treatment • Death: • Alveolar hemorrhage • GI, Heart, Kidney • Relapse: • 34% of cases MPA

  27. Salehi I Vasculitis syndrome Churg-Strauss syndrome:(CSS) • Other name: • Allergic angiitis & granulomatosis • Definition: • Small vessel vasculitis • Multisystem involvement • PMH of asthma • Eosinophilia • Extravascular granuloma • Epidemiology: • Incidence: 1-3/1000,000 • Mean age: 48 yrs • F/M ratio: 1.2/1

  28. Salehi I Vasculitis syndrome Three clinical phases of CSS • Prodromal phase: • Allergic features • Rhinitis, polyposis, asthma • Blood & tissue eosinophilia: • Similar to Loffler’s syndrome • Eosinophilic pneumonia • Eosinophilic gastroenteritis • Systemic vasculitis: • Similar to Wegener’s G.

  29. Salehi I Vasculitis syndrome • Lab. Data: • Eosinophilia> 1000/micL :80% • p-ANCA :50-70%, c-ANCA • Other similar to PAN • Classification criteria: • Asthma • Eosinophilia> 10% • Mono/polyneuropathy • Pulmonary infiltrates(non-fixed) • Paranasal sinus abnormality • Extravascular eosinophils • > 4 of 6, sens.=85%, spec.=99.7% CSS

  30. Salehi I Vasculitis syndrome • 5-year survival: • Without treatment: 25% • With treatment: 75% • Death: • Heart: 40-50% • MI, CHF • Treatment: • Similar to WG or PAN • CSS

  31. Salehi I Vasculitis syndrome Temporal Arteritis: • Other name: • Giant cell arteritis • Cranial arteritis • Definition: • Large-vessel vasculitis • Multisystem involvement • Carotid artery branches • Predilection to temporal artery

  32. Salehi I Vasculitis syndrome • Epidemiology: • Almost all in > 50 yrs • Almost all in whites • F/M ratio = 2/1 • Sibling: x 10 • 80s/50s: x 10 • Incidence: 7-33/100,000 in > 50 yrs • Pathophysiology: • Genetic: white, HLA-DR4, HLA-DRB104 • Aging: Endothelial disruption • Viruses: Parainfl., EBV, HSV, RSV • AECA: Large-vessel EC GCA

  33. Salehi I Vasculitis syndrome Clinical features: • 4 categories: • Fever/wasting syndrome • Isolated Polymyalgia Rheumatica(PMR) • Cranial Arteritis • Large-vessel GCA/ aortitis • Overlap GCA

  34. Salehi I Vasculitis syndrome • Fever/wasting syndrome: • Fever & chills(FUO) • Anorexia, weight loss • Night sweats • Weakness • Depression • Isolated PMR: • Pain & stiffness: in 2 of below 3 • Neck • Shoulder girdle • Pelvic girdle • Age > 50 yrs/old • ESR > 40 mm/hr • Duration > 1 month GCA

  35. Salehi I Vasculitis syndrome • Cranial Arteritis: • Headaches • Scalp(temporal) tenderness • Tender nodular, pulseless & Thickened temporal artery • Ischemic optic neuritis: blindness • Jaw claudication • CNS ischemia: stroke • PMR GCA

  36. Salehi I Vasculitis syndrome • Large-vessel GCA/ aortitis: • Arm claudication • Pulselessness • Raynaud’s phenomenon • Aortic aneurysm • Aortic insufficiency • Myocardial infarction • Mononeuritis multiplex • PMR GCA

  37. Salehi I Vasculitis syndrome • Typical case of GCA • A patient with: • Fever • Headache • Anemia • ESR > 50 mm/hr & • Age > 50 yrs • Lab. Data: • CBC • ESR • LFT

  38. Salehi I Vasculitis syndrome Pathology: • Biopsy of abnormal temporal artery • A segment of 3-5 cm • Mononuclear cell infiltration • Granuloma with • Multinucleated giant cell • Intimal proliferation • Disrupted internal elastic lamina • Segmental involvement GCA

  39. Salehi I Vasculitis syndrome Classification criteria of GCA • Age at onset > 50 yrs • New headache • Temporal artery abnormality • Tender or • Pulseless • ESR > 50 mm/hr • Abnormal temporal artery biopsy • MNC infiltration or • Granuloma with multinucleated giant cell • 3 of 5, sens.: 93.5%,Spec.: 91.2%

  40. Salehi I Vasculitis syndrome Treatment: • Prednisolone: • 1 mg/kg/day for 1 month • Gradual tapering to 7.5-10 mg/day • Continued for a few years • Cytotoxics: MTX • The most morbidity is Blindness • In the setting of ocular involvement • Therapy before Biopsy • Biopsy show vasculitis even after >14 days of glucocorticoid therapy GCA

  41. Salehi I Vasculitis syndrome Takayasu’s Arteritis: • Other names: • Aortic arch syndrome • Pulseless disease • Definition: • Large-vessel vasculitis • Multisystem involvement • Predilection for aortic arch & its branches • Pulseless extremities

  42. Salehi I Vasculitis syndrome Epidemiology: • Incidence: • 1.2-2.6/1000,000 • F/M ratio : 8/1 • Age: 10-30 yrs (< 40) • More common in the orient TA

  43. Salehi I Vasculitis syndrome • Types of TA: • Type I : Shimizo sano: Aortic arch • Type II: Kimoto: Descending Aorta • TypeIII: Inada: Combination of I & II • TypeIV: Any type + Pulmonary artery • Clinical stages of TA: • Prepulseless: constitutional • Pulseless: Inflammatory • Pulseless: Fibrotic TA

  44. Salehi I Vasculitis syndrome • Constitutional: • Fever, weight loss • malaise, anorexia, Arthralgia • Pulseless: • Claudication, Raynaud’s ph. • Decreased brachial pulse • Bruits, Asymmetric BP • Headache, TIA, syncope, CVA • HTN, CHF, AI • Visual change • Abdominal pain, nausea, vomiting • Lab. Data: • High ESR, Anemia • Doppler ultrasound • Spiral CT, Angiography • NO Biopsy TA

  45. Salehi I Vasculitis syndrome ACR criteria for TA: • Age at onset < 40 yrs • Claudication of extremities • Decreased brachial pulse • BP difference > 10 mm Hg • Bruit over subclavian a. or aorta • Arteriogram abnormality • 3 of 6, sens.:90.5, spec.:97.8

  46. Salehi I Vasculitis syndrome • Treatment: • Prednisolone(MDS-HDS) • MTX • Pentoxifylline • Vasodilator, Anti HTN • Surgery: Angioplasty • Death: • CHF • CVA • MI • Aneurysm rupture • Renal failure TA

  47. Salehi I Vasculitis syndrome Idiopathic Cutaneous vasculitis(ICV): • Other names: • Hypersensitivity vasculitis • Leukocytoclastic vasculitis • Definition: • Small vessel vasculitis • Skin involvement • Epidemiology: • Most common vasculitis • Secondary: 70% • Drugs, infection, CTD, neoplasm • Idiopathic: 30%

  48. Salehi I Vasculitis syndrome Pathology of ICV: • Smal vessel inflammation • PMN infiltration • Fibrinoid necrosis • Leukocytoclasis • RBC extravasation

  49. Salehi I Vasculitis syndrome • Clinical feature: • Palpable purpura • Macules, Papules • Vesicles, Bullae, Ulcer • Subcutaneous nodules • Recurrent or chronic urticaria • Hydrostatic forces ==> lower > upper • Lab. Data: • Diagnosis: • Hx., Ph.Ex., Biopsy

  50. Salehi I Vasculitis syndrome Classification Criteria of ICV: • Age at onset > 16 yrs • Medication at disease onset • Palpable purpura • Maculopapular rash • Biopsy of skin (arteriole, venule) • 3 of 5, sens.:71%, spec.: 83.9% • Treatment:

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