1 / 58

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

montague
Download Presentation

Vasculitis syndromes

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript


  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:

More Related