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The Vasculitis Syndromes

The Vasculitis Syndromes. Definition. Inflammation and damage of blood vessels Single organ→ skin Several organ systems Primary Secondary Heterogeneity Overlap. Primary Vasculitis Syndromes. Wegener ´ s granulomatosis Churg-Strauss syndrome Polyarteritis nodosa Microscopic polyangitis

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The Vasculitis Syndromes

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  1. The Vasculitis Syndromes

  2. Definition • Inflammation and damage of blood vessels • Single organ→ skin • Several organ systems • Primary • Secondary • Heterogeneity • Overlap

  3. Primary Vasculitis Syndromes • Wegener´s granulomatosis • Churg-Strauss syndrome • Polyarteritis nodosa • Microscopic polyangitis • Giant cell arteritis • Takayasu´s arteritis • Henoch-Schönlein purpura • Idiopthic cutaneous vasculitis • Essential mixed cryoglobulinemia • Behcet´s syndrome • Isolated vasculitis of the CNS • Cogan´s syndrome • Kawasaki disease

  4. Secondary Vasculitis Syndromes • Drug-induced vasculitis • Serum sickness • Vasculitis associted with other primary diseases, infection, malignancy, rheumatic disease

  5. Pathophysiology and Pathogenesis • Immunpathogenic mechanisms • Pathogenic immune complex formation and/or deposition:Hepatitis B PAN, Hepatitis C Essential mixed cryoglob. • Production of antineutrophilic cytoplasmic antibodies: Wegener´s, Churg-Strauss, Microsc. polyang. • Pathogenic T lymphocyte responses and granuloma formation:Giant cell, Takayasu´s, Wegener, Churg-Strauss

  6. Approach to the patient: Vasculitis • Unexplained systemic illness • Angiogram of organs • Definitive diagnosis→biopsy of involved tissue

  7. Wegener´s granulomatosis

  8. Incidence and Prevalence • Prevalence: 3 per 100.000 • Age of onset: 40 years • Primary vasculitis syndrome→ damage of vessels • Immunopathogenic mechanisms • ANCA= antineutrophil cytoplasmic antibody • c-ANCA= cytoplasmic ANCA • (diffuse, granular cytoplasmic staining by immunofluorescence microscopy) • Detectable antibodies to proteinase-3 • Granuloma formation

  9. Pathology and pathogenesis • Necrotizing vasculitis of small arteries and veins + granuloma formation • Lung: multiple, bilateral, nodular cavitary infiltrates • Biopsy: necrotizing granulomatous vasculitis • Upper airway lasions: sinuses, nasopharynx • Kidney: rapidly progressive crescentic glomerulonephritis

  10. Clinical manifestations • Upper airways 95% • Purulent or bloody nasal discharge • Nasal septal perforation • Saddle nose deformity • Serous oitis media, hearing loss • Subglottic tracheal stenosis→severe airway obstruction • Pulmonary involvement:hemoptysis, dyspnea 85-90% • Eye: scleritis, conjunctivitis, retroorbital mass laesion • Skin: palpable purpura, papules, vesicles, subcutaneous nodules • Heart: pericarditis, coronary vasculitis • CNS: mononeuritis multiplex, cerebral vasculitis • Kidney disease: 77%, proteinuria, hematuria, red blood cell casts, rapidly progressive renal failure

  11. Laboratory findings • ESR↑ • Mild anemia, leukocytosis, thrombocytosis • 90% positive antiproteinase-3 c-ANCA

  12. Diagnosis of WG • Tissue biopsy: necrotizing granulomatous vasculitis • c-ANCA positivity

  13. Treatment of WG • Cyclophosphamide induction for severe disease: 2 mg/kg per day orally + steroids • Cyclophosphamide for 1 year following the induction of complete remission • Prednisone 1 mg/kg per day • Improvement: 90% • Complete remissions: 75% • Later relapses • Remission maintenance: Azathioprine, Methotrexate 2 years past remission

  14. Treatment of WG 2 • Mycophenolate mofetil 1000 mg twice a day • Rituximab (anti-CD20) • Trimethoprim-sulfamethoxazole

  15. Churg-Strauss syndrome

  16. Definition • Allergic angiitis and granulomatosis • Asthma, peripheral and tissue eosinophilia, extravascular granuloma formation, vasculitis of multiple organs

  17. Incidence and Prevalence • 1-3 cases per million • Onset: 48 years

  18. Pathology and Pathogenesis • Necrotizing vasculitis • Small and medium-sized muscular erteries, capillaries, veins, and venules • Granuloma formation • Infiltration of the tissues with eosinophils • Lung, skin, heart, kidney. Peripheralnervous system, GI

  19. Clinical and Laboratory Manifesttions • Asthma, pulmonary infiltrates • Mononeuritis multiplex • Allergic rhinitis and sinusitis • Skin lesions: purpura, subcutaneous nodules • Eosinophilia • ESR↑ • p-ANCA = perinuclear ANCA (antimyeloperoxidase)

  20. Diagnosis • Asthma • Peripheral blood eosinophilia • Biopsy

  21. Treatment • Steroids • Cyclophosphamide • Survival rate:72%

  22. Polyarteritis nodosa

  23. Definition • Multisystem, necrotizing vasculitis • Small and medium-sized muscular arteries • Renal and visceral arteries

  24. Incidence and Prevalence • Very uncommon disease

  25. Pathology and Pathogenesis • Segmental lesions • Bifurcations and branching of arteries • Aneurysmal dilatations up to 1 cm in size • Kidney: arteritis without glomerulonephritis • Hepatitis B antigenemia: 10-30% • Circulating immune complexes composed of hepatitis B antigen

  26. Clinical manifestations • Fever, weight loss • Kidney→renal failure, hypertension • Joint→arthritis, arthralgia, myalgia • Peripheral neuropathy, mononeuritis multiplex • GI→abdominal pain, bleeding, bowel infarction and perforation, liver infarction, pancreatic infarction • Skin→purpra, nodules, cutaneous infarcts, livedo reticularis • Heart→congestive heart failure, myocardial infarction, pericardiis • Testicular and ovarian pain • CNS→cerebral vascular accident, seizure, altered mental status

  27. Laboratory tests • Leukocyte count↑ • ESR↑ • Anemia of chronic disease • Hypergammaglobulinemia • HBsAg pozitivity 30%

  28. Diagnosis • Vasculitis on biopsy material of involved organs • Angiography→aneurysms of small and medium-sized arteries

  29. Treatment • Steroid • Cyclophosphamide • PAN + Hepatitis B virus pozitivity: Antiviral drugs+ steroid+plasma exchange

  30. Microscopic polyangitis • Glomerulonephritis→renal failure • Pulmonary capillaritis→hemoptysis • Mononeuritis multiplex • GI vasculitis • Cutaneous vasculitis • Age of onset: 57 years • pANCA positivity 75%

  31. Diagnosis and Treatment • Kidney,lung: histologic evidence of vasculitis • Steroid +Cyclophosphamide

  32. Giant cell arteritis and Polymyalgia rheumatica

  33. Definition • Temporal arteritis or Cranial arteritis • Inflammationof medium- and large-sized arteries • Temporal artery • Aorta and its main branches • Polymyalgia rheumatica: pain in the muscles of the neck, shouldersm lower back • >50 years

  34. Clinical and Laboratory Manifestations • Fever,anemia • ESR↑ • Headache • Temporal artery→tender, thickened, nodular • Ischemic optic neuropathy→visual loss→blindness • Increased risk of aortic aneurysm→ dissection

  35. Diagnosis • Biopsy of the temporal artery • Giant cell arteritis

  36. Treatment • Steroid: Prednisone40-60 mg/day 1 month • Treatment for≥ 2 years • ESR↓ • Polymyalgia rheumatica: Prednisone 10-20 mg/day • ESR↓

  37. Takayasu´s arteritis • Inflammatory and stenotic disease of medium- and large-sized arteries • Aortic arch and its branches • =Aortic arch syndrome • Incidence: 1-2 cases/million/year • Angiography

  38. Frequency of angiographic abnormalities • Subclavian: 93% • Common carotid: 58% • Abdominal aorta: 47% • Aortic arch: 35% • Vertebral: 35% • Pulmonary: 10-40% • Coronary < 10%

  39. Clinical manifestetions • Panarteritis with inflammatory mononuclear cell infiltrates • Narrowing of the lumen • Pulses absent • ESR↑, anemia, gammaglobulin↑

  40. Diagnosis • Young woman • Decrease or absence ofperipheral pulses • Discrepancies in blood pressure • Arterial bruits • Arteriography→irregular vessel walls, stenosis, poststenotic dilatation, aneurysm formation, occlusion, evidence of increased collateral circulation

  41. Treatment • Steroid: prednisone 40-60 mg/day • Methotrexate 25 mg/week • Surgical correction of stenosed arteries

  42. Henoch-Schönlein purpura • Anaphylactoid purpura • Palpable purpura over the buttocks and lower extremities • Arthralgias • GI signs→colicky abdominal pain, melena/hematochesia • Glomerulonephritis→proteinuria, microscopic hematuria, red blood cell casts • Small-vessel vasculitis • Children 4-7 years • Immune-complex deposition • Serum IgA↑

  43. Diagnosis • Clinical symptoms • Skin biopsy→ leukocytoclastic vasculitis

  44. Treatment • Do not require therapy • Steroids: Prednisone 1 mg/kg/day

  45. Idiopathic cutaneous vasculitis • =Hypersensitivity vasculitis • =Cutaneous leukocytoclastic vasculitis • Dermis, small vessels • Only skin involvement→palpable purpura, chronic urticaria • Diagnosis: biopsy→vasculitis • Etiology: drug, infection, underlying disease • Treatment:microbe→antimicrobal therapy, steroids

  46. Essential mixed cryoglobulinemia • Cryoglobulins: cold-precipitable monoclonal or polyclonal immunoglobulins • Cutaneous vasculitis, palpable purpura, arthralgias, glomerulonephritis • Hepatitis C infection • Treatment:IFN-α, Ribavirin

  47. Behcet´s syndrome • Recurrent, painful aphthous oral ulcerations in the oral cacity • Recurrent genital ulcers • Iritis • Cutaneous lesions • Treatment: topical steroids, Prednisone

  48. Isolated vasculitis of the CNS • Severe headache, focal neurologic defects • Abnormal MRI of the brain • Abnormal liquor • Abnormal cerebral angiogram→vasculitis • Brain biopsy • Treatment:Prednisone+Cyclophosphamide

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