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APPROACH TO VASCULITIS

APPROACH TO VASCULITIS. DR. Emad Abokhabar Lecturer of Internal Medicine & Nephrology Sohag University. Introduction. Vasculitis- Inflammation of blood vessels characterised by leucocytic infiltration of the vessel walls

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APPROACH TO VASCULITIS

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  1. APPROACH TO VASCULITIS DR. Emad Abokhabar Lecturer of Internal Medicine & Nephrology Sohag University

  2. Introduction • Vasculitis- Inflammation of blood vessels characterised by leucocytic infiltration of the vessel walls • Different patterns of vessels’ involvement in different entities • Vessel lumen compromisedischemia of the corresponding organ

  3. Pathogenesis • 3 main groups of pathogenetic mechanisms behind vasculitis- • Immune complex formation • ANCA mediated (ANTINEUTROPHILIC CYTOPLASMIC ANTIBODIES) • T lymphocyte mediated with Granuloma formation

  4. Immune complex formation • HenochSchonleinpurpura- IgA mediated • SLE & other collagen vascular diseases- ANA • Serum sickness • PolyarteritisNodosa • Essential Mixed Cryoglobinemia

  5. ANCA MEDIATED • Churg Strauss vasculitis • Microscopic Polyangiitis • Wegener’s granulomatosis

  6. Granuloma formation (T lymphocyte mediated) • Giant cell arteritis • Takayasu’s arteritis • Wegener’s granulomatosis • Churg Strauss vasculitis

  7. APPROACH TO VASCULITIS

  8. STEP 1 “LEARN TO RECOGNISE VASCULITIS”

  9. Know the common features of vasculitis!!! • Palpable purpura (cutaneous vasculitis) • Pulmonary infiltrates • Glomerulonephritis (microscopic hematuria) • Mononeuritis multiplex • Unexplained ischemic events- Myocardial Infarction, Stroke, Raynaud’s phenomena, Digital gangrene, Mesentric Ischemia

  10. Palpable purpura

  11. Pulmonary infiltrates

  12. Microscopic hematuria

  13. STEP 3 RULE OUT SECONDARY CAUSES OF VASCULITIS!! i.e- diseases where vasculitis is one of the clinical manifestations of the respective disease

  14. Secondary Vasculitis • Infections • Malignancies • Thrombotic Microangiopathies • Drugs • Others

  15. Infections • Bacterial endocarditis • Gonococcal Infection • Syphilis • Rickettsial diseases • Histoplasmosis • Coccidiomycosis • Whipple’s • Lyme’s

  16. Malignancies • Atrial Myxomas • Carcinomatosis • Lymphomas Thrombotic Microangiopathies • TTP • HUS

  17. Drugs • Cocaine • Phenytoin • Sulfa drugs • Penicillins • Hydralazine • Allopurinol • Propylthiouracil • Thiazides

  18. Others • SLE • Amyloidosis • Sarcoidosis • Migraine • Atheroembolic Disease

  19. STEP 3 THE PATTERN OF VESSEL INVOLVEMENT (Large vessel, Medium vessel, Small vessel)

  20. Large vessel vasculitis • Giant cell arteritis • Takayasu’s arteritis

  21. Medium vessel Vasculitis • Poly Arteritis Nodosa • Kawasaki’s vasculitis

  22. Small vessel Vasculitis ANCA mediated • Wegener’s Granulomatosis • Churg Strauss vasculitis • Microscopic Polyangiitis Immune complex mediated • HenochSchonleinPurpura • Essential Mixed Cryoglobulinemia • SLE and other collagen c=vascular diseases related vascultis

  23. Other primary vasculitides • ThrombAngiitisObliterans • Behcet’s disease • Idiopathic Cutaneous vasculitis • Isolated Vasculitis of CNS • Relapsing Polychondritis • Polyangiitis overlap syndromes (features of more than 1 vasculitis)

  24. STEP 4 Learn the characteristic presentations of each vasculitis !!!

  25. Giant cell arteritis • Temporal arteritis • Elderly persons more than 50 yrs. of age • Non specific symptoms, Headache, Elevated ESR • BLINDNESS-most serious complication • Jaw claudication, Scalp pain, Scalp Tenderness • Polymyalgia Rheumatica- different end of the spectrum of Giant Cell Arteritis

  26. Takayasu’s Arteritis • Pulseless Disease • Middle aged females • Aorta and its branches mainly involved • Subclavian vessels, Carotid vessels, Mesentric vessels • Chronic and Relapsing course

  27. Poly Arteritis Nodosa • Renal arteries most commonly involved leading to renovascular hypertension • Pulmonary vessels NEVER involved • Association with patients of • Hepatitis B • Hairy cell leukemia

  28. Kawasaki’s Vasculitis • MucoCutaneous Lymph node syndrome • Children < 5 years of age mostly • Desquamative erythematous rashes involving the skin, mucus membranes, cervical lymphadenopathy • 25 % develop coronary artery aneurysms in the convalescent stage of the illness

  29. ANCA MEDIATED Vasculitis Usually Pulmonary capillaritis PLUS Glomerulonephritis • Granulomas +, Asthma +  Churg Strauss • Granulomas +, NO asthma  Wegener’s • NO granulomas, NO asthma  Microscopic Polyangiitis

  30. Wegener’s Granulomatosis • Classical triad  URT + LRT + renal • Chronis sinusitis, Pulmonary nodules, Pulmonary cavities, Rapidly Progressive Glomerulonephritis • Cutaneous vasculitis, Eye lesions may be present • Non specific symptoms may predominate

  31. Churg Strauss Vasculitis • Asthma, Eosinophilia with pulmonary infiltrates , glomerulonephritis • Myocardial involvement  most common cause of death Microscopic Polyangiitis • Pulmonary alveolar capillariitis, glomerulonephritis

  32. HenochSchonleinPurpura • 2nd decade • Palpable purpura over lower limbs, • Gastrointestinal complaints (abd.colicky pain, blood in stools), • Fever, polyarthralgia • Increased IgA levels in blood

  33. Essential Mixed Cryoglobulinemia • 5 % of Chronic Hepatits C pts. Have EMC • Cryoglobulins formed agianst HCV RNA • Pulmonary, renal ( MPGN ), cutaneous vasculitis

  34. Other primary vasculitides • Behcet’s disease (Recurrent OculoOroGenital ulcerations with vasculitis) • Idiopathic Cutaneous vasculitis • Isolated Vasculitis of CNS • Relapsing Polychondritis • Polyangiitis overlap syndromes (features of more than 1 vasculitis)

  35. Summary of 4 steps • Step 1- Recognise vasculitis • Step 2- Rule out Sec. Vasculitis • Step 3- Study the pattern of vessels involved in the patient • Step 4- Remember the characteristic presentations of each primary vasculitis

  36. Step 5 How to diagnose vasculitis???

  37. Common Blood Counts • Mild Anemia – Anemia of Chronic Disease • Differential Leucocyte Count: Predominant eosinophils- Churg Strauss, HSP ESR • Increased • Non specific • But useful test to suggest presence of underlying inflammatory process

  38. Acute Phase Reactants Highly sensitive C reactive Protein, Alpha 2 globulin • Chest X ray / HRCT thorax: -Pulmonary infiltrates- small vessel vasculitis -Pulmonary cavities- Wegener’s granulomatosis • Xray Para Nasal Sinuses -Sinusitis of Wegener’s

  39. Urine routine- • RBCs with active sediments suggest Glomerulonephritis (Renal involvement of small vessel vasculitis) • Viral Markers • Hep. B Poly Arteritis Nodosa • Hep.C Essential Mixed Cryoglobulinemia

  40. Immunoglogulin levels (IgG, M, A) • Usually hyper gammaglobulinemia seen • Elevated IgA levelsHenochSconleinPurpura • Cryoglobulins- Essential Mixed Cryoglobulinemia • Rheumatoid Factors -To detect secondary vasculitisRheumatoidArthrits -Significantly raised in Essential Mixed Cryoglobulinemia also

  41. Complement levels (reduced in immune compex mediated diseases)- EMC, HSP • ANCA • P-ANCA: Wegener’s Granulomatosis • C-ANCA: Microscopic polyangiitis, Churg Strauss, Wegener’s vasculitis • ANA -screening of SLE, collagen vascular disorders in suspicion of secondary vasculitis

  42. BIOPSY • Renal Biopsy- to detect glomerulonephritis especially in small vessel vasculitis • RPGN- seen in pauci immune vasculitis • MPGN- seen in EMC • Skin Biopsy- to detect “leukocytoclasis” in cutaneous vasculitis all small vessel and secondary vasculitides

  43. BIOPSY • Temporal Artery Biopsy- Giant Cell Arteritis • Pulmonary tissue Biopsy- Small vessel vascultides • Upper Airway biopsies- Wegener’s Vasculitis * Main purpose of biopsy is to study presence of leukocytoclasis, characterisitc pathological alterations in tissues, GRANULOMAS * Immunofluorescence also helps to study immune complex deposition, IgA deposition, Complement deposition

  44. ARTERIOGRAPHY • Helps specially in in arteries that cannot be biopsied easily like Aorta, Coronary artery, Mesentric vessels • Presence of vascular patency, Aneurysms • Aortic Angiography- Takayasu’s • Cerebral Angiography- Isolated CNS vascultis • Renal Angiography- PAN • Coronary Angiography- Kawasaki’s • Lower limb arteriography-Buerger’s Disease (TAO)

  45. The last step-STEP 6 TREATMENT

  46. Principles of Treatment • Immuno Suppression • Glucocorticoids- oral / IV methyl prednisolone • Cyclophosphamide • Methotrexate • Azathioprine • Cyclosporine • Rituximab- anti CD 20 ab • AntiTNF therapies- Infliximab, Adalimumab, Etanacerpt, Certulizumab

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