1 / 55

Approach to Vasculitis: Recognition, Causes, and Presentation

Learn the pathogenesis, types, and characteristics of vasculitis to effectively recognize, differentiate, and manage various forms. Understand the vessel involvement patterns, secondary causes, and typical presentations for proper diagnosis and treatment.

danieldickson
Download Presentation

Approach to Vasculitis: Recognition, Causes, and Presentation

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. 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

More Related