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  2. 2. Introduction• Vasculitis- Inflammation of blood vesselscharacterised by leucocytic infiltration of thevessel walls• Different patterns of vessels’ involvement indifferent entities• Vessel lumen compromisedischemia of thecorresponding organ
  3. 3. Pathogenesis• 3 main groups of pathogenetic mechanismsbehind vasculitis-1.Immune complex formation2.ANCA mediated3.T lymphocyte mediated with Granulomaformation
  4. 4. Immune complex formation• Henoch Schonlein purpura- IgA mediated• SLE & other collagen vascular diseases-ANA• Serum sickness• Polyarteritis Nodosa- Hepatitis B ag• Essential Mixed Cryoglobinemia- HepatitisC virion*deposition of immune complexes in theblood vesselsactivation ofcomplementsdestruction of vessel wall(acute & chronic inflammation)
  5. 5. ANCA• P-ANCA (anti-proteinase 3)- Wegener’s• C-ANCA (anti-MPO)- Churg Strauss vasculitis- Microscopic Polyangiitis- Wegener’s granulomatosis* Aberrant expression of proteinase 3 and MPOover the surface of the neutrophilsformationof antibodiesdestruction ofneutrophilsvessel wall damage
  6. 6. Granuloma formation(T lymphocyte mediated)• Giant cell arteritis• Takayasu’s arteritis• Wegener’s granulomatosis• Churg Strauss vasculitis*classical granuloma formation (giant cells andepitheloid cells in a backround of fibrinoidnecrosis) can be demonstrated in thecorresponding vessel biopsy
  9. 9. Know the common featuresof vasculitis!!!• Palpable purpura (cutaneous vasculitis)• Pulmonary infiltrates• Glomerulonephritis (microscopic hematuria)• Mononeuritis multiplex• Unexplained ischemic events- MyocardialInfarction, Stroke, Raynaud’s phenomena,Digital gangrene, Mesentric Ischemia
  10. 10. Palpable purpura
  11. 11. Pulmonary infiltrates
  12. 12. Microscopic hematuria
  13. 13. STEP 2RULE OUT SECONDARY CAUSES OF VASCULITIS!!i.e- diseases where vasculitis is one of the clinicalmanifestations of the respective disease
  14. 14. Secondary Vasculitis• Infections• Malignancies• Thrombotic Microangiopathies• Drugs• Others
  15. 15. Infections• Bacterial endocarditis• Gonococcal Infection• Syphilis• Rickettsial diseases• Histoplasmosis• Coccidiomycosis• Whipple’s• Lyme’s
  16. 16. Malignancies• Atrial Myxomas• Carcinomatosis• LymphomasThrombotic Microangiopathies• TTP• HUS
  17. 17. Drugs• Cocaine• Phenytoin• Sulfa drugs• Penicillins• Hydralazine• Allopurinol• Propylthiouracil• Thiazides
  18. 18. Others• SLE• Amyloidosis• Sarcoidosis• Migraine• Atheroembolic Disease
  19. 19. STEP 3THE PATTERN OF VESSEL INVOLVEMENT(Large vessel, Medium vessel, Small vessel)
  20. 20. Large vessel vasculitis• Giant cell arteritis• Takayasu’s arteritis
  21. 21. Medium vessel Vasculitis• Poly Arteritis Nodosa• Kawasaki’s vasculitis
  22. 22. Small vessel VasculitisPauci-immune (ANCA mediated)Wegener’s GranulomatosisChurg Strauss vasculitisMicroscopic PolyangiitisImmune complex mediatedHenoch Schonlein PurpuraEssential Mixed CryoglobulinemiaSLE and other collagen c=vascular diseasesrelated vascultis
  23. 23. Other primary vasculitides• Thromb Angiitis Obliterans• Behcet’s disease• Idiopathic Cutaneous vasculitis• Isolated Vasculitis of CNS• Relapsing Polychondritis• Polyangiitis overlap syndromes (features ofmore than 1 vasculitis)
  24. 24. STEP 4Learn the characteristic presentations of each vasculitis !!!
  25. 25. Giant cell arteritis• Temporal arteritis• Elderly persons more than 50 yrs. of age• Non specific symptoms, Headache, ElevatedESR• BLINDNESS-most serious complication• Jaw claudication, Scalp pain, Scalp Tenderness• Polymyalgia Rheumatica- different end of thespectrum of Giant Cell Arteritis
  26. 26. Takayasu’s Arteritis• Pulseless Disease• Middle aged females• Aorta and its branches mainly involved• Subclavian vessels, Carotid vessels, Mesentricvessels• Chronic and Relapsing course
  27. 27. Poly Arteritis Nodosa• Renal arteries most commonly involvedleading to renovascular hypertension• Pulmonary vessels NEVER involved• Association with patients ofo Hepatitis Bo Hairy cell leukemia
  28. 28. Kawasaki’s Vasculitis• MucoCutaneous Lymph node syndrome• Children < 5 years of age mostly• Desquamative erythematous rashes involvingthe skin, mucus membranes, cervicallymphadenopathy• 25 % develop coronary artery aneurysms inthe convalescent stage of the illness
  29. 29. Pauci immune VasculitisUsually Pulmonary capillaritis PLUSGlomerulonephritis•Granulomas +, Asthma +  Churg Strauss•Granulomas +, NO asthma  Wegener’s•NO granulomas, NO asthma  MicroscopicPolyangiitis
  30. 30. Wegener’s Granulomatosis• Classical triad  URT + LRT + renal• Chronis sinusitis, Pulmonary nodules,Pulmonary cavities, Rapidly ProgressiveGlomerulonephritis• Cutaneous vasculitis, Eye lesions may bepresent• Non specific symptoms may predominate
  31. 31. Churg Strauss Vasculitis• Asthma, Eosinophilia with pulmonary infiltrates ,glomerulonephritis• Myocardial involvement  most common causeof deathMicroscopic Polyangiitis• Pulmonary alveolar capillariitis,glomerulonephritis
  32. 32. Henoch Schonlein Purpura• 2nd decade• Palpable purpura over lower limbs,• Gastrointestinal complaints (abd.colicky pain,blood in stools),• Fever, polyarthralgia• Increased IgA levels in blood
  33. 33. Essential Mixed Cryoglobulinemia• 5 % of Chronic Hepatits C pts. Have EMC• Cryoglobulins formed agianst HCV RNA• Pulmonary, renal ( MPGN ), cutaneousvasculitisThromb Angiitis Obliterans• Chronic heavy Smokers• Inflammation of arteries, veins, nerves• Upper and lower limb gangrene, Instepclaudication, rest pain
  34. 34. Other primary vasculitides• Behcet’s disease (Recurrent OculoOroGenitalulcerations with vasculitis)• Idiopathic Cutaneous vasculitis• Isolated Vasculitis of CNS• Relapsing Polychondritis• Polyangiitis overlap syndromes (features ofmore than 1 vasculitis)
  35. 35. Summary of 4 steps• Step 1- Recognise vasculitis• Step 2- Rule out Sec. Vasculitis• Step 3- Study the pattern of vessels involvedin the patient• Step 4- Remember the characteristicpresentations of each primary vasculitis
  36. 36. Step 5How to diagnose vasculitis???
  37. 37. Common Blood Counts• Mild Anemia – Anemia of Chronic Disease• Differential Leucocyte Count:Predominant eosinophils- Churg Strauss, HSPESR• Non specific• But useful test to suggest presence ofunderlying inflammatory process
  38. 38. • Acute Phase ReactantsHighly sensitive C reactive Protein, Alpha 2globulin• Chest X ray / HRCT thorax:-Pulmonary infiltrates- small vessel vasculitis-Pulmonary cavities- Wegener’s granulomatosis• Xray Para Nasal Sinuses-Sinusitis of Wegener’s
  39. 39. • Urine routine- RBCs with active sedimentssuggest Glomerulonephritis (Renalinvolvement of small vessel vasculitis)• Viral Markers- Hep. B Poly Arteritis Nodosa- Hep.C Essential Mixed Cryoglobulinemia
  40. 40. • Immunoglogulin levels (IgG, M, A)- Usually hyper gammaglobulinemia seen- Elevated IgA levelsHenoch Sconlein Purpura• Cryoglobulins- Essential Mixed Cryoglobulinemia• Rheumatoid Factors-To detect secondary vasculitisRheumatoidArthrits-Significantly raised in Essential MixedCryoglobulinemia also
  41. 41. • Complement levels (reduced in immune compexmediated 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 insuspicion of secondary vasculitis
  42. 42. BIOPSY• Renal Biopsy- to detect glomerulonephritisespecially in small vessel vasculitisRPGN- seen in pauci immune vasculitisMPGN- seen in EMC• Skin Biopsy- to detect “leukocytoclasis” incutaneous vasculitis all small vessel andsecondary vasculitides
  43. 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 ofleukocytoclasis, characterisitc pathologicalalterations in tissues, GRANULOMAS* Immunofluorescence also helps to study immunecomplex deposition, IgA deposition, Complementdeposition
  44. 44. ARTERIOGRAPHYHelps specially in in arteries that cannot bebiopsied 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. 45. The last step-STEP 6TREATMENT
  46. 46. Principles of Treatment• Immuno SuppressionGlucocorticoids- oral / IV methyl prednisoloneCyclophosphamideMethotrexateAzathioprineCyclosporineRituximab- anti CD 20 abAntiTNF therapies- Infliximab, Adalimumab,Etanacerpt, Certulizumab
  47. 47. Principles of Treatment• Choice of therapy depends onSeverity of organ damageExtent of Multi System InvolvementThe vascular bed involved (renal, ocular,coronary)• Cyclophosphamide + Glucocorticoid therapypreferred for severe / serious complications• Glucocorticoids alone will suffice for isolatedmild vascultis like “idiopathic cutaneousvascultis”
  48. 48. Principles of Treatment• Wherever possible secondary causes(infections, malignancies) should be soughtand treated• Anti viral therapy (HCV, HBV)• ASPIRIN therapy – Kawasaki’s, Giant cellarteritis• Intravenous Immunogloguloin Therapy-Prevents coronary aneurysms in Kawasaki’s
  49. 49. Principles of Treatment• Major toxic side effects of all prescribed drugsneed to be kept in mind(Osteoporosis, growth retardation, bonemarrow suppression, hepatic toxicity, renaltoxicity, bladder cancer, cystitis …)• Long term toxicities need to be prevented• Long term prescription of a single group ofdrug to be avoided change over to a drugwith lesser toxicity profile as soon assymptoms are controlled
  50. 50. Principles of Treatment• Regular Monitoring of Blood Counts, Renaland hepatic functions• Most of the Primary vasculitides have onething in common“Chronic, Responsive to treatment, ButNotoriously Relapsing”
  51. 51. SUMMARY OF STEPS• Step 1- Recognise vasculitis• Step 2- Rule out Sec. Vasculitis• Step 3- Study the pattern of vessels involvedin the patient• Step 4- Remember the characteristicpresentations of each primary vasculitis• Step 5- How to Diagnose• Step 6- Principles of treatment