CLASSIFICATION OF VASCULITIS BASED ON PATHOGENESIS

1. CLASSIFICATION OF VASCULITIS BASED ON PATHOGENESIS • DIRECT INFECTION : • Bacterial (e.g. Neisseria ) • Rickettsial (e.g. Rocky mountain spotted fever) • Spirochetal ( e.g. Syphilis ) • Fungal (e.g. Aspergillosis, Mucormycosis ) • Viral (e.g. Herpes zoster – varicella )

2. CLASSIFICATION OF VASCULITIS BASED ON PATHOGENESIS • IMMUNOLOGIC • Immune complex–mediated • Infection-induced (e.g., hepatitis B and C virus) • Henoch-Schonlein purpura • SLE and rheumatoid arthritis • Drug-induced • Cryoglobulinemia • Serum sickness

3. CLASSIFICATION OF VASCULITIS BASED ON PATHOGENESIS • ANTINEUTROPHIL CYTOPLASMIC ANTIBODY (ANCA)–MEDIATED - Wegener granulomatosis - Microscopic polyangiitis (microscopic polyarteritis) - Churg-Strauss syndrome • DIRECT ANTIBODY MEDIATED - Goodpasturesyndrome (anti-GBM antibodies) - Kawasaki disease (anti-endothelial antibodies) • CELL-MEDIATED - Organ allograft rejection • INFLAMMATORY BOWEL DISEASE • PARANEOPLASTIC VASCULITIS

4. CLASSIFICATION OF VASCULITIS BASED ON PATHOGENESIS • CELL-MEDIATED - Organ allograft rejection • INFLAMMATORY BOWEL DISEASE • PARANEOPLASTIC VASCULITIS • UNKNOWN : - Giant cell ( temporal arteries ) • Takayasu arteries • Polyarteritisnodosa ( classic

5. CHARACTERISTICS OF VASCULITIS • LARGE CELL VASCULITIS : • Giant cell : Granulomatousarteritis of ( temporal) the aorta and its major branches arteritis ( Age above 50 ) • Takayasu : Granulomatousarteritis of the arteritis aorta and its major branches ( Age above 50 )

6. CHARACTERISTICS OF VASCULITIS • MEDIUM SIZED VESSEL VASCULITIS: • Polyarteritis : Necrotising inflammation of nodosa medium sized and small (classic ) arteries. - Kawasaki : Arteritis involving large, disease medium sized and small arteries and associated with mucocutaneous lymph node syndrome.

7. CHARACTERISTICS OF VASCULITIS • SMALL CELL VASCULITIS : • Wegner : Granulomatous inflammation granulomatosisinvolving the respiratory tract and necrotisingvasculitis affecting small to medium sized vessels.

8. GIANT CELL (TEMPORAL) ARTERITIS • Most common form of systemic vasculitis. • Chronic granulomatous inflammatory arteries. • Large, medium and small arteries involved. Temporal arteries, Opthalmic arteries, Vertebral arteries, Aaorta, Axillary, Branchial, Femoral , Mesentric etc.

9. GIANT CELL (TEMPORAL) ARTERITIS • PATHOGENESIS: T cell mediated immune reaction against component of arterial wall ( ? Elastin). • PATHOLOGY : Nodular thickening of wall with in the lumen ± thrombosis.

10. GIANT CELL (TEMPORAL) ARTERITIS • MICROSCOPY: Granulomatous inflammation with Langhan cells, usually around int. elastic lamina, full circumference involvement , fragementation of int. elastic lamina. Neutrophils ± . • CLINICAL FEATURES : Age ˃ 50 . Fever, fatigue, headache, Diplopia, Vision loss , painful temporal artery.

11. GIANT CELL (TEMPORAL) ARTERITIS

12. TEMPORAL ARTERITIS

13. TAKAYASU ARTERITIS • Involves aortic arch and other heavilly elastic arteries, i.e., chief thoracic aorta branches, most commonly young Asian women • FEMALES <40 • “PULSELESS” disease • NECROSIS, Giant Cells also

14. TAKAYASU ARTERITIS

15. WEGENER GRANULOMATOSIS • A form of necrotisingvasculitischaracterised by a clinicopatholgical triad: • Acute necrotisinggranulomas of upper respiratory tract (sinuses) and / or lower respiratory tract ( Lung ). • Focal NecrotisingVasculitis of small and medium sized vessels of lung and URT (capillaries ,arterioles, venule ). • Focal necrotising / crescenticglomerulitis. In ‘limited form’ Renal pathology is absent.

16. WEGENER GRANULOMATOSIS • PATHOGENESIS : Cell mediated immune reaction against unknown agents in affected sites. • PATHOLOGY: Severe sinusitis, ulceration of nose, pharynx, palate etc. Other organ involvement ( Joints, eye, skin, CVS, ear, nerves etc. )

17. WEGENER GRANULOMATOSIS • MICROSCOPY: • Necrotisinggranulomas of tissue Necrotisingvasculitis with or without granulomas. • Presence of fibrionoid necrosis, neutrophilsMNcells , fibroblasts , giant cells. • Vasculitis is segmental or circumferential. • Renal lesions- focal necrotisingglomerulitis or crescenticglomerulonephritis.

18. WEGENER GRANULOMATOSIS • CLINICAL FEATURES : • Age 40 – 60 M ˃ F • Persistent Pneumonitis ( 95 % ) • Chronic sinusitis ( 90 % ) • Nasopharyngeal mucosal ulcers ( 75 % ) • Sinusitis ( 90 % ) • Others : Muscle pain, skin rash, arthralgia , polyneuritis etc.

19. WEGENER GRANULOMATOSIS necrosis granulomas

20. POLYARTERITIS NODOSA (PAN) • SYSTEMIC VASCULITIS OF SMALL OR MEDIUM SIZED ARTERIES ( NOT ARTERIOLES , CAPILLARIES ,VENULES ). • RENAL, HEPATIC GI ARTERIES- MOST COMMON. • NEVER PULMONARY.

21. POLYARTERITIS NODOSA (PAN) • PATHOLOGY: Involvement of part of arterial wall circumference, predeliction for bifurcation, aneurysm formation, rupture , haemorrhage with infarction of affected area.

22. POLYARTERITIS NODOSA (PAN) • MICROSCOPY : - Neutrophils , eosinophils , MN cells • Fibrinoid necrosis. • Fibrosis of vessel wall in chronic phase. • CLINICAL FEATURES : Children and young adults. Acute , subacute and chronic forms. Fever malaise, weight loss, abdominal pain, melena, hypertension, Myalgia, peripheral neuritis, renal manifestations.

23. POLYARTERITIS NODOSA (PAN)

24. KAWASAKI DISEASE(MUCOCUTANEOUS LYMPHNODE SYNDROME ) • Acute or subacute disease of young children and adults ( 80 % ˂ 4 years old ). • Coronary artery involvement prominent. • Common in North America and Japan.

25. KAWASAKI DISEASE • PATHOGENESIS : ? Viral infection in genetically succeptible children T cell activation against unknown antigen cytokine secretion B cell activation formation of auto antibodies. • PATHOLOGY : Severe inflammation of full thickness of blood vessel, necrosis, resolution common , rarely aneurysm, thrombosis myocard. infarction

26. KAWASAKI DISEASE • CLINICAL FEATURES : • Oral erythema • Erosion • Oedema of hands, feet • Erythema of palm, sole , cervical lymph node • CVS manifestations. • Fever

27. KAWASAKI DISEASE

28. THROMBOANGITIS OBLITERANS ( BURGER DISEASE ) • Segmental thrombosing , acute and chronic inflammation of medium and small arteries , tibial and radial , leading to vascular in sufficiency.

29. THROMBOANGITIS OBLITERANS • ETIOLOGY AND PATHOGENESIS: • Genetic tendency • Strong relation to smoking • Increases prevelence in HLA – A 9 and HLA B5 types. • Hypersesitivity to tobacco products in arterial wall vaculitis Thrombosis.

30. THROMBOANGITIS OBLITERANS • PATHOLOGY: Inflammation and thickening of small and medium arteries of extremities in a segmental pattern , inflammation of nerves. • MICROSCOPY : Acute and chronic inflammation with super added thrombosis, necrosis, abscess formation. • CLINICAL FEATURES : M > F , Intermittent cloudication , pain , ulceration , gangrene .

31. THROMBOANGITIS OBLITERANS

32. CLASSIFICATION OF VASCULAR TUMOURS AND TUMOUR LIKE CONDITIONS

33. CLASSIFICATION OF VASCULAR TUMOURS AND TUMOUR LIKE CONDITIONS

34. CLASSIFICATION OF VASCULAR TUMOURS AND TUMOUR LIKE CONDITIONS

35. VASCULAR TUMOURS • CAPILLARY HEMANGIOMA : SITES : SKIN, SUBCUTANEOUS TISSUE, LIP, ORAL MUCUS MEMBRANE, LIVER, SPLEEN KIDNEY. GROSS : FEW MM TO FEW CMS. RED OR BLUE SWELLING MICRO : AGGREGATES OF THIN WALLED CAPILLARIES LINED BY SINGLE LAYER OF ENDOTHELIAL CELLS SURROUNDED BY SCANT FIBROUS TISSUE STROMA.

36. VASCULAR TUMOURS (CONTD) • CAVERNOUS HEMANGIOMA SITES : BRAIN, LIVER, SUBCUTANEOUS TISSUE, PANCREAS ETC. GROSS : 1 CM TO TWO CM, SOFT BLUISH SWELLING MICRO : SHARPLY DEFINED BUT NOT ENCAPSULATED. LARGE DILATED BLOOD FILLED VASCULAR SPACES SURROUNDED BY FIBROUS TISUE STROMA.

37. PROGRESSIVE AND MICROSCOPIC STAGES OF KAPOSI SARCOMAPATCH PLAQUE NODULAR

38. KAPOSI SARCOMA

40. PROGRESSIVE AND MICROSCOPIC STAGES OF KAPOSI SARCOMA • PATCH STAGE : Irregular dilated and angulated Resembles blood vessels lined by single granulation layer of endothelial cells tissue surrounded by infiltrate of lymphocytes plasma cells and macrophages.

41. PROGRESSIVE AND MICROSCOPIC STAGES OF KAPOSI SARCOMA • PLAQUE STAGE : Dermal dilated and jagged vascular channels lined by plump spindle cells surrounded by perivascular aggregates of similar cells Similar infiltrate as in Stage 1 is seen between the vascular channels. Pink globules can be seen in the macrophages and spindle cells.

42. PROGRESSIVE AND MICROSCOPIC STAGES OF KAPOSI SARCOMA • NODULAR STAGE : Bundles of spindle cells , slit like and small vascular spaces , containing rows of red cells and hylaine droplets . Inflammatory infiltrate + Mitotic figures +

43. FORMS OF KAPOSI SARCOMA • 1. CHRONIC , CLASSIC OR EUROPEAN • 2. LYMPHOADENOPATHIC , ENDEMIC OR AFRICAN • 3. TRANSPLANT ASSOCIATED • 4. AIDS ASSOCIATED OR EPIDEMIC

44. ANGIOSARCOMA • SITES : LIVER, SKIN, BREAST, SOFT TISSUE GROSS : LARGE RED OR GREY FLESHY MASS HAVING ILLDEFINED MARGINS MICRO : IRREGULAR VASCULAR CHANNELS LINED BY ENDOTHELIAL CELLS EXHIBITING VARYING DEGREES OF ANAPLASIA AND ATYPIA

45. ANGIOSARCOMA

46. ANEURYSMS • Permanent abnormal dilatation of blood vessels • Due to congenital or aquired weakening or distruction of the vessel wall • CLASSIFICATION 1.Depending upon the composition of the wall. True False

47. 2.Depending upon the shape Saccular Fusiform Cylindrical Surpentine Racemose

48. Based on the pathogenetic mechanisms • Atheroslerotic • Syphilitic • Dissecting aneurysms • Mycotic aneurysms • Berry aneurysms