Vasculitis  Dr Basavaraj P Bommanahalli Assistant Professor Dept of Pathology  SSIMS RC Davanagere Karnataka
<ul><li>Def: inflammation of wall of the vessels  </li></ul><ul><li>c/f constitutional signs & symptoms </li></ul><ul><li>...
<ul><li>Pathogenesis  </li></ul><ul><li>Direct infection:  </li></ul><ul><ul><li>Bacterial </li></ul></ul><ul><ul><li>Rock...
<ul><li>Immunologic   </li></ul><ul><ul><li>Immune complex mediated  </li></ul></ul><ul><ul><li>Antineutrophil cytoplasmic...
Classification  <ul><li>Large vessel vasculitis   </li></ul><ul><ul><ul><li>Giant cell arteritis </li></ul></ul></ul><ul><...
<ul><li>Giant cell (temporal) arteritis   </li></ul><ul><li>Most common systemic arteritis  </li></ul><ul><li>Acute, chron...
<ul><li>Segmental involvement  </li></ul><ul><li>Nodular thickening  & narrow lumen  </li></ul><ul><li>Granulomatous infla...
<ul><li>C/F  </li></ul><ul><li>Always > 50 yrs  </li></ul><ul><li>Increased ESR </li></ul><ul><li>Pain & tenderness, nodul...
<ul><li>Takayasu arteritis  </li></ul><ul><li>Granulomatous inflammation  </li></ul><ul><li>Characterised by  </li></ul><u...
<ul><ul><ul><li>Arch of aorta & its branches  </li></ul></ul></ul><ul><ul><ul><li>Pulmonary artery  </li></ul></ul></ul><u...
<ul><li>C/F  </li></ul><ul><li>Lower BP & weak pulses in upper limbs  </li></ul><ul><li>Total blindness  </li></ul><ul><li...
<ul><li>Polyarteritis nodosa (PAN) </li></ul><ul><li>Small & medium sized muscular arteries  </li></ul><ul><li>Most common...
<ul><li>C/F  </li></ul><ul><li>Fever </li></ul><ul><li>Myalgia  </li></ul><ul><li>Weight loss  </li></ul><ul><li>HTN </li>...
<ul><li>Kawasaki disease   </li></ul><ul><li>Young children (<4yrs) & infants  </li></ul><ul><li>Coronary arteries are inv...
<ul><li>Coronary vasculitis </li></ul><ul><li>Coronary aneurysms  </li></ul><ul><li>Rupture / thrombosis  </li></ul><ul><l...
<ul><li>Wegener granulomatosis   </li></ul><ul><li>Classic type  </li></ul><ul><li>Triad  </li></ul><ul><ul><li>Acute necr...
<ul><li>Pathogenesis  </li></ul><ul><ul><li>Immune complex mediated  </li></ul></ul><ul><ul><li>Cell mediated  </li></ul><...
<ul><li>Thromboangitis obliterans /  Buerger disease  </li></ul><ul><li>Characterised by  </li></ul><ul><ul><li>Segmental ...
<ul><li>Begins < 35yrs </li></ul><ul><li>Strongly associated with smoking  </li></ul><ul><ul><li>Hypersensitivity to intra...
<ul><li>Segmental acute & chronic vasculitis  </li></ul><ul><li>Lower & upper extremities  </li></ul><ul><li>Thrombosis – ...
<ul><li>Raynaud phenomenon  : </li></ul><ul><li>Paroxysmal pallor, cyanosis of digits of hands or feet and tips of nose & ...
<ul><li>Primary   </li></ul><ul><li>Exaggeration of normal central and local vasomotor response to cold & emotion  </li></...
<ul><li>Tumors of vessels   </li></ul><ul><li>Benign neoplasms/ developmental /acquired conditions   </li></ul><ul><li>Hem...
<ul><li>Kaposi sarcoma   </li></ul><ul><li>Low grade malignancy </li></ul><ul><li>Locally aggressive  </li></ul><ul><li>Va...
<ul><li>Classic/ European type </li></ul><ul><li>Eastern Europe  </li></ul><ul><li>Not associated with HIV </li></ul><ul><...
<ul><li>Lymphadenopathic / African/ endemic  </li></ul><ul><li>Bantu families  </li></ul><ul><li>Localised / generalised l...
<ul><li>Immunosuppression associated </li></ul><ul><li>Transplant associated  </li></ul><ul><li>LN + mucosa + visceral org...
<ul><li>AIDS associated   </li></ul><ul><li>Seen in 1/3 rd of AIDS  pts  </li></ul><ul><li>Male homosexuals  </li></ul><ul...
<ul><li>Gross   </li></ul><ul><li>Patch  </li></ul><ul><li>Plaque  </li></ul><ul><li>Nodular lesions  </li></ul><ul><li>Mi...
<ul><li>KS associated Herpes virus (KSHV) / HHV-8 </li></ul><ul><ul><li>Inhibits apoptosis  </li></ul></ul><ul><ul><li>Inc...
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types of vasculitis

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  1. 1. Vasculitis Dr Basavaraj P Bommanahalli Assistant Professor Dept of Pathology SSIMS RC Davanagere Karnataka
  2. 2. <ul><li>Def: inflammation of wall of the vessels </li></ul><ul><li>c/f constitutional signs & symptoms </li></ul><ul><li>fever, myalgia, arthralgia </li></ul><ul><li>signs of ischemia </li></ul>
  3. 3. <ul><li>Pathogenesis </li></ul><ul><li>Direct infection: </li></ul><ul><ul><li>Bacterial </li></ul></ul><ul><ul><li>Rocky Mountain Spotted fever </li></ul></ul><ul><ul><li>Syphilis </li></ul></ul><ul><ul><li>Fungal : aspergillosis, mucormycosis </li></ul></ul><ul><ul><li>Viral: herpes zoster- varicella </li></ul></ul>
  4. 4. <ul><li>Immunologic </li></ul><ul><ul><li>Immune complex mediated </li></ul></ul><ul><ul><li>Antineutrophil cytoplasmic antibody (ANCA) mediated </li></ul></ul><ul><ul><li>Cell mediated </li></ul></ul><ul><ul><li>Direct antibody mediated </li></ul></ul><ul><li>Idiopathic </li></ul>
  5. 5. Classification <ul><li>Large vessel vasculitis </li></ul><ul><ul><ul><li>Giant cell arteritis </li></ul></ul></ul><ul><ul><ul><li>Takayasu arteritis </li></ul></ul></ul><ul><li>Medium sized vessel vasculitis </li></ul><ul><ul><ul><li>Polyarteritis nodosa </li></ul></ul></ul><ul><ul><ul><li>Kawasaki disease </li></ul></ul></ul><ul><li>Small vessel vasculitis </li></ul><ul><ul><ul><li>Wegener granulomatosis </li></ul></ul></ul><ul><ul><ul><li>Churg- stauss syndrome </li></ul></ul></ul><ul><ul><ul><li>Microscopic polyangitis </li></ul></ul></ul>
  6. 6. <ul><li>Giant cell (temporal) arteritis </li></ul><ul><li>Most common systemic arteritis </li></ul><ul><li>Acute, chronic granulomatous lesion </li></ul><ul><li>Large arteries </li></ul><ul><ul><ul><li>Aorta & its branches </li></ul></ul></ul><ul><ul><ul><li>Temporal artery </li></ul></ul></ul><ul><ul><ul><li>Vertebral artery </li></ul></ul></ul><ul><ul><ul><li>Ophthalmic artery </li></ul></ul></ul>
  7. 7. <ul><li>Segmental involvement </li></ul><ul><li>Nodular thickening & narrow lumen </li></ul><ul><li>Granulomatous inflammation </li></ul><ul><ul><ul><li>Giant cells (foreign body & Langhans) </li></ul></ul></ul><ul><ul><ul><li>Lymphocytes </li></ul></ul></ul><ul><li>Fragmentation of internal elastic lamina </li></ul><ul><li>Later fibrosis </li></ul>
  8. 8. <ul><li>C/F </li></ul><ul><li>Always > 50 yrs </li></ul><ul><li>Increased ESR </li></ul><ul><li>Pain & tenderness, nodular temporal A </li></ul><ul><li>Black outs, permanent visual loss </li></ul><ul><li>Biopsy is confirmatory </li></ul><ul><li>Anti-inflammatory drugs are effective </li></ul>
  9. 9. <ul><li>Takayasu arteritis </li></ul><ul><li>Granulomatous inflammation </li></ul><ul><li>Characterised by </li></ul><ul><ul><li>Ocular disturbances </li></ul></ul><ul><ul><li>Weakening of pulses in the upper extremities </li></ul></ul><ul><ul><li>pulseless disease </li></ul></ul><ul><ul><li>Fibrous thickening of arch of aorta & branches </li></ul></ul><ul><li>Associated with HLA A24-B52-DR2 </li></ul>
  10. 10. <ul><ul><ul><li>Arch of aorta & its branches </li></ul></ul></ul><ul><ul><ul><li>Pulmonary artery </li></ul></ul></ul><ul><ul><ul><li>Coronary & renal arteries </li></ul></ul></ul><ul><li>Morphology similar to giant cell arteritis with necrosis </li></ul><ul><li>Younger age group </li></ul>
  11. 11. <ul><li>C/F </li></ul><ul><li>Lower BP & weak pulses in upper limbs </li></ul><ul><li>Total blindness </li></ul><ul><li>HTN </li></ul><ul><li>Claudication </li></ul>
  12. 12. <ul><li>Polyarteritis nodosa (PAN) </li></ul><ul><li>Small & medium sized muscular arteries </li></ul><ul><li>Most commonly visceral & renal arteries involved </li></ul><ul><li>Arterioles, capillaries & venules are not involved </li></ul><ul><li>Segmental transmural necrotising inflammation </li></ul><ul><ul><li>Neutrophils </li></ul></ul><ul><ul><li>Eosinophils </li></ul></ul><ul><ul><li>Mononuclear cells </li></ul></ul><ul><ul><li>Fibrinoid necrosis </li></ul></ul>
  13. 13. <ul><li>C/F </li></ul><ul><li>Fever </li></ul><ul><li>Myalgia </li></ul><ul><li>Weight loss </li></ul><ul><li>HTN </li></ul><ul><li>30% are associated with hepatitis B </li></ul><ul><li>Renal involvement is seen </li></ul><ul><li>No glomerulonephritis </li></ul>
  14. 14. <ul><li>Kawasaki disease </li></ul><ul><li>Young children (<4yrs) & infants </li></ul><ul><li>Coronary arteries are involved </li></ul><ul><li>Associated with mucocutaneous lymph node syndrome </li></ul><ul><ul><li>Fever </li></ul></ul><ul><ul><li>Conjunctival & oral erythema </li></ul></ul><ul><ul><li>Skin rashes </li></ul></ul><ul><ul><li>Cervical lymphadenopathy </li></ul></ul><ul><li>PAN like inflammation </li></ul>
  15. 15. <ul><li>Coronary vasculitis </li></ul><ul><li>Coronary aneurysms </li></ul><ul><li>Rupture / thrombosis </li></ul><ul><li>MI, sudden death </li></ul>
  16. 16. <ul><li>Wegener granulomatosis </li></ul><ul><li>Classic type </li></ul><ul><li>Triad </li></ul><ul><ul><li>Acute necrotising granulomas of upper respiratory tract </li></ul></ul><ul><ul><li>Granulomatous vasculitis of lung & upper respiratory tract </li></ul></ul><ul><ul><li>Focal necrotising crescentic glomerulonephritis </li></ul></ul><ul><li>Limited type : restricted to respiratory tract </li></ul>
  17. 17. <ul><li>Pathogenesis </li></ul><ul><ul><li>Immune complex mediated </li></ul></ul><ul><ul><li>Cell mediated </li></ul></ul><ul><li>C/F </li></ul><ul><ul><li>M >F </li></ul></ul><ul><ul><li>4 th – 5 th decade </li></ul></ul><ul><ul><li>c-ANCA + in 95 % of cases </li></ul></ul>
  18. 18. <ul><li>Thromboangitis obliterans / Buerger disease </li></ul><ul><li>Characterised by </li></ul><ul><ul><li>Segmental thrombosing acute & chronic inflammation of medium sized & small arteries : tibial/ radial a </li></ul></ul><ul><ul><li>Extending to veins & nerves of extremities </li></ul></ul>
  19. 19. <ul><li>Begins < 35yrs </li></ul><ul><li>Strongly associated with smoking </li></ul><ul><ul><li>Hypersensitivity to intradermally tobacco extract </li></ul></ul><ul><li>Associated with HLA-A9, HLA-B5 </li></ul><ul><li>Prevalent in Israel, Japan, India than US & Europe </li></ul>
  20. 20. <ul><li>Segmental acute & chronic vasculitis </li></ul><ul><li>Lower & upper extremities </li></ul><ul><li>Thrombosis – recanalisation </li></ul><ul><li>Contiguous involvement of veins & nerves </li></ul><ul><li>C/F </li></ul><ul><li>Gangrene </li></ul><ul><li>Chronic ulcerations </li></ul><ul><li>Severe pain: resting pain : nerve involvement </li></ul>
  21. 21. <ul><li>Raynaud phenomenon : </li></ul><ul><li>Paroxysmal pallor, cyanosis of digits of hands or feet and tips of nose & ear </li></ul><ul><li>Due to cold induced vasoconstriction precapillary arterioles & digital arteries </li></ul><ul><li>Long standing cases associated with atrophy of skin, subcutaneous tissue & muscle </li></ul>
  22. 22. <ul><li>Primary </li></ul><ul><li>Exaggeration of normal central and local vasomotor response to cold & emotion </li></ul><ul><li>3-5% of population </li></ul><ul><li>Median age-14yrs </li></ul><ul><li>No arterial pathology </li></ul><ul><li>Secondary </li></ul><ul><li>Associated with SLE, systemic sclerosis, atherosclerosis, Buerger disease </li></ul><ul><li>> 30 yrs </li></ul><ul><li>+ vascular pathology </li></ul>
  23. 23. <ul><li>Tumors of vessels </li></ul><ul><li>Benign neoplasms/ developmental /acquired conditions </li></ul><ul><li>Hemangioma </li></ul><ul><ul><li>Capillary </li></ul></ul><ul><ul><li>Cavernous </li></ul></ul><ul><ul><li>Pyogenic </li></ul></ul><ul><li>Lymphangioma </li></ul><ul><li>Vascular ectasia </li></ul><ul><li>Bacillary angiomatosis </li></ul><ul><li>Intermediate grade neoplasms </li></ul><ul><li>Kaposi sarcoma </li></ul><ul><li>Hemangioendothelioma </li></ul><ul><li>Malignant neoplasm </li></ul><ul><li>Angiosarcoma </li></ul><ul><li>Hemangiopericytoma </li></ul>
  24. 24. <ul><li>Kaposi sarcoma </li></ul><ul><li>Low grade malignancy </li></ul><ul><li>Locally aggressive </li></ul><ul><li>Variants </li></ul><ul><ul><li>Classic/ European type </li></ul></ul><ul><ul><li>Lymphadenopathic / African/ endemic </li></ul></ul><ul><ul><li>Immunosuppression associated </li></ul></ul><ul><ul><li>AIDS associated type </li></ul></ul>
  25. 25. <ul><li>Classic/ European type </li></ul><ul><li>Eastern Europe </li></ul><ul><li>Not associated with HIV </li></ul><ul><li>But homosexuals are at risk </li></ul><ul><li>Localised to skin </li></ul>
  26. 26. <ul><li>Lymphadenopathic / African/ endemic </li></ul><ul><li>Bantu families </li></ul><ul><li>Localised / generalised lymphadenopathy </li></ul><ul><li>Sparse skin lesions </li></ul><ul><li>Seen in both HIV +/ - </li></ul><ul><li>Extremely aggressive </li></ul>
  27. 27. <ul><li>Immunosuppression associated </li></ul><ul><li>Transplant associated </li></ul><ul><li>LN + mucosa + visceral organs </li></ul><ul><li>Skin lesions are absent </li></ul>
  28. 28. <ul><li>AIDS associated </li></ul><ul><li>Seen in 1/3 rd of AIDS pts </li></ul><ul><li>Male homosexuals </li></ul><ul><li>LN + mucosa + skin + viscera </li></ul><ul><li>Early widespread dissemination </li></ul>
  29. 29. <ul><li>Gross </li></ul><ul><li>Patch </li></ul><ul><li>Plaque </li></ul><ul><li>Nodular lesions </li></ul><ul><li>Microscopy </li></ul><ul><li>Dilated irregular, angulated blood vessels lined by endothelial cells </li></ul><ul><li>Spindled cells containing pink hyaline globules </li></ul><ul><li>Hemosiderin laden macrophages </li></ul><ul><li>Mitotic figures </li></ul>
  30. 30. <ul><li>KS associated Herpes virus (KSHV) / HHV-8 </li></ul><ul><ul><li>Inhibits apoptosis </li></ul></ul><ul><ul><li>Increases production of p53 inhibitors </li></ul></ul><ul><li>Rx : excision + radiotherapy + chemotherapy </li></ul>
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