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VASCULAR PATHOLOGY

VASCULAR PATHOLOGY. By DR. OLA OMRAN ASSOCIATE PROFESSOR PATHOLOGY CONSULTANT PATHOLOGY DEPARTMENT. VASCULITIS. DEFINITION: It means “ inflammation of the walls of the vessels ” and can be associated to many different clinical conditions

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VASCULAR PATHOLOGY

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  1. VASCULAR PATHOLOGY By DR. OLA OMRAN ASSOCIATE PROFESSOR PATHOLOGY CONSULTANT PATHOLOGY DEPARTMENT

  2. VASCULITIS DEFINITION: Itmeans “inflammation of thewalls of thevessels” and can beassociatedtomanydifferentclinicalconditions 20 primary forms of vasculitis are recognized Classificationsaccording to vessel size, role of immune complexes, presence of specific autoantibodies, granuloma formation, organ tropism, and even population demographics. Several vasculitides tend to affect only vessels of particular caliber or tissue beds

  3. VASCULITIS The two most common pathogenic mechanisms of vasculitis are: immune-mediated inflammation direct invasion of vascular walls by infectious pathogens. Predictably, infections can also indirectly induce a noninfectious vasculitis, for example, by generating immune complexes or triggering cross-reactivity. Physical and chemical injury, such as from irradiation, mechanical trauma, and toxins, can also cause vasculitis.

  4. VASCULAR PATHOLOGY VASCULITIS . CLASSIFICATION OF VASCULITIS BASED ON THE PATHOGENESIS: • *DirectInfection. -Bacterial(Neisseria) -Rickettsial(spottedfever) -Spirochetal(Syphilis) -Fungal(aspergillosis) -Viral(herpes zoster)

  5. *Immunologic. A. Immunecomplex- mediated. -Infection-induced(Hepatitis B,C) -Henoch-Schonlein purpura -SLE, etc. -Drug-induced -Cryoglobulinemia(Ig, IgM) -Serumsickness B. ANCA-mediated. -Wegenergranulomatosis -Microscopicpolyangeitis -Churg-Strauss syndrome C. Direct Ab-induced -Goodpasturesyndrome(anti-GBM Abs) -Kawasaki disease(anti-endoth.Ab)

  6. VASCULAR PATHOLOGY D. Cell-mediated. -Organ-allograftrejection -Inflammatoryboweldisease -Paraneoplastic vasculitis • *Unknown. -Giant.cell arteritis -Takayasu arteritis -Polyarteritisnodosa

  7. Noninfectious Vasculitis (immune-mediated inflammation) Immune complex deposition 1.  Vasculitis associated with drug hypersensitivity (e.g., penicillin), antibodies directed against the drug-modified self proteins lead to the formation of immune complexes. Manifestations frequently involving the skin and can be mild and self-limiting or severe and even fatal. Discontinuation of the offending agent is often curative. 2. vasculitis associated with viral infections, antibody to viral proteins may form immune complexes detectable in the serum and in the vascular lesions; for example, as many as 30% of patients with polyarteritisnodosahave an underlying hepatitis B infection with vasculitis attributable to complexes of hepatitis B surface antigen (HBsAg) and antibodies to HBsAg.

  8. 2. Antineutrophil Cytoplasmic Antibodies (ANCA): Many patients with vasculitis have circulating antibodies that react with neutrophilcytoplasmic antigens, so-called ANCAs. ANCAs are a heterogeneous group of autoantibodies directed against constituents (mainly enzymes) of neutrophil primary granules, monocytelysosomes, and endothelial cells. Two general types of ANCAs based on immunofluorescence staining patterns: Cytoplasmic localization (c-ANCA),wherein the most common target antigen is p roteinase-3 (PR3), a neutrophil granule constituent Perinuclear localization (p-ANCA),wherein most of the autoantibodies are specific for myeloperoxidase (MPO). c-ANCA in Wegener granulomatosis& p-ANCA in microscopic polyangiitis and Churg-Strauss syndrome.  ANCAs is useful quantitative diagnostic markers for the ANCA-associated vasculitides, and their levels reflect the degree of inflammatory activity.  Although the mechanisms are unknown, ANCAs can directly activate neutrophils and stimulates neutrophils to release reactive oxygen species and proteolytic enzymes.

  9. 3. Anti-Endothelial Cell Antibodies Antibodies to ECs may predispose to certain vasculitides, for example Kawasaki disease. Another Classificationsaccording to vessel size:………

  10. 1. GIANT CELL (TEMPORAL) ARTERITIS. • MOST commonform of systemic vasculitis • Adults males over 50ys • acuteorchronic • largesizedvessels ( affects temporal arteries, ophtalmic(blindness) and aorta (aneurysm) • GP: tender, nodular thickening of theartery reduction of thelumenthrombosis. • MP: Granulomatousinflammation in innerhalf of media withgiantcells.

  11. GIANT CELL (TEMPORAL) ARTERITIS. Thickened, nodular, and tender arteries

  12. Temporal (giant cell) arteritis thickened, nodular, and tender segment Giant cells & granuloma at the degenerated internal elastic membrane in active arteritis H&E Elastic tissue stain demonstrating focal destruction of internal elastic membrane (arrow) and intimal thickening (IT) characteristic of long-standing or healed arteritis

  13. Giant cell arteritis Elastic tissue staindemonstrating: • focal destruction of internal elastic membrane (arrow) and • intimal thickening (IT) characteristic of long-standing or healed arteritis.

  14. GIANT CELL ARTERITIS...(cont.) CLINICAL FEATURES: • Oldpatientswithfever, fatigue, loss of weight, withor w/o facial pain and headache. • More severeform ….involvement of ophtalmicartery diplopiaorblindness of abruptonset.

  15. 2. TAKAYASU ARTERITIS. • Predominant in females • below 50´s • Propableautoimmunemechanism, • characterizedby ocular manifestations/weakness of pulses in upperextremities (reducedbrachial pulse), dueto vasculitis of AorticArch+ branches narrowing obliteration.

  16. TAKAYASU ARTERITIS...(cont.) • MICROSCOPIC: • Mononuclearinfiltration of adventecia. • Giant-cellgranulomatousinflammation NOT distinguishablefromgiant-cell arteritis. • CLINICAL: • Reducedbrachial pulse, difference in BP between R & L arm>10 mm Hg, coldness+ numbness of fingers,, • Visual defects  blindness.

  17. A, Aortic arch angiogram showing narrowing of the brachiocephalic, carotid, and subclavian arteries (arrows). B, Gross photograph of two cross-sections of the right carotid artery demonstrating marked intimal thickening with minimal residual lumen. C, Destruction and fibrosis of the arterial media and an infiltrate of mononuclear inflammation, including giant cells. Takayasu arteritis

  18. 3. POLYARTERITIS NODOSA (PAN). • Mediumsized arteritis affectingmultipleorgans (skin, peripheralnerves, gut, kidney and heart. • Childhoodlate adulthood (average 40 ys) • Associatedwith Hepatitis B, C orboth (mostcommon in injectiondrugabusers). • Probablymediatedbyimmunecomplexes (Igs + viral Ags)  circulating and deposited in inflammedvessels.

  19. 3. POLYARTERITIS NODOSA (PAN). ...(cont.) CLINICAL FEATURES: Onsetis gradual (wks mos.) • Non specific: malaise,fever, weightloss, abdominal pain, melena, myalgias, muscular weakness, diastolicpressure >90 mm Hg, • Specific: • Renal involvementhypertension • GI tract abdominal angina (hemorrhage, perforation). • heart: myocarditis/myocardialinfarction. • Eye: scleritis. • About 1/3 of patients  HBAb+.

  20. POLYARTERITIS NODOSA..(cont.) Diagnosis: • *Elevated BUN orcreatinine • *Proven hepatitis B/C virus infection • *Angiographicsigns of aneuryms/vascular occlusion (60% sensitive) • *MP: Demonstration of necrotizinginflammation (segmentalfibrinoid necrosis) & granulocytes in mediumsizedvessels (biopsy).

  21. Polyarteritisnodosa • There is segmental fibrinoid necrosis and thrombotic occlusion of the lumen of this small artery. • Note that part of the vessel wall at the upper right (arrow) is uninvolved

  22. 4. WEGENER´S GRANULOMATOSIS. • Granulomatousinflammation & necrotizing vasculitis • Averageageisabout 40 yrs. • Affectsupperrespiratorytract, lowerrespiratorytract and kidneys. • Otherorgansaffected: • Eye (proptosis/diplopia), • Skin (ulcers, purpura) • Itmayremainlocalizedtoonesiteforyrs./mos.  systemiclater (fever, fatigue, loss of weight, anemia, leukocytosis andincreasedESR

  23. 4. WEGENER´S GRANULOMATOSIS….CONT CLINICAL FEATURES: • *Chronic sinusitis(90%) • *Chronicpneumonitis in about 95% of patients. • *Mucosalulcerations of nasopharynx (75%) • *Renal disease (80%)

  24. WEGENER´S GRANULOMATOSIS

  25. Wegener granulomatosisinflammation of a small artery along with adjacent granulomatous inflammation, in which epithelioid cells and giant cells (arrows)

  26. 5. BUERGER´S DISEASE (Thromboangiitis obliterans) • Characterizedbysegmentalthrombosis + acute / chronicinflammation of medium and smallarteries MOSTLY tibial and radial arteries, and secondarilyinvolvement of veins and nerves of limbs. • Apparently heavy cigarette-smokers are MOST frequentlyaffected (endoth. cellshypersensitivity?) • Moreover increasedprevalence of HLA-A9/ HLA-B5 in thesepatients and in Israel, Japan, India (genetictrait?)

  27. Buerger diseaseThe lumen is occluded by a thrombus containing two abscesses (arrows). The vessel wall is infiltrated with leukocytes.

  28. BUERGER´S DISEASE..(cont.) Microscopically: • acute + chronicinflammation in arterial walls + thrombosisorganization/recanalization. Also, thrombosiscontainsmicroabscesses + granulomatousinflammation extensiontoveins/nerves. • Late complication: chroniculceration of toes orfingers  gangrene.

  29. BUERGER´S DISEASE...(cont.) CLINICAL FEATURES: • Claudication in feet and/orhandssometimescentrallyradiated. • Numbness and tingling in limbs + Raynaud´sphenomenon. • Skinulcerations + gangrene of digits

  30. TUMORS OF BLOOD VESSELSCavernoushemangioma • Large blood filled vascular spaces • Separated by mild to moderate amount of CT stroma • Intravascular thrombosis may be seen.

  31. Kaposi sarcoma Coalescent red-purple macules and plaques of the skin sheets of plump, proliferating spindle cells

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