Vasculitis PDF

Vasculitis Dr. Leyla Cinel  Vasculitis is a general term for vessel wall inflammation with variable manifestations depending on the vascular bed affected. The lumen of the involved vessel is usually narrowed and may lead to ischemia  Vasculitis can result from infections but more commonly...

Vasculitis Dr. Leyla Cinel  Vasculitis is a general term for vessel wall inflammation with variable manifestations depending on the vascular bed affected. The lumen of the involved vessel is usually narrowed and may lead to ischemia  Vasculitis can result from infections but more commonly has an immunologic basis.  Physical and chemical injury, (including that due to radiation, mechanical trauma, and toxins), also can cause vasculitis. Classification according to vessel size, role of immune complexes, presence of specific autoantibodies, granuloma formation, tissue tropism.  Infectious Vasculitis Infectious agents usually are bacteria or fungi, (Aspergillus and Mucor spp.) How are infections vasculitis diagnosed?  PAS  GMS (Gomori Metenamin silver)  Noninfectious Vasculitis  The main immunologic mechanisms are  Immune complex deposition  Antineutrophil cytoplasmic antibodies(ANCA)  Anti-endothelial cell antibodies  Autoreactive T cells  Immune Complex–Associated Vasculitis  This form of vasculitis is seen in immunologic disorders such as systemic lupus erythematosus that are associated with autoantibody production.  There is an immune comlex deposition in the vessel wall  In most of the cases, antigen is not known  Drug hypersensitivity vasculitis.  Secondary to viral infections. (Ab to microbial constituents can form immune complexes that circulate and deposit in vascular lesions.)  In up to 30% of patients with polyarteritis nodosa, the vasculitis is attributable to immune complexes composed of hepatitis B surface antigen (HBsAg) and anti-HBsAg ab.  Anti-Neutrophil Cytoplasmic Antibodies  ANCAs are autoantibodies directed against constituents (mainly enzymes) of neutrophil primary granules, monocyte lysosomes, and endothelial cells.  ANCAs are very useful diagnostic markers Two of ANCAs are most important:  Antiproteinase-3 (PR3-ANCA)/c-ANCA are associated with Wegener granulomatosis  Anti-myeloperoxidase (MPO-ANCA)/p-ANCA are associated with microscopic polyangiitis and Churg-Strauss syndrome.  The ANCA autoab don't form circulating immune complexes!.  The vascular lesions don't contain ab and C; therefore ANCA-associated vasculitides are often described as “pauci-immune.”  ANCAs can directly activate neutrophils, stimulating the release of ROS and proteolytic enzymes; in vascular beds, this may lead to endothelial cell injury.  Anti-Endothelial Cell Antibodies Kawasaki disease  Acute, febrile, usually self-limited Which one of the illness of infancy and fallowing sentences are correct about childhood KAWASAKI disease ? It is seen in adults and  Large to medium- usually persents with sized vessels. fever Usually effect coronary arteries and cause aneurysm and  Coronary arteritis rapture can cause aneurysms and rupture or thrombose In genetically susceptible persons, mostly viral infectious agents trigger the disease.  Cytokine production and polyclonal B cell activation result in autoantibodies to endothelial cells and smooth muscle cells. In kawasaki disease the produced antibodies act as oxidizariin agents od LDL producing atherosclerosis. False What are the diagnostic criteria of kawasaki disease  Conjunctival and oral erythema  erythema of the palms and soles, a desquamative rash, and  cervical lymph node enlargement (mucocutaneous lymph node syndrome).  There is a dense transmural inflammatory infiltrate  In giant cell arteritis you will have eldery patients in contract in Kawasaki disease you will Giant Cell (Temporal) Arteritis face infents  The most common form of vasculitis among the elderly.  Chronic, typically granulomatous, inflammation of large to medium size arteries, especially the temporal arteries.  Kawasaki disease we have ctokine and colonical b antibodies outo AB against sm and  T cell–mediated endothelial cells while in giant cell arteritis we immune response to have T Cell mediated response an uncharacterized vessel wall antigen.  Nodular intimal thickening that reduce the lumen diameter and cause Kawasaki diseasein giant cell arteritis we will see distal ischemia. lumon narrwoing in intimal layer due to nodual but granulomatous  Classic lesions inflammation are seen with in tunica media exhibit granulomatous inflammation within the inner tunica media.  Which one the below is wrong about giant tcell arteritis It is mediated by T cells We can see lumon narrowing We can see granulomatus in inner tunica media We can see multiuclated  There is an infiltrate of giant cells and internal leastic lamina fragmentation lymphocytes and Diagnosis is perimerly done by biobsy macrophages, with multinucleate giant cells, and fragmentation of the internal elastic lamina  Patients are older than 50 years  Diagnosis depends on biopsy and histology  Because involvement in temporal arteritis is patchy, a negative biopsy result doesn't exclude the diagnosis!.  Takayasu Arteritis (pulseless disease) - Granulomatous vasculitis of medium-sized and larger arteries – Ocular disturbances and marked weakening of the pulses in the upper extremities – Younger than 50 years Aortic arch angiogram  Where do we see takayas arteritis Transmural scarring and thickening of the aorta (particularly the aortic arch and great vessels) with luminal narrowing of the major branch vessels  In early stage, granulomatous inflamation, zonal medial necrosis  In late stage, transmural fibrous In giant cell arteitis we see thickening zonal medial necrosis.false  Narrowing of the coronary ostia can lead to myocardial infarction, and  Involvement of the renal arteries causes systemic hypertension in roughly half of the patients  Patient visits ER with haptic b what is the Polyarteritis nodosa (PAN) probable vasculitis condition fallowing it Immuncomplex mediated systemic vasculitis of small or medium-sized What is the underlying muscular arteries that cause of polyarteritis typically involves the renal nodosa ? and visceral vessels Capillaries, veins and venules are not involved. A third of the patients Polar artitis nodosa is and immune epic have chronic hepatitis B system. Flase infection The cause is unknown in the remaining cases.  Segmental, transmural, necrotizing inflammation, often with superimposed thrombosis. Kidney, heart, liver, and gastrointestinal tract vessels are affected in descending order of frequency.  Fibrinoid necrosis and luminal thrombosis (also What are the common findings occur Wegener and between polyarterits Churg-Strauss) nodas and wenger chrug stratus  PAN is primarily a disease of young adults. A “classic” presentation can involve some combination of rapidly accelerating hypertension (due to renal artery involvement); abdominal pain and bloody stools (caused by vascular gastrointestinal lesions); diffuse muscular pains; and peripheral neuritis, (predominantly affecting motor nerves).  Microscopic Polyangiitis (hypersensitivity vasculitis or leukocytoclastic vasculitis) Necrotizing vasculitis that generally affects capillaries, as well as small arterioles and venules. In microscopic pilyangitis we comonly see granloumarus Unlike in PAN, all lesions inflammation. False it is esoniphilic of microscopic polyangiitis tend to be of the same age in any given patient. Most patients have p- ANCA (+)  Microscopic Polyangiitis What are the common persentations of The skin, mucous Microscopic polyangitis ? membranes, lungs, brain, heart, gastrointestinal tract, kidneys, and muscle all can be involved; necrotizing glomerulonephritis (seen in 90% of patients) and pulmonary capillaritis are particularly common.  Granulomatous inflammation is absent! These lesions resemble those of PAN but spare medium-sized and larger arteries, so that macroscopic infarcts are uncommon. In some areas (typically postcapillary venules), infiltrating neutrophils that frequently undergo fragmentation are seen, giving rise to the term leukocytoclastic vasculitis  Wegener Granulomatosis (Granulomatosis with polyangiitis) necrotising vasculitis, necrotising parenchymal lesions are characterized by a specific findings: Granulomas of the lung and/or the upper respiratory tract (ear, nose, sinuses, throat) Vasculitis of small to medium- sized vessels most prominently in the -lungs and - upper respiratory tract -Glomerulonephritis  Wegener Granulomatosis The typical patient is a 40-year old man “Limited” or “widespread”forms Clinically, this resembles PAN with the additional feature of respiratory involvement If untreated, the mortality rate at 1 year is 80%.  Wegener Granulomatosis Wegener granulomatosis is likely to be initiated as a cell- mediated hypersensitivity response directed against inhaled infectious or environmental antigens. PR3-ANCAs (c-ANCA) are present in almost 95% of cases Upper respiratory tract lesions: granulomatous sinusitis, ulcerative lesions of the nose, palate, or pharynx  Wegener Granulomatosis Lung findings; ranging from diffuse parenchymal infiltrates to granulomatous nodules with central cavitation  Wegener Granulomatosis Multifocal necrotizing granulomatous vasculitis with a surrounding fibroblastic In which one of ghw vascultitis do wee fibroblastic proliferation lesion  Wegener Granulomatosis The renal lesions range What is the unique from mild, focal biopsy finding fir wenger glomerular necrosis Granulomatosis (focal and segmental necrotizing glomerulonephritis) to more advanced glomerular lesions with forming epithelial crescents (crescentic glomerulonephritis)  Churg-Strauss syndrome (allergic granulomatosis and angiitis/eosinophilic granulomatosis with polyangiitis) A small vessel necrotizing vasculitis classically associated with *asthma, *allergic rhinitis, *lung infiltrates, *peripheral eosinophilia, *extravascular necrotizing granulomas, *a striking infiltration of vessels and perivascular tissues by eosinophils.  Churg-Strauss syndrome (allergic granulomatosis and angiitis) Cutaneous involvement (with palpable purpura), gastrointestinal bleeding, and renal disease (primarily as focal and segmental glomerulosclerosis) are the major associations. Cardiac involvement is seen in 60% of patients and is a major cause of morbidity and death.  The vascular lesions differ from those of polyarteritis nodosa or microscopic polyangiitis by the presence of granulomas and eosinophils.  Thromboangiitis obliterans (Buerger disease) Focal acute and chronic inflammation of medium and Tnromboangitis small arteries, especially the usually happe s in tibial and radial arteries, small arteries. False associated with thrombosis Frequently results in vascular insufficiency and gangrene of the extremities.  Thromboangiitis obliterans (Buerger disease) Buerger disease occurs almost exclusively in heavy tobacco smokers and usually develops before age 35. The etiology is unknown. Direct endothelial cell toxicity caused by some component of tobacco is suspected; alternatively, a reactive compound in tobacco may modify vessel wall components and induce an immune response.  In early stages, mixed inflammatory infiltrates are accompanied by luminal thrombosis; small microabscesses, The inflammation often The inflammatory extends into contiguous veins and nerves (a feature that is rare in other forms of vasculitis). With time, thrombi can organize and recanalize, and eventually the artery become encased in fibrous tissue  Vasculitis Associated with Other Noninfectious Disorders Vasculitis resembling hypersensitivity vasculitis or classic PAN can be associated with many other diseases, including malignancies and immunologic disorders such as rheumatoid arthritis, systemic lupus erythematosus, antiphospholipid antibody syndrome, and Henoch- Schönlein purpura.