vasculitis Patomorfologia vasculitis (arteritis) caused by infections vasculitis (arteritis) caused by infections

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1 Patomorfologia Lecture 12 prof hab. n. med. Andrzej Marszałek VASCULITIS infectious vasculitis non-infectious causes: Aspergillus Rickettsiae vasculitis (arteritis) caused by infections Mucormycosis syphylis bacteriemia infectious emboli ("Roth's spots" eye fundic) in a course of e.g. pneumonia wall of abscess vasculitis (arteritis) caused by infections complications: thrombosis infarction aneurysma (è aneurysma mycotica) vasculitis (arteritis) caused by infections aneurysma mycotica/mycotic aneurysm usually without fungal infection usually caused by implantation of infectious material (embolus) è infection è wall destruction è aneurysm 1

2 immunology in pathology infectious vasculitis non-infectious disease with immunologica mechanism of tissue injury hypersensitivity reactions: type I type II type III type IV non-infectious vasculitis large vessels giant cell / temporal arteritis Takayasu arteritis medium-sized vessels (panca uj) small vessels Wegener's Granulomatosis (canca+) microscopic polyangiitis (panca+) Churg-Strauss (eozynofile, zwykle panca +) Henoch-Schonlein cryoglobulinemia leukocytoclastic vasculitis temporal arteritis (giant cell a.) TEMPORAL ARTERITIS ü rare disease ü usually after 50yrs (most common arteritis) ü cause? ü type IV hypersensitivity to Ag in a. wall ü role of T cells? ü IL-1 & IL-6 ü more common in patients with HLA-DR4 ü some familial cases 2

3 temporal arteritis (giant cell a.) involved vessels: ü large (and medium) size mainly in head area: ü temporal a. ü vertebral a. ü occular a. ü aorta temporal arteritis (giant cell a.) clinic: ü symptoms after age of 50 ü systemic ü local: ü headache ü facial pain ü intermittent mastication ü disturbances (up to loss) of vision ü 50% patients: pain + shoulder and hip stifness (polymyalgia reumatica) temporal arteritis (giant cell a.) : ü granuloma formation ü numerous giant cells ü injury of lamina elastica interna è fibrosis ü In acute phase: thrombosis TAKAYASU ARTERITIS Takayasu arteritis (aortic arch syndrome, occlusive thromboarthropathy; pulse-less disease) ü rare disease ü usually young adults ü cause? ü Takayasu disease & temporal a. one disease??? ü before 50 yrs (90% below age of 30) ü F:M = 9:1 ü Increased risk in patients with HLA-DR4 Takayasu arteritis (aortic arch syndrome, occlusive thromboarthropathy; pulse-less disease) ü systemic symptoms ü symptoms related to involved vessels: ü aortic arch + branches ü also: ü pulmonary aa. (50%) ü kidney aa. ü coronary aa. 3

4 Takayasu arteritis (aortic arch syndrome, occlusive thromboarthropathy; pulse-less disease) Morphology: ü 30% only involvement of aortic arch ü 10% whole aorta involvement ü inflammatory infiltrates in media and adventitia (mainly arroud vasa vasorum): ü neutrophils ü lymphocytes ü plasma cells ü giant cell granuloma (rarely) ü fibrosis of whole arterial wall POLYARTERITIS NODOSA ü infalammation (systemic) of muscular type vessels of medium and small diameter ü most common involves aa. of kidney and viscera ü NO LUNG aa. involvement ü most common involved vessels of: kidney, heart, liver, GI, pancreas, testis, skeletal muscles, CNS, skin Etiology ü type III hypersensitivity (immune-complexes) ü 30-40% patients HBV+ ü HCV??? ü bacteries??? ü rare (5% of vasculitis) ü ANA+ (??) ü p-anca+ (??) clinic: ü young adults (arround 40 yrs) ü F:M = 1:1 ü acute/sub-acute/chronic, è relapses ü symptoms ü malaise, ü fever, ü loss of body mass, ü disseminated vascular lesions (èmelena) ü most common cause of death: è kidney insufficiency (without symptoms of GN!) 4

5 ü regional vesculitis (medium and small aa.) + necrosis ü Inflammation within whole vessel wall ü infiltration first in peripheral part (often in place of division) ü histology: ü acute phase: fibrinoid necrosis, neutrophils, mononuclear cells ü chronic phase: fibrosis (including adventitia) ü consequences: ü abnormal perfussion leading to ulcers, infarction, atrophy from ischemia or hemorrhagies ü inflammatory process causes artery wall weakness è dilatation (aneurysma) or rupture ü vascular lumen could be closed by thrombosis ü characteristically all stages of activity could be found paralelly in same vessels or different vesells! KAWASAKI DISEASE ü acute febrile disease ü self-limitting ü immulogic background ü majority of patients at the age of 2-5yrs (80% before 4 yrs) diagnosis: fever > 5 dni + 4 symptoms from below: edema/conjunctivitis (bilateral) inflammatory changes on mucous membranes (pharynx, tongue, lips) lesion on legs (edema, redness, peeling on palms and soles) skin rush lymphadenopathy cervical (at leas 1 of minimal diam. of 1,5 cm) 5

6 symptoms: artritis (7-25%) small & large sterille uretritis CNS involvement sterille memingitis nerve paralysis defness symptoms: GI involvement: abdominal pain, diarrhorea, nausea, hepatitis heart involvement coronary aa. aneurysm myocardial disfunction infarctions : like polyaretritis nodosa intense inflammatory infiltrate (full wall thickness) sometimes present fibronid necrosis GRANULOMATOIS WITH POLYANGITIS granulomatosis with polyangiitis (Wegener granulomatosis) rare disease, often involves multiple organs, most commonly (90% cases): respiratory system, vasculitis GN sometimes skin lesions granulomatosis with polyangiitis (Wegener granulomatosis) depends on site of involvement (diveristy) oral lesions (about 5%) dark-cherry gum hypertrophy in other localizations might lead to ulcers with abundant necrosis 6

7 granulomatosis with polyangiitis (Wegener granulomatosis) lesions granuloma-like histiocytes + multinucleated giant cells neutrophils hyperplastic changes in epithelium in vascular wall leukocytoclastic changes (mainly in arterioles) more common in kidney then in lungs granulomatosis with polyangiitis (Wegener granulomatosis) in vasculitis is characteristic finely granular basophylic geographic necrosis surrounded by rim of histiocytes + collagenolytic necrosis; vascular lessions found in all stages of disease depending on stage vascular wall fibrinoid necrosis, after healing fibrosis (increased wall thickness, narrowing) 85% patients have c-anca + HENOCH-SCHONLEIN PURPURA Henoch-Schonlein purpura most common vasculitis in children immune background IgA immune complex deposites often selflimitting rare in Summer in about 50% infection by Streptococcus viruses? Henoch-Schonlein purpura symptoms GI edema, hemorrhagies in mucosa kidney (50% cases) from hematuria to end-stage insufficiency (below 1%) 7

8 BUERGER S DISEASE Buerger s disease (thromabangitis obliterans) focal medium size and small vasculitis rare in UE, USA, often in Israel, Japan and India young males (20-30yrs) smokers!!!! Buerger s disease (thromabangitis obliterans) changes regional arterial changes + neighbouring vains and nerves in acute phase: edema + neutrophils infiltration in vascularnerve bundle thrombosis + microabscesses in chronic phase: fibrosis vessel occlusion Buerger s disease (thrombangitis obliterans) clinic symptoms related to ischemia remittent claudication calve muscle pain (after exercise, then lowers) disases progress è rest pain è atrophic skin changes è dry gangrene 8