Create
Learn
Share

Vasculitides

rename
darodri6's version from 2016-09-05 05:10

Section

Vasculitis TypeMicroscopic DescriptionVessels InvolvedClinical HxClinical PresentationTreatment
Giant-cell (temporal) arteritisGranulomatous inflammation of media; segmental nodular thickening, reducing luminal diameter; internal elastic fragmentationBranches of the carotidElderly females (> 40), +/-polymyalgiaClinically: Patients >50, 65% Female, may be associated with polymyalgia rheumatic. Fevers, fatigue, weight loss with facial pain/HA/Jaw pain.
-Tenderness to palpation of temporal artery. With ophthalmic artery involvement, visual symptoms/blindness in 50%.

***most common form of vasculitis in older people in the US/Europe
corticosteroids prior to temporal artery biopsy to prevent blindness.
Takayasu arteritisGranulomatous inflammation of media; marked intimal thickening; chronically collagenous scaring w/chronic inflammatory infiltrates in all three layers of vessel.Large vessels: aortic arch and proximal great vesselspatients younger than 40 (asian/african females)Systemic general symptoms. -Reduced BP/pulses in bilateral UEs, ocular disturbance, neurologic defects, if distal aorta  claudication of legs.surgery/immunosuppresion/stents
Polyarteritis NodosaCharacterized by segmental transmural fibroid necrosis with mixed infiltrate of neutrophils/eosinohpils/mono in small-to-med arts -Chronically, the inflammation is replaced by fibrous thickening of the vessel wall.
-Complications of inflammation are aneurysms, rupture, and thrombosis. All stages of activity present. (Lesions of DIFFERENT AGES).
-Systemic vasculitis of Med/small muscular arteries, involving RENAL vessels and visceral vessels (SPARES PULMONARY VESSELS).Etiology: Idiopathic/unknown cause. PAN associated with chronic hepatitis 30% have HepB +HBsAg-HbsAb complexes. Cutaneous forms of PAN.

Disease of young adults, can also occur in children/adults. Acute/chronic.
Episodic with long symptom free intervals.
-Malaise, fever, weight loss, other symptoms depending on affected organs (Kidney>heart>liver>GI). Untreated is fatal.
:
Kawasaki DiseaseInflammation affecting entire wall thickness with less prominent Fibrinoid necrosis than PAN. Healed lesions thicken the intima.-Arteritis in childhood a predilection for coronary artery involvement (Coronary arteritis can cause aneurysms which can rupture/thrombose/MI)Leading cause of ACQUIRED heart disease in children. Acute febrile illness, self-limited, occurs in infancy/childhood. (80% younger than 4).

-A viral infection may possibly trigger the autoimmunity causing disease. Vascular damage by Tcells/monocytes. AutoAb to endothelium/SM.
Clinically: Also called mucocutaneous lymph node syndrome, presents with conjunctival/oral erythema/erosion. Edema of hands/feet. -Erythema of palms/soles. Desquamative rash/cervical node involvement. If untreated, 20% develop cardiovascular sequelae.ASA and/or IVIG
Buerger Disease (aka Thromboangiitis Obliterans)microabscesses; nerves and/or veins may become encased in fibrosisextremities of med/small arteries...may extend to veins/nerves. mainly affects tibial/radial artery (but hand vasculitis is rare)-Occurs almost exclusively in HEAVY SMOKERS, mostly male, less than 35-45 years old. More prevalent in Japan/Isreal/India.

-Etiology: Increased incidence of HLA-A9/HLA-B5. Possibly due to direct endothelial cell toxicity from a component of tobacco.
Superficial nodular phlebitis, cold sensitivity (Reynaud-like) to hands, instep claudication. SEVERE PAIN even at rest (nerves).
-Chronic ulcerations of toes, feet, fingers, followed by frank gangrene.
Smoking cessation
Granulomatosis w/polyangiitis (Wegener)Triad: Acute necrotizing granulomas of the ear, nose, sinuses, throat, lung. Necrotizing/Grans vasculitis affecting small-med vessels in lungs.
-Focal necrotizing glomerulonephritis, often crescentic.

Morphology: Upper airway with mucosal granulomas/ulcerative lesions of nose/pharynx. Granulomas with central necrosis/vasculitis surrounded
by a zone of fibroblastic proliferation with giant cells and leukocyte infiltrate. Renal involvement with various glomerulonephritis.
-Necrotizing vasculitis involving UPPER AND LOWER AIRWAYS and THE KIDNEYS.Clinically: Males>females. Average age 40 years. Persistent pneumonitis, sinusitis, mucosal ulceration of nasopharynx and evidence of renal dz.
-Other: rashes, myalgias, fever, arthralgia. 80% mortality. Chronic remitting and relapsing disease with treatment.
-Etiology: Probable T-cell mediated hypersensitivity reaction to inhaled agent. PR3-ANCA/C-ANCA in 95%.cyclophosphamide, corticosteroids
Eosinophilic granulomatosis w/polyangiitis (aka Churg-Strauss)Small vessel necrotizing vasculitis associated with ASTHMA/Allergic rhinitis, lung infiltrates, peripheral hypereosinophilia, extravascular necrotizing granulomas. Eosinophils present in vascular inflammation as well as macrophages/lymphocytes.small vesselsasthma, atopyMPO-ANCA/P-ANCA in less than 50%. Heart involved in 60% of patients with eosinophil infiltrate. Skin/GI tract, kidneys, and other organs involved.:
Microscopic polyangiitis-Morphology: Segmental Fibrinoid necrosis of the media with focal transmural necrotizing lesions and NO granulomatous inflammation.Necrotizing vasculitis affecting arterioles, venules, and capillaries. Lesions are the SAME AGE. Multiple organs affected. -Necrotizing glomerulonephritis in 90%. PULMONARY CAPILLARIES IS COMMON. (Affects RENAL AND PULMONARY-think Hemoptysis)ANY-Associated with MPO-ANCA/P-ANCA. Lesions are PAUCI-IMMUNE (devoid of immune complexes).

Clinically: Symptoms depend on organ. With kidney we get hematuria/proteinuria. Lungs --> Hemoptysis.
cyclophosphamide, corticosteroids
Behcet DiseaseVasculitis with neutrophilic infiltrate involving veins/caps/small/med areries.veins/caps/small-med arteriesPrevalence highest in Middle East/Japan, 25-30 years old.Classic triad of findings: Recurrent oral aphthous ulcers, genital ulcers, and uveitis. May have GI/pulm/CNS involvement.

Infectious agents triggers auto-Ab formation. Associated with HLA-B51/HLA-B5.
::
memorize