Vasculitis Flashcards
Signs of vasculitis
Fever, myalgias, arthralgias, malaise
Pathogenesis of non’infectious vasculitis
Abs to viral proteins form immune complexes that deposit in vascular lesions
30% of pats with poly arthritis bodies have underlying Hep B
Anti-myeloperoxidase (MPO-ANCA)
Microscopic polyangiitis and Church-Strauss syndrome
Anti-proteinase-3 (PR3-ANCA)
Typically found in polyagiitis with granulomatosis
Giant cell arteritis
Most common
Chronic, often granulomatous
Commonly arteries in the head (TEMPORAL ARTERY)
Ophthalmic artery (blindness)
Giant cell arteritis morphology
Modular intimal thickenings with reduction of lumen
Medial granulomatous inflammation
Elastic laminate fragmentation
Segmental inflammatory lesions along vessel
Giant cell arteritis clinical
MEDICAL EMERGENCY- diagnose and treat to prevent blindness
Affected artery is modular and tender to palpating
Diagnose by biopsy of involved artery
Takayasu Arteritis
Rare, granulomatous vasculitis
<50
Women > men
Involves aortic arch
Takayasu Arteritis Clinical
Weakening of pulses in the upper extremities, reduced BP
Ocular disturbances
Claudication of legs
Polyarteritis nodoosa
Involves small and medium arteries
No ANCA association
About 30% have chronic HepB with HbsAg-HbsAb complexes in affected vessels
Polyarteritis nodosa morphology
Segmental necrotizing inflammation of small and medium vessels
Descending distribution Kidneys, heart, liver, GI tract
Impaired perfusion: ulceration, infarcts, atrophy, hemorrhage’s in distribution of affected vessels
Aneurysmal nodules
Acute polyarteritis nodosa
Transmittal inflammation with fibrinoid necrosis
Later polyarteritis nodosa
Acute inflammation with fibrinoid necrosis
Clinical polyarteritis nodosa
Young adults > kids or elderly
Vasculitis in renal arteries
No glomerulonephritis
Major cause of death along with hypertension
Polyarteritis nodosa treatment
Corticosteroids and cyclophosphamide
Kawasaki syndrome
Large, medium sized and small arteries
Young children and infants (#1 cause of acquired heart disease in children)
Acute, febrile, usually self limited
Morphology of Kawasaki syndrome
Resembles polyarteritis nodosa
Transmittal inflammation but less fibrinoid necrosis
Range from intimal lesions to severe transmittal destruction
Kawasaki syndrome clinical
Fever, congested conjunctival, changes in lips and oral mucosa, rash, cervical lymphadenopathy
Edema of hands, feet, erythema of palms and soles
Untreated Kawasaki syndrome
Asymptomatic vasculitis of coronaries
Giant coronary aneurysm
MI or sudden death
Kawasaki syndrome treatment
Aspirin, IV immunoglobulin
Microscopic Polyangiitis
Usually affects arteriolosclerosis, capillaries and venules
All lesions tend to be SAME AGE
MPO-ANCA is usually present
Microscopic polyangiitis pathogenesis
Some cases from antibody response to antigens such as:drugs, microorganisms, heterologous proteins, tumor antigens
Immune complex decomposition
Triggers secondary immune response (MPO-ANCAs)
Most lesions are pauci-immune
Microscopic polyangiitis morphology
Segmental fibrinoid necrosis of the media
No granulomatous inflammation
Similar to PAN but muscular and large arteries are spared
May only see infiltrating and fragmenting of neutrophils
Microscopic polyangiitis clinical
Hemostasis, Hematuria, and proteinuria
Bowel pain and bleeding
Muscle pain or weakness
Palpable purpura
Microscopic polyangiitis treatment
Immunosuppression, improves long term survival
Church Strauss syndrome
small vessel necrotizing vasculitis
Associated with allergic rhinitis, bronchial asthma and eosinophilia
Intra and extra-vascular granulomas
Infiltration of vessels and perivascular tissues by eosinophils
Granulomatous with Polyagiitis
Respiratory tract involvement with acute necrotizing granulomas of upper and/or lower
Focal necrotizing or granulomatous vasculitis of small to med sized vessels
Renal disease
(Limited forms restricted to respiratory tract)
Granulomatosis with polyangiitis pathogenesis
PR3-ANCA in 95% of patients
Granulomatosis with polyangiitis clinical
M>F average age 40
Persistent pneumonia is with bilateral modular and cavitary infiltrates
Chronic sinusitis
Mucosal ulceration of the nasopharyngeal
Rapidly fatal if untreated (immunosuppressive, cyclophosphamide)
Thromboangiitis obliterans (Buerger Disease)
Segmental , thrombosis, acute and chronic inflammation of med sized and small arteries (Tibial and radial arteries)
MEN WHO ARE HEAVY CIGARETTE SMOKERS
Begins before 35
Thromboangiitis obliterans morphology
The onus contains microabscesses with central focus of neutrophils surrounded by granulomatous inflammation
Inflammation extends to adjacent veins and nerves
Thromboangiitis obliterans clinical
Superficial modular phlebitis
Cold sensitivity in hands
Instep claudication
Chronic ulceration of toes, feet, fingers
