Vasculitis Flashcards
General causes of vasculitis
Immune mediated inflammation
Direct invasion of vascular wall by infectious pathogens
Physical and chemical injury
Types of large vasculitides
Giant cell arteritis
Takayasu arteritis
Types of medium vasculitis
Polyarteritis nodosa
Kawasaki disease/mucocutaneous lymph node syndrome
Types of small vasculitides
Microscopic polyangiitis
Granulomatosis with polyangiitis/Wegner’s granulomatosis
Allergic granulomatosis with polyangiitis/Churg Strauss Syndrome
IgA vasculitis/Henock Schonlein purpura
Mixed cryoglobulinemia
Diagnostic marker of granulomatosis with polyangiitis (Wegner’s granulomatosis)
c-ANCA/PR3-ANCA
Diagnostic marker of microscopic polyangiitis or allergic granulomatosis with polyangiitis (Churg Strauss syndrome)
p-ANCA/MPO-ANCA
Main arteries affected in giant cell/temporal arteritis
Temporal
Vertebral
Ophthalmic
Vasculitis characterized by medial granulomatous inflammation centered on the internal elastic lamina, which produces elastic fragmentation
Giant cell/temporal arteritis
Vasculitis commonly associated with polymyalgia rheumatica
Giant cell/temporal arteritis
Granulomatous vasculitis of medium and large arteries that commonly involves the aortic arch
Takayasu arteritis
Large vessel vasculitis more common in females of Asian descent, under the age of 50.
Takayasu arteritis
Vasculitis of small and medium sized muscular arteries that involves renal and visceral vessels, but spares pulmonary circulation
Polyarteritis nodosa
Vasculitis associated with chronic hep B in 30% of cases
Polyarteritis nodosa
Microscopy shows segmental transmural necrotizing inflammation of small to medium sized arteries, vessels at various stages of acute and chronic inflammation, and fibrinoid necrosis in acute stages
Polyarteritis nodosa
Treatment of polyarteritis nodosa
Corticosteroids
Resistant cases –> cyclophosphamide
Acute, febrile, self-limited vasculitis in children <4 yo
Kawasaki disease
Toddler-aged pt presents with conjunctival and oral erythema, strawberry tongue, blistering and edema of hands and feet, and desquamative rash of palms and soles. Microscopy shows dense transmural inflammatory infiltrate.
Kawasaki disease
Increased risk of what is associated with Kawasaki disease
Coronary A aneurysms
Treatment for Kawasaki disease
IV immunoglobulin
Aspirin –> reduce risk of coronary A aneurysm
Pt presents with palpable purpura, renal disease, and GIT bleeding. They have asthma. Chest x-ray shows lung infiltrates. They have increased serum IgE.
Eosinophilic granulomatosis with angiitis (Churg Strauss)
Small vessel necrotizing vasculitis with MPO-ANCAs present in <50% of cases.
Eosinophilic granulomatosis with angiitis (Churg Strauss)
Most common vasculitis in children (1-15 yo)
Immunoglobulin A vasculitis (Henoch Schoenlein purpura)
Vasculitis associated with HLA-B51
Behcet disease
Triad of Behcet disease
Recurrent oral aphthous ulcers
Genital ulcers
Uveitis
(can’t kiss, can’t pee, can’t see)
Necrotizing vasculitis that generally affects capillaries where all lesions tend to be the same age. Associated with MPO-ANCA in most cases.
Microscopic polyangiitis
Necrotizing glomerulonephritis in 90%. MPO-ANCA positive.
Microscopic polyangiitis
Vasculitis characterized by necrotizing granulomas of URT and/or LRT, necrotizing granulomatous vasculitis of small/medium vessels, and focal necrotizing/crescentic glomerulonephritis.
Granulomatosis with polyangiitis
Vasculitis that is a form of T cell mediated hypersensitivity. Associated with PR3-ANCA in 95% of cases.
Granulomatosis with polyangiitis
Microscopy of lungs show granulomas with geographic patterns of central necrosis and accompanying vasculitis. Granulomas coalesce, forming cavitary nodules. CXR shows large nodular densities
Granulomatosis with polyangiitis
Pt presents with chronic sinusitis, hemoptysis, dyspnea, and hematuria. They have a nasal septum perforation on exam. They are positive for PR3-ANCA.
Granulomatosis with polyangiitis
Segmental, thrombosing, acute and chronic inflammation of medium sized and small arteries, extending into contiguous veins and nerves. Exclusively seen in male heavy cigarette smokers, <40 yo.
Thromboangiitis obliterans (Buerger disease)
Vasculitis associated with hepatitis due to mixed IgG and IgA complex deposition. Characteristic triad of palpable purpura, weakness, and arthralgias.
Mixed cryoglobulinemia
Causes of secondary Raynaud phenomenon
SLE
Scleroderma
Buerger disease
Atherosclerosis
