Pathology of Vasculitis Flashcards
What population is generally affected by Kawasaki disease?
Young children and infants
What is a longterm complication of Kawasaki disease?
Damage to coronary arteries that may cause acute MI in children
How can microscopic polyangiitis and polyarteritis nodosa be differentiated histologically?
Granulomatous inflammation is absent with microscopic polyangiitis
What population is most affected by Henoch-Schonlein purpura (IgA vasculitis) and when?
Children 1-15yo with peak incidence in the spring
These antibodies reflect the degree of inflammatory activity in vasculitis.
Antineutrophilic Cytoplasmic Antibodies (ANCA)
What population is typically affected by giant cell arteritis?
Older adults ( >50yo)
These antibodies are present in SLE and Kawakasi disease and may predispose individuals to certain types of vasculitis.
Anti-endometrial cell antibodies
What population is generally affected by Takayasu arteritis?
Females under 40yo
What is the leading cause of acquired heart disease in North America?
Kawasaki disease
What anitbodies are associated with granulomatosis with polyangiitis (Wegener granulomatosis)?
PR3-ANCA (c-ANCA)
How do antineutrophilic cytoplasmic antibodies contribute to vasculitis?
ANCAs directly activate neutrophils to release ROS and proteolytic enzymes, causing destructive interactions between inflammatory and endothelial cells
What vessels are impacted by polyarteritis nodosa?
Small and medium arteries. Smaller arteries, capillaries, venules, and pulmonary vessels are spared
What infections are most associated with infectious arteritis?
Bacterial pneumonia and TB. Common species include Pesudomonas, Aspergillus, Mucor
Permanent blindness is a complication of this type of vasculitis.
Giant cell arteritis
What is the primary cause of morbidity and mortality due to Churg-Strauss (allergic granulomatosis and angitiis?
Coronary arteritis and myocarditis due to eosinophilic infiltration of the heart
Hepatitis B antigen is associated with what type of vasculitis?
Polyarteritis nodosa
What is the pathophysiology of Takayasu arteritis?
Granulomatous vasculitis of medium and large arteries that may involve with aorta and aortic arch and its branches
This type of vasculitis generally follows a viral URI or pharyngitis due to group A strep.
Henoch-Schonlein purpura (IgA vasculitis)
What antibodies are present with Churg-Strauss (allergic granulomatosis and angitiis?
MPO-ANCA (p-ANCA)
What antibodies are present in Kawasaki disease?
Anti-smooth muscle and anti-endothelial antibodies
What antibodes are present with microscopic polyangiitis?
MPO-ANCA (p-ANCA)
What is a potential complication of infectious arteritis?
Mycotic aneurysms (weakening of vessel wall due to infection)
What histologic findings are presnet with Kawasaki disease?
Segmental transmural necrotizing inflammation with fibrinoid necrosis (same as polyarteritis nodosa)
What is the pathophysiology of giant cell arteritis?
Granulomatous inflammation with fragmentation of the internal elastic membrane
What vessels are involved in microscopic polyangiitis?
Arteries, capillaries, and venules
This type of vasculitis is characterized by necrotizing vasculitis involving the lungs and upper respiratory tract.
Granulomatosis with polyangiitis (Wegener granulomatosis)
A 30yo Asian woman presents with confusion, diminished radial pulse, cold arms, and vision problems. The attending tells you she has a type of vasculitis. What is the diagnosis?
Takayasu arteritis - note diminished upper extremity pulse, occular symptoms, and potential neuro symptoms
What symptoms are specific for Churg-Strauss (allergic granulomatosis and angitiis?
Allergic rhinitis, bronchial asthma, eosinophilia
This type of vasculitis is most common in male smokes under 35yo.
Thromboangiitis obliterans (Buerger disease)
Glomerulonephiritis and pulmonary capillaries are potential complications of this type of vasculitis with MPO-ANCA (p-ANCA).
Microscopic polyangiitis - these are primary differences between microscopic polyangiitis and PAN
What is the pathophysiology of thromboangiitis obliterans (Buerger disease)?
Inflammation permeates the vessel wall leading to thrombosis that occludes the lumen and contains microabscesses
What is the most common cause of death from polyarteritis nodosa?
Vasculitis of the renal artery
What is the clinical presentation of polyarteritis nodosa?
Rapidly accelerating hypertension, abdominal pain, and GI hemorrhage in a young adult
What are complications of granulomatosis with polyangiitis (Wegener granulomatosis)?
Glomerulonephritis, sinusitis, hemoptysis due to alveolar hemorrhage
What are the two types of antineutrophilic cytoplasmic antibodies?
Anti-proteinase 3 (c-ANCA) & Anti-myeloperoxidase (p-ANCA)
What population is most affected by polyarteritis nodosa?
Young adults
This type of vasculitis is characterized by segmental transmural necrotizing inflammation with fibrinoid necrosis in young adults.
Polyarteritis nodosa