Vasculitis, Veins, Lymphatics, DVT, Vascular Tumors Flashcards
Clinical presentation of vasculitis
Depends on the vascular bed affected — but generally presents as constitutional sx: fever, malaise, arthralgias, myalgias
What primary form of vasculitis affects the aorta?
Giant cell arteritis
Diagnosis of what primary form of vasculitis REQUIRES the presence of neutrophils?
Behcet disease
Diagnosis of what primary form of vasculitis REQUIRES the presence of eosinophils?
Churg-Strauss Syndrome
Diagnosis of what primary form of vasculitis REQUIRES the presence of granulomas?
Granulomatosis with Polyangiitis
Diagnosis of what primary form of vasculitis REQUIRES the feature of thrombosis?
Buerger disease
A clinical history of >40 y/o, +/- polymyalgia rheumatica may clue you into what primary form of vasculitis?
Giant cell arteritis
A clinical history including asthma and/or atopy may clue you into what primary form of vasculitis?
Churg-Strauss syndrome
A clinical history including a young male smoker may clue you into what primary form of vasculitis?
Buerger disease
A clinical history including orogenital ulcers may clue you into what primary form of vasculitis?
Behcet disease
Pathogenesis of immune complex vasculitis
Autoantibody production and formation of immune complexes — deposition of Ag-Ab complexes in vascular walls — this incites an inflammatory reaction within the wall, the antigen is often unidentified
Immune complex vasculitis may be seen in what conditions?
Systemic immunologic diseases (e.g.,SLE)
Drug hypersensitivity — e.g., penicillin (streptokinase acts as foreign protein) - always consider this because if drug is stopped, vasculitis resolves!
Exposure to infectious agent — so Abs to microbial constituents form immune complexes that deposit in vascular lesions (e.g., polyarteritis nodosa — associated with HBsAg and anti-HBsAg)
Heterogenous group of antibodies reactant with cytoplasmic enzymes found in neutrophil granules, monocytes, and endothelial cells, —and associated with noninfectious vasculitis
Antineutrophil cytoplasmic antibodies (ANCA)
2 types of ANCA antibodies and the conditions they are associated with
PR3-ANCA (anti-proteinase-3) — associated with polyangiitis
MPO-ANCA (anti-myeloperoxidase) — associated wtih microscopic polyangiitis and Churg-Strauss
ANCA titers usually follow disease _____
Severity
Why are ANCA-associated vasculitides considered “pauci-immune”?
ANCA Abs are directed against cellular constituents and DO NOT form circulating immune complexes — thus vascular lesions do not typically contain Ab and complement
Most common vasculitis among older patients that may present with constitutional symptoms (fever, fatigue, weight loss), as well as facial pain and headache
Giant cell (temporal) arteritis and aortitis
Pathogenesis of giant cell arteritis and aortitis
Chronic T-cell mediated inflammation of arteries in the head, especially temporal arteries
Medial granulomatous inflammation occurs, often with multinucleated giant cells
Fragmentation of elastic lamina and intimal thickening
Sites of involvement within an artery may be patchy and focal
Why might pts with Giant cell arteritis complain of double vision?
Involvement of the ophthalmic artery — may lead to vision loss and is a medical emergency!
Noninfectious vasculitis characterized by ocular disturbances and marked weakening of pulses of the upper extremities (pulseless disease); may present with weak pulse and low BP in upper extremities
Takayasu arteritis
Takayasu has similar histologic findings (giant cells) as those seen in giant cell arteritis, with what exceptions?
It involves the aortic arch (aortitis) and major branch vessels — occasionally causing dilation and aortic valve insufficiency (leads to cardiac symptoms)
Pulmonary artery is involved in 1/2 the cases, coronary and renal arteries may also be involved
Younger age group (<50)
Noninfectious, systemic vasculitis, likely immune-complex mediated, tends to involve renal vessels, heart, liver, and/or GI tract — BUT pulmonary vessels are spared
[most common is for just renal vessels to be involved]
Polyarteritis nodosa (PAN)
Polyarteritis nodosa may affect any age group, but classically affects _______; almost 1/3 of the patients have _______
Young adult; chronic hepatitis B (HBsAg-HBsAb complexes are found in the involved vessels)
What type of inflammation characterizes polyarteritis nodosa?
Segmental transmural necrotizing inflammation (neutrophils, eosinophils, lymphocytes, and macrophages) — with predilection for branch points
Ulcerations, infarcts, ischemic atrophy, or hemorrhage may be first sign of dz
Sites of inflammation are typically NOT circumfirential; inflamed vessel walls are susceptible to thrombus formation/occlusion, aneurysm, rupture
What type of necrosis is seen with polyarteritis nodosa?
Fibrinoid necrosis
Acute arteritis of infants and small children (80% affecting < 4 years), often involving the coronary arteries which may end up forming aneurysms that thrombose or rupture leading to acute MI
Kawasaki disease
Clinical presentation and prognosis of kawasaki disease
Usually erythema of the conjunctiva, oral mucosa, PALMS AND SOLES; desquamative rash
Also may see cervical lymph node enlargement (“mucocutaneous lymph node syndrome”)
Px: usually self-limited but IVIg and aspirin are indicated to lower the risk of a coronary event
Necrotizing vasculitis involving arterioles, capillaries, and venules characterized by fragmented PMNs and associated with Henoch-Schonlein purpura, essential mixed cryoglobulinemia, and other CT disorders
Microscopic polyangiitis
Microscopic polyangiitis affects vessels of many organ systems, but which are most common?
Renal glomeruli and lung capillaries
Most cases of microscopic polyangiitis are associated with _______ Abs. Pathologic pattern includes segmental necrotizing inflammation with _____ necrosis. Many apoptotic _____ are usually seen — and it is often called “leukocytoclastic vasculitis”
MPO-ANCA; fibrinoid; neutrophils
Small vessel necrotizing vasculitis associated with asthma, allergic rhinitis, hypereosinophilia, lung infiltrates, extravascular granulomas, and may resemble PAN or microscopic polyangiitis but with the addition of eosinophils and granulomas
Churg-Strauss syndrome
T/F: less than 1/2 of Churg Strauss pts show ANCA positivity
True — when they do it is usually MPO-ANCA
Organ systems involved with Churg Strauss syndrome and potentially fatal complication
Many organ systems may be involved; multisystem disease with cutaneous involvement (palpable purpura), GI tract bleeding, and renal disease (primarily as FSGS)
Myocardial involvement may give rise to cardiomypathy; heart is involved in 60% of pts, and accounts for almost half of all deaths in the syndrome
Behcet dz is a noninfectious vasculitis of small to medium vessels. What is the classic triad associated with this disease?
Aphthous ulcers of the oral cavity
Genital ulcers
Uveitis
[there are also GI and pulmonary manifestations]