What things should make you think vasculitis?
fever of unknown origin and/or unexplained constitutional symptoms;
tissue ischemia (especially in a young person);
mononeuritis multiplex (dropped wrist/foot);
skin lesions/mucosal ulcerations;
rapidly progressive organ dysfunction
Phys exam findings of vasculitis
Splinter hemorrhages, digital infarctions, mononeuritis multiplex, oral ulcers, punched out ulcers in skin, palpable purpura, livedo reticularis (lacy appearance from blood vessels), Subcutaneous nodules, Hemorrhagic nodules, Urticaria lasting for > 24hrs
Ex of large vessel vasculitides
Giant Cell Arteritis
Ex of medium vessel vasculitides
Ex of small vessel vasculitides, ANCA associated
Granulomatosis with polyangiitis (formerly Wegener's Granulomatosis)
Ex of small vessel vasculitides, non ANCA associated
Henoch - Schonlein Purpura (covered in the peds lecture)
Essential cryoglobulinemic vasculitis
Cutaneous leukocytoclastic vasculitis
What is ANCA and its relevance in vasculitides?
Anti-neutrophil cytoplasmic antibodies
p-ANCA = perinuclear (p-ANCA against MPO associated with microscopic polyangiitis)
c-ANCA = cytoplasmic (c-ANCA against PR3 associated with granulomatosis with polyangiitis/Wegener's)
What is Essential cryoglobulinemic vasculitis?
Weakness and Fatigue--severe constitutional sx; Palpable Purpura (Esp Lower Extremities); Arthralgias; Peripheral Sensory Neuropathy; acral gangrene and raynaud's
Associated with Hep C and Sjogrens
Pathogenesis of essential cryoglobulinemic vasculitis
Ab/immune complex mediated
Find mixed cryglobulinemia (IgM, IgG), RF +
Hypocomplement low C3, C4
Tx of essential cryoglobulinemic vasculitis
Plasmapharesis to clear the immune complexes
Cytotoxins: Cyclophosphamide, Azathioprine, Rituximab
What is Cutaneous leukocytoclastic vasculitis?
ONLY involves the skin (palpable purpura, more severe in gravity dependent areas)
Can be caused by drugs (thiazides, allopurinol), infections (viral), and malignancies, but generally benign process
Need to check to make sure there is no other organ involvement (meaning another disease)
What is Behcet disease?
NOT a vasculitis
Multisystem inflammatory disease that may affect arteries and veins of all sizes.
Age of onset usually 20-35 y.o., affects persons of Asian and Mediterranean descent
Characterized by recurrent aphthous ulcers, recurrent genital ulcers, uveitis, cutaneous lesions, or positive pathergy test
Serious complication: blindness, GI ulcerations, thrombosis and aneurysms
What is pathergy test?
Use sterile small needle to prick skin
If local reaction/induration within 24 hours, + and means Behcet
What things can mimic vasculitides?
--infectious endocarditis (ECHO, blood cultures)
--cholesterol emboli/atrial myxoma (ECHO)
--antiphospholipid syndrome (aPL Abs)
--thrombotic thrombocytopenic purpura (smear)
--calciphylaxis (vasc calcification, common in dialysis pts)
--medications, cocaine, amphetamines (Urine tox)
--HIV, hepatitis C, hepatitis B
--Meningitis/encephalitis (LP)--if they have CNS sx
Tests suggestive of inflammation
CBC: anemia of chronic disease, thrombocytosis, neutrophilia, eosinophilia
ESR > 100mm/hr and CRP > 10mg/ml strongly suggest vasculitis if r/o infection
Low albumin - goes down with systemic inflammation