rheumatology & immunology Flashcards
What disease is associated with giant cell arteritis?
polymyalgia rheumatica (PMR): 40–50% of patients with giant cell arteritis also have PMR
Immediate administration of what is crucial in preventing what in GCA
Immediate administration of high-dose glucocorticoids is crucial to prevent permanent vision loss in patients with giant cell arteritis!
Large vessel vasculitis
Giant cell arteritis (TA)
Takayasu arteritis
What is takayasu arteritis
granulomatous inflammation of the aorta and branches
Features of Takayasu arteritis
Typically affects women aged 30–40. Presents with fever, arthralgia, weight loss, poor peripheral pulses (decrease bilateral brachial and radial pulses) limb ischemia, hypertension, and/or arterial bruits.
Medium-sized vessel vasculitis
Kawasaki disease
Polyarteritis nodosa
Thromboangiitis obliterans
Polyarteritis nodosa
often associated with Hep B or C infection
tissue ischemia affecting skin, muscles joints kidneys
lungs are not involved
young adult presenting with stroke or MI what rheum disorder should be considered
Polyarteritis nodosa
Thromboangiitis obliterans
Smoking most important risk factor
young men
located in distal arteries of Upper and lower extremities
has normal ESR and CRP
young man with intermittent claudication, Raynaud’s phenomenon, and migratory thrombophlebitis has what
Thromboangiitis obliterans
Imaging of thromboangiitis obliterans shows what?
arteriography shows segmental lesions that occlude distal vessels of extremities with corkscrew-shaped collateral vessels around the site of occlusion
Wegener granulomatosis
C’ disease: Curvy nose (saddle-nose deformity), Chronic sinusitis, Cough, Conjunctivitis and Corneal ulceration, Cardiac arrhythmias, nonCaseating granulomas on biopsy, cANCA, Corticosteroids and Cyclophosphamide as treatment.
Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
pANCA and polyneuropathy (foot or wrist drop), allergic rhinitis/sinusitis/asthma, vasculitis, eosinophilia, and skin nodules
Microscopic polyangiitis
pauci-immune glomerulonephritis; hypertension
pulmonary vasculitis with hemoptysis
palpable purpura
spares nasopharynx and no granulomas
ANCA-associated small vessel vasculitis
Granulomatosis with polyangiits–> cANCA
Eosinophilic granulomatosis with polyangiitis–>pANCA
Microscopic polyangiitis–> pANCA
Difference between microscopic polyangiitis adn Wegner’s
presentation of microscopic polyangiitis is similar to that of Wegener granulomatosis, but it does not affect vessels in the upper and lower respiratory tract (no sinusitis or rhinitis), does not involve granuloma formation, and is associated with pANCA, not cANCA.
Microscopic polyangiits has what positive
has MyeloPeroxidase antibodies (i.e., p-ANCA).
Non-ANCA-associated vasculitis of small vessels
Henoch-Schonlein purpura
Cryoglobulinemic vasculitis
Behcet disease
Immunoglobulin A vasculitis/Henoch-Schonlein purpura typically presents how?
It is often preceded by an upper respiratory tract infection and typically presents with a tetrad of symptoms: palpable purpura, arthritis/arthralgia, abdominal pain, and renal disease.
why is HSP different from most purpura
platelet count in HSP is normal or elevated, as opposed to other causes of purpura!
Cryoglobulinemic vasculitis
characterized by temperature-dependent deposition of immune complexes in blood vessel walls and subsequent inflammation of involved vessels and surrounding tissue
What is is cryoglobulienmia highly associated with?
HCV
Patient with fatigue, palpable purpura with increase RF and hx of Hep c disease has what?
Cryoglobulinemic vasculitis
Behcet disease associated with
HLA-B51
in 20-40 yos
Behcet mnemonic=
PATHERGY
Positive pathergy test, Apthous mouth ulcers Thrombosis Hemoptysis (pulmonary artery aneurysm) Eye lesions (uveitis) Genital ulcers Young at presentation
65 yo female presents with new blurry vision, HA, and tender temporal artery with bilateral pain in shoulders
what does she ahve
Giant cell arteritis
Labs of Giant cell arteritis
elevated ESR
Temporal artery biopsy (gold standard) shows granulomatous inflammation with giant cells and intima proliferation that results in stenosis.
35 yo female presents with different blood pressure in two arms, abdominal bruit
with syncope and angina
what does she have and how to treat
Takayasu arteritis
tx with glucocorticoids
How to treat most vasculitis
Corticosteroids
