Vasculitides (are da worst) Flashcards
Giant cell temporal arteritis demographics
Elderly females
GCA symptoms
Unilateral headache, jaw claudication, can cause unilateral/irreversible blindness
MOA of blindness in GCA
Opthalmic artery occlusion (disease affects branches of carotid a. most frequently)
GCA association
Polymyalgia rheumatica
Histology of GCA
Granulomatous inflammation along the internal elastic lamina –> focal destruction and fibrosis of vessel wall –> wall thickening, luminal narrowing
Takayasu arteritis demographic
Asian females <40yrs
Takayasu arteritis signs
Weak upper extremity pulses, fever, night sweats, arthritis, myalgias, skin nodules, ocular disturbances
Takayasu arteritis histology
Mononuclear inflammation w/ giant cells through full thickness of vessel wall (aortic arch and branches), w/ fibrosis, assc. w/ wall thickening and luminal narrowing
Granulomatosis w/ polyangiitis test
C-ANCA (anti PR3)
Granulomatosis w/ polyangiitis other name
Wegeners
Granulomatosis w/ polyangiitis signs
Upper RT: perforation of nasal septum, chronic sinusitis, otitis media, mastoiditis, saddle nose
Lower RT: hemoptysis, cough, dyspnea
Kidneys: hematuria, red cell casts
Granulomatosis w/ polyangiitis histology
Necrotizing granulomas of small vessels, necrotizing glomerulonephritis, necrotizing granulomas in lung/upper airway
Polyarteritis nodosa demographics
Young/middle aged men
Polyarteritis nodosa association
Hep B pos in 30%
Polyarteritis nodosa signs
Fever, wt. loss, malaise, headache
GI: abdominal pain, melena
HTN, neuro dysfxn, cutaneous eruptions, renal damage
NO LUNGS
Polyarteritis nodosa histology
Fibrinoid necrosis and thrombosis alternating w/ regions of vascular wall fibrosis
Kawasaki disease demographics
Asian child <4yrs
Kawasaki other name
Mucocutaneous lymph node syndrome
Kawasaki signs
Conjunctival injxn, rash (polymorphous–>desquamating), Adenopathy (cervical), Strawberry tongue, Hand/foot changes (edema, erythema), fever
CRASH and burn
Kawasaki complications
Coronary artery aneurysms, thrombosis, rupture –> death
Tx of Kawasaki
IVIG and aspirin
Microscopic polyangiitis signs
Involvement of kdiensy, lung, skin -- pauci-immune glomerulonephritis and palpable purpura NO NASOPHARYNGEAL (like in GwP)
Microscopic polyangiitis histology
Similar to polyarteritis nodosa w. fibrinoid necrosis except w/ smaller vessels, fragmented neuts (leukocytoclasia), and similar age of lesions
Henoch schonlein purpura demographic
Most common systemic vasculitis in kiddos
Henoch schonlein purpura association
Follow URI, IgA nephropathy (Berger disease)
Triad of Henoch Schonlein Purpura
Skin – palpable purpura
Arthralgias
GI: abdominal pain
Henoch Schonlein purpura histology
Necrotizing vasculitis w/ depositions of IgA immune complexes and C3 (also in glomerular mesangium)
Thromboangiitis obliterans other name
Buerger disease
Thromboangiitis obliterans demographic
heavy smoking men under 40
Thromboangiitis obliterans signs
Intermittent claudication–>gangrene/autoamputation of digits
Thromboangiitis obliterans histology
Segmental thrombosing vasculitis, fibrosis, extension into veins/nerves –> painful
Eosinophilic granulomatosis w/ polyangiitis other name
Churg Strauss
Eosinophilic granulomatosis w/ polyangiitis histology
Lots and lots of eo’s w/ granulomas
Eosinophilic granulomatosis w/ polyangiitis signs
Asthma, sinusitis, skin nodules, purpura, peripheral neuropathy (foot/wrist drop), heart, GI, kidneys (pauci-immune glomerulonephritis)
Eosinophilic granulomatosis w/ polyangiitis demographic
Middle aged w/ long history of asthma
Eosinophilic granulomatosis w/ polyangiitis association
p-ANCA (anti-MPO), increaed IgE
Hereditary hemorrhagic telangietasia findings
Blanching skin lesions, recurrent epistaxis, skin discolorations, AVMs, GI bleeding, hematuria
Hereditary hemorrhagic telangiectasia other name
Osler Weber Rendu
