Car 23 - Vascular Disease Flashcards
What is Microscopic polyangiitis?
A small vessel vasculitis, it affects the kidneys, causing Pauci-immune crescentic glomerulonephritis. Usually (+) P-ANCA.
What is the difference b/w p-ANCA vs c-ANCA?
ANCA is Anti-Neutrophil Cytoplasmic Antibodies. Perinuclear-ANCA (p-ANCA) is made up of Ab against antigen Myeloperoxidase (MPO), therefore sometimes called MPO-ANCA. Cytoplasmic ANCA (c-ANCA) is made up of Ab against Proteinase 3 (PR3), therefore AKA PR3-ANCA.
What is Granulomatosis w/ Polyangiitis (GPA Or Wegener’s granulomatosis)?
A small vessel vasculitis, it has a Classic Triad: Focal necrotizing vasculitis, granulomas in lung and upper airway, glomerulonephritis. Granulomas in soft palate, oropharynx, nasal cavity, sinuses. Can also cause collapse of the bridge of the nose (saddle nose). Dyspnea, hemoptysis. Hematuria. (+) c-ANCA.
What disease affects both the lungs and kidney?
Granulomatosis w/ polyangiitis (Wegener’s). Goodpasture syndrome.
What is the treatment for Granulomatosis w/ polyangiitis (GPA)?
Cyclophosphamide. Corticosteroids.
What is Churg-Strauss syndrome?
A small vessel vasculitis, it has vasculitis with eosinophilia. Associated w/ asthma, sinusitis, skin lesions, peripheral neuropathy. (+) p-ANCA.
What is Henoch-Schonlein purpura?
A small vessel vasculitis, it is the most common form of systemic vasculitis in childhood. Classic tetrad: Causes rash on legs and buttocks made up of palpable purpura. Arthralgias. Abdominal pain (intestinal hemorrhage). Renal disease (IgA complexes in the kidney). Associated w/ recent URI.
What is Polyarteritis Nodosa (PAN)?
A medium-sized vasculitis, it is pretty nonspecific. Involves kidneys and visceral vessels (skin, GI tract, heart). However, it spares the lungs. Strongly associated w/ hepatitis B/C and (-) ANCA.
What is the treatment for Polyarteritis nodosa (PAN)?
Corticosteroids. +/- cyclophosphamide.
What is Kawasaki disease?
A medium-sized vasculitis, AKA Mucocutaneouse lymph node syndrome. Causes necrotizing vasculitis in infants, especially in Asian descent. Fever that last for at least 5 days and 4 of the following 5 symptoms: [CRASH] Conjunctivitis (bilateral nonexudative), Rash (trunk), Adenopathy (cervical), Strawberry tongue, Hands and feet (erethyma and descamation). Can cause coronary aneurysm resulting in rupture or thrombosis.
What is the treatment for Kawasaki disease?
IV immunoglobulin (IVIG). High-dose aspirin (to prevent thrombosis of coronary artery aneurysms).
What is Buerger disease?
A small and medium-sized veins, seen in male smokers in their 40s. AKA thromboangiitis obliterans. Causes Intermitten claudication. Superficial nodular phlebitis. Cold sensitivity.
What is the treatment for Buerger disease?
Stop smoking.
What is Temporal Arteritis?
A large-sized vasculitis, AKA Giant cell arteritis. Most common form of vasculitis. Causes inflammation of branches of external carotid artery, typically seen in elderly women. Causes unilateral headache and jaw claudication. Can progress to occlusion of the ophthalmic artery, causing vision loss and irreversible blindness. Causes elevation in ESR. Associated w/ PMR (pain and joint stiffness, affecting the proximal joints).
How do we diagnose Temporal arteritis?
Temporal artery biopsy.
What is the treatment for temporal arteritis?
High-dose steroids for several months.
What is Takayasu arteritis?
A large-sized arteritis, AKA pulseless disease. Causes granulomatous inflammation of arteries off the aortic arch, leading to poor pulses in extremities. Seen in young Asian women (teens to 20s). Causes elevation of ESR.
What vasculitis can you suspect in 2 y.o Asian female?
Kawasaki.
What vasculitis can you suspect in 20 y.o Asian female?
Takayasu.
What vasculitis can you suspect in someone with asthma?
Churg-Strauss.
What vasculitis can you suspect in young male smoker?
Buerger.
What vasculitis can you suspect that is associated w/ polymyalgia rheumatica?
Giant cell.
What vasculitis can you suspect that is associated w/ IgA nephropathy?
Henoch-Scholen purpura.
What vasculitis can you suspect that is associated w/ hepatitis B/
PAN.
What vasculitis can you suspect in elderly woman w/ jaw claudication and vision loss?
Giant cell.