Vasculitis & Angitis Lecture Powerpoint Flashcards
2 key large vessel vasculitis
Takayasu arteritis
Giant cell arteritis
2 key medium vessel vasculitis
Polyarteritis nodosa
Kawasaki disease
Key ANCA positive small vessel vasculitis (3)
- microscopic polyangitis
- granulomatosis with polyangitis (wegeners)
- eosinophilic granulomatosis with polyangitis
Key Immune complex small vessel vasculitis (2)
- Anti glomerular basement membrane dz
- IgA vasculitis
Vasculitis etiology and pathogenesis
Antigenic stimulation results in immune complex formation, autoantibody production and or cell mediated immunity. Formation of pathogenic immune complexes, antiendothelial antibody mediated vascular injury, cell mediated immune resposne with granuloma formation
Giant cell arteritis definition
Granulomatous vasculitis of medium to large arteries most often temporal artery or opthalmic arteries, can also involve arteries that originate from aortic arch
Giant cell arteritis clinical presentation (9)
- age >50
- headache
- systemic symptoms
- polymyalgia rheumatica (muscle aches in proximal musculature of upper and lower extremities)
- visual symptoms
- jaw or limb claudication
- markedly elevated sed rate
- tender or swollen artery
- temporal cell biopsy shows thickening with multinucleated giant cells and skip lesions
Giant cell arteritis treatment options (3)
- high dose prednisone followed by slow taper
- tocilizumab
- methotrexate
Polymyalgia rheumatica (PMR) definition and treatment (1)
Proximal upper and lower limb myalgias present for at least 1 month in patients over 50, sed rate elevated (over 50) upon exclusion of other conditions and see excellent response to moderate dosage of prednisone
Giant cell arteritis and polymyalgia rheumatica etiology
Unknown
Polymyalgia rheumatica (PMR) clinical presentation (4)
- good health prior to onset
- low grade fever and weight loss
- begins in one area and then involves symmetrical and proximal muscles of upper and lower extremities
- morning stiffness common
Polyarteritis nodosa
Medium size blood vessel vascularity, necrotizing panarteritis (involving all 3 layers of blood vessel) often presenting with skin, joints, peripheral nerves, gut, and kidney involvement, see panmural necrotizing lesions with predilection for vessel bifurcation but distinct from ANCA associated vasculitis (microscopic polyangitis)
Polyarteritis nodosa clinical presentation (8)
- peak age 40-60
- fever, milaise
- skin purpura or ulcerations
- renal disease (proteinuria and hypertension)
- neuropathy (foot or wrist drop***)
- GI bleeding due to ischemia
- elevated ESR, anemia, thrombocytosis
- beaded appearance on angiogram esp. at bifurcation
Polyarteritis nodosa (2) treatment
- high dose prednisone and taper
- cyclophosphamide when severe
Eosinophilic granulamatosis with polyangitis (EGPA) definition
ANCA associated vasculitis often begins with prodromal period of months to years of rhinitis, polyposis, asthma, 2nd stage of eosinophilia, eosiniphilic pneumonia or gastroenteritis, followed by 3rd phase which is frank systemic vasculitis
Eosinophilic granulomatosis with polyangitis (EPGA) treatment options (1)
-prednisone 40-60 mg daily
Granulomatosis with polyangitis (wegener’s granulomatosis) definition and treatment options (3)
Positive c-ANCA necrotizing granulomas of respiratory tract upper and lower, glomerulonehprhitis, and joints/other organs, limited movement initlaly and more generalized dz eventually, requires biopsy for diagnosis, treated with steroids and cyclophosphamide or rituximab
Microscopic polyangiitis and treatment (3)
Small vessel pauci immune necrotizing vasculitis onsetting age of 40-60 treated with high dose prednisone or cyclophosphamide and rituximab
IgA vasculitis (Henoch schonlein purpura) definition
Multisystem disorder characterized by palpable purpura (common on lower extremities and buttocks), abdominal pain, and hematuria, peak incidence ages 3-15 but can affect any age, self limited course but an relapse, just need supportive care via glucocorticoids
Leukocytoclastic vasculitis (hypersensitivty vasculitis)
Biopsy finding of small blood vessels usually in the skin typically having skin develop characteristic palpable purpura at the same stage, often idiopathic but can be due to serum sickness or if IgA then considered henoch schonlein purpura
Think of ___ when 2 or more seemingly unrelated organ systems are involved in pathology
vasculitis