Vaculitis Flashcards
What are the types of large vessel vasculitis?
- giant cell arteritis
- takayasu arteritis
What vessels are affected in giant cell arteritis?
Large & medium sized vessels of the extracranial branches of the carotid artery
Seen after 50 years of age
What are the predisposing factor to giant cell arteritis?
- genetic predisposition
- viral infections
- 50% also have PMR
- over 50 years of age
What is the most important pathophysiological characteristic of giant cell arteritis?
Focal granulomatous inflammation
What are the clinical features of giant cell arteritis?
- constitutional symptoms
- headaches
- scalp tenderness
- JAW CLAUDICATION
- VISION LOSS
- symptoms of PMR
What will be seen on lab diagnostics in case of giant cell arteritis?
- increased ESR & CRP
- TEMPORAL ARTERY BIOPSY (gold) -> mononuclear cell infiltration with giant cells
- duplex US of temporal arteries
If suspected do NOT WAIT FOR RESULTS & TREAT IMMEDIATELY
How is giant cell arteritis managed?
High dose corticosteroids for 1-2 years titrated based on symptoms & ESR
What are the complications of giant cell arteritis?
- vision loss
- aortic aneurysm
What are the vessels involved in Takayasu arteritis?
Pulseless aortitis & vasculitis of the large vessels & branches of the aorta
Seen in women in east asia
What are the symptoms of Takayasu arteritis?
- fever
- malaise
- myalgia
- arthralgia
- weight loss
- progressive claudication (in limbs)
How is Takayasu arteritis diagnosed & managed?
Diagnosed by -> angiography of aorta
Treated -> high dose corticosteroids, methotrexate, TNF inhibitors
What are the types of medium vessel vasculitis?
- polyarteritis nodosa
- thromboangitis obliterans
What are the clinical features of Polyarteritis Nodosa?
- fever, weight loss, malaise, muscle & joint pain
- KIDNEY: hypertension & renal impairment
- SKIN: livedo reticularis, nodules, ulcerations
- neuro: mononeuropathies
- GI: abdominal pain, melena, nausea, vomiting
How is PAN diagnosed?
- increased ESR & CRP
- ANCA
- hepatitis B
Angiography -> aneurysmal dilations of affected arteries (Rosary beads) usually in renal & mesenteric
How is PAN treated?
- high dose corticosteroids
- cytotoxic immunosuppressive agents (cyclophosphamide)
- antiviral therapy for HBV infection
What is the classic triad of thromboangitiis obliterans?
1- intermittent claudication
2- Raynaud phenomena
3- migratory thrombophlebitis
Usually in male smokers
Treatment is discontinuing smoking
What are the organs involved in granulomatosis with polyangiitis?
Necrotizing granulomatous arteritis mostly in the kidneys & upper & lower respiratory tract
What are the clinical features of GPA?
- upper respiratory tract -> sinusitis, epistaxis, otitis media, gingivitis, stridor, mastoiditis, saddle nose
- lungs -> cough, hemoptysis, diffuse alveolar hemorrhage, dyspnea, tracheal stenosis
- kidneys -> rapidly progressive crescentic glomerulonephritis, hematuria
- arthritis, scleritis, skin rashes
How is GPA diagnosed?
- ESR increased
- C ANCA (anti proteinase 3) positive
- biopsy -> necrotizing granulomatous
- x ray -> pulmonary nodules or cavities
- UA with active sedatives
How is GPA managed?
INDUCTION -> cyclophosphamide + corticosteroids and/or rituximab
REMISSION -> methotrexate, azathioprine
What are the complications of GPA?
- kidney failure
- diffuse alveolar hemorrhage could be life-threatening
What organs are most involved in eosinophilic granulomatosis with polyangitis (Churg-Strauss syndrome)?
small and medium vessel necrotizing vasculitis & eosinophilia most commonly in LUNGS & SKIN
What are the clinical features of EGPA?
- asthma, nasal polyps, allergic rhinitis
- mononeuritis multiplex
- cough, dyspnea, angina pectoris
How is EGPA diagnosed & treated?
- EOSINOPHILIA
- MPO-ANCA & pANCA
- XRAY -> fleeting pulmonary infiltrates
- biopsy -> tissue eosinophilia
Treat with immunosuppression + glucocorticoids +/- cyclophosphamide
What are the characteristics of microscopic polyangitis?
NONGRANULOMATOUS vasculitis of small vessels mostly in lungs, kidneys, & skin
How is microscopic polyangitis diagnosed & treated?
Diagnosis
- BIOPSY: fibrinoid necrosis with infiltration of neutrophils & no granuloma
- MPO-ANCA & pANCA
Treatment
- immunosuppression with corticosteroids & cyclophosphamide
What are the characteristics of Behcet disease?
- oral ulcers
- recurrent painful genital ulcerations
- eye or cutaneous lesions
- parhergy test
TREAT WITH systemic corticosteroids
