Vasculitis Flashcards
Temporal (giant cell) arteritis epidemiology/presentation
- Generally elderly females
- Unilateral headache (temporal artery), jaw claudication
- May lead to irreversible blindness due to opthalmic artery occlusion.
- Associated with polymyalgia rheumatica
Temporal (giant cell) arteritis pathology/labs
- Large vessel vasculitis
- most commonly affects branches of carotid artery
- focal granulomatous inflammation
- increased ESR
Temporal (giant cell) arteritis Treatment
Treat with high-dose corticosteroids
Takayasu’s arteritis epidemiology/presentation
- Asian females <40 years of age
- “pulseless disease” (weak upper extremity pulses), fever, night sweats, arthritis, myalgias, skin nodules, ocular disturbances
Takayasu’s arteritis Pathology/Labs
- Large-vessel vasculitis
- Granulomatous thickening of aortic arch, proximal great vessels
- Increased ESR
Takayasu’s arteritis Treatment
Treat with corticosteroids
Polyarteritis nodosa epidemiology/presentation
- young adults
- hepatitis B seropositivity in 30% of patients
- fever, weight loss, malaise, headache
- GI: abdominal pain, melena
- Hypertension, neurological dysfunction, cutaneous eruptions, renal damage
Polyarteritis nodosa pathology/labs
- Medium-vessel vasculitis
- Typically involves renal and visceral vessels, NOT pulmonary arteries.
- Immune complex mediated
- Transmural inflammation of the arterial wall with fibrinoid necrosis
- Lesions are of different ages.
- Many aneurysms and constrictions on arteriogram.
Polyarteritis nodosa Treatment
Treat with corticosteroids, cyclophosphamide
Kawaski disease epidemiology/presentation
- Asian children < 4 years of age.
- fever, cervical lymphadenitis, conjunctival injection, changes in lips/oral mucosa (“strawberry tongue”), hand-foot erythema and desquamating rash.
Kawaski disease pathology/labs
- Medium vessel vasculitis
- May develop coronary aneurysms -> MI, rupture
Kawaski disease Treatment
Treat with IV immunoglobulin and aspirin
Buerger’s disease (thromoangitis obliterans) epidemiology/presentation
- Heavy smokers, males <40 years of age
- Intermittent claudication may lead to gangrene, autoamputation of digits, superficial nodular phlebitis
- Raynaud’s phenomenon is often present
Buerger’s disease (thromoangitis obliterans) Pathology/labs
- Medium-vessel vasculitis
- Segmental thrombosis vasculitis
Buerger’s disease (thromoangitis obliterans) Treatment
Treat with smoking cessation
Microscopic Polyangitis Epidemiology/presentation
- Necrotizing vasculitis commonly involving lung, kidneys, and skin with pauci-immune glomerulonephritis and palpable purpura.
Microscopic Polyangitis pathology/labs
- Small-vessel vasculitis
- NO granulomas
- p-ANCA positive
Microscopic Polyangitis Treatment
Treat with cyclophosphamide and corticosteroids
Wegener’s granulomatosis (granulomatosis with polyangiitis) epidemiology/presentation
- Upper respiratory tract: perforation of nasal septum, chronic sinusitis, otitis media, mastoiditis
- Lower respiratory tract: hemoptysis, cough, dyspnea
- Renal: hematuria, red cell casts.
Wegener’s granulomatosis (granulomatosis with polyangiitis) Pathology/labs
- Small-vessel vasculitis
- Triad:
- Focal necrotizing vasculitis
- Necrotizing granulomas in the lung and upper airway
- Necrotizing glomerulonephritis
- c-ANCA positive
- Chest X-ray: large nodular densities
Wegener’s granulomatosis (granulomatosis with polyangiitis) Treatment
Treat with cyclophosphamide, corticosteroids
Churg-Strauss syndrome epidemiology/presentation
- asthma, sinusitis, palpable purpura, peripheral neuropathy (ex. wrist/foot drop).
- can also involve heart, GI, kidneys (pauci-immune glomerulonephritis).
Churg-Strauss syndrome pathology/labs
- small-vessel vasculitis
- Granulomatous, necrotizing vasculitis with eosinophilia
- **p-ANCA positive, elevated IgE level. **
Henoch-Chonlein purpura epidemiolog/presentation
- Most common childhood systemic vasculitis
- Often follows URI
-
Classic triad:
- Skin: palpable purpura on buttocks/legs
- Arthralgia
- GI: abdominal pain, melena, multiple lesions of same age.
Henoch-Schonlein pupura Pathology/labs
- Small-vessel vasculitis
- Vasculitis secondary to IgA complex deposition.
- Associated with IgA nephropathy.