Vasculitis Flashcards
Mechanisms of vasculitis - cell mediated, immune complex mediated, ANCA associated
- Cell mediated: GCA, takayasu, primary CNS vasculitis
- Immune complex mediated: PAN, HSP, cryoglobulinemic vasculitis
- ANCA associated: granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis
What are the typical presentations of large, medium and small vessel?
- Large vessel: limb claudication, bruits, asymmetric blood pressure, absence pulses
- Medium vessel: cutaneous nodules, ulcers, livedo reticularis, digital gangrene, mononeuritis multiplex, renovascular htn
- Small vessel: palpable purpura, urticaria, glomerulonephritis, alveolar haemorrhage, scleritis
Large vessel vasculitis - compare GCA with takayasu
- Granulomatous arteritis in patient > 50yo = giant cell arteritis
- Granulomatous arteritis in patient < 50yo especially if female = takayasu
How do you differentiate small vessel vasculitis
Immune complexes in vessels
- Cryoglobulins in blood and vessels: cryoclobulin vasculitis
- IgA dominant vessel wall deposits: HSP
- SLE or RA: SLE/RA vasculitis
Paucity of vascular Ig (often with ANCA)
- Vasculitis with no asthma or granulomas: microscopic polangiitis (pANCA, MPO)
- Granulomas and no asthma: granulomatosis with polyangiitis (cANCA-PR3)
- Eosinophilia, asthma, granulomas - churg strauss, eosinophilic granulomatous with polyangiitis (pANCA, MPO)
Symptoms of GCA
- PMR
- Headache (unilateral or bilateral)
- Scalp tenderness
- Jaw claudication
- Diplopia or blindness, constitutional symptoms
Eyes: affects the POSTERIOR CILIARY ARTERIES causing anterior ischaemic optic neuritis
Diagnosis of GCA
- ESR ++
- CRP
- Doppler US: halo sign , compression sign
- Temporal artery biopsy: gold standard but can have false negatives due to skip lesions
Treatment for GCA
- Empirical: PO Prednisone
- If vision threatening: IV methypred 500g for 3 days while awaiting confirmatory test
- Aspirin to prevent ischaemic events
- Pred sparing include methotrexate or tocilizumab (IL6)
Histopathology of GCA biopsy
- 50% granulomatous inflammation of the inner half of the media with fragmentation of the internal elastic membrane
- 60% giant cells
- Non specific pan-arteritis
- Fibrinoid necrosis not seen (a sign of PAN)
- Negative biopsy does not exclude GCA
In a 75yo woman presenting with unilateral temporal headache, which of the following clinical features is most specific for the diagnosis of temporal arteritis?
A. Unilateral headache
B. Blurred vision
C. Jaw claudication
D. Pain and stiffness around the hips and shoulders
E. Fever
Jaw claudication
58yo woman with unilateral headache, vision loss and morning stiffness has a biopsy of her temporal artery. What are the histologic findings you would expect on biopsy?
A. Large vessel giant cell vasculitis with fragmentation of elastic lamina
B. Small vessel eosinophilic vasculitis
C. Medium vessel vasculitis with fibrinoid necrosis
D. Large vessel granulomatous vasculitis with massive intimal fibrosis
E. Fibrinoid necrosis of the external and internal elastic lamina
A. Large vessel giant cell vasculitis with fragmentation of elastic lamina
72yo male presents with unilateral headache, jaw pain on chewing and shoulder and hip girdle stiffness. Commenced on pred and responds initially but symptoms reoccur. Which of the following drugs would be most appropriate to add in addition to pred? A. Adalimumab B. Secukinumab C. Tocilizumab D. Golimumab E. Abatacept F. Tofacitinib
C
34yo female presents with malaise, fever, arm pain, loss of appetite and blurred vision. Exam reveals reduced pulsation and low BP on the upper extremities. Pulses and BP on the lower extremities are normal. Which of the following are the most likely morphologic changes within the affected arteries?
A. Segmental necrotising inflammation
B. Necrotizing inflammation with thrombosis and abscess formation
C. Necrotising inflammation with eosinophilia
D. Granulomatous inflammation and treponemas
E. Granulmatous inflammation and AFB
F. Granulomatous inflammation
F- granulomatous inflammation
AFB: TB
Treponema: Syphillis
Characteristics of takayasu
- Young women, especially asian, <50yo
- Triphasic presentation
Constitutional: fever, arthralgia, weight loss
Vessel inflammation: pain, tenderness
Fibrotic: bruits, ischaemia
• Primarily granulomatous inflammation of aorta and its branches initially proximal subclavian artery then others
• Abdominal aorta effected eventually in 50%
• Aneurysmal dilatation or scarred stenosed vessel
• Renal involvement: Involvement of the renal arteries leads to
renovascular hypertension in > 50% cases
Investigations for takayasu
- elevated ESR
- Angiography: detects vascular stenosis
- Biopsy
Granulomatous inflammation
Infiltrate localised to outside (rather than inside for GCA)
T lymphocytes and NK cells
Treatment for takayasu
Steroids and methotrexate are first line.
Mycophenolate, azathioprine, cyclophsphamide or biologics also used
Sometimes for irreversible stenosis- Percutaneous transluminal
angioplasty or bypass grafts
Characteristics and symptoms of polyarteritis nodosa
- 40-60yo, M=F
Clinical Features
- Fever, constitutional symptoms
- Neuropathy - normally mononeuritis multiplex, sensorimotor polyneuropathy
- Rash - livedo reticularis
- Renal failure
- Renal HTN (don’t biopsy), GI involvement (especially Hep B), orchitis, iritis
- Lungs generally spared (can have infiltrates, nodules)
- Livedo reticularis, haematuria, renal failure
Bloods - ANCA negative (if MPO thinking of MPA) - Hep B* - HIV
Imaging: MESENTERIC or RENAL angiography would show stenosis and microaneurysms
Histo: focal segmental necroitising vasculitis of medium arteries (fibrinoid necrosis with predominantly neutrophils)
In PAN the Pulmonary Arter is Not involved, PANmural inflammation of the arterial wall is present and PAN is often associated with Hepantitis B.
Treatment of PAN
- PAN (no HBV): steroids, cyclophosphamide, azathioprine, methotrexate, mycophenolate, infliximab
HBV associated PAN:
- Prednisone to control symptoms (lower dose with rapid taper)
- Plasmapheresis (to remove immune complexes)
- Anti-viral agents
Prognosis very poor without therapy
Characteristicis of thromboangiitis obliterans (Buerger disease)
- Men <45 yo
- Inflammatory, obliterative, nonatheromatous vascular disease (small and medium arteries)
- Characterised by inflammatory thombus formation
- Strong association with tobacco use
- Intermittent claudication due to severe limb ischaemia, acute necrosis of toes and fingers often requiring amputation
- Superficial thrombophlebitis and Raynaud phenomenon in 40%
- Angiography (confirmatory test: non atherosclerotic segmental occlusion of arteries - focal stenosis or occlusion with otherwise normal proximal vessels
- Tx: stop smoking, vasodilators (eg: calcium channel blocker, sildenafil, iloprost)
55yo female, non smoker diagnosed with systemic vasculitis affecting medium sized arterities. Biopsy shows transmural inflammatory infiltrate without giant cells and fibinoid necrosis of the media. Normal upper airway and aortic arch. Most likely dx A. HSP B. Temporal arteritis C. Polyarteritis nodosa D. Thromboangiitis obliterans E. Granulomatosis with polyangiitis
C. Polyarteritis nodosa
