Medicine chapters
Vasculitis syndromes (HSP, PAN, hypersensitivity vasculitis, granulomatosis with polyangiitis, giant cell arteritis, Takayasu, Buerger disease)
Inflammation of blood vessel walls, classified by vessel size: giant cell and Takayasu arteritis (large), polyarteritis nodosa (medium), granulomatosis with polyangiitis, Henoch-Schonlein purpura and hypersensitivity vasculitis (small), and Buerger disease in young smokers.
- Vasculitis is classified by vessel size: large (giant cell arteritis over 50, Takayasu under 50), medium (polyarteritis nodosa, Kawasaki), small (ANCA-associated: GPA, MPA, EGPA; immune complex: HSP, hypersensitivity, cryoglobulinaemic).
- Giant cell arteritis: elderly, headache, scalp tenderness, jaw claudication, very high ESR; give high-dose steroids at once to prevent blindness, then biopsy.
- GPA = nose/sinus + lung + kidney with c-ANCA (PR3); PAN = medium arteries, hepatitis B, microaneurysms, lungs spared, ANCA negative; HSP = child, palpable purpura on buttocks and legs, arthritis, abdominal pain, IgA nephritis; Buerger = young male smoker, distal ischaemia; stop smoking.
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Overview
Vasculitis means inflammation of blood vessel walls. Inflamed vessels narrow, block, bleed or form aneurysms, so the organs they supply become ischaemic. Most systemic vasculitides cause fever, fatigue, weight loss, arthralgia and a raised ESR, plus organ-specific features.
The key to exam questions is the vessel size and the ANCA status.
Classification (Chapel Hill, simplified)
| Vessel size | Disease | Key clue |
|---|---|---|
| Large (aorta and main branches) | Giant cell (temporal) arteritis | Over 50, headache, jaw claudication |
| Takayasu arteritis | Under 50, young woman, pulseless arms | |
| Medium (main visceral arteries) | Polyarteritis nodosa | Hepatitis B, microaneurysms, spares lungs |
| Kawasaki disease | Child under 5, coronary aneurysms | |
| Small, ANCA-associated | Granulomatosis with polyangiitis (GPA, Wegener) | ENT + lung + kidney, c-ANCA |
| Microscopic polyangiitis (MPA) | Lung + kidney, p-ANCA, no granulomas | |
| Eosinophilic GPA (Churg-Strauss) | Asthma + eosinophilia, p-ANCA | |
| Small, immune complex | IgA vasculitis (Henoch-Schonlein purpura) | Child, purpura, IgA |
| Hypersensitivity (cutaneous leucocytoclastic) vasculitis | Drug or infection, purpura on legs | |
| Cryoglobulinaemic vasculitis | Hepatitis C | |
| Anti-GBM disease (Goodpasture) | Lung haemorrhage + nephritis | |
| Variable | Behcet disease; Buerger disease (thromboangiitis obliterans) | Ulcers; young smokers |
Bigger vessel = older patient (except Takayasu): giant cell in grandparents, Takayasu in young women, Kawasaki in toddlers.
Large-vessel vasculitis
Giant cell (temporal) arteritis
Who: almost always over 50; women more than men. Strongly linked with polymyalgia rheumatica (PMR).
Pathology: granulomatous inflammation of the intima and media of large arteries (branches of the external carotid, ophthalmic artery, aorta) with giant cells, CD4 T cells and macrophages, and fragmentation of the internal elastic lamina. Lesions are patchy (skip lesions).
Clinical features
- New headache, usually temporal.
- Scalp tenderness (painful hair combing).
- Jaw claudication (pain on chewing): the most specific symptom.
- Thickened, tender, nodular temporal artery with a weak or absent pulse.
- Visual loss: sudden, painless, in one eye, from anterior ischaemic optic neuropathy (posterior ciliary/ophthalmic artery occlusion). May be preceded by amaurosis fugax or diplopia.
- Systemic: fever, malaise, weight loss; PMR symptoms.
- Aortic involvement: aneurysm, aortic regurgitation, arm claudication.
Polymyalgia rheumatica: over 50, sudden pain and stiffness of shoulder and hip girdles, worse in the morning; no true weakness; raised ESR/CRP; CK normal; dramatic response to low-dose prednisolone (about 15 mg daily) within 1–2 days.
Investigations
- ESR very high (often 50–120 mm/h) and high CRP; normocytic anaemia; raised ALP.
- Temporal artery biopsy: definitive test. Take a long segment (over 1 cm) because of skip lesions; a negative biopsy does not exclude GCA. Do it within about 7 days of starting steroids (findings persist).
- Temporal artery ultrasound ("halo sign") increasingly replaces biopsy.
Management
- Start high-dose prednisolone immediately (60–100 mg daily in some regimens; IV methylprednisolone if vision is threatened). Do not wait for the biopsy.
- Taper slowly over 12–18 months, guided by symptoms and ESR/CRP.
- Low-dose aspirin, and bone protection (calcium, vitamin D, bisphosphonate) while on steroids.
- Steroid-sparing: tocilizumab (anti-IL-6 receptor), methotrexate.
In suspected GCA, give steroids first, then biopsy. Delay risks permanent blindness in the other eye.
An elderly patient with headache, jaw claudication and very high ESR = giant cell arteritis; the feared complication is blindness.
Takayasu arteritis ("pulseless disease", aortic arch syndrome)
- Young women (usually under 40–50), most common in Asia (including South Asia) and Japan.
- Granulomatous inflammation of the aorta and its main branches (subclavian, carotid, renal).
- Early (systemic) phase: fever, night sweats, weight loss, arthralgia, raised ESR.
- Late (occlusive) phase:
- Absent or weak pulses, usually in the arms; arm claudication.
- Unequal blood pressure between the two arms.
- Bruits over subclavian arteries, carotids or aorta.
- Hypertension (renal artery stenosis).
- Aortic regurgitation, dizziness, syncope, visual disturbance, stroke.
- Investigations: raised ESR/CRP; MR or CT angiography (or conventional angiography) shows long smooth stenoses, occlusions and aneurysms; PET shows active wall inflammation.
- Treatment: corticosteroids, with methotrexate, azathioprine or anti-TNF/tocilizumab as steroid-sparing agents; angioplasty or bypass surgery for fixed stenoses once inflammation is quiet; control hypertension.
A young woman with a difference in arm blood pressures, absent radial pulse and bruits has Takayasu arteritis, not aortic dissection or coarctation.
Medium-vessel vasculitis
Polyarteritis nodosa (PAN)
- Necrotising inflammation of medium-sized arteries, without glomerulonephritis, and sparing the lungs and capillaries.
- Usually middle-aged men. Classic link with hepatitis B (immune complexes).
- Pathology: fibrinoid necrosis with lesions of different ages; microaneurysms, thrombosis and infarction.
Clinical features
- Fever, malaise, weight loss, myalgia.
- Mononeuritis multiplex (e.g. foot drop) from arteritis of the vasa nervorum.
- Abdominal pain (mesenteric ischaemia, worse after meals), GI bleeding, bowel infarction.
- Kidney: renal artery involvement → hypertension, renal infarcts, haematuria and kidney impairment (but not glomerulonephritis).
- Skin: livedo reticularis, tender nodules, ulcers, digital gangrene.
- Heart: coronary arteritis → MI, heart failure.
- Testicular pain.
Investigations
- High ESR/CRP, anaemia, neutrophil leucocytosis.
- ANCA usually negative in classic PAN.
- Hepatitis B surface antigen.
- Angiography (mesenteric, renal, hepatic): microaneurysms and beading.
- Biopsy of affected tissue (skin, muscle, nerve).
Treatment: corticosteroids plus immunosuppression (cyclophosphamide or azathioprine) for severe disease. In hepatitis B-related PAN: antiviral therapy with a short course of steroids and plasma exchange.
PAN = medium arteries + hepatitis B + microaneurysms + mononeuritis multiplex + hypertension, with normal lungs and negative ANCA.
Small-vessel vasculitis
Granulomatosis with polyangiitis (GPA, formerly Wegener granulomatosis)
- Necrotising granulomatous vasculitis of small (and medium) vessels.
- Classic triad: upper airway, lower respiratory tract and kidney.
| Site | Features |
|---|---|
| Nose and sinuses | Chronic sinusitis, bloody nasal crusting, nasal septal perforation, saddle-nose deformity, otitis media, deafness |
| Lungs | Cough, haemoptysis, pleuritic pain; multiple nodules that cavitate, infiltrates, alveolar haemorrhage |
| Kidney | Rapidly progressive (crescentic) glomerulonephritis: haematuria, red cell casts |
| Others | Eye (scleritis, proptosis from orbital mass), skin purpura, mononeuritis multiplex, subglottic stenosis |
- Investigations: c-ANCA (cytoplasmic, anti-proteinase 3) in most active cases; very high ESR (often over 100 mm/h); urine for blood and casts; chest X-ray/CT; biopsy (nose, lung or kidney) shows necrotising granulomas and vasculitis; kidney shows pauci-immune crescentic GN.
- Treatment: induction with high-dose corticosteroids plus cyclophosphamide or rituximab; plasma exchange for severe kidney failure or lung haemorrhage; maintenance with azathioprine, methotrexate or rituximab. Untreated, it is usually fatal.
c-ANCA = Classic Wegener (GPA); p-ANCA = Polyangiitis (microscopic) and Churg-Strauss.
Related ANCA vasculitides
- Microscopic polyangiitis (MPA): lungs (alveolar haemorrhage) and kidneys (GN) like GPA, but no granulomas and no ENT disease; p-ANCA (anti-MPO).
- Eosinophilic granulomatosis with polyangiitis (Churg-Strauss): late-onset asthma, allergic rhinitis, blood eosinophilia, then vasculitis (neuropathy, skin, heart); p-ANCA in some.
IgA vasculitis (Henoch-Schonlein purpura, "anaphylactoid purpura")
- The commonest vasculitis of childhood; also occurs in adults (often more severe kidney disease).
- Often follows an upper respiratory tract infection. IgA immune complexes deposit in small vessels of skin, gut, joints and kidney mesangium.
Tetrad
- Palpable purpura on the buttocks and extensor surfaces of the legs, with normal platelet count.
- Arthritis/arthralgia (knees, ankles).
- Abdominal colic, GI bleeding; risk of intussusception.
- Glomerulonephritis: haematuria, proteinuria (like IgA nephropathy); occasionally nephrotic or nephritic syndrome.
- Investigations: platelets and clotting normal (rules out ITP); urine dipstick and BP for kidney involvement; serum IgA may be high; skin biopsy shows leucocytoclastic vasculitis with IgA; kidney biopsy shows mesangial IgA.
- Treatment: usually self-limiting within weeks: rest, analgesia (paracetamol). Steroids for severe abdominal pain; immunosuppression for severe nephritis. Follow urine and BP for months because kidney disease can appear late.
Purpura with a normal platelet count in a child = HSP (vasculitis). Purpura with low platelets = ITP or leukaemia.
Hypersensitivity vasculitis (cutaneous leucocytoclastic vasculitis)
- Small-vessel vasculitis of dermal post-capillary venules, mostly limited to the skin.
- Triggers: drugs (penicillins, sulfonamides, allopurinol, thiazides), infections (streptococcus, hepatitis B and C), connective tissue disease, malignancy; serum sickness is a systemic form.
- Palpable purpura on the lower legs appearing a week or so after a new drug, sometimes with fever, arthralgia and mild GN.
- Biopsy: leucocytoclastic vasculitis (neutrophils and nuclear dust around venules).
- Treatment: stop the drug or treat the cause; usually settles in weeks; antihistamines/NSAIDs; short steroid course if severe.
Buerger disease (thromboangiitis obliterans)
- Young men (often under 30) who smoke heavily; common in South Asia and the Middle East.
- Segmental inflammatory thrombosis of small and medium arteries and veins of the hands and feet (radial, ulnar, tibial, plantar arteries).
- Features:
- Claudication of the foot (instep) or hand, then rest pain.
- Ischaemic ulcers and gangrene of the fingers and toes.
- Migratory superficial thrombophlebitis.
- Raynaud phenomenon.
- Investigations: angiography shows segmental occlusions of distal vessels with "corkscrew" collaterals and normal proximal arteries without atheroma.
- Treatment: complete and permanent smoking cessation (including other tobacco); this halts but does not reverse the disease. Supportive: foot care, analgesia, prostacyclin analogue (iloprost), sympathectomy. Amputation if gangrene progresses.
In Buerger disease, the only treatment that changes the outcome is stopping smoking.
General principles of treatment
| Severity | Treatment |
|---|---|
| Skin only | Stop trigger; observe; short steroids if needed |
| Organ or life-threatening | High-dose steroids + cyclophosphamide or rituximab; plasma exchange in some |
| Maintenance | Azathioprine, methotrexate, rituximab; slow steroid taper |
| Infection-related | Treat the virus (hepatitis B in PAN; hepatitis C in cryoglobulinaemia) |
Expect: classification by vessel size; GCA in the over-50s with jaw claudication, scalp tenderness, very high ESR and risk of blindness, treated with steroids before biopsy; PMR responding to low-dose prednisolone; Takayasu in young women with unequal arm pulses and BP; PAN with hepatitis B, microaneurysms, mononeuritis multiplex and spared lungs; GPA with sinusitis, cavitating lung nodules, GN and c-ANCA; MPA and EGPA with p-ANCA; HSP with palpable purpura on buttocks and legs, normal platelets, abdominal pain and IgA nephritis; drug-induced leucocytoclastic vasculitis; and Buerger disease in young smokers treated by stopping smoking.
Quick revision
- Large: GCA (over 50), Takayasu (young women). Medium: PAN, Kawasaki. Small: ANCA (GPA, MPA, EGPA) and immune complex (HSP, hypersensitivity, cryoglobulin).
- GCA: jaw claudication, ESR high, blindness; steroids now, biopsy later.
- Takayasu: pulseless arms, BP difference, bruits; steroids, bypass.
- PAN: hepatitis B, microaneurysms, mononeuritis multiplex, hypertension; lungs spared; ANCA negative.
- GPA: nose + lung + kidney; c-ANCA (PR3); steroids + cyclophosphamide or rituximab.
- HSP: child, palpable purpura (normal platelets), arthritis, colic, IgA GN.
- Hypersensitivity vasculitis: drug, leg purpura; stop the drug.
- Buerger: young male smoker, distal gangrene, thrombophlebitis; stop smoking.
Written by The House Job from standard references (Kumar and Clark's Clinical Medicine; Bailey and Love's Short Practice of Surgery).
FAQ
Should treatment wait for the temporal artery biopsy in suspected GCA?
No. High-dose steroids are started at once to protect vision; the biopsy stays positive for several days after starting treatment.
Why are the lungs important in telling PAN from GPA?
PAN involves medium arteries and spares the lungs, while GPA commonly causes lung nodules, cavities and haemorrhage along with sinus and kidney disease.
What does c-ANCA target?
Proteinase 3 in neutrophil granules; it is typical of granulomatosis with polyangiitis.
How is HSP purpura told apart from ITP?
HSP purpura is palpable, sits on the buttocks and legs, and the platelet count is normal; in ITP the platelet count is low.
Can Buerger disease be cured with drugs?
No drug replaces stopping smoking; complete abstinence from tobacco is essential to halt the disease and avoid amputation.