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Vasculitis

Vasculitis. Joanna Zalewska. Definition. Group of a rare conditions characterized by inflammation of blood vessels. Classification. Epidemiology. Diseases of the extremes age Kawasaki diseases and Henoch- Schoenlein purpura occur in childhood, whilst GCA occurs in those aged >65 years

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Vasculitis

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  1. Vasculitis Joanna Zalewska

  2. Definition • Group of a rare conditions characterized by inflammation of blood vessels

  3. Classification

  4. Epidemiology • Diseases of the extremes age • Kawasaki diseases and Henoch- Schoenlein purpura occur in childhood, whilst GCA occurs in those aged >65 years • The other types are much rare, but also tend to occur in older people

  5. Clinical features • In the early stages- the symptoms can be non-specific • The age is important (Takayasu, GCA) • Systemic- fever, weight loss, myalgia, arthralgia • ENT- epistaxis, nasal crusting, deaffness, sinusitis • Pulmonary- cough, haemoptysis, dyspnoea, wheeze • Late onset asthma- CSS • Cutaneous- purpuric rash, ulcers

  6. Neurological- peripheral neuropathy, headache- especially located in the temple, new onset or of a different character to previous haedache • Vascular- jaw claudication, limb claudication, carotidynia, difficulty in obtaining blood pressure readings

  7. Key featues on examination • Cutaneous- purpuric rash, ulcers, digital gangrene • Musculoskeletal- synovitis • Neurological- motor or sensory neuropathy, mononeuritis multiplex • Vascular- tender non-pulstile temporal arteries, absent peripheral pulses, bruits, difficulty in obtaining BP measurement • ENT- nasal bridge collapse

  8. General investigations • FBC anaemia, leucocytosis, eosinophilia • Acute phase response (ESR and CRP) • Liver function

  9. Assessment of organ involvement • Urinalysis (proteinuria, haematuria, red cell casts) • Renal function (creatinine clearance, quantification of protein leak if present using either 24-h protein excretion or urine/ creatinine ratio) • CXR- infiltrates, haemorrhage, granuloma • Liver function • Nervous system • Cardiac function (ECG, echocardioography) • Gut (celiac axis angiography)

  10. Serological investigations • cANCA- strongly associated with WG (>90%) • pANCA- MPA and CSS • ANA • RF- may be positive in cryoglobulinaemic vasculitis or systemic rheumatoid vasculitis • Anticardiolipin antibodies • Complement (low in cryoglobulinaemic vasculitis) • Cryoglobulins

  11. Giant cell arteritis (GCA) • The most common of systemic vasculititides and is characterized by envolvement of large vessels particularly the extra cranial branches of the aorta • Often involves temporal artery, aorta and its major branches • Patients older than 50

  12. ACR criteria of GCA (>3 criteria) • Age onste >50 years • New headache • Temporal artery abnormality (tenderness to plaplation or decreased pulsation) • Increase in ESR > 50 mm/h • Abnormal artery biopsy- predominance of mononuclear infiltration or granulomatous inflammation

  13. Clinical features • In early stages the symptoms are non-specific (fever, weight loss, myalgia, morning stiffness) • Headache • Visual disturbance- suddenly, without warning and may be bilateral, transient visual loss • Jaw claudication- pain on eating • Arm or leg claudication

  14. Examination • The temporal arteries- tender, thickened and non pulsatile • Eye- ischaemic optic neuropathy

  15. Investigations • FBC anaemia, thrombocytosis • Acute phase response (ESR and CRP) • Temporal artery biopsy- as soon as possible • Ultrasonography • Angiography

  16. Prognosis • Blindness risk (reduced by early corticosteroids therapy) Treatment • High dose of corticosteroids starting 0.7- 1 mg/kg (60- 80 mg) oral • Methylprednisolone (15 mg/kg) for 3 days – 1 g per day • Low dose of aspirin- reduces the risk of thrombosis • Azathioprine or methotrexate

  17. Takayasu arteritis • Rare systemic, vasculitis of unknown aetiology, characterized by large vessel vasculitis • Granulomatous inflammation of the aorta and its major branches • Patients younger than 50

  18. Criteria >3 • Age <40 years old • Claudication of extremities- development and worsening of fatigue and discomfort in muscles of one or more extermity while in use, espacially the upper extremities • Decreased brachial arterialpulse • BP difference >10 mmHg in systolic BP between arms • Bruit over subclavian arteries or aorta • Arteriogram abnormality- narrowing or occlusion

  19. Occurence of stroke in a young person especcially when associated with a high ESR or CRP • Fever, weight loss, myalgia, arthralgia • Absence or asymmetry of peripheral pulses • Claudication of arms or legs • TIA and stroke

  20. Examination • BP inequality between arms and legs • Hypertension new onset or evidence of renal artery stenosis • Vascular bruits most often heard over the carotid artries • Carotidynia • Neurological symptoms

  21. FBC anaemia • ESR and CRP elevation • Angiography- gold standard- localized narrowing or irregularity of the lumen, stenosis and aneurysms • MR- angiography • PET • High resolution Doppler ultrasound • ANCA, ANA, RF, anticardiolipin antibodies- negative

  22. Remisson can be achieved in the majority of patients using corticosteroids, but 73% of patients required additional immunosuppressants

  23. Oral corticosteroids start at 1 mg/kg (maximum dose 60- 80 mg) and taper quickly; aim for 10 mg/day at 6 months • Immunosuppression with methotrexate or azthioprine should be started at the same time as oral corticosteroids • Control hypertension • Surgery- bypass procedure or stenting

  24. Wegener’s granulomatosis • Rare systemic vasculitis of unknown aetiology, characterized by involvement of the upper airways with granuloma formation • Granulomatous inflammation involving the respiratory tract and necrotizing vasculitis affectin small to medium sized vessels

  25. Criteria >2 • Nasal or oral inflammation- oral ulcers or purulent/ bloody nasal discharge • Abnormal chest radiograph- nodules, fixed infiltrates or cavities • Urinary sediment- microhaematuria (>5 red cells per high power field) or red cell casts in urinary sediment • Granulomatous inflammation- histological changes showing granulomatous on biopsy inflammation within the wall artery or in the perivascular or extravascular area

  26. Clinical features • Systemic- fever, weight loss, myalgia, arthralgia • ENT- epistaxis, nasal crusting, deaffness, sinusitis • Pilmonary- cough, haemoptysis, dyspnoea • Cutaneous- purpuric rash, ulcers • Peripheral neuropathy

  27. Skin- purpura, ulcers • Arthritis • Eye- scleritis, proptosis- retroorbital mass • ENT- nasal collapse, destructive sino-nasal disease

  28. Anaemia, leucocytosis, eosinophilia • ESR and CRP- elevation • Liver function • Urinalysis (proteinuria, haematuria, red cell casts) • Renal function (creatinine clearance, quantification of protein leak if present using either 24-h protein excretion or urine protein/creatinine ratio) • CXR- infiltrates, haemorrhage, granuloma • Nervous system- nerve conduction • Cardiac function- ECG, echocardiography • Skin- biopsy

  29. cANCA- strongly associated with WG >90% • ANA, RF, anticardiolipin antibodies, complement • cryoglobulins

  30. Differential diagnosis • Other types of vasculitis • Vasculitis mimics- malignancy, atrial myxoma, calciphylaxis • Infection • Blood cultures • Viral serology (HBV, HCV, HIV, CMV) • Echocardiography

  31. Churg- Strauss Syndrome • Eosinophil- rich and granulomatous inflammation involving the respiratory tract and necrotizing vasculitis affecting small to medium- sized vessels associated with asthma and eosinophilia • Male • Peak age onset- 65- 74 years • Aetiology is unknown- leukotriene inhibitors

  32. ACR Criteria >4 • Asthma- history of wheezing or diffuse high pitch rales on expiration • Eosinophilia>10 % on white cell differential count • Mononeuropathy or polyneuropathy- development of mononeuropathy, multiple menoneuropathies or polyneuropathy • Pulmonary infiltrates • Paranasal sinus abnormality- history of acute or chronic paranasolor tenderness • Extravascular eosinophilis- biopsy including artery

  33. Key points on history • Adult onset asthma- difficult to control • Systemic symptoms • Pulmonary- cough, wheeze, dyspnoea, upper respiratory tract symptoms • Cutaneous- purpuric rash or cutaneous granulomata occur in up to 50% • Neurological- peripheral neuropathy or mononeuritis multiplex • Cardiac- dyspnoea, cardiac failure, arrythmia

  34. Key points on examination • Skin- purpuric vasculitic rash or palpable granulomata • Musculoskeletal- arthritis • Ophtalmic- scleritis • ENT- nasal polyps • Neurological- mononeuritis multiplex, sensory or motor neuropathy

  35. Anaemia, leucocytosis, eosinophilia >1.5 x109 • ESR and CRP- elevation • Liver function • ANCA- 50% of patients are ANCA positive

  36. Assessment of organ involvement • Urinalysis (proteinuria, haematuria, red cell casts) • Renal function (creatinine clearance, quantification of protein leak, 24-h protein excretion or urine protein/ creatinine ratio) • CXR- infiltrates, haemorrhage, granuloma • Liver function • Nervous system • Cardiac function • Skin- biopsy

  37. Differential diagnosis • Other causes of hypereosinophilia- hypereosinophilic syndrome, eosinophilic leukaemia, chronic parasitic infection

  38. Treatment • Induction, consolidation, maintenance of remission • immunosuppression

  39. Microscopic polyangiitis • Necrotizing vasculitis, with few or no immune deposits affecting small vessels

  40. Key features on history • Systemic- fever, weight loss, myalgia, arthralgia • Pulmonary- cough, haemoptysis, dyspnoea • Renal involvement • Cutaneous- purpuric rash • Neurological- peripheral neuropathy

  41. Key features on examination • Cutaneous- purpuric rash, digital ischaemia, ulcers, livedo reticularis • Neurological- mononeuritis multiplex • Pulmonary- intersitial lung disease

  42. Assessment of organ involvement • Urinalysis (proteinuria, haematuria, red cell casts) • Renal function (creatinine clearance, quantification of protein leak, 24-h protein excretion or urine protein/ creatinine ratio) • CXR- infiltrates, haemorrhage, granuloma • Liver function • Nervous system • Cardiac function • Skin- biopsy

  43. Five factor score • Proteinuria >1g/ 24h • Serum creatinine> 140 umol/l • Gastrointestinal involvement • Cardiomyopathy • CNS involvement

  44. Treatment- vasculitis • Induction- oral corticosteroids combined with methotrexate, mycophenolate mofetil or cyclophosphamide • Prophylaxis against pneumocystis carinii, PPI against gastric ulceration, Mesna against haemorrhagic cystitis, prophylaxis against osteoporosis • Plasma exchange • Azathioprine

  45. Polyarteritis nodosa • Necrotizing inflammation of medium- sized or small arteries without glomerulonephritis or vasculitis in capillaries or venules

  46. Criteria • Weight loss • Livedo reticularis • Testicular pain or tenderness • Myalgias, weakness or leg tenderness • Mononeuropathy or polyneuropathy • Diastolic BP>90 mmHg • Elevated blood urea or creatinine • Hepatitis B virus • Arteriographic abnormality • Biopsy of small or medium vessel

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