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vasculitis

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vasculitis

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vasculitis

  1. 1. Seminar-Vasculitis Abdul Waris Khan
  2. 2. Definition • Vasculitis is a histological term describing inflammation of the vessel wall. • Characterized by widespread vasculitis leading to systemic symptoms and signs, generally requiring treatment with corticosteroids and/ or immunosuppressive drugs. • Two main features are helpful in classifying these vasculitides:- – The size of the blood vessels involved – The presence or absence of anti-neutrophil cytoplasmic antibodies (ANCA) in the blood
  3. 3. Types
  4. 4. Clinical features
  5. 5. PMR & GCA • Polymyalgia rheumatica (PMR) and giant cell (temporal) arteritis are systemic illnesses of the elderly. • Both are associated with the finding of a giant cell arteritis on temporal artery biopsy.
  6. 6. Polymyalgia rheumatica (PMR)  PMR causes a sudden onset of severe pain and stiffness of the shoulders and neck, and of the hips and lumbar spine.  These symptoms are worse in the morning, lasting from 30 minutes to several hours.  Clinical history is usually diagnostic and the patient is always over 50 years old.  Approximately one-third of patients develop systemic features of tiredness, fever, weight loss, depression and occasionally nocturnal sweats.
  7. 7. Investigation of PMR  A raised ESR and/or CRP is a hallmark of this condition.  Serum alkaline phosphatase and γ-glutamyltranspeptidase may be raised as markers of the acute inflammation.  Anaemia often present.  Temporal artery biopsy shows giant cell arteritis in 10–30% of cases, but is rarely performed unless GCA is also suspected.
  8. 8. Giant cell arteritis  GCA is inflammatory granulomatous arteritis of large cerebral arteries which occurs in association with PMR.  The patient may have current PMR, a history of recent PMR, or be on treatment for PMR.
  9. 9. Clinical features of GCA  It is extremely rare under 50 years of age.  Presenting symptoms include:  Severe headaches  Tenderness of the scalp or of the temple  Claudication of the jaw when eating  Tenderness and swelling of one or more temporal or occipital arteries.  The most feared manifestation is sudden painless temporary or permanent loss of vision in one eye due to involvement of the ophthalmic artery.  Systemic manifestations of severe malaise, tiredness and fever occur.
  10. 10. Investigation of GCA • A temporal artery biopsy from the affected side is the definitive diagnostic test. This should be taken before, or within 7 days of starting, high doses of corticosteroids. • Anemia may be present • ESR is usually raised and the CRP very high. • Liver biochemistry: Abnormalities occur, as in PMR
  11. 11. Histological features of GCA  The histological features of GCA are:  Cellular infiltrates of CD4+ T lymphocytes, macrophages and giant cells in the vessel wall.  Granulomatous inflammation of the intima and media  Breaking up of the internal elastic lamina  Giant cells, lymphocytes and plasma cells in the internal elastic lamina.
  12. 12. Treatment of PMR or GCA  Corticosteroids produce a dramatic reduction of symptoms of PMR within 24–48 hours of starting treatment, provided the dose is adequate.  This treatment should reduce the risk of patients who have PMR developing GCA.  In GCA, corticosteroids are obligatory because they significantly reduce the risk of irreversible visual loss and other focal ischaemic lesions, but much higher doses are needed than in PMR.
  13. 13.  Sometimes biopsy is not obtainable, so the treatment should not be delayed, especially if there have already been episodes of visual loss or stroke.  PMR: 10–15 mg prednisolone as a single dose in the morning.  GCA: 60–100 mg prednisolone, usually in divided doses.  Most patients will eventually be able to stop corticosteroids after 12–18 months.  Calcium and vitamin D supplements and sometimes bisphosphonates are necessary to prevent osteoporosis while high-dose steroids are being used
  14. 14. Takayasu’s disease  This is rare, except in Japan.  It is of unknown aetiology and occurs in females.  There is a vasculitis involving the aortic arch as well as other major arteries.  There is also a systemic illness, with pain and tenderness over the affected arteries.  Absent peripheral pulses and hypertension are common.  Corticosteroids help relieve symptoms.  Treatment may require a surgical bypass to improve perfusion of the affected areas.  Eventually heart failure and strokes may occur but most patients survive for at least 5 years
  15. 15. Medium-sized vessel vasculitis
  16. 16. Polyarteritis nodosa (PAN)  Classical PAN is a rare condition which usually occurs in middle-aged men.  its occasional association with hepatitis B antigenaemia suggests a vasculitis secondary to the deposition of immune complexes.  Pathologically, there is fibrinoid necrosis of vessel walls with microaneurysm formation, thrombosis and infarction.
  17. 17. Clinical features  These include fever, malaise, weight loss and myalgia. These initial symptoms are followed by dramatic acute features that are due to organ infarction.  Neurological: mononeuritis multiplex is due to arteritis of the vasa nervorum.  Abdominal: pain due to arterial involvement of the abdominal viscera, mimicking acute cholecystitis, pancreatitis or appendicitis. Gastrointestinal haemorrhage occurs because of mucosal ulceration.
  18. 18. Renal: presents with haematuria and proteinuria. Hypertension and acute/chronic kidney disease occur. Cardiac: coronary arteritis causes myocardial infarction and heart failure. Pericarditis also occurs. Skin: subcutaneous haemorrhage and gangrene occur. A persistent livedo reticularis is seen in chronic cases. Cutaneous and subcutaneous palpable nodules occur, but are uncommon. Lung: involvement is rare.
  19. 19. Investigations  Blood count: Anaemia, leucocytosis and a raised ESR occur.  Angiography: Demonstration of microaneurysms in hepatic, intestinal or renal vessels if necessary.  Other investigations: as appropriate (e.g. ECG and abdominal ultrasound), depending on the clinical problem. ANCA is positive only rarely in classic PAN.
  20. 20. Treatment Is with corticosteroids, usually in combination with immunosuppressive drugs such as azathioprine.
  21. 21. Kawasaki’s disease • This is an acute systemic vasculitis involving medium-sized vessels, affecting mainly children under 5 years of age. • It is very frequent in Japan, and an infective trigger is suspected. • It occurs worldwide and is also seen in adults.
  22. 22. Clinical features  Fever lasting 5 days or more  Bilateral conjunctival congestion 2–4 days after onset  Dryness and redness of the lips and oral cavity 3 days after onset  Acute cervical lymphadenopathy accompanying the fever  Polymorphic rash involving any part of the body  Redness and oedema of the palms and soles 2–5 days after onset
  23. 23. Diagnosis  The persistent fever plus at least 4/5 features should be present to make the diagnosis, or < 4 if coronary aneurysms can be seen on two dimensional echocardiography, MRI or angiography.  Cardiovascular changes in the acute stage include pancarditis and coronary arteritis leading to aneurysms or dilatation.  Anti-endothelial cell autoantibodies are often detectable.  Other features include diarrhoea, albuminuria, aseptic meningitis and arthralgia and, in most, there is a leucocytosis, thrombocytosis and a raised CRP.
  24. 24. Treatment • Is with a single dose of high-dose intravenous immunoglobulin (2 g/kg), which prevents the coronary artery disease, followed after the acute phase by aspirin 200–300 mg daily. • There is no evidence that steroid treatment improves the outcome.
  25. 25. Small vessel vasculitis
  26. 26. This can be separated into those that are positive or negative for anti-neutrophil cytoplasmic antibody (ANCA)
  27. 27. ANCA-positive small vessel vasculitis  Wegener’s granulomatosis  Churg–Strauss granulomatosis  Microscopic polyangiitis ANCA-negative small vessel vasculitis  Henoch–Schönlein purpura  Cryoglobulinaemic vasculitis  Cutaneous leucocytoclastic vasculitis
  28. 28. ANCA-positive small vessel vasculitis
  29. 29. Wegener’s granulomatosis  It is characterized by lesions involving the upper respiratory tract, lungs and kidneys.  It often starts with severe rhinorrhoea, with subsequent nasal mucosal ulceration followed by cough, haemoptysis and pleuritic pain.  Etiology is unknown  Single or multiple nodular masses or pneumonic infiltrates with cavitation are seen on chest X-ray.  The typical histological changes are usually best seen on renal biopsy, which shows necrotizing microvascular glomerulonephritis.  This disease responds well to treatment with cyclophosphamide 150–200 mg daily.  Rituximab is also being used.  A variant of Wegener’s granulomatosis called ‘midline granuloma’ affects the nose and paranasal sinuses and is particularly mutilating; it has a poor prognosis.
  30. 30. Churg–Strauss syndrome  This condition classically occurs in males in their 4th decade, who present with rhinitis and asthma, eosinophilia and systemic vasculitis.  The aetiology is uncertain.  Typically, it involves the lungs, peripheral nerves and skin, but renal involvement is uncommon.  Transient patchy pneumonia-like shadows may occur.  Skin lesions include tender subcutaneous nodules as well as petechial or purpuric lesions.  ANCA is usually positive.  The disease responds well to corticosteroids.
  31. 31. Microscopic vasculitis (polyangiitis) This involves the kidneys and the lungs where it results in recurrent haemoptysis. ANCA is usually positive.
  32. 32. ANCA-negative small vessel vasculitis
  33. 33. Henoch–Schönlein Purpura • This clinical syndrome comprises a characteristic skin rash, abdominal colic, joint pain and glomerulonephritis. • Approximately 30–70% have clinical evidence of renal disease with haematuria and/or proteinuria • The renal lesion is a focal segmental proliferative glomerulonephritis, sometimes with mesangial hypercellularity. • In more severe cases, epithelial crescents may be present. • Immunoglobulin deposition is mainly IgA in the glomerular mesangium distribution, similar to IgA nephropathy. • There is no treatment of proven benefit; steroid therapy is ineffective. • Treatment is usually supportive but with crescentic GN aggressive immunosuppression has been tried with variable outcome.
  34. 34. Leucocytoclastic vasculitis • (LCV) is the most common cutaneous vasculitis affecting small vessels. • This usually appears on the lower legs as a symmetrical palpable purpura. • It can be caused by drugs (15%), infection (15%), inflammatory disease (10%) or malignant disease (<5%) but often no cause is found (55–60%). • Investigations are only necessary with persistent lesions or associated signs and symptoms. • LCV often settles spontaneously, treatment with analgesia, support stockings, dapsone or prednisolone may be needed to control the pain and to heal up any ulceration.
  35. 35. References • Kumar & Clark's clinical medicine 8th edition

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