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Chapter: Medicine Study Notes : Musculo-Skeletal

Vasculitis - Connective Tissue Diseases

Occurs in non-organ specific autoimmune diseases (eg RA, SLE)

Vasculitis

 

·        Associations:

o   Occurs in non-organ specific autoimmune diseases (eg RA, SLE)

o   Principal feature of other connective tissue diseases that may or may not be autoimmune

o   Also occurs in conditions not usually included in connective tissue diseases (eg drug reactions)

·        Types:

o   Large vessel vasculitis: Giant cell arteritis, Takayasu‟s arteritis

o   Medium sized vessels: Polyarteritis Nodosa (PAN), Kawasaki‟s disease 

o   Small vessel vasculitis: Wegener‟s Granulomatosis, Microscopic Polyarteritis, Henoch-Schonlein purpura

 

Giant Cell arteritis/Temporal Arteritis

 

·        Medium and small arteries (especially temporal arteries ® medical emergency – affects retinal arteries).

 

·        From 50 years, peaking at 75.

 

·        Clinical: Initially persistent headache, then superficial pain and tenderness over temporal arteries, unilateral visual disturbance, arthritic pain, jaw claudication, fever, malaise, ­ESR (Age and ESR both over 60 in 3/4 cases)

 

·        Immune reaction with internal elastic lamina

 

·        Diagnosed by biopsy showing giant cells engulfing the IEL and inflamed media. Biopsy is critical as treatment should continue for 2 years and therefore want to be sure of diagnosis

 

·        Presumptive treatment with steroids. Immediate risk is blindness, but longer-term morbidity is due to steroid treatment!

 

·        Overlaps with Polymyalgia Rheumatica in 25% of cases (® stiff proximal muscles in the morning). 

 

Polyarterititis nodosa

 

·        Affects young adults.  M:F = 4:1.

·        Presents with non-specific symptoms – fever malaise, abdominal pain, renal failure, purpura.

·        Immune complex mediated arteritis (type 3 hypersensitivity)

·        40% associated with Hep B 

·        Necrotising vasculitis of medium sized arteries (not arterioles). Involves smaller arteries – kidney, heart, liver, GI. Often patchy distribution. Macroscopic: small nodules. Microscopic: fibrinoid necrosis, intimal proliferation, media destruction, inflammation of adventitia, scarring if chronic. 

·        Investigations: FBC, biopsy of affected organ, ECG, ANCA may be +ive

·        Treatment: steroids/immunosuppressives (azathioprine/cyclophosphamide)

 

Kawasaki Disease

 

·        = Mucocutaneous Lymph Node Syndrome ~ Childhood Polyarteritis Nordosa

·        Immune mediated injury to vascular endothelium, including coronary artery arteritis

·        ?post viral 

·        Fever in kids (usually < 5) for > 5 days with bilateral, non-purulent conjunctival infection, oral mucosal changes, cervical lymphadenopathy, changes in the extremities (eg swelling of hands), & generalised rash 

·        Investigations: Echo for coronary aneurysm, FBC (­WBC, ­platelets), ­CRP 

·        Differential: Scarlet fever, EBV

·        Complications: pancarditis, aneurysms or dilatation

·        Treatment: none, or high dose IgG/steroids


Wegener’s Granulomatosis

 

·        Generalised necrotising arteritis of small-medium sized arteries of the respiratory tract and kidney with non-caseating granuloma formation 

·        Wegner‟s triad: aseptic necrosis of the lower and upper respiratory tract and focal glomerulonephritis of the kidney

·        Presentation: 

o  Upper airways disease (chronic rhinitis/epistaxis/sinusitis/mouth ulcers) unresponsive to therapy. CXR shows spots. May have haemoptysis. Progresses to ulceration of nasal mucosa, perforation of the septum, heavy nose bleeds, granulomatous invasion of large bronchi ® bronchial stenosis

o  Glomerulonephritis.  If untreated then slow progression to end stage renal failure

o  Systemic: fever, night sweats, weight loss, et

o  Non-deforming arthritis and arthralgia

·        Progression highly variable

·        Investigations: 

o  ­ESR, c-ANCA positive, CXR (nodular masses, cavitation)

o  Renal biopsy: necrotising glomerulonephritis: may be focal and crescentic.  Immunoflouresence is   –ive Þ pauci-immune 

·        Treatment: steroids +/- cyclophosphamide ® 90% remission but frequent relapse. Continue for a year then taper off

 

Microscopic Polyarteritis

 

·        Vasculitis of small-medium sized vessels

·        Multisystem involvement including glomerulonephritis 

·        Kidney involvement: crescentic rapidly progressive GN common, no immune deposits on immunoflouresence (ie pauci immune)

·        Biopsy: fibrinoid necrosis and cellular proliferation within capillaries

·        Frequently positive for p-ANCA

·        Treatment: similar to Wegener‟s

 

Henoch-Schonlein Purpura

 

·        Leukocytoclastic vasculitis of small vessels with deposition of IgA immune complexes in the skin, gut and kidney

·        Usually in young children, associated with URTIs

·        Palpable purpuric rash over the buttocks and ankles, abdominal pain and arthralgia 

·        Renal involvement: macroscopic or microscopic vasculitis, mesangial proliferative glomerulonephritis, maybe crescentic, IF +ive for mesangial IgA deposition 

·        Usually self-limiting, otherwise steroids

 

Others

 

·        Hypersensitivity angiitis (Leukocytoclastic vasculitis): Type 3 immune injury.  Associated with


·        medicines, lupus, HBV.  Microscopically: neutrophils, fibrinoid necrosis

 

·        Takayasu’s arteritis: Aortic thickening with autoimmune granulomas = Pulseless Disease. Rare, in young females, hypertension, pain of affected artery

 

·        Thromboangiitis Obliterans = Buerger‟s disease. Neurovascular bundles  mainly in legs and arms of young/middle aged smokers – become inflamed and thrombosed

 

·        Behcet’s Disease: Systemic vasculitis, commoner in Turkey and Japan, oral and genital ulcers, eye lesions, arthritis of knee, ankles, wrists and elbows


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