Home | | Medicine Study Notes | Vasculitis - Connective Tissue Diseases

Chapter: Medicine Study Notes : Musculo-Skeletal

Vasculitis - Connective Tissue Diseases

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



·        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




·        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

Study Material, Lecturing Notes, Assignment, Reference, Wiki description explanation, brief detail
Medicine Study Notes : Musculo-Skeletal : Vasculitis - Connective Tissue Diseases |

Privacy Policy, Terms and Conditions, DMCA Policy and Compliant

Copyright © 2018-2024 BrainKart.com; All Rights Reserved. Developed by Therithal info, Chennai.