Transcript Vasculitis and Connective Tissue Disease

Vasculitis and connective
tissue disease – just a taster!!
The common and the rare!!
Polymyalgia rheumatica (PMR)
• Common clinical syndrome of middleaged & elderly (2-3 / GP)
• characterized by pain & stiffness neck,
shoulder & pelvic girdles
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• dramatic response to small doses of
steroids
Giant cell arteritis
• vasculitis underlying polymyalgia
rheumatica and temporal arteritis
• Other terms cranial arteritis &
granulomatous arteritis
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• Early recognition & treatment can
prevent blindness & other
complications resulting from occlusion
or rupture of involved arteries
Polymyalgia rheumatica
• musculoskeletal symptoms usually bilateral & symmetric
• Stiffness predominant feature; particularly EMS may prevent patient
getting out of bed
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• Muscular pain often diffuse; pain at night common
• Corticosteroid treatment usually required for at least 2 years.
Sometimes 4-5 years
• Systemic features include low-grade fever, fatigue, weight loss & an
increased erythrocyte sedimentation rate
• Prednisolone 20 – 30mg / day ( tapering over 2 years or
so)
Giant cell arteritis
• wide range of symptoms, clinical findings
related to involved arteries
• Frequently fatigue, headaches, jaw
claudication, loss of vision, scalp
tenderness, AND polymyalgia rheumatica
• Risk is loss of vision – this is a medical
emergency!
• Prednisolone 40 – 60 mg / day ( tapering over 2
years or so)
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Figure 157.2 Granulomatous vasculitis. (a) Brain biopsy findings of granulomatous vasculitis side by side with a polyarteritis-type necrotizing arteritis (open arrow). (b)
Close-up view of (a) showing foreign body (short arrow) & foreign body (long arrow) giant cells in granulomatous vasculitis. Hematoxylin & eosin, × 64 & 400
respectively. (With permission from Calabrese et al. 4)
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'vasculitis'
• heterogeneous group of disorders characterized
inflammatory alteration of blood vessel wall
• primary event (primary vasculitis) - e.g. in ANCAassociated vasculitides
• or secondary to other diseases (secondary
vasculitis), e.g. in RA or SLE
• underlying events mediated by immunologic
mechanisms
• supported by response to various potent
immunosuppressives
• Aggressive treatment to prevent permanent organ
damage
Few clues to aetiology of primary
vasculitides
• Hep B virus implicated as causative agent in
polyarteritis nodosa (PAN) & Hep C in essential mixed
cryoglobulinemia (EMC)
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• circumstantial evidence for causative role of infection in
Wegener's granulomatosis: seasonal variation in
incidence of proteinase 3 autoantibodies (PR3-ANCA)
suggests that an infectious agent prevalent in spring or
autumn might trigger WG
• genetic predisposition suggested by studies showing link
between small vessel vasculitis & HLA-DQw7, between
WG & DR1, & between HLA-Bw52 & giant cell arteritis
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To name a few……..
Giant cell arteritis
Takayasu arteritis
Polyarteritis nodosa
Kawasaki disease
Wegeners Granulomatosis
Churg-Strauss Syndrome
Microscopic polyangiitis
Henoch-Schonlein purpura
Essential cryoglobulinaemic vasculitis
Behcets disease
Cutaneous leukocytoclastic vasculitis
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Figure 155.6 Leukocytoclastic vasculitis with ulceration in a patient with rheumatoid arthritis.
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Figure 146.4 Polyarteritis involving the gallbladder artery, showing pleomorphic inflammatory cell infiltration & fibrinoid necrosis. (With permission from Conn 10.)
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Figure 146.6 Vasculitis pathogenesis
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Connective tissue disease
• group of conditions which affect many organs or tissues
• widespread inflammation
• Have some symptoms in common
• All patients have features of general inflammation : fever,
weight loss, tiredness & anaemia
• Most patients also have inflammation of joints
• Vasculitis of small blood vessels also common
• Raynaud’s phenomenon
• Involvement of the kidney may result in hypertension
• If inflammation settles all symptoms improve
• However, if process continues scarring occurs in
affected tissues - will be damaged permanently
• As well as symptoms common to whole
group, each CTD has own particular
symptoms
• All can be distinguished clinically &
serologically
• All these CTD are rare
• All more common in women
• All treated with immunosuppressant drugs
• SCLERODERMA; excessive fibrosis of tissues; skin is
hard, & loses its ability to stretch. The fingertips are
thickened & skin around the mouth tightens
• POLYMYOSITIS; inflammation mainly in muscles; pain &
weakness are main symptoms. Lung fibrosis
• SLE; skin, joints, fatigue, mouth ulcers, alopecia,
glomerulonephritis, lung fibrosis, CNS involvement
• Sjogrens; sicca symptoms, joints, fatigue, lymphoma risk
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• Also may have symptoms from different disorders;
MIXED CONNECTIVE TISSUE DISEASE
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Figure 122.6 Mouth ulcers in a patient with SLE.
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