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Summary Connective Tissue Diseases
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Connective Tissue Diseases Summary
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Inflammatory Muscle DiseaseImmune system attacks muscles inflammation
Polymyositis – inflammation of the proximal muscles (35+)
Dermatomyositis – PM + skin involvement (5-15, 35+)
Inclusion body myositis – both proximal and distal muscles
Sx
1. Proximal muscle weakness
2. Dysphagia, asphyxiation – if oesophageal muscles are effect
3. Respiratory muscle damage, failure
4. Gottron’s papules (on hands), heliotrope rash (on upper eyelids) – Dermatomyositis
Dx
Gold standard: Muscle biopsy
CK will be elevated (would also be elevated in rhabdo)
Anti-jo1, anti-mi2
DM often has a solid organ malignance
Can do EMG
DDx –
Fibromyalgia – café au lait spots
PMR – girdle pain, associated with temporal arteritis, high ESR
Hypothyoid/steroid induced myopathy
SCLERODERMA
Immune system attacks skin’s connective tissue – elastin becomes replaced with fibrin resulting in
thickening and hardening of the skin
Limited SSC – inflammation of just the skin, usually peripheral to proximal, more common,
CREST ++
Diffuse SSC – earlier internal organ involvement, less common and more deadly
Cutaneous Manifestations
Thickening of hardening of skin
Sclerodactyly
Atrophy
Telangiectasia
Face of no expression
Vascular
Raynaud’s
Thromboemboli
CREST – Calcinosis cutis, raynaud’s, oesophageal dysmotility, sclerodactyly, telangiectasia
Internal organs –
Lung – Pulmonary fibrosis
MSK: Arthralgia
Kidney: Scleroderma renal crisis due to collagen deposition
Heart: restrictive CM
Dx – UEC, renal US, echo, antibody testing (anti-centromere – limited, anti-scl70 – diffuse)
SJOGREN’S SYNDROME
Immune system attacks parotid and salivary glands through lymphocytic infiltration; slightly
increased risk of non-Hodgkin’s lymphoma
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