Summary Connective Tissue Diseases

Inflammatory Muscle Disease
Immune 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