Week 2 Systemic sclerosis Flashcards
Define system sclerosis.
- systemic connective tissue disease
- a heterogenous group of disorders, with key feature being:
- Scleroderma=thickened hardened skin
- divided into limited and diffuse systemic scleroderma
Describe limited scleroderma.
-limited skin sclerosis: hands, distal forearm>face, chest
-visceral involvement is delayed
-associated with pulmonary hypertension
CREST
1. Calcinosis- Ca2+ deposition can be anywhere
2. Raynaud’s phenomenon: reduced blood flow due to cold/temp changes: white (constriction)–>blue (cyanosis)–>red (hyper fusion). Can be painful
3. Esophageal dysfunction: fibrosis of lower 2/3 smooth muscle, food can get stuck, regurg, increased incidence of acid reflux
4. Sclerodactyly: contractors and ulcerations, hypopigmentation, swelling, tapering, ischemia
5. telangiectasia: small dilated blood vessels
Describe diffuse systemic sclerosis.
- diffuse skin thickening of face,neck, chest, hands, arms, legs. Significant early extra-cutaneous manifestations
1. Face involvement - mask like facies
- thinned and/or retracted lips
- decreased oral aperture
- skin is tight with waxy texture
2. Skin involvement - edema
- induration-increased in fibrosis, thickened dermis seen
- atrophy
3. can have systemic sclerosis w/out skin involvement - extra cutaneous features
Describe the extra cutaneous features of diffuse systemic sclerosis: MSK
MSK
- arhritis: symmetric, polyarticular, small/large joints
- tendon rubs
- muscle atrophy and or myositis
Describe the extra cutaneous features of diffuse systemic sclerosis: GI
due to atrophy, fibrosis, inflammation
- esophagus: dilatation, impaired mobility, lower esophageal sphincter dysfunction, GERD
- Stomach: delayed emptying, gastric antral vascular ectasia “watermelon stomach”
- small intestines: atony with obstruction, stasis with malabsorption, pneumotosis cytoides
- large intestines: pseduo-diverticula, infarction
Describe the extra cutaneous features of diffuse systemic sclerosis: pulmonary
- pulmonary fibrosis
- pulmonary hypertension
- superimposed infections
- microaspirations
- lung cancer
Describe the extra cutaneous features of diffuse systemic sclerosis: CV
- myocardial fibrosis
- conduction deficits
- pericarditis
- pulmonary hypertension–>heart failure
Describe the extra cutaneous features of diffuse systemic sclerosis: renal
- malignant hypertension due to overactive renin-angiotensin system
- renal insufficiency
- the two combined =scleroderma renal crisis
- seen in patients with rapidly progressive skin disease
- can be precipitated by steroid use
- intimal hyperplasia and fibrinoid necrosis of renal blood vessels (medium sized)
- treat with ACE inhibitors
Describe localized scleroderma.
- morphoea: plaque like, most common, oval lesion on 1 area of body
- linear scleroderma: most common type in children, longitudinal band like lesions usually on limbs, can lead to joint contractors and muscle atrophy
What is the pathogenesis of systemic sclerosis?
- complex, not really understood
- immune activation, vascular damage, excessive synthesis of extracellular matrix
- 95% have antibodies
- -Scl 70 topoisomerase: diffuse
- centromere antibodies: limited
