Systemic Sclerosis Flashcards
What are other names for systemic sclerosis?
- progressive systemic sclerosis
- scleroderma (old name; implies skin only)
What is the 3rd most common autoimmune condition?
systemic sclerosis
What is the etiology of systemic sclerosis?
autoimmune
Systemic sclerosis is a ____-____ disorder
collagen-vascular
(excess collagen + vasculitis)
What demographic is primarily affected by systemic sclerosis?
- onset 40-50 yrs
- F>M (3-8:1)
What are the general systems affected by systemic sclerosis?
- GI
- pulmonary, renal & cardiac (fibrosis)
- MSK
- vascular
What are the possible clinical manifestations of systemic sclerosis involving the GI tract?
- Xerostomia (“desert mouth”)
- loss of peristalsis (dysphagia)
- severe reflux (GERD)
What is xerostomia?
aka “desert mouth”
dry mouth due to lack of saliva
(in systemic sclerosis, caused by fibrosis of mucus glands)
What is dysphagia?
difficulty swallowing
What is a potential sequela of GI involvement of systemic sclerosis?
severe reflux –> Barrett esoph. –> esoph. carcinoma
What is the most common cause of death in patients with systemic sclerosis?
lung disease (COPD)
eg. emphysema w/ no Hx of smoking
What are the possible clinical manifestations of systemic sclerosis involving the renal system?
renal insufficiency & malignant HTN
What are the possible clinical manifestations of systemic sclerosis involving the MSK system?
- swelling & polyarthralgia
- synovitis
- loss of AROM
- deformity (reversible; swan neck, boutonniere, hitch hiker)
- fatigue
- exacerbation/remission pattern
What is systemic sclerosis over-diagnosed as?
fibromyalgia (general fatigue & achiness)
How does vasculitis present in patients with systemic sclerosis?
Raynaud phenomenon (finger/toe tips, ears, nose)
How do fibroblastic and vascular changes present in patients with systemic sclerosis?
fibrosis of skin (sweat/oil glands dry up) causes leathery/tight skin
- Acro-osteolysis
- osteonecrosis/soft tissue necrosis
- loss of facial expression
What is acro-osteolysis and how does it present in patients with systemic sclerosis?
- erosion of distal bone (tips of fingers/toes) due to retraction of tight skin pulling on bone
- presents as ungual tuft resorption radiographically
- “mouse-like facies” (erosion of cheek bones & chin)
How does soft tissue calcification in systemic sclerosis appear radiographically?
in “working areas” (high contact areas) such as finger pads
What are the radiographic characteristics of systemic sclerosis?
- Acro-osteolysis (ungual tuft resorption)
- soft tissue calcification (of necrosed areas)
- possible deformity (swan neck, boutonniere, hitch hiker)
What are the relevant lab findings for systemic sclerosis?
- ^ESR/CRP
- anemia of chronic dz
- RF neg.
- ANA pos. (95%)
- ^creatinine & liver enzymes (reduced renal function)
Dry mouth and severe GERD are seen mostly in what condition?
progressive systemic sclerosis
