Systemic Sclerosis (SSc) Flashcards
What is systemic sclerosis?
autoimmune connective tissue disease involving inflammation and fibrosis (hardening or scarring) of the connective tissues, skin and internal organs
Previously known as scleroderma.
What causes systemic sclerosis?
Increased fibroblast activity resulting in abnormal growth of connective tissue leading to vascular damage and fibrosis.
What are the two main subtypes of systemic sclerosis?
- Limited cutaneous SSc
- Diffuse cutaneous SSc
What is another name for limited scleroderma?
CREST syndrome
What are the components of CREST syndrome?
- Calcinosis Cutis
- Raynaud’s phenomenon
- Esophageal dysmotility
- Sclerodactyly
- Telangiectasia
How long do Raynaud’s symptoms usually precede scleroderma in limited scleroderma?
Many years - development of PA HTN after a mean of 10 years of symptoms
What characterizes diffuse scleroderma?
Sudden onset of skin involvement, proximal to the elbows and knees.
What is the risk associated with diffuse scleroderma?
Higher risk of mortality.
What are the typical inflammatory marker results in systemic sclerosis?
Usually normal.
What imaging is used to evaluate calcinosis in systemic sclerosis?
X-ray hands
What tests are used to assess pulmonary disease in systemic sclerosis?
- CXR
- HRCT
- PFT
What cardiac evaluations are performed for systemic sclerosis?
- ECG
- ECHO
To look for PA HTN, heart failure, myocarditis and arrhythmias
What percentage of patients with systemic sclerosis have positive ANA?
90%
Which antibody is strongly associated with limited systemic sclerosis?
Anti-centromere antibody
Which antibodies are associated with diffuse systemic sclerosis? (2)
- Scl-70 (topoisomerase)
- Anti RNA polymerase III antibodies
Is there a cure for systemic sclerosis?
No cure
What treatment may reduce Raynaud’s symptoms in systemic sclerosis?
- calcium antagonist
- sildenafil
- prostaglandin infusion - iloprost
Which medications may reduce skin thickening in systemic sclerosis? (2)
- Methotrexate
- Mycophenolate mofetil
What is the role of ACE inhibitors in systemic sclerosis?
Prevent hypertensive crisis & reduce mortality from renal failure.
What is morphoea?
A localized form of scleroderma.
How much more common is scleroderma in women?
Five times more common.
What vascular involvement occurs in systemic sclerosis?
- Raynaud’s phenomenon
- Ischemic digital ulcers
- Hypertensive crisis
- Pulmonary arterial hypertension
True or False: Absence of Raynaud’s phenomenon makes the diagnosis of systemic sclerosis very unlikely.
True
What is scleroderma renal crisis?
A serious condition with features of accelerated hypertension leading to renal failure if not treated promptly.
When is scleroderma renal crisis seen in patients with diffuse SSc?
Early in the course of the disease.
What treatment can be used for SSc flares
Short course of prednisolone
What treatment can be used for GI symptoms of SSc
PPIs
what does diffuse cutaneous SSc involve
also includes CREST features and affects internal organs:
- CVS: HTN, CAD
- lung: p.HTN, p. fibrosis
- kidney: scleroderma renal crisis
what is scleroderma
hardening of the skin, giving the appearance of shiny, tight skin without the normal skin folds
- changes are most notable on the hands and face.
what is sclerodactyly
describes skin changes in the hands:
- skin tightening around the joints which restricts ROM and reduces function
- fat pads on fingers lost
- skin can break and ulcerate
what is telangiectasia
dilated blood vessels in the skin measuring less than 1mm in diameter