Scleroderma Flashcards
What are the two main subtypes of systemic scleroderma?
Diffuse cutaneous scleroderma and limited cutaneous scleroderma.
What are the two major categories of scleroderma?
Systemic and localized
How does diffuse cutaneous scleroderma affect the body?
It is characterized by proximal and distal skin involvement with internal organ dysfunction of the GI tract, lung, heart, and kidney.
What is another name for limited cutaneous scleroderma?
CREST syndrome
How does limited cutaneous scleroderma affect the body?
It is characterized by calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasias.
What is the classic triphasic progression of Raynaud phenomenon?
Raynaud phenomenon is a spasm of the digital arteries with blanching and numbness or pain of the fingers, often precipitated by cold. The triphasic progression is: white, blue, then red on rewarming.
How do children with systemic scleroderma usually present?
They usually present with Raynaud phenomenon. Fingertip ulcerations occur frequently.
What is the classic progression of skin findings in systemic scleroderma?
Gradual thickening of the skin of the distal extremities occurs and slowly progresses, eventually involving the face and trunk.
Which part of the GI tract is most commonly affected in systemic scleroderma?
The distal esophagus is frequently affected. Involvement of the distal smooth muscle leads to dysphagia and GERD.
Which lung finding is more common in patients with diffuse cutaneous scleroderma?
Pulmonary interstitial fibrosis.
Which lung finding is more common in patients with limited cutaneous scleroderma?
Pulmonary HTN leading to right heart failure.
Which class of antihypertensive do you use to reduce the incidence of scleroderma renal crisis?
ACE inhibitors
In patients with which subtype of scleroderma is renal crisis most likely to occur?
Renal crisis occurs in only 10% of patients with scleroderma, and almost exlusively in those with diffuse cutaneous scleroderma.
What findings are used to make a clinical diagnosis of systemic scleroderma?
Sclerodactyly (stiffness and tightness of the skin of the fingers), periungual tortuous dilated loops in the capillary nail beds, and evidence of internal organ involvement.
Which laboratory findings are helpful in the diagnosis of systemic scleroderma?
Laboratory testing shows that 80% of patients have a nucleolar or speckled ANA, and 50% have antibodies to SCL-70 (topoisomerase 1). Anticentromere antibodies may be seen in patients with limited cutaneous scleroderma.
