Systemic Sclerosis (Scleroderma)
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Introduction
The Case of...
a simple case introducing clincial presentation and calling for a differential diagnosis to get students thinking.
Causes and Risk Factors
Pathophysiology
types of scleroderma
limited cutaneous scleroderma
- Raynauds', hands, feet, and face
vascular phenomenon tend to be grouped together: Raynaud's, telangiectasia, pulmonary hypertension
CREST syndrome
diffuse cutaneous scleroderma
often centromere antibody
- Raynaud's, followed by puffy or hidebound skin changes
- trucal and acral skin involvement
- early and significant incidence of renal, interstitial lung, diffuse gastrointestinal, and myocardial disease
Renal crisis
can lead to microangiopathic hemolytic anemia:
- thrombocytopenia; normal hemoglobin and WBC
- treat wirh ACEi (ie captopril, quick and short acting
- avoid steroids >15 mg daily
Signs and Symptoms
- history
- physical exam
History
Esophageal fibrosis can lead to dysphagia.
Physical Exam
an begin in hands, face, or mouth
most commonly affected systems
skin - scleroderma, calcinosis, telangiectasia, Raynaud's disease.
lungs - interstitial lung disease, pulmonary hypertension, pleural effusions
kidneys - scleroderma renal crisis
heart - arrhythmias, pericarditis, myocardial fibrosis
Investigations
- lab investigations
- diagnostic imaging
Lab Investigations
autoantibodies include ANA, anti-centromere, anti-Scl-70, and anti-RNP
Urinalysis
micro
Diagnostic Imaging
Differential Diagnosis
Treatments
Consequences and Course
Resources and References
Topic Development
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