Fast Five Quiz: Presentation and Diagnosis of Systemic Sclerosis

Dirk M. Elston, MD


February 04, 2021

Systemic sclerosis (SSc) is a rare, heterogenous autoimmune disease characterized by sclerodermatous skin changes, vasculopathy, and involvement of the lungs and other organs. The cause of SSc is largely unknown, but both genetic and environmental factors are believed to be involved. The disease has two principal forms: limited SSc (lSSc) or diffuse SSc (dSSc), which is typically progressive.

The patient workup for SSc uses various laboratory and imaging studies; gastrointestinal surveys (eg, scoping of the digestive tract, gastric emptying); and pulmonary and cardiovascular testing.

Several disorders have clinical similarities and thus mimic SSc. These include:

  • Nephrogenic systemic fibrosis

  • Eosinophilic fasciitis and eosinophilia-myalgia syndrome

  • Graft-versus-host disease

  • Generalized morphea

  • Diabetic cheiroarthropathy

  • Several varieties of scleroderma, such as scleromyxedema, scleredema adultorum of Buschke, and scleredema diabeticorum

  • Radiation exposure, contrast agents with gadolinium, and several drugs and chemicals

Are you familiar with the presentation of SSc and the initial workup required when SSc is suspected? Test your knowledge with this quick quiz.


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