Scleroderma-Expanded analysis (527)

Tests in package

Fibrillarin antibodies, PM/Scl antibodies (P100), RNA Polymerase antibodies, Th/To antibodies

Indication

Suspicion of scleroderma or systemic sclerosis. In-depth analysis is ordered when diagnosis has been established or if there is a strong suspicion of scleroderma/systemic sclerosis and when it is desired to differentiate between sub-entities. As shown in the investigation algorithm below, the analysis may be extended with determination of antibodies against PM-Scl (p100), Th/To, RNA polymerase, and Fibrillarin (see these analyses in the Individual tests list).

Clinical background

A disease with an unclear etiology, which affects many organ systems, mainly the skin and the blood vessels but the lungs and the gastrointestinal tract may also be affected. Kidney, heart and muscle symptoms are less common. The most common clinical characteristics are dermatofibrosis of varying intensity and extent and circulation disorders in fingers and toes (Raynaud’s phenomenon). The disease is usually divided into different forms, such as:

  1. Limited form (usually in the face and distally toward the elbow and knee) fits in with CREST syndrome.
  2. Diffuse forms of skin symptoms, proximally to the elbow and on the body.
  3. Overlap syndrome.

Serological investigation:

 

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View other packages Scleroderma/systemic sclerosis (523).

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