Keyword for Investigation, Referral to specialist and Writing hospital records by Systemic Sclerosis 5/5 (1)

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ICD-10: M34.0 (diffuse form) M 34.1 (Limited Crest Form)

Diagnosis is based on

  • Disease history
  • Medical Examination Findings
  • Antibodies

Disease history

Time of symptom debut (Raynaud's phenomenon and other symptoms) and for diagnosis

* (Star) marks the symptoms and research findings that are included in ACR-EULAR Criteria (2013):


Clinical examination

Supplementary medical investigations

Radiological examinations

  • X-ray examination of the esophagus (dynamic with ingestion of contrast agent) or manometry (dysmotility, stenoses, reflux)
  • CT examination / HRCT of lungs (milk glass, fibrosis or extended pulmonary artery (more than 3,5cm) at suspected Pulmonary hypertension)
    • More than 20% lung tissue infection suggests serious lung involvement
  • MRI of the heart
    • Can detect fibrosis in the heart muscle

Lung function tests

  • Lung function tests (FEV1, FVC, DLCO% of expected)
    • Severe lung manifestation: FVC less than 70% and CT examination show more than 20% attacked lung tissue. Similarly, if FVC and DLCO fall by 10% and 15%, respectively, each year


  • Mega capillaries, bleeding, "bushy" capillaries

6-minute walk test

  • The distance traveled during the 6 minute maximum, but even times
  • Can be done in a corridor with a measured distance of trained health personnel with emergency medical access
  • Registration of oxygen saturation and Borg's Dyspne Index (scale 0-10) before and immediately after the test
  • Reference: ATS statement: guidelines for the six-minute walk test


Cardiac examination

Blood tests

Follow-up for systemic sclerosis

Referral to specialist

Local rheumatology department should be preferred. In Oslo: Rheumatology Department, Rikshospitalet: Postbox 4950 Nydalen, 0424 OSLO. The department receives patients from the Health Region South East and from all over the country when the capacity allows.

Uncertain diagnosis?

Norwegian Directorate of Health (Supervisor for referrals)

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