[Pulmonary arterial hypertension in collagen disease: experiences of the German Network for Systemic Scleroderma]

Dtsch Med Wochenschr. 2006 Dec 8;131(49 Suppl 9):S325-7. doi: 10.1055/s-2006-957203.
[Article in German]


The German Network for Systemic Scleroderma (Deutsches Netzwerk Für Systemische Sklerodermie [DNSS]) has as its aim to improve and standardize the diagnosis and treatment of this rare disease. 14.6 % of patients in the DNSS register have been shown to have pulmonary hypertension (PHT). Comparable international data range from 12 % to 26.7 %. The DNSS recommends annual echocardiography for patients with scleroderma and right-heart catheterization if the systolic pulmonary arterial pressure is higher than 35 mmHg. Aggressive immunosuppressive treatment--in the first instance with cyclophosphamide--should be given only if there is also progressive fibrosis. Drugs that lower the level of PHT also have a favourable effect on other signs of scleroderma.

Publication types

  • English Abstract
  • Review

MeSH terms

  • Catheterization, Swan-Ganz
  • Connective Tissue Diseases / complications*
  • Connective Tissue Diseases / physiopathology
  • Echocardiography
  • Germany / epidemiology
  • Humans
  • Hypertension, Pulmonary / diagnosis
  • Hypertension, Pulmonary / epidemiology
  • Hypertension, Pulmonary / etiology*
  • Hypertension, Pulmonary / therapy
  • Immunosuppressive Agents / therapeutic use*
  • Prognosis
  • Pulmonary Fibrosis / drug therapy
  • Pulmonary Fibrosis / etiology
  • Pulmonary Wedge Pressure
  • Scleroderma, Systemic / complications*
  • Scleroderma, Systemic / physiopathology
  • Scleroderma, Systemic / therapy
  • Societies, Medical / organization & administration


  • Immunosuppressive Agents