Pulmonary hypertension in connective tissue diseases, new evidence and challenges

Eur J Clin Invest. 2021 Apr;51(4):e13453. doi: 10.1111/eci.13453. Epub 2020 Dec 5.


Pulmonary arterial hypertension is a lethal complication of different connective tissue diseases such as systemic sclerosis, mixed connective tissue disease and systemic lupus erythematosus. Although the treatment possibilities for patients with pulmonary arterial hypertension have increased in the last two decades and survival of patients with idiopathic pulmonary arterial hypertension has improved, the latter is not the case for patients with pulmonary arterial hypertension associated with connective tissue disease. In this narrative review, we review recent literature and describe the improvement of early diagnostic possibilities, screening modalities and treatment options. We also point out the pitfalls in diagnosis in this patient category and describe the unmet needs and what the focus of future research should be.

Keywords: connective tissue disease; pulmonary hypertension; systemic lupus erythematosus; systemic sclerosis.

Publication types

  • Review

MeSH terms

  • Connective Tissue Diseases / complications
  • Connective Tissue Diseases / therapy*
  • Dermatomyositis / complications
  • Dermatomyositis / therapy
  • Disease Management
  • Humans
  • Hypertension, Pulmonary / diagnosis
  • Hypertension, Pulmonary / etiology
  • Hypertension, Pulmonary / physiopathology
  • Hypertension, Pulmonary / therapy*
  • Lupus Erythematosus, Systemic / complications
  • Lupus Erythematosus, Systemic / therapy
  • Mass Screening
  • Mixed Connective Tissue Disease / complications
  • Mixed Connective Tissue Disease / therapy
  • Prognosis
  • Pulmonary Arterial Hypertension / diagnosis
  • Pulmonary Arterial Hypertension / etiology
  • Pulmonary Arterial Hypertension / physiopathology
  • Pulmonary Arterial Hypertension / therapy
  • Scleroderma, Systemic / complications
  • Scleroderma, Systemic / therapy
  • Sjogren's Syndrome / complications
  • Sjogren's Syndrome / therapy