BMJ Case Reports 2018; doi:10.1136/bcr-2017-223972
  • Reminder of important clinical lesson

Systemic sclerosis: severe pulmonary arterial hypertension and pericardial effusion at diagnosis

  1. Arturo Botella
  1. Internal Medicine, Hospital São Francisco Xavier, Centro Hospitalar Lisboa Ocidental/NOVA Medical School, Faculdade de Ciências Médicas, Universidade Nova de Lisboa, Lisbon, Portugal
  1. Correspondence to Dr Patricia Moniz, patricia.moniz25{at}
  • Accepted 23 April 2018
  • Published 14 May 2018


A 51-year-old female patient with a recent hospital admission reported to the emergency room (ER) with progressive worsening of fatigue, dyspnoea and chest discomfort. She had been recently admitted and discharged with the diagnosis of pericarditis and medicated with non-steroidal anti-inflammatory drugs and diuretics. She returned to the ER with persisting symptoms. Echocardiography was repeated and showed signs of elevated right ventricular systolic pressure and a slightly increased moderate/severe pericardial effusion without signs of cardiac tamponade. The patient was admitted and further evaluation confirmed an underlying case of advanced systemic sclerosis with skin, vascular, pulmonary and cardiac involvement. The patient was referred to specialised consults in autoimmune pathology and pulmonary arterial hypertension. She was started on bosentan and corticosteroids, presenting a favourable clinical evolution although symptoms of exertional dyspnoea persist.


  • Contributors All authors participated in the patient’s management during hospital admission. PM wrote the case report and carried out the bibliographic review. AB, DR and JR were consulted during the writing process and assisted in the manuscript’s revision.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.

Register for free content

The full text of all Editor's Choice articles and summaries of every article are free without registration

The full text of Images in ... articles are free to registered users

Only fellows can access the full text of case reports (apart from Editor's Choice) - become a fellow today, or encourage your institution to, so that together we can grow and develop this resource

Don't forget to sign up for content alerts so you keep up to date with all the case reports as they are published, and let us know what you think by commenting on the Editor's blog

Navigate This Article