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Spontaneous intrapulmonary haemorrhage in vascular Ehlers-Danlos syndrome
  1. Ping Wang1,
  2. Zhilan Meng2,
  3. Ruie Feng2,
  4. Juhong Shi1,
  5. Wenbing Xu1
  1. 1 Department of Pulmonary and Critical Care Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing, China
  2. 2 Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing, China
  1. Correspondence to Dr Ping Wang, Department of Pulmonaryd Critical Care Condition, Peking Union Medical College Hospital, Chinese Academy of Medical Science & Peking Union Medical College, Beijing, China; iarentree{at}sina.com

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A 17-year-old male patient presented with a 2-year history of intermittent self-resolving left or right pleuritic chest pain, radiating to the back and with variable duration from several minutes to days. He was a non-smoker and had no known medical or family history. Physical examination was remarkable for hypermobility of small joints (figure 1A) and translucent skin with visible veins (figure 1B). Two chest CT scans, separated by 2 years, revealed waxing and waning bilateral multiple nodules and cavities with surrounding ground glass opacity (figure 1C–F). Absence of aortic and arterial aneurysms was revealed by the enhanced CT. Pulmonary function tests revealed restrictive ventilation dysfunction and no diffusion impairment. Bronchoscopy showed visible tortuous mucosal vessels (figure 1G,H) and multiple telangiectasias-like and haemorrhagic foci (figure 1I) on the smooth mucosal surface of the trachea and bilateral bronchus. Bronchoalveolar lavage fluid (BALF) was haemorrhagic and …

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Footnotes

  • Contributors PW contributed to data collection and analysis; statistical analysis; and writing, review and approval of the final manuscript. ZM and RF contributed to data collection, review and approval of the final manuscript. JS and WX contributed to writing, review and approval of the final manuscript.

  • Funding Beijing Natural Science Foundation (7222132), National High Level Hospital Clinical Research Funding (2022-PUMCH-A-130).

  • Competing interests None declared.

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