You are here

Article Text

Article menu

Download PDFPDF

Unilateral interstitial lung disease in a woman aged 35 years
Free
  1. Daniel John O'Hare1,
  2. Timothy Scanlon2,
  3. Cormac Thomas O'Connor3,
  4. Aidan O'Brien4
  1. 1Department of Medicine, University Hospital Limerick, Limerick, Ireland
  2. 2Department of Radiology, University Hospital Limerick, Limerick, Ireland
  3. 3Cardiology Department, University Hospital Limerick, Limerick, Ireland
  4. 4University Hospital Limerick, Limerick, Ireland
  1. Correspondence to Dr Daniel John O'Hare, drdanielohare{at}gmail.com

https://doi.org/10.1136/bcr-2015-211289

Statistics from Altmetric.com

    Request Permissions

    If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.

    Description

    A woman aged 35 years presented to the respiratory services with a history of asthma and recurrent lower respiratory tract infections. Chest X-ray (figure 1) showed mediastinal shift, volume reduction and increased interstitial markings throughout the right lung. Pulmonary function testing revealed a mixed obstructive and restrictive pattern.

    Figure1
    Figure1

    Chest X-ray.

    CT scan demonstrated the absence of the right main pulmonary artery (figure 2). Collateral supply to the right lung was provided from the enlarged right internal mammary and inferior phrenic arteries (figure 3). Fine reticular peripheral interstitial markings, focal areas of ground glass and bronchiolitis were seen in the right lung (figure 4).

    Figure 2
    Figure 2

    CT thorax showing the absence of the right pulmonary artery.

    Figure 3
    Figure 3

    CT showing enlarged inferior phrenic artery.

    Figure 4
    Figure 4

    CT showing interstitial markings.

    Radiographical findings were consistent with a diagnosis of interruption of the right pulmonary artery. This is a rare congenital condition caused by a development anomaly of the sixth branchial arch.1

    The condition may present with recurrent respiratory infections. Despite collateral circulation, the affected lung is often hypoplastic, and interstitial pulmonary pathology including bronchestasis is revealed on CT imaging.2

    Complications include haemoptysis due to the rupture of collateral vessels. Management is primarily conservative. In the presence of large volume haemoptysis, systemic collateral vessels may be embolised.3

    Learning points

    • Agenesis of a pulmonary artery can lead to unilateral interstitial lung disease.

    • CT allows evaluation of pulmonary vasculature and lung parenchyma when investigating an abnormal chest X-ray.

    References

    1. ↵
      1. Bouros D,
      2. Pare P,
      3. Panagou P, et al
      . The varied manifestation of pulmonary artery agenesis in adulthood. Chest 1995;108:670–6. doi:10.1378/chest.108.3.670
      OpenUrlCrossRefPubMedWeb of Science
    2. ↵
      1. Ten Harkel AD,
      2. Blom NA,
      3. Ottenkamp J
      . Isolated unilateral absence of a pulmonary artery: a case report and review of the literature. Chest 2002;122:1471–7. doi:10.1378/chest.122.4.1471
      OpenUrlCrossRefPubMedWeb of Science
    3. ↵
      1. Reñé M,
      2. Sans J,
      3. Dominguez J, et al
      . Unilateral pulmonary artery agenesis presenting with hemoptysis: treatment by embolization of systemic collaterals. Cardiovasc Intervent Radiol 1995;18:251–4. doi:10.1007/BF00239422
      OpenUrlPubMedWeb of Science
    View Abstract

    Footnotes

    • Contributors DJOH is the primary author of the paper. CTOC aided critical appraisal of the submitted article and provided literature review help. TS is the consultant radiologist who reported the patient's scans and reached the correct diagnosis. AOB is the consultant respiratory physician who managed the care of the patient, and provided critical appraisal of the final article.

    • Competing interests None declared.

    • Patient consent Obtained.

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