Thoracic involvement in generalised lymphatic anomaly (or lymphangiomatosis)

Eur Respir Rev. 2016 Jun;25(140):170-7. doi: 10.1183/16000617.0018-2016.

Abstract

Generalised lymphatic anomaly (GLA), also known as lymphangiomatosis, is a rare disease caused by congenital abnormalities of lymphatic development. It usually presents in childhood but can also be diagnosed in adults. GLA encompasses a wide spectrum of clinical manifestations ranging from single-organ involvement to generalised disease. Given the rarity of the disease, most of the information regarding it comes from case reports. To date, no clinical trials concerning treatment are available. This review focuses on thoracic GLA and summarises possible diagnostic and therapeutic approaches.

Publication types

  • Review

MeSH terms

  • Biopsy
  • Diagnosis, Differential
  • Disease Progression
  • Humans
  • Lung Diseases / congenital*
  • Lung Diseases / diagnosis
  • Lung Diseases / epidemiology
  • Lung Diseases / therapy
  • Lymphangiectasis / congenital*
  • Lymphangiectasis / diagnosis
  • Lymphangiectasis / epidemiology
  • Lymphangiectasis / therapy
  • Palliative Care
  • Predictive Value of Tests
  • Prognosis
  • Rare Diseases* / diagnosis
  • Rare Diseases* / epidemiology
  • Rare Diseases* / therapy
  • Risk Factors
  • Tomography, X-Ray Computed

Supplementary concepts

  • Lymphangiectasia, pulmonary, congenital