"End-stage" pulmonary fibrosis in sarcoidosis

Alvin T. Teirstein, Adam S. Morgenthau

Research output: Contribution to journalArticlepeer-review

29 Scopus citations


Pulmonary fibrosis is an unusual "end stage" in patients with sarcoidosis. Fibrosis occurs in a minority of patients, and presents with a unique physiologic combination of airways dysfunction (obstruction) superimposed on the more common restrictive dysfunction. Imagin techniques are essential to the diagnosis, assessment and treatment of pulmonary fibrosis. Standard chest radiographs and CT scans may reveal streaks, bullae, cephalad retraction of the hilar areas, deviation of the trachea and tented diaphragm. Positive gallium and PET scans indicate residual reversible granulomatous disease and are important guides to therapy decisions. Treatment, usually with corticosteroids, is effective in those patients with positive scans, but fibrosis does not improve with any treatment. With severe functional impariment and patient disability, pulmonary hypertension and right heart failure may supervene for which the patient will require treatment. Oxygen, careful diuresis, sildenafil and bosentan may be salutary. These patients are candidates for lung transplantation.

Original languageEnglish
Pages (from-to)30-36
Number of pages7
JournalMount Sinai Journal of Medicine
Issue number1
StatePublished - 2009


  • Airways obstruction
  • Bullae
  • Fibrosis
  • Gallium and PET scans
  • Hilar retraction
  • Pulmonary hypertension
  • Right heart failure
  • Tented diaphragm


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