Diffuse cystic lung disease: Part II

Nishant Gupta, Robert Vassallo, Kathryn A. Wikenheiser-Brokamp, Francis X. McCormack

Research output: Contribution to journalArticlepeer-review

62 Scopus citations


The diffuse cystic lung diseases have a broad differential diagnosis. A wide variety of pathophysiological processes spanning the spectrum from airway obstruction to lung remodeling can lead to multifocal cyst development in the lung. Although lymphangioleiomyomatosis and pulmonary Langerhans cell histiocytosis are perhaps more frequently seen in the clinic, disorders such as Birt-Hogg-Dubé syndrome, lymphocytic interstitial pneumonia, follicular bronchiolitis, and light-chain deposition disease are increasingly being recognized. Obtaining an accurate diagnosis can be challenging, and management approaches are highly disease dependent. Unique imaging features, genetic tests, serum studies, and clinical features provide invaluable clues that help clinicians distinguish among the various etiologies, but biopsy is often required for definitive diagnosis. In part II of this review, we present an overview of the diffuse cystic lung diseases caused by lymphoproliferative disorders, genetic mutations, or aberrant lung development and provide an approach to aid in their diagnosis and management.

Original languageEnglish (US)
Pages (from-to)17-29
Number of pages13
JournalAmerican journal of respiratory and critical care medicine
Issue number1
StatePublished - Jul 1 2015


  • Birt-Hogg-Dubé
  • Follicular bronchiolitis
  • High-resolution computed tomography
  • Lymphoid interstitial pneumonia
  • Sjögren syndrome
  • Syndrome

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Critical Care and Intensive Care Medicine


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