Cystic lung disease is not uncommon in men with tuberous sclerosis complex

Research output: Contribution to journalArticle

11 Citations (Scopus)

Abstract

Objective: To assess the frequency of cystic lung disease suggestive of pulmonary lymphangiomyomatosis in men with tuberous sclerosis complex. Patients and methods: Retrospective review of CT scans of the chest and abdomen on 29 men with tuberous sclerosis complex encountered during a 13-year period, 1998 to 2010. Results: Cystic lung disease (defined as ≥4 cysts) was seen in 11 of 29 men (38%) with tuberous sclerosis complex. The mean age of those with cystic lung disease was 46.3 ± 19.1 years. None of the patients had experienced pneumothorax or chylothorax and none had undergone a lung biopsy for evaluation of cystic lung disease. Lymphangiomyomatosis had been diagnosed in two of 11 patients with cystic lung disease. Renal angiomyolipomas were demonstrated in 14 of 25 patients (56%) with renal imaging available; all 14 had multiple bilateral renal involvement. In this limited cohort of patients, the presence of cystic lung disease did not correlate with age, smoking exposure or the presence of renal AMLs. Conclusion: We conclude that tuberous sclerosis complex -related lymphangiomyomatosis may not be rare in men but is milder in severity.

Original languageEnglish (US)
Pages (from-to)1586-1590
Number of pages5
JournalRespiratory Medicine
Volume106
Issue number11
DOIs
StatePublished - Nov 2012

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Tuberous Sclerosis
Lymphangioleiomyomatosis
Kidney
Chylothorax
Angiomyolipoma
Lung
Pneumothorax
Abdomen
Cystic Disease Of Lung
Cysts
Thorax
Smoking
Biopsy

Keywords

  • Angiomyolipoma
  • Lymphangioleiom-yomatosis
  • Tuberous sclerosis

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine

Cite this

Cystic lung disease is not uncommon in men with tuberous sclerosis complex. / Ryu, Jay H; Sykes, Anne-Marie Gisele; Lee, Augustine S.; Burger, Charles Dwayne.

In: Respiratory Medicine, Vol. 106, No. 11, 11.2012, p. 1586-1590.

Research output: Contribution to journalArticle

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abstract = "Objective: To assess the frequency of cystic lung disease suggestive of pulmonary lymphangiomyomatosis in men with tuberous sclerosis complex. Patients and methods: Retrospective review of CT scans of the chest and abdomen on 29 men with tuberous sclerosis complex encountered during a 13-year period, 1998 to 2010. Results: Cystic lung disease (defined as ≥4 cysts) was seen in 11 of 29 men (38{\%}) with tuberous sclerosis complex. The mean age of those with cystic lung disease was 46.3 ± 19.1 years. None of the patients had experienced pneumothorax or chylothorax and none had undergone a lung biopsy for evaluation of cystic lung disease. Lymphangiomyomatosis had been diagnosed in two of 11 patients with cystic lung disease. Renal angiomyolipomas were demonstrated in 14 of 25 patients (56{\%}) with renal imaging available; all 14 had multiple bilateral renal involvement. In this limited cohort of patients, the presence of cystic lung disease did not correlate with age, smoking exposure or the presence of renal AMLs. Conclusion: We conclude that tuberous sclerosis complex -related lymphangiomyomatosis may not be rare in men but is milder in severity.",
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