High frequency of pulmonary lymphangioleiomyomatosis in women with t\iberous sclerosis complex

Lisa C. Costello, Thomas E. Hartman, Jay H Ryu

Research output: Contribution to journalArticle

Abstract

• Objective: To determine the frequency of pulmonary lymphangioleiomyomatosis (LAM), a rare cystic lung disorder that occurs almost exclusively in women of reproductive age, in women with tuberous sclerosis complex (TSC), an inheritable multiorgan hamartomatosis. • Patients and Methods: In this retrospective cohort study, the medical records of 78 women with definite TSC were reviewed, and pertinent information was recorded, including the demographic data, clinical manifestations, results of lung biopsies and autopsies, and findings on imaging studies of the chest and abdomen. All available computed tomographic (CT) scans of the chest and abdomen were reviewed. • Results: Of 78 women with definite TSC seen from 1977 to 1998, 20 (26%) had evidence of LAM. Surgical lung biopsy or autopsy in 7 patients confirmed the diagno-sis of their lung disease. Characteristic CT findings of LAM were noted in 13 additional patients. Twelve of these 20 patients with TSC-associated LAM had respiratory symptoms, including exertional shortness of breath and spontaneous pneumothorax, that eventually led to their pulmonary diagnosis. • Conclusions: The frequency of lung involvement (LAM) in women with TSC is substantially higher than previously suspected and may be even higher than reported in this retrospective study. These findings support the recommendation for a screening CT of the chest for all women with TSC.

Original languageEnglish (US)
Pages (from-to)291-294
Number of pages4
JournalMayo Clinic Proceedings
Volume75
Issue number6
StatePublished - 2000

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Lymphangioleiomyomatosis
Tuberous Sclerosis
Sclerosis
Lung
Thorax
Abdomen
Autopsy
Retrospective Studies
Biopsy
Pneumothorax
Dyspnea
Lung Diseases
Medical Records
Cohort Studies
Demography

ASJC Scopus subject areas

  • Medicine(all)

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High frequency of pulmonary lymphangioleiomyomatosis in women with t\iberous sclerosis complex. / Costello, Lisa C.; Hartman, Thomas E.; Ryu, Jay H.

In: Mayo Clinic Proceedings, Vol. 75, No. 6, 2000, p. 291-294.

Research output: Contribution to journalArticle

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