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Related Experiment Videos

Pulmonary tuberous sclerosis

M Castro1, C W Shepherd, M R Gomez

  • 1Division of Thoracic Diseases and Internal Medicine, Mayo Clinic, Rochester, MN 55905.

Chest
|January 1, 1995
PubMed
Summary
This summary is machine-generated.

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Pulmonary involvement in tuberous sclerosis complex (TSC) often presents with respiratory symptoms and requires delayed diagnosis. Hormonal therapy may benefit patients with moderate to severe airflow obstruction and declining pulmonary function.

Area of Science:

  • Pulmonology
  • Genetics
  • Radiology

Background:

  • Tuberous sclerosis complex (TSC) is a genetic disorder with multi-systemic manifestations.
  • Pulmonary involvement, including lymphangioleiomyomatosis (LAM), can significantly impact patient morbidity and mortality.
  • Diagnosis of pulmonary TSC is often delayed, impacting timely management.

Purpose of the Study:

  • To describe the clinical presentation, diagnostic findings, and outcomes of pulmonary involvement in tuberous sclerosis complex.
  • To evaluate the efficacy of hormonal therapy in patients with pulmonary TSC.
  • To assess the long-term survival and clinical course of patients with pulmonary TSC.

Main Methods:

  • Retrospective review of nine female patients with pulmonary involvement in TSC.

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  • Analysis of clinical presentation, pulmonary function tests (PFTs), chest radiography, and computed tomography (CT) findings.
  • Evaluation of response to hormonal therapy and patient survival over an average follow-up of 17 years.

    • Main Results:

    • Average diagnostic delay of 8 years; common symptoms include seizures, pneumothorax, dyspnea, and skin changes.
    • PFTs revealed airflow obstruction and reduced diffusing capacity; CT showed diffuse interstitial infiltrates and cystic changes.
    • Five of seven symptomatic patients received hormonal therapy, with three showing clinical response; two untreated patients died of respiratory failure.

    Conclusions:

    • Pulmonary involvement in TSC typically follows a slowly progressive course.
    • Hormonal therapy may be beneficial for symptomatic patients or those with declining pulmonary function.
    • Tuberous sclerosis complex should be considered in patients diagnosed with lymphangioleiomyomatosis.