Treatment of Lymphangioleiomyomatosis: A Meta-analysis

doi:10.1378/chest.96.6.1352

Chest

Volume 96, Issue 6, December 1989, Pages 1352-1355

https://doi.org/10.1378/chest.96.6.1352 Get rights and content

Lymphangioleiomyomatosis is a rare disease which afflicts young women of childbearing age. It is sufficiently uncommon that randomization or any other systematic evaluation of regimens of treatment has been difficult. Review of scattered case reports implies that a number of hormonal manipulations may be equally effective. A comprehensive review of the literature revealed 30 cases of LAM treated with eight regimens of treatment. Evaluation with predetermined criteria (meta-analysis) shows that administration of progesterone or oophorectomy or both are the most effective treatments, resulting in improvement or stabilization of the disease in the majority of cases.

Section snippets

Index Case

A 25-year-old white woman first noted episodes of hemoptysis in October 1985. The hemoptysis occurred approximately once per month and was always associated with running. Running would provoke a coughing spell which produced about one teaspoon of heavily blood-tinged sputum. The patient also noted mild dyspnea on exertion but could run two miles in 19 minutes. She denied a regular cough, production of mucus, wheezing, and chest pain. She was referred for evaluation in March 1986. The findings

Methods

The search for reports of LAM was performed by a computer-assisted examination of the literature. Keywords used were lymphangioleiomyomatosis and lymphangiomyomatosis. The reference lists of each retrieved report were scanned for other potential reports. A manual search of Index Medicus was performed as well.

Results

A review of all identified reports revealed 30 cases of LAM treated with the eight different regimens listed in Table 2. Figure 1 details the combination and sequential application of therapies in each case.

Twenty-three of the 30 patients received only one treatment regimen. Four patients were treated with two sequential regimens. Two patients received three sequential attempts at therapy, and one patient had four separate trials of treatment. These 30 patients thus represented 41 separate

Meta-analysis Methods

To determine if therapy was adequate, we set these criteria: (1) adequate dose (equivalent of progesterone at 10 mg/day or tamoxifen at 20 mg/day); (2) minimum duration of therapy at least three months; and (3) therapy initiated sufficiently early in the course of the disease to allow a chance for therapeutic effect (eg, no evidence of cor pulmonale, honeycomb fibrosis on roentgenogram, or refractory hypoxemia).
If therapy was considered to be adequate based on these criteria, then the success

Meta-analysis Results

After meta-analysis, only 12 of the 30 cases (23 trials of treatment) were judged to be evaluable. The most common reasons to exclude a case were institution of therapy too late in the course of disease, insufficient data on the specifics of therapy, or inadequate documentation of response. Many cases contained no information on dosage, timing, or duration of therapy and suffered from lack of data or unclear presentation of the facts. These omissions unfortunately made them uninterpretable and

Discussion

Lymphangioleiomyomatosis is a devastating disease which strikes young women of childbearing age. Because of the implications of the diagnosis, one should be cautious in labelling a patient as having this disorder. This most often requires an open lung biopsy; however, in the proper clinical setting of interstitial pulmonary disease in a young woman with other characteristic findings (chylous effusions; intra-abdominal leiomyomata; air trapping), a transbronchial lung biopsy may be adequate.

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Pulmonary lymphangiomyomatosis: a review
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The lymphangiomyomatosis syndrome
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Pulmonary lymphangiomyomatosis: treatment with castration
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There are more references available in the full text version of this article.

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The opinions or assertions contained herein are the private views of the authors and are not to be construed as official or as reflecting the views of the Department of the Army or the Department of Defense.

Manuscript received January 26; revision accepted April 27.

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Chest

Clinical InvestigationsTreatment of Lymphangioleiomyomatosis: A Meta-analysis

Section snippets

Index Case

Methods

Results

Meta-analysis Methods

Meta-analysis Results

Discussion

Br J Dis Chest

Pulmonary lymphangiomyomatosis: a review

Am J Pathol

The lymphangiomyomatosis syndrome

Cancer

Lymphangiomyomatosis, a clinicoanatomical entity

Cancer

Pulmonary lymphangiomyomatosis: treatment with castration

Cancer

Clinical Investigations
Treatment of Lymphangioleiomyomatosis: A Meta-analysis