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Sporadic lymphangioleiomyomatosis: Treatment and prognosis

Authors
Francis X McCormack, MD
Nishant Gupta, MD
Section Editor
Talmadge E King, Jr, MD
Deputy Editors
Geraldine Finlay, MD
Helen Hollingsworth, MD

INTRODUCTION

Lymphangioleiomyomatosis (LAM) is a rare multisystem disorder that mostly afflicts women. The term sporadic LAM is used for patients with LAM who do not have tuberous sclerosis complex (TSC), while TSC-LAM refers to LAM that occurs in patients with TSC. The management of LAM is generally based upon supportive care, use of sirolimus to slow progression of respiratory impairment, and prevention or treatment of complications [1,2].

The management, monitoring, and prognosis of sporadic LAM and its complications are reviewed here. The epidemiology, pathogenesis, clinical presentation, and diagnostic evaluation are discussed separately. (See "Sporadic lymphangioleiomyomatosis: Epidemiology and pathogenesis" and "Sporadic lymphangioleiomyomatosis: Clinical presentation and diagnostic evaluation".)

GENERAL MEASURES

Given the complexities and multisystem nature of LAM, we encourage patient referral to centers with expertise in this disorder for shared care between LAM experts and local pulmonologists. (See 'Additional resources' below.)

Supportive care — Several supportive measures are useful in managing patients with LAM:

Avoidance of cigarette smoking – Patients with LAM should be advised to avoid cigarette smoking and passive exposure to tobacco smoke, given the potential for accelerating disease progression (table 1). (See "Overview of smoking cessation management in adults" and "Pharmacotherapy for smoking cessation in adults" and "Behavioral approaches to smoking cessation".)

                                   

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Literature review current through: Nov 2016. | This topic last updated: Fri Sep 23 00:00:00 GMT+00:00 2016.
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