--- title: "Update on the treatment of lymphangioleiomyomatosis. | Sutter Health" description: "We describe the clinical, physiologic, radiologic, and pathologic characteristics of lymphangioleiomyomatosis, and discuss current and possible treatments." url: "https://www.sutterhealth.org/research/publications/update-treatment-lymphangioleiomyomatosis-1055312862" --- 1. [Home](https://www.sutterhealth.org/) / 2. [Research](https://www.sutterhealth.org/research) / 3. [Research publications](https://www.sutterhealth.org/research/publications) / 4. Update on the treatment of lymphangioleiomyomatosis. # Update on the treatment of lymphangioleiomyomatosis. Krishna G, Berry G, Kao P, Rosen G, & Raffin TA. _Clin Pulmon Med. 6(2):126-132._ 1999-03-01 ## Abstract Lymphangioleiomyomatosis (LAM) is a rare disease of unknown origin that affects women of reproductive age. The presence of spontaneous pneumothorax, hemoptysis, and chylous effusion or ascites in a young woman prompts the diagnosis. Most patients present with progressive dyspnea and cough. High-resolution CT usually shows bilateral diffuse cystic airspace disease with cysts of variable sizes. Pulmonary function tests show predominantly an obstructive pattern. The treatments available for LAM at this point are not clearly effective. Since its description more than 50 years ago, various treatment modalities including corticosteroids and cytotoxic agents have been tried without any significant benefit. Because the disease predominantly affects women of childbearing age, hormonal manipulation has been attempted with questionable success. Recent advances in molecular biology have thrown some light on the pathophysiology of this disease and may pave the way for future therapies. Most patients who have LAM die within 10 to 15 years of diagnosis because of progressive respiratory failure. More than 40 patients with LAM have had lung transplantation. LAM-specific post-transplant complications occur, and there are rare reports of recurrence of the disease in the transplanted lung. In this review, we describe the clinical, physiologic, radiologic, and pathologic characteristics of this disease and discuss the various currently available treatment options as well as future possibilities in the treatment of this rare and progressive disease. [PubMed abstract](http://journals.lww.com/clinpulm/Abstract/1999/03000/Update_on_the_Treatment_of.6.aspx) ![Ganesh Krishna, M.D., FCCP](https://dyn.mktgcdn.com/p/7HZoN6h0d4phO4VQYcU6TE-_Cn72_cBpIS3NRGru51E/fit=crop,gravity=auto,dpr=2,width=188,height=250,quality=100) Ganesh Krishna, M.D., FCCP Principal investigator [View profile](https://www.sutterhealth.org/research/investigators/ganesh-krishna-md-fccp-1049681082) ## Research topics - [Disease Management](https://www.sutterhealth.org/research-search-results?query=%22%5C%22Disease+Management%5C%22%22) - [Lung Diseases](https://www.sutterhealth.org/research-search-results?query=%22%5C%22Lung+Diseases%5C%22%22) ## Related publications [View all publications](https://www.sutterhealth.org/research-search-results?verticalKey=research_publication) - Publication ### [Smoking cessation therapy with varenicline.](https://www.sutterhealth.org/research/publications/smoking-cessation-therapy-with-varenicline-1055312920) Mohanasundaram UM, Chitkara R, Krishna G. Int J Chron Obstruct Pulmon Dis. 2008;3(2):239-51. 2008 Feb 01 - Publication ### [The dying thoracic patient.](https://www.sutterhealth.org/research/publications/dying-thoracic-patient-1055312967) Krishna G, Raffin TA. Chest Surg Clin N Am. 8(3):723-39. 1998 Aug 01 - Publication ### [Electromagnetic navigational bronchoscopy spares a drunken crab from the surgeon's knife.](https://www.sutterhealth.org/research/publications/enb-saves-drunken-crab-fr-surgery-1055312421) Parikh MS, Seeley E, Krishna G. J Bronchology Interv Pulmonol. \[Epub ahead of print\] 2016 Apr 11 - Publication ### [PG490-88, a derivative of triptolide, blocks bleomycin-induced lung fibrosis.](https://www.sutterhealth.org/research/publications/pg490-88-blocks-bleomycin-induced-lung-fibrosis-1055312795) Krishna G, Liu K, Shigemitsu H, Gao M, Raffin TA, Rosen GD. Am J Pathol. 158(3):997-1004. 2001 Mar 01 - Publication ### [Advances in the management of chronic obstructive pulmonary disease.](https://www.sutterhealth.org/research/publications/advances-mgmt-copd-1055312934) Meinke L, Chitkara R, Krishna G. Expert Opin Pharmacother. 8(1):23-37. 2007 Jan 01 [View all publications](https://www.sutterhealth.org/research-search-results?verticalKey=research_publication) ```json {"@context":"https://schema.org","@type":"MedicalOrganization","name":"Sutter Health","@id":"https://www.sutterhealth.org/#medicalorganization","url":"https://www.sutterhealth.org","logo":"https://www.sutterhealth.org/static/images/sutter-health-logo.svg","address":{"@type":"PostalAddress","streetAddress":"2200 River Plaza Drive","addressLocality":"Sacramento","addressRegion":"CA","postalCode":"95833","addressCountry":"US"},"areaServed":{"@type":"AdministrativeArea","name":"California"},"sameAs":["https://www.facebook.com/SutterHealth","https://www.instagram.com/sutterhealth/","https://x.com/SutterHealth","https://www.youtube.com/sutterhealth","https://www.linkedin.com/company/sutter-health","https://en.wikipedia.org/wiki/Sutter_Health"]} ``` ```json {"@context":"https://schema.org","@type":"BreadcrumbList","@id":"https://www.sutterhealth.org/research/publications/update-treatment-lymphangioleiomyomatosis-1055312862#breadcrumblist","itemListElement":[{"@type":"ListItem","position":1,"name":"Home","item":"https://www.sutterhealth.org/"},{"@type":"ListItem","position":2,"name":"Research","item":"https://www.sutterhealth.org/research"},{"@type":"ListItem","position":3,"name":"Research publications","item":"https://www.sutterhealth.org/research/publications"},{"@type":"ListItem","position":4,"name":"Update on the treatment of lymphangioleiomyomatosis."}]} ``` ```json {"@context":"https://schema.org","@type":"MedicalScholarlyArticle","@id":"https://www.sutterhealth.org/research/publications/update-treatment-lymphangioleiomyomatosis-1055312862#medicalscholarlyarticle","name":"Update on the treatment of lymphangioleiomyomatosis.","headline":"Update on the treatment of lymphangioleiomyomatosis.","abstract":"Lymphangioleiomyomatosis (LAM) is a rare disease of unknown origin that affects women of reproductive age. The presence of spontaneous pneumothorax, hemoptysis, and chylous effusion or ascites in a young woman prompts the diagnosis. Most patients present with progressive dyspnea and cough. High-resolution CT usually shows bilateral diffuse cystic airspace disease with cysts of variable sizes. Pulmonary function tests show predominantly an obstructive pattern. The treatments available for LAM at this point are not clearly effective. Since its description more than 50 years ago, various treatment modalities including corticosteroids and cytotoxic agents have been tried without any significant benefit. Because the disease predominantly affects women of childbearing age, hormonal manipulation has been attempted with questionable success. Recent advances in molecular biology have thrown some light on the pathophysiology of this disease and may pave the way for future therapies. Most patients who have LAM die within 10 to 15 years of diagnosis because of progressive respiratory failure. More than 40 patients with LAM have had lung transplantation. LAM-specific post-transplant complications occur, and there are rare reports of recurrence of the disease in the transplanted lung. In this review, we describe the clinical, physiologic, radiologic, and pathologic characteristics of this disease and discuss the various currently available treatment options as well as future possibilities in the treatment of this rare and progressive disease.","description":"We describe the clinical, physiologic, radiologic, and pathologic characteristics of lymphangioleiomyomatosis, and discuss current and possible treatments.","keywords":["Disease Management","Lung Diseases"],"datePublished":"1999-03-01","url":"https://www.sutterhealth.org/research/publications/update-treatment-lymphangioleiomyomatosis-1055312862","inLanguage":"en-US","isPartOf":{"@type":"Periodical","name":"Clin Pulmon Med"},"author":[{"@type":"Person","@id":"https://www.sutterhealth.org/research/investigators/ganesh-krishna-md-fccp-1049681082","name":"Krishna G","url":"https://www.sutterhealth.org/research/investigators/ganesh-krishna-md-fccp-1049681082"},{"@type":"Person","name":"Berry G"},{"@type":"Person","name":"Kao P"},{"@type":"Person","name":"Rosen G"},{"@type":"Person","name":"Raffin TA"}],"sourceOrganization":{"@type":"MedicalOrganization","name":"Palo Alto Medical Foundation Research Institute"},"about":[{"@type":"DefinedTerm","name":"Disease Management","url":"https://www.sutterhealth.org/research-search-results?facets=%5B%7B%22i%22%3A%22c_researchTopics.name%22%2C%22v%22%3A%5B%22Disease+Management%22%5D%7D%5D&verticalKey=research_publication"},{"@type":"DefinedTerm","name":"Lung Diseases","url":"https://www.sutterhealth.org/research-search-results?facets=%5B%7B%22i%22%3A%22c_researchTopics.name%22%2C%22v%22%3A%5B%22Lung+Diseases%22%5D%7D%5D&verticalKey=research_publication"}],"mainEntityOfPage":{"@type":"WebPage","@id":"https://www.sutterhealth.org/research/publications/update-treatment-lymphangioleiomyomatosis-1055312862","breadcrumb":{"@id":"https://www.sutterhealth.org/research/publications/update-treatment-lymphangioleiomyomatosis-1055312862#breadcrumblist"}}} ```