Lymphangioleiomyomatosis (LAM)
LAM is a rare, progressive, cystic lung disease caused by mutations in the TSC1 or TSC2 genes, leading to mTORC1 hyperactivation and abnormal proliferation of smooth muscle-like LAM cells throughout the lungs and lymphatics. It almost exclusively affects premenopausal women and is characterized by bilateral pulmonary cysts, spontaneous pneumothorax, chylothorax, and renal angiomyolipomas.
Table of Contents
- Disease Overview and Classification
- Pathogenesis: TSC Mutations and mTORC1
- Pulmonary Manifestations
- Extrapulmonary Manifestations
- Symptoms and Clinical Presentation
- Diagnosis: HRCT, VEGF-D, and Biopsy
- Pulmonary Function and Disease Progression
- Treatment: mTOR Inhibitors and Supportive Care
- Prognosis and Transplantation
- References & Research
- Featured Videos
Disease Overview and Classification
Lymphangioleiomyomatosis (LAM) is a multisystem disorder characterized by the uncontrolled proliferation of abnormal smooth muscle-like cells — LAM cells — throughout the lung parenchyma, lymphatics, and kidneys. The disease was first described in 1937 and remained a medical curiosity until the 1990s, when the discovery of TSC gene mutations provided its molecular basis and eventually led to targeted mTOR inhibitor therapy.
Sporadic LAM (sLAM)
Sporadic LAM accounts for approximately 80–85% of all cases. It arises from a somatic (acquired) mutation in the TSC2 gene occurring in a single progenitor cell. The LAM cells bearing this mutation then proliferate, spread hematogenously and lymphatically, and seed distant organs. sLAM is almost exclusively a disease of women in their reproductive years, with a median age at diagnosis of 35–40 years. The strong estrogen dependence of sLAM is evidenced by its rarity before menarche, acceleration during pregnancy, and occasional stabilization after menopause. No germline mutations are present and sLAM does not carry an increased risk for family members.
Tuberous Sclerosis Complex-Associated LAM (TSC-LAM)
TSC-LAM affects individuals with tuberous sclerosis complex (TSC), an autosomal dominant neurocutaneous syndrome caused by germline mutations in TSC1 (hamartin) or TSC2 (tuberin). Among women with TSC, approximately 30–40% will develop radiographically detectable LAM by age 40. TSC-LAM can affect males, although the disease is far less severe in men — a difference attributed to the lack of estrogen-driven LAM cell proliferation. TSC patients also carry the full burden of extrapulmonary TSC manifestations: cortical tubers causing epilepsy, subependymal giant cell astrocytomas, cardiac rhabdomyomas (often regressing spontaneously in childhood), and renal angiomyolipomas.
Epidemiology
LAM is genuinely rare, with prevalence estimates of 3–7 per million women. Because of this rarity and the nonspecific early presentation, diagnosis is frequently delayed by 3–5 years. The LAM Foundation patient registry has been critical in characterizing the natural history of this disease in an otherwise understudied population.
Pathogenesis: TSC Mutations and mTORC1
The molecular basis of LAM is hyperactivation of mTORC1 (mechanistic target of rapamycin complex 1), a master regulator of cell growth, protein synthesis, and autophagy. Understanding this pathway is essential to understanding both the disease and the rationale for its treatment.
The TSC1/TSC2 Tumor Suppressor Complex
TSC1 (hamartin) and TSC2 (tuberin) form an obligate heterodimer that functions as a GTPase-activating protein (GAP) for the small GTPase Rheb. When active, the TSC1/TSC2 complex converts Rheb-GTP to inactive Rheb-GDP, preventing Rheb from activating mTORC1. Loss-of-function mutations in either TSC1 or TSC2 eliminate this brake: Rheb remains bound to GTP, constitutively activating mTORC1. mTORC1 activation then drives phosphorylation of S6K1 and 4E-BP1, leading to increased ribosomal biogenesis, protein synthesis, cell growth, and inhibition of autophagy — the cellular machinery underlying the hyperproliferative LAM cell phenotype.
LAM Cell Identity and Spread
LAM cells are smooth muscle-like cells that express neural crest markers (HMB-45, a melanoma antigen), smooth muscle markers (alpha-smooth muscle actin, SMA), and sex hormone receptors (estrogen receptor, ER; progesterone receptor, PR). Their precise tissue of origin remains debated, but the leading hypothesis is that they derive from a uterine or pelvic source and spread to the lungs via lymphatic and hematogenous routes. Evidence for this metastatic spread model comes from the observation that LAM recurs in transplanted lungs — new LAM cells from the recipient colonize the donor lung. The recurrence risk post-transplant is low (approximately 1–2%), but it excludes the possibility that LAM is simply a locally generated pulmonary disease.
Estrogen's Role
Estrogen promotes LAM cell survival and proliferation through ER-mediated transcriptional effects and possibly through mTORC1-independent pathways. Clinical observations supporting estrogen dependence include: the near-exclusive premenopausal female predominance, documented disease progression during pregnancy (when estrogen levels are maximally elevated), anecdotal evidence of disease acceleration with exogenous estrogen administration, and case reports of disease stabilization after oophorectomy. However, controlled trials of anti-estrogen therapies (progesterone, tamoxifen, oophorectomy) failed to show benefit in the pre-mTOR inhibitor era, suggesting estrogen modulation alone is insufficient to halt LAM progression.
Pulmonary Manifestations
The lungs bear the brunt of LAM pathology. LAM cells infiltrate the lung parenchyma along lymphatics and airway walls, causing progressive destruction of normal lung architecture and replacement with thin-walled cysts.
Pulmonary Cysts: Formation and Distribution
The hallmark of LAM on imaging is bilateral, diffuse, thin-walled, round-to-oval pulmonary cysts distributed throughout both lungs. The cysts range from a few millimeters to several centimeters in diameter and characteristically have a normal-appearing parenchyma between them — a feature that distinguishes LAM from emphysema (where alveolar destruction eliminates the normal intervening lung). The mechanism of cyst formation involves LAM cell infiltration and partial obstruction of small airways, creating a check-valve effect that traps air distally; protease release by LAM cells then destroys adjacent alveolar walls.
Obstructive Physiology: A Paradox
Despite being classified as an interstitial lung disease, LAM produces an obstructive ventilatory defect on pulmonary function testing — airflow limitation with reduced FEV1/FVC ratio and air trapping (elevated residual volume and total lung capacity). This is paradoxical because most interstitial lung diseases produce restriction. The explanation lies in the mechanism: LAM cells obstruct small airways directly, and the loss of elastic recoil from cyst formation reduces the driving pressure for expiratory airflow, mimicking emphysema physiology. The diffusing capacity for carbon monoxide (DLCO) is reduced early and tracks disease severity. FEV1 decline rate in untreated LAM averages 70–90 mL/year — faster than in COPD — but is highly variable between patients.
Spontaneous Pneumothorax
Spontaneous pneumothorax occurs in 25–40% of LAM patients, often before or at the time of diagnosis. It is the most dramatic acute presentation of LAM and results from rupture of subpleural cysts. Unlike primary spontaneous pneumothorax (which tends not to recur after the first episode if managed correctly), LAM-associated pneumothorax has a recurrence rate exceeding 70% after the first episode — a rate that profoundly affects management. Current guidelines recommend early pleurodesis (chemical or surgical) after a first LAM-associated pneumothorax to prevent recurrence, rather than the "watchful waiting" approach used in primary spontaneous pneumothorax.
Chylothorax and Chylous Ascites
Chylothorax — accumulation of chylous (lymph-rich) fluid in the pleural space — is a hallmark complication occurring in approximately 20–30% of LAM patients. It results from LAM cell infiltration and obstruction of thoracic lymphatic vessels, causing lymph to leak into the pleural space. Chylothorax is milky-white in appearance, with triglyceride levels typically exceeding 110 mg/dL and chylomicrons on lipoprotein electrophoresis. Chylous ascites reflects the same process in the peritoneal lymphatics. Both respond to mTOR inhibitor therapy — sirolimus reduces lymphatic complications in addition to its pulmonary effects.
Extrapulmonary Manifestations
LAM is a multisystem disease. Extrapulmonary manifestations reflect LAM cell infiltration of abdominal and pelvic lymphatics and parenchymal organs, and in TSC-LAM, the full systemic burden of tuberous sclerosis complex.
Renal Angiomyolipomas (AML)
Angiomyolipomas are benign hamartomas composed of abnormal blood vessels, immature smooth muscle, and fat. They occur in 50–60% of women with sLAM and in over 80% of TSC-LAM patients. AMLs can be identified on CT by their fat content (Hounsfield units less than -10 in the fat component) or on MRI using fat-suppression sequences. The clinical significance of AMLs relates to their propensity to bleed spontaneously — the abnormal vasculature within AMLs lacks the normal elastic lamina and is prone to aneurysm formation and rupture. AMLs exceeding 4 cm in diameter are at highest risk for spontaneous hemorrhage (Wunderlich syndrome), which can be life-threatening. Management of large or symptomatic AMLs includes mTOR inhibitor therapy (first line, as sirolimus reduces AML volume by 50–70%) or selective embolization; surgical resection is avoided to preserve renal parenchyma.
Lymphangioleiomyomas
Lymphangioleiomyomas are LAM cell-lined cystic structures that form within the lymphatic system, most commonly in the retroperitoneum, pelvis, and mediastinum. They appear on CT as thin-walled fluid-filled structures and can grow to considerable size. Symptoms depend on location: retroperitoneal lymphangioleiomyomas may cause abdominal pain, early satiety, or bowel compression. They are distinct from simple lymphoceles and respond to mTOR inhibitor therapy.
TSC-Specific Manifestations (TSC-LAM Only)
In TSC-LAM, the full spectrum of tuberous sclerosis manifestations may be present. Cortical tubers are epileptogenic lesions that cause seizures, often beginning in infancy, and vary widely in severity — from pharmacologically controlled epilepsy to refractory status epilepticus. Subependymal giant cell astrocytomas (SEGAs) can obstruct CSF flow causing hydrocephalus and require treatment with mTOR inhibitors (everolimus is FDA-approved for SEGA) or surgery. Cutaneous features include facial angiofibromas (adenoma sebaceum, the classic butterfly facial rash), ash leaf macules (hypomelanotic macules, visible under Wood's lamp), shagreen patches (connective tissue hamartomas on the lower back), and periungual fibromas. Cardiac rhabdomyomas, present in fetal life, frequently involute spontaneously. Retinal hamartomas are incidental findings.
Symptoms and Clinical Presentation
LAM presents insidiously in most patients, with symptoms often attributed to more common conditions such as asthma or anxiety. The average delay from first symptoms to diagnosis is 3–5 years, partly because many physicians have never encountered the disease. Awareness of LAM's characteristic presentations can dramatically shorten this diagnostic odyssey.
Exertional Dyspnea
Progressive exertional dyspnea is the most common presenting symptom, reported by over 80% of patients at diagnosis. It follows an insidious course — initially noticed only during strenuous exercise, then during activities of daily living. The mechanism is multifactorial: obstructive airflow limitation, reduced DLCO from cyst destruction of the alveolar-capillary membrane, and impaired gas exchange efficiency during exercise. Unlike asthma, the dyspnea does not have the classic episodic bronchospastic character, though bronchodilator responsiveness is present in some patients.
Spontaneous Pneumothorax as First Presentation
A spontaneous pneumothorax in a young woman — particularly if recurrent, or occurring without obvious precipitant — should always prompt consideration of LAM. Approximately 25% of LAM patients present with a pneumothorax before any other diagnosis, often being evaluated for "primary spontaneous pneumothorax" for months before the underlying LAM is recognized. The clue is recurrence: primary spontaneous pneumothorax recurs in 25–30% of cases; LAM-associated pneumothorax recurs in over 70%.
Chylous Effusion Presentation
Chylothorax presenting as a unilateral or bilateral pleural effusion — particularly in a young woman — is a highly specific presentation for LAM (or other lymphatic abnormalities). The effusion is often massive, causing dyspnea and reduced exercise tolerance. Thoracentesis revealing milky fluid with high triglycerides confirms chylous origin and should immediately prompt HRCT and VEGF-D testing.
Incidental Diagnosis
An increasing proportion of LAM is diagnosed incidentally — cysts are found on CT performed for another reason, or renal AMLs are discovered on abdominal ultrasound. This shift toward incidental detection is expected to improve prognosis as the diagnosed population includes earlier-stage disease.
Diagnosis: HRCT, VEGF-D, and Biopsy
The diagnosis of LAM can often be established without surgical lung biopsy using a combination of HRCT findings, serum VEGF-D levels, and clinical context. Current ERS and ATS guidelines provide a diagnostic algorithm that spares many patients an invasive procedure.
High-Resolution CT (HRCT)
HRCT of the chest is the cornerstone of LAM diagnosis. The characteristic appearance is bilateral, diffusely distributed, thin-walled (wall thickness typically <2 mm), round-to-oval cysts ranging from 2 mm to 2 cm in diameter, with completely normal intervening lung parenchyma. The cysts are uniformly distributed throughout both lungs — upper and lower zones equally — without the upper-lobe predominance of emphysema or the lower-lobe predominance of usual interstitial pneumonia. In early disease, cysts may be few and small; in advanced disease they may replace most of the lung parenchyma. Ground-glass opacities and nodules are not prominent features of LAM (their presence should prompt consideration of an alternative diagnosis). A characteristic HRCT in the right clinical context (young woman with dyspnea, pneumothorax, or chylothorax) is sufficient to suspect LAM strongly.
Serum VEGF-D: A Diagnostic Biomarker
Vascular endothelial growth factor D (VEGF-D) is produced by LAM cells and is measurable in serum. A serum VEGF-D concentration exceeding 800 pg/mL is highly specific (approximately 98%) for LAM in the appropriate clinical context and is included in the ERS and ATS diagnostic criteria as a non-invasive confirmatory test. Combined with characteristic HRCT findings, a VEGF-D >800 pg/mL is sufficient to diagnose LAM without tissue biopsy in most cases. VEGF-D sensitivity is approximately 69% in sLAM and somewhat lower in TSC-LAM, so a normal VEGF-D does not exclude LAM. VEGF-D also tracks disease activity and may serve as a pharmacodynamic marker for mTOR inhibitor therapy.
Diagnostic Criteria (ERS/ATS)
According to current guidelines, a definitive LAM diagnosis can be made without biopsy if: characteristic or compatible HRCT findings are present plus any one of the following — (1) serum VEGF-D ≥800 pg/mL; (2) renal angiomyolipoma; (3) chylous pleural or peritoneal effusion; (4) lymphangioleiomyoma; (5) confirmed TSC diagnosis. In the absence of these features, surgical lung biopsy (video-assisted thoracoscopic surgery, VATS) is required. Biopsy specimens show LAM cell nests — spindle-shaped smooth muscle-like cells expressing HMB-45, SMA, ER, and PR — surrounding the characteristic cysts.
Additional Diagnostic Workup
Abdominal CT or MRI is essential in all LAM patients to evaluate for renal AMLs and retroperitoneal lymphangioleiomyomas. Genetic testing for TSC1/TSC2 germline mutations should be offered to all LAM patients regardless of apparent sporadic nature — TSC mutations found on germline testing redefine the diagnosis as TSC-LAM and have implications for family screening. Pulmonary function tests at diagnosis provide a functional baseline. Echocardiography should be performed if pulmonary hypertension is suspected (LAM can cause Group 5 PH through lymphatic and vascular involvement).
Pulmonary Function and Disease Progression
Monitoring lung function over time is central to LAM management because the disease is progressive and the rate of progression varies substantially between patients. Some women experience rapid FEV1 decline requiring early treatment; others remain stable for years.
Spirometry and DLCO
Pulmonary function in LAM shows an obstructive pattern: reduced FEV1/FVC ratio, increased residual volume, air trapping, and disproportionately reduced DLCO relative to FVC. Bronchodilator responsiveness (improvement in FEV1 ≥12% and 200 mL after albuterol) is present in approximately 30% of LAM patients and predicts better outcomes and greater benefit from bronchodilators. DLCO correlates with cyst burden on HRCT and with exercise capacity, making it a valuable complement to FEV1 for disease staging.
Rate of FEV1 Decline
In untreated LAM, the mean rate of FEV1 decline has been reported as 70–120 mL/year in most cohort studies — substantially faster than the 30–40 mL/year expected with normal aging or the 40–60 mL/year in COPD. There is, however, enormous interindividual variability: some patients lose over 200 mL/year, others show minimal decline over years of follow-up. The LAM Functional Classification (LFC) system uses baseline FEV1 (as %predicted) to grade severity: LFC I (>70% predicted), LFC II (51–70%), LFC III (≤50%).
Exercise Testing
Six-minute walk test (6MWT) and cardiopulmonary exercise testing (CPET) provide complementary information about functional impairment. Many LAM patients demonstrate exercise-induced oxygen desaturation disproportionate to their resting lung function — early supplemental oxygen supplementation guidance relies on exercise oximetry. CPET characteristically shows a ventilatory limitation pattern with reduced peak VO2 and VE/VCO2 slope consistent with the obstructive physiology and impaired gas exchange efficiency of LAM.
Treatment: mTOR Inhibitors and Supportive Care
The 2011 publication of the MILES trial (Multicenter International LAM Efficacy of Sirolimus trial) fundamentally transformed LAM management by demonstrating that sirolimus stabilizes lung function — ending the era of watchful waiting as the only available strategy for most patients.
mTOR Inhibitors: Sirolimus and Everolimus
Sirolimus (rapamycin) inhibits mTORC1 by binding FKBP-12 and allosterically blocking mTORC1 kinase activity, directly countering the TSC2 loss-of-function that drives LAM cell proliferation. In the MILES trial (N Engl J Med, 2011; PMID 21410393), sirolimus stabilized FEV1 over 12 months (change +1 mL in sirolimus vs −12 mL in placebo per month), improved forced vital capacity, DLCO, 6-minute walk distance, and quality of life compared to placebo. Crucially, however, lung function declined again after sirolimus discontinuation — demonstrating that mTOR inhibitors suppress but do not cure the disease, and ongoing treatment is required for sustained benefit. Sirolimus also reduces serum VEGF-D, shrinks renal AMLs by approximately 50–70%, and reduces chylous complications. Target trough serum levels are 5–15 ng/mL. Everolimus (a sirolimus analog) has demonstrated similar efficacy for renal AMLs in TSC and is used interchangeably in many patients. Common side effects include mouth ulcers (stomatitis), infections (particularly opportunistic), hyperlipidemia, proteinuria, cytopenias, impaired wound healing, and rarely pneumonitis.
Indications for mTOR Inhibitor Therapy
Not all patients with LAM require immediate mTOR inhibitor therapy. Current ATS guidelines recommend initiating treatment when any of the following are present: (1) moderate or severe lung function impairment (FEV1 <70% predicted); (2) rapid decline in lung function over observation period; (3) symptomatic or large renal AMLs (≥3 cm, or any AML with symptoms); (4) chylous complications (chylothorax, chylous ascites); (5) functional impairment limiting daily activities. Patients with preserved lung function and no complications may be observed with lung function monitoring every 6–12 months before initiating treatment.
Bronchodilators
Inhaled bronchodilators — short-acting beta-2 agonists and long-acting beta-2 agonists — are used for symptom management in patients with bronchodilator-responsive airflow obstruction. Approximately 30% of LAM patients demonstrate clinically meaningful bronchodilator responsiveness. Inhaled corticosteroids may be considered for patients with significant bronchospasm but are not standard therapy for LAM itself.
Pneumothorax Management
First LAM-associated pneumothorax should be managed with prompt pleurodesis rather than simple drainage alone, given the high recurrence rate exceeding 70%. Chemical pleurodesis (talc or doxycycline via thoracoscopy) or surgical pleurodesis (mechanical abrasion or pleurectomy via VATS) are both effective. The choice must balance effectiveness against the potential impact on future lung transplantation — prior pleurodesis increases the technical difficulty and risk of lung transplant surgery, so thoracic surgical input into the decision is important. Contralateral prophylactic pleurodesis is sometimes considered given the high bilateral risk.
Estrogen Avoidance
Women with LAM should avoid exogenous estrogen. Combined oral contraceptive pills (estrogen-progestin) are contraindicated. Progestin-only contraceptives (progestin-only pills, levonorgestrel IUD, depot medroxyprogesterone) are preferred alternatives. Pregnancy in the context of LAM carries significant risks — documented rapid disease progression during and after pregnancy — and requires thorough counseling. Assisted reproductive technologies that involve ovarian hyperstimulation should be approached cautiously. Hormone replacement therapy at menopause is generally avoided.
Lung Transplantation
Lung transplantation is an option for end-stage LAM with FEV1 <30% predicted, hypoxemia at rest, or rapidly declining function despite optimal medical therapy. LAM-specific considerations include: (1) LAM recurs in the transplanted lung in approximately 1–2% of cases, due to recipient LAM cells colonizing the donor lung; (2) prior pleurodesis increases surgical complexity; (3) young patients may outlive first transplant allografts and require retransplantation. Overall post-transplant survival in LAM is comparable to or better than other transplant indications, with 5-year survival approximately 65–70% in experienced centers.
Prognosis and Transplantation
The natural history of LAM has been substantially altered by mTOR inhibitor therapy, but the disease remains progressive. Understanding prognostic factors helps guide counseling and treatment intensity decisions.
Prognostic Factors
Worse prognosis is associated with: severe baseline lung function impairment (FEV1 <50% predicted at diagnosis), rapid FEV1 decline in the pre-treatment period, diffuse cyst score on HRCT, symptomatic renal AMLs, and absence of bronchodilator responsiveness. Conversely, incidental diagnosis (by definition, early disease), preserved DLCO, and good functional capacity at diagnosis are favorable features. Postmenopausal women, while rare, generally have more indolent disease, consistent with the estrogen dependence of LAM cell proliferation.
Life Expectancy in the Modern Era
Historical data from the pre-sirolimus era suggested median survival from symptom onset of approximately 10 years, but this dramatically underestimates the survival of patients diagnosed today — many of whom are diagnosed earlier, receive mTOR inhibitors, and may live near-normal lifespans with preserved lung function for decades. Registry data suggest that LAM-attributable 10-year mortality is now below 10% in patients with mild-to-moderate disease who receive appropriate therapy. Death from LAM is most commonly due to respiratory failure from progressive cystic destruction.
Monitoring and Follow-Up
Regular monitoring should include pulmonary function tests and 6MWT every 6–12 months (more frequently in patients on mTOR inhibitors or with rapid decline), annual abdominal imaging for AML surveillance, HRCT every 2–3 years or with clinical change, and serum VEGF-D levels as a pharmacodynamic marker during mTOR inhibitor therapy. Patients should be enrolled in the LAM Foundation registry and, where eligible, in clinical trials — research into LAM remains active despite the small patient population.
References & Research
- McCormack FX, Inoue Y, Moss J, et al. Efficacy and safety of sirolimus in lymphangioleiomyomatosis. N Engl J Med. 2011;364(17):1595–1606. PMID: 21410393
- Johnson SR, Cordier JF, Lazor R, et al. European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis. Eur Respir J. 2010;35(1):14–26. PMID: 20044458
- Gupta N, Finlay GA, Kotloff RM, et al. Lymphangioleiomyomatosis diagnosis and management: high-resolution chest computed tomography, transbronchial lung biopsy, and pleural disease management. Ann Am Thorac Soc. 2017;14(7):1175–1182. PMID: 28504576
- Young LR, Almoosa KF, Pollock-Barziv S, Coutinho M, McCormack FX, Sahn SA. Patient perspectives on management of pneumothorax in lymphangioleiomyomatosis. Chest. 2006;129(5):1267–1273. PMID: 16685019
- Taveira-DaSilva AM, Steagall WK, Moss J. Lymphangioleiomyomatosis. Cancer Control. 2006;13(4):276–285. PMID: 17075565
- Bissler JJ, McCormack FX, Young LR, et al. Sirolimus for angiomyolipoma in tuberous sclerosis complex or lymphangioleiomyomatosis. N Engl J Med. 2008;358(2):140–151. PMID: 18184959
- Henske EP, McCormack FX. Lymphangioleiomyomatosis — a wolf in sheep's clothing. J Clin Invest. 2012;122(11):3807–3816. PMID: 23114603
- Baldi BG, Freitas CSG, Araujo MS, et al. Clinical course and characterisation of lymphangioleiomyomatosis in a Brazilian reference centre. Sarcoidosis Vasc Diffuse Lung Dis. 2014;31(2):129–135. PMID: 25078550
- Ando K, Okada Y, Akiba M, et al. Lung transplantation for lymphangioleiomyomatosis in Japan. PLoS One. 2013;8(6):e64594. PMID: 23724058
- Young L, Lee HS, Inoue Y, et al. Serum VEGF-D concentration as a biomarker of lymphangioleiomyomatosis severity and treatment response. Eur Respir J. 2013;42(1):143–152. PMID: 23100500
- Oprescu N, McCormack FX, Byrnes S, Kinder BW. Clinical predictors of mortality and cause of death in lymphangioleiomyomatosis: a population-based registry. Lung. 2013;191(1):35–42. PMID: 23070100
- Taveira-DaSilva AM, Moss J. Management of lymphangioleiomyomatosis. F1000Prime Rep. 2014;6:116. PMID: 25580264