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Rare Disease

lung lymphangioleiomyomatosis

MONDO:0006277

Also known as:Lymphangioleiomyomatosislung lymphangiomyomatosispulmonary lymphangioleiomyomatosis

Find Clinical TrialsTrials

Overview

Lymphangiomyomatosis involving the lungs and local lymph nodes. Patients usually present with chylous pleural effusion. The clinical course is variable. Patients with resectable lesions usually have a favorable clinical outcome. Patients with diffuse lesions usually have a progressive clinical course.

Clinical Trials13 registered · 9 recruiting

Diagnosis

Biomarker and diagnostic research for lung lymphangioleiomyomatosis has been reported in the published literature.

Prognosis

Estimated prevalence: 1-9 in 1,000,000 (Rare).

Research Landscape

Sourced from ClinicalTrials.gov and PubMed. Publication types are classified using NLM-curated article metadata; research type breakdowns reflect the nature of published work for this disease.

13 clinical trials registered, 9 recruiting. Interventions under study include other interventions, drug therapy, procedural interventions, and medical devices. Pipeline includes 1 PHASE3, 4 PHASE1. Research is primarily sponsored by academic and government institutions.

NCT ID	Title	Phase	Sponsor	Status
[NCT02432560](https://clinicaltrials.gov/study/NCT02432560)

Data assembled from 4 of 12 sources · Last updated Sep 19, 2026, 7:26 PM UTC

MONDOHPOClinicalTrials.govFDAPubMedHGNCClinVarClinGenGeneReviewsOrphanetOMIMRUSP

Medical References

External Resources

Orphanet

European rare disease database

Orphanet:538

GARD

Genetic and Rare Diseases Info Center

GARD:3319

Questions for Your Care Team

→Are any of the 13 active clinical trials a good fit for my situation?
→Are there specialists or centers of excellence for this condition in my area?
→Is there a patient registry I should consider joining?

← Back to All Diseases

Research Type	Count	% of Total
Research summaries	35	34%
Laboratory research	16	15%
Disease patterns and progression	15	14%
Testing and diagnosis research	13	13%
Patient case studies	10	10%
Other research	9	9%
Clinical study results	4	4%
New treatment approaches	2	2%

Kisho

lung lymphangioleiomyomatosis

Overview

Diagnosis

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Questions for Your Care Team

Questions for Your Care Team

Recent Publications

Latest News & Developments

Multidisciplinary Management of Lymphangioleiomyomatosis Presenting With Severe Bilateral Pneumothorax During Pregnancy: A Case Report.

Bilateral Lung Transplantation for Lymphangioleiomyomatosis With Secondary Pulmonary Hemosiderosis: A Case Report.

A Case Series of Lymphangioleiomyomatosis, a Rare Cystic Lung Disease.

lung lymphangioleiomyomatosis

Overview

Diagnosis

Prognosis

Research Landscape

Medical References

External Resources

Orphanet

GARD

Related Diseases

Questions for Your Care Team

Questions for Your Care Team

Recent Publications

Latest News & Developments

Multidisciplinary Management of Lymphangioleiomyomatosis Presenting With Severe Bilateral Pneumothorax During Pregnancy: A Case Report.

Bilateral Lung Transplantation for Lymphangioleiomyomatosis With Secondary Pulmonary Hemosiderosis: A Case Report.

A Case Series of Lymphangioleiomyomatosis, a Rare Cystic Lung Disease.