Lymphangioleiomyomatosis
New lymphangioleiomyomatosis research, summarized each month in plain language.
2026 so far: So far in 2026 (January 2026 to September 2026), routine-care reports linked sirolimus with stable lung function and clearer improvements in lymphatic and tumor findings. Structured exercise improved walking distance, and fatigue was linked to muscle performance. Read the 2026 review
We also cover clinical trials.
Yearly update
2026 so far
- Sirolimus outcomes. Evidence shifted toward multi-organ outcomes, not just spirometry.
- Exercise and fatigue. Management focus expanded from lungs to function and fatigue.
- Pleural fluid care. Case reports detailed varied pleural fluid patterns and targeted procedures.
- Function and risk. Walking distance repeatedly diverged from FEV1 change patterns.
- Getting the diagnosis. Reports emphasized pattern-based diagnosis and limits of single tests.
New
September 2026
- Pleural fluid drainage. In one case, targeted treatment improved drainage, and the lung fully re-expanded after further drainage and repeat pleurodesis.
- Atypical test results. Unusual imaging still matched LAM when other tests supported it.
- Sirolimus response pattern. Some lymphatic lesions shrank, while lung tests changed little.
August 2026
- Muscle weakness, fatigue. In LAM, weaker muscles matched higher fatigue reports.
- Endurance and activity. Muscle endurance aligned with walking distance and exercise capacity.
- Energy use during exercise. Lower fat use during exercise was linked to worse fatigue.
- Stopping sirolimus. In one severe TSC-LAM case, symptoms worsened after abrupt stopping.
July 2026
- Collapsed lung sign. A collapsed lung led to scans showing many lung cysts.
- Chylothorax finding. The same workup also found chylothorax (milky chest fluid).
- Lymph node finding. LAM was found in lymph nodes during other cancer care.
- Robotic lung surgery. One case reported no ongoing air leak after robotic surgery.
June 2026
- Sirolimus results. Routine-care data linked sirolimus to stable lungs and smaller tumors.
- Exercise effects. Structured exercise increased 6-minute walk distance; other changes were unclear.
- Test interpretation. One biopsy marker was consistent, but a blood test can mislead.
May 2026
- Breath test patterns. Breath testing could separate several lung diseases, but LAM was harder for the model to classify.
- Biopsy stain consistency. Some tissue stains were consistent, while HMB-45 staining was often patchy and easier to miss in small areas.
- Stable spirometry, lower walking distance. Over long follow-up, breathing test numbers stayed fairly stable while walking distance declined.
April 2026
- Europe-wide LAM registry proposal. A European group proposed a shared LAM registry to collect more standardized data across countries.
- IL-6 and lung function. Higher IL-6 levels were reported to be associated with worse lung function in LAM.
- Estrogen and IL-6 in lab studies. In laboratory models, estrogen signaling and IL-6 were reported to reinforce LAM-like cell behavior.
- Diagnosis can require tissue testing. Some reported LAM diagnoses depended on detailed testing of lung tissue, especially when other lung conditions were also present.
March 2026
- Urine protein signal. One urine protein (pro‑cathepsin K) was higher in a small LAM group, but it was not clearly linked to lung measurements or useful for monitoring.
- Bone density link. In the same small study, higher urine pro‑cathepsin K was associated with femur bone density scores in people with LAM.
- LAM can look different. Case reports showed LAM can resemble other conditions and may be diagnosed in less typical settings.
- Sirolimus in clinic follow-up. A small real‑world clinic series reported improved breathing symptoms and higher FEV1 after sirolimus in several patients.
February 2026
- Confirm true lung cysts first. A step-by-step CT review focused first on confirming that the findings are true lung cysts before narrowing the cause, including LAM, to avoid confusion with similar-looking changes.
- VEGF-D supports, but does not rule out. VEGF-D was usually higher in definite LAM than in non-LAM conditions, but some people with typical LAM features still had VEGF-D below 800 pg/mL.
- TSC-related findings can mimic LAM. Case reports showed that TSC-related tumors can affect multiple organs and sometimes resemble LAM outside the lungs, and genetic plus pathology testing helped clarify the diagnosis.
January 2026
- Sirolimus blood levels. Lower sirolimus levels showed similar lung function change.
- 6-minute walk distance. Shorter walk distance was linked to worse survival.
- Blood protein markers. New protein markers helped separate LAM from healthy controls.
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