September 2026 Research Update

This month, the clearest pattern was that pleural fluid problems in LAM can be complicated and may not look the same from person to person, but several reports showed they can sometimes be improved with carefully targeted approaches. Across cases, chylous (milky, lymph-fluid) effusions and trapped or separated fluid pockets were central drivers of shortness of breath and repeated hospital care. These reports add practical detail about how teams confirmed the cause of the fluid and what was tried when routine drainage was not enough. [1] [2] [3]

In one post-lung transplant situation, contrast-enhanced ultrasound was used to tell apart non-blood-supplied fibrin walls from normal pleural tissue, so the walls splitting the fluid into pockets could be punctured with a fine needle and urokinase (a clot-dissolving medicine) injected precisely into the pockets. The report described opening of the fluid pockets and improved drainage after targeted treatment. Following additional drainage and repeat pleurodesis to address the leak, imaging showed full lung re-expansion without recurrence. This adds an early example of how real-time imaging might help make pleural procedures more targeted when effusions are loculated and refractory. [1]

Two other reports highlighted that chylous pleural effusions can occur alongside broader lymphatic involvement, and diagnosis can be challenging when resources are limited. One person had recurrent chylothorax with atypical lung imaging (not the classic widespread lung cyst pattern), but very high VEGF-D and biopsy findings supported LAM with lymphatic involvement, and extrapulmonary lymphatic lesions became much smaller on sirolimus while lung function changed little in the short term. Another person was first treated as tuberculosis without improvement, then pleural fluid testing and CT imaging supported LAM; symptoms improved soon after starting sirolimus, although the medicine was later stopped due to cost. [2] [3]

For patients, these reports reinforce that ongoing or recurrent pleural fluid buildup in LAM may have different underlying features, such as chylous fluid or fluid trapped in separate pockets, and that figuring out which one is present can change what the care team tries next. The evidence here is early and based on individual cases, so it does not prove what will work for most people, but it does clarify what options have been attempted when standard drainage is not enough. What remains uncertain is how often approaches like contrast-enhanced ultrasound-guided fibrinolysis help, and which patients are most likely to benefit, especially outside specialized centers. For now, this mainly adds to shared medical knowledge about real-world problem-solving rather than establishing a new standard approach. [1] [2] [3]

This month also added examples of how LAM may surface during major life events or alongside other conditions, where symptoms trigger urgent evaluation and multidisciplinary care. The reports show that diagnosis sometimes required combining imaging with tissue testing, and that resource limits affected which tests could be done. Together, these cases expand the picture beyond "typical" presentations by showing the broader context in which LAM can be recognized and managed. [4] [5]

One report described a pregnant person at 17 weeks with severe breathing difficulty and bilateral pneumothorax, where emergency chest drainage and a thoracoscopic lung biopsy confirmed LAM, and sirolimus was started after stabilization. The case emphasized that LAM during pregnancy can present dramatically and require coordination across lung, surgical, and obstetric teams; some common tests, such as VEGF-D, were not available. The person was discharged in improved condition, but longer-term lung function tracking was not reported. [4]

Another report centered on tuberous sclerosis complex (TSC), where abnormal uterine bleeding led to discovery of multiple organ involvement, including pulmonary LAM and kidney angiomyolipomas. The bleeding was attributed to uterine PEComas, and the report described partial improvement in bleeding with hormonal treatment and iron, plus kidney surgery for an angiomyolipoma. This case adds a reminder that in some people, LAM appears as one part of a multi-organ condition, and the first symptom bringing someone to care may not be lung-related. [5]

For patients and caregivers, these reports help clarify that LAM can intersect with pregnancy decisions and with TSC-related multi-organ monitoring, and that diagnosis may rely on whatever tests are realistically available. Because these are single-person reports, they do not predict what will happen to others, but they do show the kinds of situations where LAM is sometimes first recognized or confirmed. What remains uncertain is how widely the described pathways of care apply across different health systems, especially where key lab tests or follow-up breathing tests are hard to access. For now, this information mainly helps people understand the range of real-world presentations rather than changing day-to-day care for most patients. [4] [5]