Evidence map›Paper›PMID 31437431›Full record

ArticleChest2019

Immunotherapy for Lymphangioleiomyomatosis and Tuberous Sclerosis: Progress and Future Directions.

Heng-Jia Liu, Vera P Krymskaya, Elizabeth P Henske

Open access · bronzeAbstract read
In one paragraph

Article in Chest, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 11 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
11citing papers in PubMed, 1 pooled it
1.8field-weighted citation impact, top 14% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

11 citing papers in PubMed, 1 synthesis or guideline pooled it, 21 citations in OpenAlex.

  1. Brazilian Thoracic Association recommendations for the management of lymphangioleiomyomatosis.Jornal brasileiro de pneumologia : publicacao oficial da Sociedade Brasileira de Pneumologia e Tisilogia · 2025
    Guideline
  2. Lymphangioleiomyomatosis: a metastatic lung disease.American journal of physiology. Cell physiology · 2023
    Review
  3. Article
  4. Article
  5. Review
  6. Article
  7. Article
  8. Article
  9. Interleukin-6 mediates PSAT1 expression and serine metabolism in TSC2-deficient cells.Proceedings of the National Academy of Sciences of the United States of America · 2021
    Article
  10. Article
  11. Adoptive T-Cell Transfer to Treat Lymphangioleiomyomatosis.American journal of respiratory cell and molecular biology · 2020
    Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

3 authors at 3 institutions in 1 country.

Heng-Jia LiuPulmonary and Critical Care Medicine, Department of Medicine, Brigham and Women's Hospital and Harvard Medical School, Boston, MA.
Vera P KrymskayaLung Biology Institute and Pulmonary, Allergy and Critical Care Division, Department of Medicine, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA.
Elizabeth P HenskePulmonary and Critical Care Medicine, Department of Medicine, Brigham and Women's Hospital and Harvard Medical School, Boston, MA. Electronic address: ehenske@bwh.harvard.edu.
Brigham and Women's Hospital · USHarvard University · USUniversity of Pennsylvania · US

Funding

mTORC1 and WNT in lung mesenchymeR01HL158737 · NHLBI · UNIVERSITY OF PENNSYLVANIA · PI KRYMSKAYA, VERA P · 2021 to 2024
$2.5M
mTOR signaling in lung homeostasis, aging and diseaseR01HL151467 · NHLBI · UNIVERSITY OF PENNSYLVANIA · PI KRYMSKAYA, VERA P · 2020 to 2023
$2.0M
NHLBI NIH HHS R01 HL151467NHLBI NIH HHS R01 HL158737
6 · The paper itself

Abstract

Pulmonary lymphangioleiomyomatosis (LAM) is a rare genetic multisystem disease characterized by the nodular proliferation of smooth muscle-like LAM cells, progressive cystic changes of the lung, lymphatic abnormalities, and renal angiomyolipomas (AMLs). LAM can arise sporadically or in women with the autosomal dominant disorder, tuberous sclerosis complex (TSC), in which hamartomatous tumors of brain, heart, skin, kidney, and lung are found. LAM and TSC are caused by mutations in the TSC1 or TSC2 tumor suppressor genes leading to elevated mechanistic/mammalian target of rapamycin complex activity. Recent data indicate that T cells within LAM nodules and renal AMLs exhibit features of T-cell exhaustion, with coinhibitory receptor programmed cell death protein 1 (PD-1) expression on tumor-infiltrating T cells. Treatment of animal models of TSC and LAM with anti-PD-1 antibodies or with the combination of anti-PD-1 and anti-CTLA4 antibodies has led to remarkable results, suppressing TSC2-null tumor growth and inducing tumor rejection. Here we review our current knowledge about the potential for immunotherapy for the treatment of LAM and TSC and highlight critical unknowns and key next steps.

Indexed as

ImmunotherapyAnimalsCell Cycle CheckpointsForecastingHumansLung NeoplasmsLymphangioleiomyomatosisTuberous Sclerosiscancer immunotherapyCTLA-4immune checkpoint blockadelymphangioleiomyomatosis (LAM)PD-1PD-L1tuberous sclerosis complex (TSC)

Identifiers

PMID31437431
PMCPMC13169335
OpenAlexW2969333873

What OpenQuestion holds

Textmetadata
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.