Evidence map›Paper›PMID 39593123›Full record

ArticleRespiratory research2024

Pulmonary lymphangiomatosis: insights into an ultra-rare disease.

M Polke, N Polke, S Piel, E Brunnemer, J Wälscher, K Buschulte, A Warth, C P Heussel, M Eichinger, L Frankenstein and 4 more

Abstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in Respiratory research, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 3 papers.

0numbers the graph read from it
0cells of the map it votes in
3citing papers in PubMed
–field-weighted citation impact
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

3 citing papers in PubMed.

  1. Article
  2. Repeat misdiagnosis of chylous pneumonia: a case description.Quantitative imaging in medicine and surgery · 2026
    Article
  3. Review
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

14 authors.

M PolkeCenter for Interstitial and Rare Lung Diseases, Pneumology and Critical Care Medicine, Thoraxklinik, University of Heidelberg, Heidelberg, Germany. markus.polke@med.uni-heidelberg.de.
N PolkeCenter for Interstitial and Rare Lung Diseases, Pneumology and Critical Care Medicine, Thoraxklinik, University of Heidelberg, Heidelberg, Germany.
S PielCenter for Interstitial and Rare Lung Diseases, Pneumology and Critical Care Medicine, Thoraxklinik, University of Heidelberg, Heidelberg, Germany.
E BrunnemerCenter for Interstitial and Rare Lung Diseases, Pneumology and Critical Care Medicine, Thoraxklinik, University of Heidelberg, Heidelberg, Germany.
J WälscherCenter for Interstitial and Rare Lung Diseases, Pneumology Department, Ruhrlandklinik, University Hospital, University of Essen, Essen, Germany.
K BuschulteCenter for Interstitial and Rare Lung Diseases, Pneumology and Critical Care Medicine, Thoraxklinik, University of Heidelberg, Heidelberg, Germany.
A WarthTranslational Lung Research Center Heidelberg, Member of the German Center for Lung Research DZL, Heidelberg, Germany.
C P HeusselTranslational Lung Research Center Heidelberg, Member of the German Center for Lung Research DZL, Heidelberg, Germany.
M EichingerTranslational Lung Research Center Heidelberg, Member of the German Center for Lung Research DZL, Heidelberg, Germany.
L FrankensteinDepartment of Cardiology, University of Heidelberg, Heidelberg, Germany.
M EichhornDepartment of Thoracic Surgery, University of Heidelberg, Heidelberg, Germany.
S MiliauskasDepartment of Pulmonology, Lithuanian University of Health Sciences, Kaunas, Lithuania.
F J F HerthCenter for Interstitial and Rare Lung Diseases, Pneumology and Critical Care Medicine, Thoraxklinik, University of Heidelberg, Heidelberg, Germany.
M KreuterCenter for Pulmonary Medicine, Department of Pneumology, Mainz University Medical Center, Mainz, Germany.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundPulmonary lymphangiomatosis (PL) is an ultrarare disease characterized by diffuse infiltration of the lung, pleura and/or mediastinum by abnormal lymphatic proliferation. Consented diagnostic or treatment approaches are not established. We therefore aimed to collect data on diagnostics and treatments in a cohort of patients with PL from a tertiary center for rare lung diseases.

methodsClinical, radiological and outcome data from PL patients were collected retrospectively.

results12 patients were diagnosed between 1996 and 2022 in our center. PL was diagnosed more commonly in female (58%), never smokers (75%) and younger patients (mean age 42 years). Main clinical symptoms comprised haem- and chyloptysis (58%) and dyspnea on exertion (83%). Pulmonary function was mostly restrictive (mean VC 59%) with impaired DLCO (mean 65%). Radiological assessment mainly showed mediastinal involvement (83%), and pleural effusion (67%), pleural thickening (67%) and bronchial wall thickening (67%) while interstitial changes were rare. Diagnosis was confirmed by surgical or transbronchial cryobiopsy. 8 patients were treated with sirolimus, 3 of these combined with a surgical intervention and in one case surgical intervention was necessary 9 months after initiation of sirolimus. Clinical and radiological improvement was demonstrated for all patients treated with sirolimus. 1 patient received a lung transplant due disease progression. Survival rates were 90% after a mean follow up of at least 3 months.

conclusionThis case series illustrates the variability of the clinical presentation of PL. Among our patients, those treated with sirolimus showed significant clinical, functional and radiological improvement. However, further investigation is needed to understand the pathogenesis of lymphangiomatosis in order to establish therapeutic approaches.

Indexed as

Rare DiseasesAdultAgedFemaleHumansLung DiseasesLymphangiectasisMaleMiddle AgedRetrospective StudiesSirolimusTreatment OutcomeYoung AdultSirolimus

Identifiers

PMID39593123
PMCPMC11600747

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Registered trials

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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.