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. 2022 Dec;54(1):2744-2751.
doi: 10.1080/07853890.2022.2128401.

Physiotherapy in lymphangioleiomyomatosis: a systematic review

Victoria Maria Garcia de Medeiros  1 , Jéssica Gonçalves de Lima  1 , Claudia Rosa  2 , Juliana Rega  2 , Mauro Felippe Felix Mediano  1   3 , Luiz Fernando Rodrigues Junior  1   4
Affiliations

Physiotherapy in lymphangioleiomyomatosis: a systematic review

Victoria Maria Garcia de Medeiros et al. Ann Med. 2022 Dec.

Abstract

Background: Lymphangioleiomyomatosis (LAM) is associated with progressive dyspnoea and exercise intolerance, but despite the central role of physiotherapy on pulmonary rehabilitation, there is a huge lack of physiotherapy approaches used specifically for LAM patients.

Objective: to identify the physiotherapeutic strategies used in the treatment of patients with LAM.

Materials and methods: This is a systematic review of literature. Searches were performed (in PubMed, Lilacs, Embase and PEDro databases) with the keywords "Lymphangioleiomyomatosis" and "Physiotherapy," and its variations. Articles describing physiotherapy interventions were included in the study. Data extracted from the studies were authors, year, country of publication, sample size, physiotherapy intervention, time/frequency/duration of intervention protocols, instruments used to measure results and main findings. Methodological quality of studies was evaluated by PEDro Scale (clinical trials), Newcastle-Ottawa Scale (NOS; observational studies) and CARE checklist (case reports), respectively.

Results: A total of 82 articles identified, three duplicates were removed, 71 studies were excluded after title and abstract reading and four after full-text reading, all due to absence of association with the study topic. Four studies were included in the present review. Cardiorespiratory physiotherapy with endurance and resistance training were identified as physiotherapeutic strategies to improve lung function, functional capacity, depression symptoms and quality of life in LAM.

Conclusions: Endurance and resistance training is the keystone for physiotherapy in patients with LAM, but despite the reported benefits, there is a huge lack of studies related to the modalities, safety and dosage of physiotherapy prescription for patients with LAM.KEY MESSAGESLymphangioleiomyomatosis (LAM), a rare disease, leads to progressive dyspnoea and exercise intolerance;Physiotherapy can improve dyspnoea and exercise intolerance in LAM through endurance and resistance exercises.

Keywords: Lymphangioleiomyomatosis; exercise; physiotherapy; rehabilitation.

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Conflict of interest statement

No potential conflict of interest was reported by the author(s).

Figures

A total of 82 articles were selected, from those three duplicates were removed and 71 were excluded after title and abstract reading due to the absence of association with the study topic. The remaining eight were read in full text for screening for eligibility, four of them were excluded for the absence of association with the study topic. So, four studies were included in this review.
Figure 1.
Study flowchart.
The box elicits the poor methodological approach of the studies related to physiotherapy in LAM.
Figure 2.
Methodological quality assessment of the studies included in the review.
See this image and copyright information in PMC

References

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    1. Diretrizes de Doenças Pulmonares Intersticiais da Sociedade Brasileira de Pneumologia e Tissologia. J Bras Pneumol. 2012;38(2):S1–S133.

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