Benefits of Pulmonary Rehabilitation in Patients With Severe Lymphangioleiomyomatosis (LAM)

Benefits of Pulmonary Rehabilitation in Patients With Severe Lymphangioleiomyomatosis (LAM) - a Retrospective Analysis

Data from patients with the orphan disease of lymphangioleiomyomatosis (LAM) which performed a pulmonary Rehabilitation program will be analyzed retrospectively. Data will be taken from the internal data base of the reference Center (Schoen Klinik Berchtesgadener Land, Schoenau, Germany) where These data were collected during clinical routine. Data will be included from the year 2000 until now. A retrospectively matched COPD cohort will be included for comparison.

Study Overview

• Status: Completed
• Conditions: Lymphangioleiomyomatosis; Pulmonary Rehabilitation; LAM
• Intervention / Treatment: Other: Pulmonary Rehabilitation

• Study Type: Observational
• Enrollment (Actual): 58

Contacts and Locations

Study Locations

• Germany
  • Schönau Am Königssee, Germany
    • Schoen Klinik Berchtesgadener Land

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years and older (Adult, Older Adult)
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Probability Sample

Study Population

patients with a confirmed diagnosis of Lymphangioleiomyomatosis

Description

Inclusion Criteria:

• confirmed diagnosis of Lymphangioleiomyomatosis
• performed a pulmonary Rehabilitation program at the reference center

Exclusion Criteria:

• missing data
• repeated stay for pulmonary Rehabilitation at the reference center

Study Plan

How is the study designed?

Design Details

• Observational Models: Cohort
• Time Perspectives: Retrospective

Number of groups / cohorts

1

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
Pulmonary Rehabilitation	Other: Pulmonary Rehabilitation; Patients will perform a comprehensive, inpatient pulmonary rehabilitation program for 4 weeks duration

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Change from baseline 6 Minute walk distance at week 4	the walked distance in meter will be the Primary outcome of the 6 Minute walk test	baseline and week 4
Change from baseline Quality of life at week 4	the physical and mental health component summary score from the Short Form 36 Health Questionnaire will be recorded. Score range from 0 to 100 with lower scores indicating worse impairment	baseline and week 4

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Change from baseline lung function at week 4	forced expiratory volume in 1 second and inspiratory vital capacity will be provided in Liter and percent predicted	baseline and week 4

Collaborators and Investigators

• Sponsor: Schön Klinik Berchtesgadener Land
• Investigators: Principal Investigator: Rembert A Koczulla, MD, Schoen Klinik Berchtesgadener Land

Publications and helpful links

General Publications

• Gloeckl R, Nell C, Schneeberger T, Jarosch I, Boensch M, Watz H, Wirtz H, Welte T, Kenn K, Koczulla AR. Benefits of pulmonary rehabilitation in patients with advanced lymphangioleiomyomatosis (LAM) compared with COPD - a retrospective analysis. Orphanet J Rare Dis. 2020 Sep 22;15(1):255. doi: 10.1186/s13023-020-01540-3.

Study record dates

Study Major Dates

• Study Start (Actual): December 3, 2019
• Primary Completion (Actual): December 23, 2019
• Study Completion (Actual): December 23, 2019

Study Registration Dates

• First Submitted: November 11, 2019
• First Submitted That Met QC Criteria: December 2, 2019
• First Posted (Actual): December 3, 2019

Study Record Updates

• Last Update Posted (Actual): January 21, 2020
• Last Update Submitted That Met QC Criteria: January 16, 2020
• Last Verified: January 1, 2020

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Immune System Diseases; Neoplasms, Connective and Soft Tissue; Neoplasms by Histologic Type; Neoplasms; Lymphoproliferative Disorders; Lymphatic Diseases; Immunoproliferative Disorders; Lymphangiomyoma; Lymphatic Vessel Tumors; Perivascular Epithelioid Cell Neoplasms; Lymphangioleiomyomatosis
• Other Study ID Numbers: LAM Trial

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No