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OBJECTIVE: To evaluate the reliability of the Dutch version of the Pediatric Evaluation of Disability Inventory (PEDI), an instrument for measuring functional status (capability and performance in self-care, mobility and social function) of young children using parent interviews. DESIGN: Inter-interviewer reliability was studied after scoring audiotaped interviews by a second researcher. For test-retest reliability the same parent was interviewed twice within three weeks; in inter-respondent reliability both parents of a child were interviewed independently within a few days. On item level, percentage identical scores were computed, and on scale level intraclass correlation coefficients (ICC) and Cronbach's alphas were calculated. SUBJECTS: Parents of 63 nondisabled and 53 disabled (various diagnosis) children aged between 7 and 88 months were interviewed. RESULTS: On scale level, all ICCs were above 0.90 and Cronbach's alpha was 0.89 for the self-care domain, 0.74 for the mobility domain and 0.87 for the social function domain. On item level for the Functional Skills Scale, the mean percentage identical scores varied from 89 to 99, and for the Caregiver Assistance Scale from 54 to 90. Different scores between interviewers resulted partially from ambiguous interpretation of the item and/or the explanation. CONCLUSIONS: Although small adaptations have to be made, the psychometric properties of the Dutch PEDI are found to be good. [ABSTRACT FROM AUTHOR]

Introduction: Idiopathic pulmonary fibrosis (IPF) is a fatal progressive fibrosing lung disease. A decreased 6-min walk distance (6MWD) and exercise-induced oxygen desaturation measured during the 6-min walk test (6MWT), are known predictors of mortality in patients with IPF. However, the use of antifibrotic drugs showed a survival benefit in IPF. Therefore, this study aimed to evaluate to what extend 6MWT-derived attributes are associated with two-year survival when antifibrotic drugs were introduced as part of standard IPF-care., Methods: This real-world data-study included patients with IPF with a 6MWT between 2015 and 2020, and used composite outcome: mortality or lung transplantation within 2 years of follow-up. Data were collected systematically, including demographics, pulmonary function tests, comorbidities, medications and 6MWT-derived attributes. The prediction attributes of 6MWT were studied with a Cox Proportional-Hazards model and Kaplan-Meier survival curves. The best discriminating attribute to predict mortality was added to the prediction model Gender-Age-Physiology (GAP)., Results: In 216 patients, 2-year transplant-free survival cut-off points were identified for the 6MWD (≥413 m), 6MWD %predicted (≥83 %), SpO 2 -nadir (≥86 %) and distance-saturation-product (≥374 m%), with the best discriminative value for SpO 2 -nadir (area under the curve: 0.761). 2-Year survival percentage of patients with SpO 2 -nadir below or above threshold (86 %) was 37.1 % and 80.0 %, respectively. Exercise-induced oxygen desaturation added to the GAP model showed an improvement in its predictive power., Conclusion: Patients with IPF who have an exercise-induced oxygen desaturation have worse prognosis. Addition of SpO 2 -nadir to the GAP model seems promising for use in clinical care of IPF patients., Competing Interests: Declaration of competing interest The author declares on behalf of all authors that they have no relevant or material financial interests related to the research described in this article., (Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Introduction: Besides dyspnoea and cough, patients with idiopathic pulmonary fibrosis (IPF) or sarcoidosis may experience distressing non-respiratory symptoms, such as fatigue or muscle weakness. However, whether and to what extent symptom burden differs between patients with IPF or sarcoidosis and individuals without respiratory disease remains currently unknown., Objectives: To study the respiratory and non-respiratory burden of multiple symptoms in patients with IPF or sarcoidosis and to compare the symptom burden with individuals without impaired spirometric values, FVC and FEV1 (controls)., Methods: Demographics and symptoms were assessed in 59 patients with IPF, 60 patients with sarcoidosis and 118 controls (age ≥18 years). Patients with either condition were matched to controls by sex and age. Severity of 14 symptoms was assessed using a Visual Analogue Scale., Results: 44 patients with IPF (77.3% male; age 70.6±5.5 years) and 44 matched controls, and 45 patients with sarcoidosis (48.9% male; age 58.1±8.6 year) and 45 matched controls were analyzed. Patients with IPF scored higher on 11 symptoms compared to controls (p<0.05), with the largest differences for dyspnoea, cough, fatigue, muscle weakness and insomnia. Patients with sarcoidosis scored higher on all 14 symptoms (p<0.05), with the largest differences for dyspnoea, fatigue, cough, muscle weakness, insomnia, pain, itch, thirst, micturition (night, day)., Conclusions: Generally, respiratory and non-respiratory symptom burden is significantly higher in patients with IPF or sarcoidosis compared to controls. This emphasizes the importance of awareness for respiratory and non-respiratory symptom burden in IPF or sarcoidosis and the need for additional research to study the underlying mechanisms and subsequent interventions., Competing Interests: Declaration of Competing Interest None., (Copyright © 2023 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: Self-management is considered important in the management of patients with idiopathic pulmonary fibrosis (IPF) or sarcoidosis. However, data about the degree of activation for self-management is lacking., Objectives: The aim of the study was to determine the degree of activation for self-management in patients with IPF or sarcoidosis using the Patient Activation Measure (PAM) and to evaluate the association between PAM scores, clinical characteristics, and health-related outcomes., Study Design and Methods: This cross-sectional prospective study assessed besides the PAM also demographics, lung function, dyspnea (modified Medical Research Council [mMRC]), fatigue (Checklist Individual Strength-Fatigue [CIS-Fatigue]), anxiety/depression (Hospital Anxiety and Depression Scale [HADS-A/HADS-D]), and generic health status (EuroQol five-dimensional-five-level [EQ-5D-5L])., Results: Mean PAM was 55.0 (9.1) points in patients with IPF (n = 59) and low levels of patient activation for self-management (PAM ≤55.1 points) were present in 56% of the patients. PAM Scores correlated significantly (p < 0.05) with mMRC (ρ = -0.476), HADS-A (ρ = -0.326), HADS-D (ρ = -0.459), and EQ-5D-5L (ρ = 0.393). In patients with sarcoidosis (n = 59), the mean PAM score was 55.7 (11.0) points, and 46% of the patients reported low PAM levels. Significant correlations were found with mMRC (ρ = -0.356), HADS-A (ρ = -0.394), HADS-D (ρ = -0.478), and EQ-5D-5L (ρ = 0.313)., Conclusion: About half of the outpatients with IPF or sarcoidosis have a low degree of activation for self-management, and these patients generally report more dyspnea, anxiety, depression, and a lower health status. Whether patients with a low degree of activation can be successful in self-managing their disease remains to be determined., (© 2021 S. Karger AG, Basel.)

In patients with interstitial lung disease (ILD) next to dyspnea, fatigue is expected to be the most prevalent symptom. Surprisingly, the prevalence of severe fatigue has been scarcely studied in ILD patients and limited information on its associated factors is available. This study aimed to determine the prevalence of severe fatigue in patients with idiopathic pulmonary fibrosis (IPF) or pulmonary sarcoidosis and to identify the relationship between fatigue, patient characteristics, and clinical parameters. In this cross-sectional study, fatigue (checklist individual strength-fatigue (CIS-Fat)), demographics, lung function, dyspnea (modified-Medical Research Council (mMRC)), sleepiness (Epworth Sleepiness Scale), anxiety/depression (hospital anxiety and depression scale (HADS-A/HADS-D)), catastrophizing (fatigue catastrophizing scale (FCS)), functional activity impairment (respiratory illness quality-of-life (QoL-RIQ-Activity)), and health status (EuroQol five-dimensional descriptive system (EQ-5D-5L)) were assessed in outpatients with ILD. Mean CIS-Fat scores were 34.1 (SD ± 11.2) in 59 IPF patients and 40.0 (12.3) in 58 sarcoidosis patients. Severe fatigue (SD ± ≥36 points) was present in IPF patients (47.5%) and sarcoidosis (69%). In IPF, CIS-Fat correlated strongly (ρ > 0.5; p < 0.01) with FCS, QoL-RIQ-Activity, and EQ-5D-5L-Health and moderately (0.3 < ρ < 0.5; p < 0.01) with EQ-5D-5L-Index, mMRC, and HADS-D. In sarcoidosis, CIS-Fat correlated strongly with EQ-5D-5L-Health, QoL-RIQ-Activity, EQ-5D-5L-Index, HADS-D, and mMRC and moderately with FCS and hospitalization <12 months. Severe fatigue is highly prevalent in ILD patients and is associated with dyspnea, depression, catastrophizing, functional activity impairments, and QoL., Competing Interests: The authors declare no conflict of interest.

Background and objective: Patients with pulmonary fibrosis (PF) have a clear exercise intolerance. The 4-meter-gait-speed (4MGS) test and the 5-repetitions-sit-to-stand (5STS) test are easy, inexpensive and reliable measures of functional performance. Both tests have been validated in healthy adults and patients with chronic obstructive pulmonary disease. 4MGS test and 5STS test have not been studied in patients with PF. Methods: In this cross-sectional clinimetric validation study 51 PF patients conducted in random order the 4MGS test, 5STS test and the 6-min walk test (6MWT) on a single day. Additionally, body weight, height, lean body mass, health-related quality of life, disease severity, handgrip strength, dyspnoea and leg fatigue were assessed. The setting was a tertiary referral center for Interstitial Lung Diseases. Results: Patients had a diagnosis of idiopathic pulmonary fibrosis (IPF, 37%), PF other than IPF (47%), or unclassified (16%). Patients walked 453±111m in six minutes. Moreover, it took the patients 2.0±0.5s to walk 4 m, and 12.0±3.8s for the 5STS test. The 4MGS test (r = 0.77; p<0.01) and the 5STS test (r = -0.41; p<0.01) correlated significantly with the distance walked in 6MWT. Indeed, 4MGS combined with handgrip strength and Medical Research Council dyspnoea grade could explain 75% of the variance in 6MWD. Conclusions: 4-meter-gait-speed and 5-repetitions sit-to-stand are significantly and independently correlated with the 6-minute walk distance in patients with pulmonary fibrosis. Indeed, 4-meter-gait-speed test may serve as a simple initial field test to assess exercise performance in patients with pulmonary fibrosis. (Sarcoidosis Vasc Diffuse Lung Dis 2018; 35: 317-326) ., (Copyright: © 2018 SARCOIDOSIS VASCULITIS AND DIFFUSE LUNG DISEASES.)