Interstitial Lung Disease With Systemic Sclerosis
Conditions
Keywords
core endurance, pelvic floor dysfunction, exercise capacity, pelvic floor knowledge, pulmonary function
Brief summary
Systemic sclerosis (SSc) is a heterogeneous multisystem disease characterized by vascular dysfunction, immune abnormalities, and progressive fibrosis of the skin and internal organs. Pulmonary involvement, particularly interstitial lung disease (ILD), is one of the most common and clinically important manifestations of SSc and may lead to impaired pulmonary function, respiratory muscle weakness, reduced diaphragmatic function, and decreased exercise capacity. In addition to pulmonary involvement, systemic inflammation, physical inactivity, nutritional disturbances, and musculoskeletal manifestations may contribute to generalized skeletal muscle dysfunction in individuals with SSc. Pelvic floor dysfunction, including urinary and fecal incontinence, pelvic organ prolapse, pelvic pain, and sexual dysfunction, has also been reported in individuals with SSc. Previous studies have suggested that vascular dysfunction, tissue fibrosis, and structural and functional abnormalities of the pelvic floor may contribute to these symptoms. Furthermore, impaired pelvic floor and sexual function in women with SSc have been associated with several disease-related characteristics, including dyspnea, interstitial lung disease, reduced physical activity, functional disability, and impaired quality of life. The pelvic floor muscles form the inferior component of the core muscle system and function synergistically with the diaphragm and abdominal muscles to regulate intra-abdominal pressure. Coordinated activity between the diaphragm and pelvic floor muscles during respiration has been demonstrated. Therefore, pulmonary and respiratory muscle involvement in SSc may potentially be associated with impaired pelvic floor function. However, the relationships between pelvic floor dysfunction and pulmonary function, respiratory muscle strength, exercise capacity, and core endurance in patients with SSc with pulmonary involvement remain insufficiently understood. Investigating these relationships may contribute to a more comprehensive understanding of pelvic floor dysfunction and its associated factors in this population.
Interventions
OTHERPelvic Floor Health Knowledge Assessment
Pelvic floor health knowledge will be assessed using the Pelvic Floor Health Knowledge Test. The questionnaire consists of 29 items covering pelvic floor function and dysfunction, risk factors and etiology, and diagnosis and treatment. Higher scores indicate greater knowledge of pelvic floor health.
OTHERPelvic Floor Dysfunction Assessment
Pelvic floor dysfunction will be assessed using the Pelvic Floor Distress Inventory-20 (PFDI-20), a 20-item questionnaire evaluating symptoms and symptom-related distress associated with pelvic floor disorders. It consists of three subscales and provides a total score ranging from 0 to 300, with higher scores indicating greater symptom burden.
Core endurance will be assessed using the McGill core endurance tests, including trunk flexor, trunk extensor, and lateral trunk endurance tests. Participants will maintain standardized test positions for as long as possible, and endurance time will be recorded in seconds.
Body composition will be assessed using bioelectrical impedance analysis with a Tanita BC-730 InnerScan body composition analyzer. Body fat mass and muscle mass will be recorded in kilograms under standardized measurement conditions.
Sexual function will be assessed using the Arizona Sexual Experiences Scale (ASEX), a five-item questionnaire evaluating sexual drive, arousal, physiological arousal, ability to reach orgasm, and satisfaction from orgasm. Higher scores indicate greater sexual dysfunction.
OTHERRespiratory Function
Respiratory function will be assessed using pulmonary function testing and DLCO according to ATS criteria. Respiratory muscle strength will be evaluated by maximal inspiratory (MIP) and expiratory (MEP) pressures using a MicroRPM device according to ATS/ERS recommendations. Cough-related quality of life will be assessed using the Leicester Cough Questionnaire (LCQ), and dyspnea using the modified Medical Research Council (mMRC) Dyspnea Scale.
OTHERFunctional Exercise Capacity Assessment
Functional exercise capacity will be assessed using the 6-Minute Walk Test (6MWT). Participants will be instructed to walk as far as possible for six minutes along a 30-meter corridor. The total distance walked will be recorded in meters.
OTHERFunctional Status Assessment
Disease-related functional status will be assessed using the Scleroderma Health Assessment Questionnaire (SHAQ). The questionnaire evaluates functional limitations and disease-specific manifestations associated with systemic sclerosis, with higher scores indicating greater functional impairment.
Sponsors
Biruni University
Study design
Observational model
COHORT
Time perspective
CROSS_SECTIONAL
Eligibility
Sex/Gender
FEMALE
Age
18 Years to 70 Years
Healthy volunteers
No
Inclusion criteria
* Meeting the 2013 European Alliance of Associations for Rheumatology/American College of Rheumatology (EULAR/ACR) classification criteria for systemic sclerosis (SSc), * Having pulmonary involvement, * Being a woman aged 18-70 years
Exclusion criteria
* Having a severe chronic comorbid condition, * Having any other rheumatologic disease, * Having hearing or visual impairments, * Having a concomitant neurological, cardiovascular, or orthopedic disorder.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Pelvic Floor Dysfunction - Pelvic Floor Distress Inventory-20 Total Score | Baseline | A 20-item questionnaire assessing pelvic floor symptoms across three subscales: POPDI-6, UDI-6, and CRADI-8 will be used. The total score ranges from 0 to 300, with higher scores indicating greater symptom-related distress and worse pelvic floor health. The Turkish validity and reliability were established. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Pulmonary Function Test - Forced Expiratory Volume in One Second | Baseline | Pulmonary function will be assessed using a spirometry system (ZAN 300 MGA) in accordance with American Thoracic Society (ATS) acceptability criteria. Forced expiratory volume in one second (FEV₁) will be recorded and reported as percent predicted (%). |
| Pulmonary Function Test - Forced Vital Capacity | Baseline | Pulmonary function will be assessed using a spirometry system (ZAN 300 MGA) in accordance with American Thoracic Society (ATS) acceptability criteria. Forced vital capacity (FVC) will be recorded as a measure of pulmonary function and reported as percent predicted (%). |
| Pulmonary Function Test - Diffusing Capacity of the Lungs for Carbon Monoxide | Baseline | Diffusing capacity of the lungs for carbon monoxide (DLCO) will be assessed using the ZAN 300 MGA system in accordance with standardized testing procedures and reported as percent predicted (%). |
| Maximum Inspriratory Pressure | Baseline | Maximum Inspiratory Pressure (MIP): Inspiratory muscle strength will be assessed using a portable electronic respiratory pressure meter (MicroRPM; Micro Medical, UK) in accordance with ATS/ERS guidelines. MIP will be measured during a maximal inspiratory effort (Müller maneuver) with the nose occluded. At least three measurements will be performed with a minimum 1-minute rest between trials. MIP will be reported in cmH₂O, with higher absolute values indicating greater inspiratory muscle strength. |
| Maximum Expiratory Pressure | Baseline | Maximum Expiratory Pressure (MEP): Expiratory muscle strength will be assessed using a portable electronic respiratory pressure meter (MicroRPM; Micro Medical, UK) in accordance with ATS/ERS guidelines. Participants will perform a maximal expiratory effort with the nose occluded. At least three measurements will be performed, with a minimum 1-minute rest between trials, and the highest valid value will be recorded. MEP will be reported in cmH₂O, with higher values indicating greater expiratory muscle strength. |
| Functional Exercise Capacity - 6-Minute Walk Test | Baseline | Functional exercise capacity will be assessed using the Six-Minute Walk Test (6MWT) in accordance with ATS guidelines. Participants will be instructed to walk as far as possible at their own pace along a 30-meter flat corridor for six minutes. A 10-minute seated rest period will be provided before the test. Resting during the test will be permitted without stopping the timer. The total distance walked in six minutes will be recorded in meters (m), with a greater distance indicating better functional exercise capacity. |
| Trunk Flexor Endurance - McGill Core Endurance Test | Baseline | Trunk flexor endurance will be assessed using the McGill Core Endurance Test. Participants will maintain a standardized position with the knees flexed to 90° and the trunk at 60°, with the arms crossed over the chest. The duration for which the position can be maintained will be recorded in seconds (s), with a longer duration indicating greater trunk flexor endurance. |
| Trunk Extensor Endurance - McGill Core Endurance Test | Baseline | Trunk extensor endurance will be assessed using the McGill Core Endurance Test. Participants will maintain the trunk horizontally unsupported in the prone position while the lower body is stabilized. The test will end when the participant is unable to maintain the required position. The duration will be recorded in seconds (s), with a longer duration indicating greater trunk extensor endurance. |
| Lateral Trunk Endurance - McGill Core Endurance Test | Baseline | Lateral trunk endurance will be assessed using a modified side-bridge test with the knees flexed. Participants will support the body on the lower elbow and knees and maintain the required position for as long as possible. The test will end when the participant is unable to maintain the position. The duration will be recorded in seconds (s), with a longer duration indicating greater lateral trunk endurance. |
| Cough-Related Quality of Life - Leicester Cough Questionnaire | Baseline | Leicester Cough Questionnaire (LCQ): The impact of cough on health-related quality of life will be assessed using the Leicester Cough Questionnaire (LCQ), a 19-item questionnaire comprising physical, psychological, and social domains. The total score ranges from 3 to 21, with higher scores indicating better cough-related quality of life and a better outcome. The Turkish version of the LCQ has been demonstrated to be valid and reliable. |
| Modified Medical Research Council Dyspnea Scale | Baseline | Modified Medical Research Council (mMRC) Dyspnea Scale: Dyspnea during activities of daily living will be assessed using the modified Medical Research Council (mMRC) Dyspnea Scale. The scale ranges from 0 to 4, with higher scores indicating greater dyspnea severity and a worse outcome. |
| Sexual Function - Arizona Sexual Experiences Scale | Baseline | Sexual function will be assessed using the Arizona Sexual Experiences Scale (ASEX), a 5-item scale evaluating sexual drive, arousal, physiological arousal, ability to reach orgasm, and satisfaction from orgasm. The total score ranges from 5 to 30, with higher scores indicating greater sexual dysfunction and a worse outcome. A total score of ≥19, a score of 5 or 6 on any single item, or a score of ≥4 on three or more items indicates sexual dysfunction. The Turkish version of the ASEX has been demonstrated to be valid and reliable. |
| Disease-Related Functional Status - Scleroderma Health Assessment Questionnaire | Baseline | Functional status and limitations in activities of daily living will be assessed using the Scleroderma Health Assessment Questionnaire (SHAQ). The questionnaire evaluates disability and disease-related limitations associated with systemic sclerosis, including Raynaud's phenomenon, digital ulcers, pulmonary symptoms, gastrointestinal symptoms, and overall disease severity. Higher scores indicate greater disability and a worse functional outcome. |
| Pelvic Floor Health Knowledge - Pelvic Floor Health Knowledge Test | Baseline | Pelvic floor health knowledge will be assessed using the Pelvic Floor Health Knowledge Quiz (PFHKQ), a 29-item questionnaire evaluating knowledge of pelvic floor function and dysfunction, risk and etiology, and diagnosis and treatment. Each correct response is scored as 1 and incorrect or "I don't know" responses as 0, resulting in a total score ranging from 0 to 29. Higher scores indicate greater pelvic floor health knowledge and a better outcome. |
Countries
Turkey (Türkiye)
Contacts
CONTACTB.Beril Kılıç
Outcome results
None listed