Scleroderma Lung
Conditions
Keywords
Scleroderma Lung, Gastroesophageal Reflux, Microaspiration, Cough
Brief summary
This is a mechanistic research study to evaluate the relationship between cough, reflux, and aspiration in patients with systemic sclerosis (scleroderma).
Interventions
None listed
Sponsors
Mayo Clinic
Study design
Observational model
COHORT
Time perspective
CROSS_SECTIONAL
Eligibility
Sex/Gender
ALL
Age
18 Years to No maximum
Healthy volunteers
No
Inclusion criteria
* Adult patients ≥ 18 years old. * Fulfilling the American College of Rheumatology criteria for SSc. * For ILD subgroup (n=6): * An upright chest-roentgenogram (chest x-ray) is abnormal and compatible with an interstitial lung disease. * HRCT of the chest confirms the presence of bilateral reticular, ground glass, and/or honeycomb abnormalities that is otherwise unexplained by an alternative process * The HRCT fibrosis score must be ≥ 7. * The total lung capacity (TLC, measured by body plethysmography) and/or the diffusing capacity of the lung for carbon monoxide (DLCO) is below the predicted normal. * The forced vital capacity (FVC) is reduced below the predicted normal. * The patient is symptomatic (i.e. dyspnea and/or a chronic cough) for more than 8 weeks that is otherwise unexplained by an alternative mechanism. * For No ILD subgroup (n=6): * No radiographic evidence of ILD on plain chest x-ray. * The HRCT fibrosis score, when performed) must be \< 7 * The TLC and FVC are within their predicted normal. * The DLCO is within the predicted normal, except when reduced in isolation by pulmonary hypertension.
Exclusion criteria
* Active smoking within the 6 months. * Pneumonia or bronchitis in past 4 weeks. * Active acute illness such as uncontrolled heart failure, infection, or asthma. * Use of more than 3 liters per minute (LPM) of oxygen by nasal cannula at rest. * Coordinator, investigator, or clinician concerns on the patient's compliance, safety, or in ability to complete study requirements. * Morbid condition not expected to live more than 1 year. * Alternative cause or diagnosis for the patient's ILD besides SSc. * Pregnancy. * Obstructive lung disease with an FEV1 (forced expiratory volume in 1 second) to FVC ratio less than 0.7, nor radiographic evidence of emphysema on CT. * Planned therapeutic procedures involving the esophagus (e.g. dilation, surgery) prior to completion of study tests
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Airway pepsin concentration | cross-sectional | Cross-sectional comparison between airway pepsin level, cough frequency, FVC, DLCO, and CT fibrosis score |
Countries
United States
Outcome results
None listed