Scleroderma Lung: Role of Gastroesophageal Reflux, Microaspiration and Cough

Fecha de inicio: 1 de agosto de 2012

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