Esophageal involvement and interstitial lung disease in mixed connective tissue disease

• Published in: Respiratory medicine 2009-06, Vol.103 (6), p.854-860
• Main Authors: Fagundes, M.N, Caleiro, M.T.C, Navarro-Rodriguez, T, Baldi, B.G, Kavakama, J, Salge, J.M, Kairalla, R, Carvalho, C.R.R
• Format: Article
• Language: English
• Subjects: Adult; Aged; Biological and medical sciences; Computed tomography scan; Dilatation, Pathologic - diagnosis; Esophageal pH Monitoring; Esophagus - pathology; Esophagus - physiopathology; Female; Gastroesophageal Reflux - complications; Gastroesophageal Reflux - physiopathology; Humans; Interstitial; Lung diseases; Lung Diseases, Interstitial - complications; Lung Diseases, Interstitial - diagnostic imaging; Lung Diseases, Interstitial - physiopathology; Male; Manometry; Medical sciences; Middle Aged; Mixed connective tissue disease; Mixed Connective Tissue Disease - complications; Mixed Connective Tissue Disease - diagnostic imaging; Mixed Connective Tissue Disease - physiopathology; Pneumology; Prospective Studies; Pulmonary/Respiratory; Respiratory Function Tests; Respiratory system : syndromes and miscellaneous diseases; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Smoking - epidemiology; Tomography, X-Ray Computed - methods; Young Adult
• ISSN: 0954-6111; 1532-3064
• DOI: 10.1016/j.rmed.2008.12.018

Summary Rationale Mixed connective tissue disease is a systemic inflammatory disorder that results in both pulmonary and esophageal manifestations. Objectives We sought to evaluate the relationship between esophageal dysfunction and interstitial lung disease in patients with mixed connective tissue disease. Methods We correlated the pulmonary function data and the high-resolution computed tomography findings of interstitial lung disease with the results of esophageal evaluation in manometry, 24-hour intraesophageal pH measurements, and the presence of esophageal dilatation on computed tomography scan. Measurements and main results Fifty consecutive patients with mixed connective tissue disease, according to Kasukawa's classification criteria, were included in this prospective study. High-resolution computed tomography parenchymal abnormalities were present in 39 of 50 patients. Esophageal dilatation, gastroesophageal reflux, and esophageal motor impairment were also very prevalent (28 of 50, 18 of 36, and 30 of 36, respectively). The presence of interstitial lung disease on computed tomography was significantly higher among patients with esophageal dilatation (92% vs. 45%; p < 0.01) and among patients with severe motor dysfunction (90% vs. 35%; p < 0.001). Conclusions Although we were not able to prove a causal relationship between esophageal and pulmonary involvement, our series revealed a strong association between esophageal motor dysfunction and interstitial lung disease in patients with mixed connective tissue disease.