title: [Use of pulmonary function tests and biomarkers studies to diagnose and follow-up interstitial lung disease in systemic sclerosis].
abstract: Interstitial lung disease (ILD) is becoming one of the main causes of death of patients with systemic sclerosis (SSc). The prevalence of ILD associated with SSc (SSc-ILD) varies from 33% to 100% according to diagnostic methods. Clinical features such as dyspnea on exertion, dry cough, and chest pains are not specific and usually late-appearing, implying more specific tests in the diagnostic, prognosis, and follow-up of ILD in patients with SSc. High resolution thoracic CT scanner (HRCT) is more sensitive than chest X-ray in the detection of SSc-ILD. Pulmonary function tests (PFT) are non-invasive and periodically used to assess the impacts of SSc on respiratory function. Diagnostic values of bronchoalveolar lavage and histological examination on lung biopsy are controversial. However, these techniques are essential for studying cellular and molecular mechanisms underlying the pathophysiology of SSc-ILD. Several biomarkers such as surfactant-A (SP-A), -D (SP-D), mucin-like high molecular weight glycoprotein (KL-6), and chemokine CCL-18 have been implicated in SSc-PID. Serum levels of these proteins are correlated with the severity of SSc-ILD, as assessed by HRCT and/or PFT. Finally, alveolar concentration of exhaled nitric oxide can be used to screen SSc patients with high risk of deterioration of respiratory function, in whom immunosuppressant treatment could be useful in preventing the evolution to irreversible lung fibrosis.
authors.affiliation: UPRES-EA 2511, service de physiologie-explorations fonctionnelles, hôpital Cochin, AP-HP, université Paris Descartes, Sorbonne Paris Cité, 75014 Paris, France.
authors.forename: T
authors.initials: T
authors.lastname: Hua-Huy
authors.affiliation: Service de médecine interne, Hôpital Saint-Antoine, université Pierre-et-Marie-Curie, 75012 Paris, France.
authors.forename: S
authors.initials: S
authors.lastname: Rivière
authors.affiliation: Hôpital privé de Vitry, site Pasteur, 94400 Vitry-sur-Seine, France.
authors.forename: K P
authors.initials: KP
authors.lastname: Tiev
authors.affiliation: UPRES-EA 2511, service de physiologie-explorations fonctionnelles, hôpital Cochin, AP-HP, université Paris Descartes, Sorbonne Paris Cité, 75014 Paris, France. Electronic address: anh-tuan.dinh-xuan@cch.aphp.fr.
authors.forename: A T
authors.initials: AT
authors.lastname: Dinh-Xuan
mesh_terms: D015415:Biomarkers / Q000378:metabolism*; D005500:Follow-Up Studies; D006801:Humans; D017563:Lung Diseases, Interstitial / Q000175:diagnosis* / Q000209:etiology / Q000378:metabolism; D012129:Respiratory Function Tests / Q000379:methods*; D012595:Scleroderma, Systemic / Q000150:complications*; D012680:Sensitivity and Specificity
chemical_list: D015415:Biomarkers
keywords: Biomarkers; Biomarqueurs; Explorations fonctionnelles respiratoires; Interstitial lung disease; Monoxyde d’azote; Nitric oxide; Pneumopathie interstitielle diffuse; Pulmonary function tests; Sclérodermie systémique; Systemic sclerosis