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2.
Semin Respir Crit Care Med ; 40(2): 184-193, 2019 04.
Article in English | MEDLINE | ID: mdl-31137059

ABSTRACT

Multidisciplinary diagnosis is now viewed as the diagnostic reference standard in interstitial lung disease (ILD). This process consists of the integration of the evidence base with clinical reasoning in the formulation of a diagnosis and requires input from clinicians, radiologists, and, in selected cases, histopathologists. In ILD associated with connective tissue disease (CTD-ILD), multidisciplinary evaluation is especially helpful when CTD is suspected but cannot be diagnosed using strict criteria. In this context, the integration of systemic clinical data, serologic information, and computed tomography and biopsy findings may allow CTD-ILD to be diagnosed. However, the value of multidisciplinary evaluation in CTD-ILD is not confined to diagnosis. The frequent coexistence of pulmonary processes other than ILD, including pulmonary vascular disease, extrapulmonic restriction, and airways disease, often has a major impact on symptoms and pulmonary function tests (PFTs). In this review, we highlight the value of multidisciplinary discussion (MDD) in reconciling clinical data, PFT, and imaging data in the accurate staging of disease severity, baseline prognostic evaluation, and the identification of progression of ILD. MDD also provides a means to combine the views of respiratory physicians and rheumatologists in formulating a treatment strategy. It is often possible to reach a robust view as to whether management should be driven by systemic disease, pulmonary disease, or both. When treatment needs to be introduced or modified for both systemic and pulmonary reasons, face-to-face discussion facilities the selection of therapeutic agents that are likely to be efficacious for both systemic and pulmonary diseases.


Subject(s)
Connective Tissue Diseases/complications , Interdisciplinary Communication , Lung Diseases, Interstitial/diagnosis , Disease Progression , Humans , Lung Diseases, Interstitial/etiology , Prognosis
3.
Eur Respir Rev ; 24(136): 327-39, 2015 Jun.
Article in English | MEDLINE | ID: mdl-26028644

ABSTRACT

The prevalence of obstructive sleep apnoea (OSA) is continuously increasing in patients with idiopathic pulmonary fibrosis (IPF) and, for the first time, the recent IPF guidelines recognise OSA as an important associated comorbidity that can affect patient's survival. Thus, it becomes conceivable that clinicians should refer patients with newly diagnosed IPF to sleep centres for the diagnosis and treatment of OSA as well as for addressing issues regarding the reduced compliance of patients with continuous positive airway pressure therapy. The discovery of biomarkers common to both disorders may help early diagnosis, institution of the most appropriate treatment and follow-up of patients. Better understanding of epigenetic changes may provide useful information about pathogenesis and, possibly, development of new drugs for a dismal disease like IPF.


Subject(s)
Idiopathic Pulmonary Fibrosis/epidemiology , Lung/physiopathology , Sleep Apnea, Obstructive/epidemiology , Sleep , Comorbidity , Genetic Markers , Genetic Predisposition to Disease , Humans , Hypertension, Pulmonary/epidemiology , Hypertension, Pulmonary/physiopathology , Hypoxia/epidemiology , Hypoxia/physiopathology , Hypoxia/therapy , Idiopathic Pulmonary Fibrosis/diagnosis , Idiopathic Pulmonary Fibrosis/genetics , Idiopathic Pulmonary Fibrosis/physiopathology , Idiopathic Pulmonary Fibrosis/therapy , Lung/pathology , Oxygen Inhalation Therapy , Phenotype , Predictive Value of Tests , Prevalence , Prognosis , Risk Factors , Sleep Apnea, Obstructive/diagnosis , Sleep Apnea, Obstructive/genetics , Sleep Apnea, Obstructive/physiopathology , Sleep Apnea, Obstructive/therapy
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