Clinical review: Idiopathic pulmonary fibrosis acute exacerbations - unravelling Ariadne's thread

Idiopathic pulmonary fibrosis (IPF) is a dreadful, chronic, and irreversibly progressive fibrosing disease leading to death in all patients affected, and IPF acute exacerbations constitute the most devastating complication during its clinical course. IPF exacerbations are subacute/acute, clinically significant deteriorations of unidentifiable cause that usually transform the slow and more or less steady disease decline to the unexpected appearance of acute lung injury/acute respiratory distress syndrome (ALI/ARDS) ending in death. The histological picture is that of diffuse alveolar damage (DAD), which is the tissue counterpart of ARDS, upon usual interstitial pneumonia, which is the tissue equivalent of IPF. ALI/ARDS and acute interstitial pneumonia share with IPF exacerbations the tissue damage pattern of DAD. 'Treatment' with high-dose corticosteroids with or without an immunosuppressant proved ineffective and represents the coup de grace for these patients. Provision of excellent supportive care and the search for and treatment of the 'underlying cause' remain the only options. IPF exacerbations require rapid decisions about when and whether to initiate mechanical support. Admission to an intensive care unit (ICU) is a particular clinical and ethical challenge because of the extremely poor outcome. Transplantation in the ICU setting often presents insurmountable difficulties

Management of patients with combined pulmonary fibrosis and emphysema

Combined pulmonary fibrosis and emphysema (CPFE) was first introduced as a distinct clinical entity to describe the coexistence of any type and grade of radiological pulmonary emphysema and the idiopathic usual interstitial pneumonia CT pattern as well as any pathologically confirmed case and has been extended to include patients with emphysema and other interstitial lung diseases (ILDs), such as nonspecific interstitial pneumonia, and connective tissue disease-ILD as well. Since no certain recommendation or guideline regarding treatment strategy for CPFE patients exists, the current therapeutic option is to follow both pulmonary fibrosis and emphysema current management approach. Smoking cessation, influenza and pneumococcal vaccination should be proposed to all patients. Bronchodilator therapy could be considered in symptomatic CPFE patients with a significant obstructive pattern component in their pulmonary function testing. Dyspneic patients with CPFE might benefit from rehabilitation programs while hypoxemic patients from supplemental oxygen. No exact data exist on treatment with specific drugs for pulmonary fibrosis or/and pulmonary hypertension. In case of acute deterioration best supportive care, extensive search for the etiologic factor and extended spectrum antimicrobials should be provided. Referral for lung transplantation should be encouraged in appropriate CPFE patients

Determinants of continuous positive airway pressure compliance in a group of Greek patients with obstructive sleep apnea

Objective: The aim of the study was to investigate the patients’ characteristics that correlate with greater compliance to CPAP use. Methods: Patients diagnosed with OSAHS and treated with CPAR who had at least one follow-up visit in the Sleep Clinic during one year, were included in the study. Demographic data, history of symptoms, comorbidities, Body Mass Index (BMI), Epworth Sleepiness Scale questionnaire (ESS), were obtained from patients before and under CPAP use. All variables were correlated with average daily CPAP use. Objective and subjective compliance were estimated and a cutoff point of 4.5 h/d was used to distinguish ‘more compliant’ from less ‘compliant’ patients. Results: Ninety eight patients, with a mean age (+/- SD) of 55.5 (+/- 11.1) years were examined. Patients’ symptoms improved after CPAP use. The objective compliance was 5.3 +/- 1.6 h/d whereas the subjective compliance was higher. Only 25% of patients were characterized as ‘more compliant’. Compliance was positively correlated in a significant way with age and female gender, and negatively correlated with neck circumference, preexisting nasal problems and minimum saturation during sleep. Patients with arterial hypertension showed a trend to better compliance. Weight gain was more frequently observed in ‘less compliant’ patients. Conclusion: To our knowledge this is the first study examining parameters of CPAP compliance in a Greek population of OSAHS patients. Age, gender and minimum saturation during sleep were related to better compliance whereas higher neck circumference and preexisting nasal problems were the parameters related to a worse adherence to treatment. (C) 2009 European Federation of Internal Medicine. Published by Elsevier B.V. All rights reserved