Estimates of epidemiology, mortality and disease burden associated with progressive fibrosing interstitial lung disease in France (the PROGRESS study).

BACKGROUND: There is a paucity of data on the epidemiology, survival estimates and healthcare resource utilisation and associated costs of patients with progressive fibrosing interstitial lung disease (PF-ILD) in France. An algorithm for extracting claims data was developed to indirectly identify and describe patients with PF-ILD in the French national administrative healthcare database. METHODS: The French healthcare database, the Système National des Données de Santé (SNDS), includes data related to ambulatory care, hospitalisations and death for 98.8% of the population. In this study, algorithms based on age, diagnosis and healthcare consumption were created to identify adult patients with PF-ILD other than idiopathic pulmonary fibrosis between 2010 and 2017. Incidence, prevalence, survival estimates, clinical features and healthcare resource usage and costs were described among patients with PF-ILD. RESULTS: We identified a total of 14,413 patients with PF-ILD. Almost half of them (48.1%) were female and the mean (± standard deviation) age was 68.4 (± 15.0) years. Between 2010 and 2017, the estimated incidence of PF-ILD ranged from 4.0 to 4.7/100,000 person-years and the estimated prevalence from 6.6 to 19.4/100,000 persons. The main diagnostic categories represented were exposure-related ILD other than hypersensitivity pneumonitis (n = 3486; 24.2%), idiopathic interstitial pneumonia (n = 3113; 21.6%) and rheumatoid arthritis-associated ILD (n = 2521; 17.5%). Median overall survival using Kaplan-Meier estimation was 3.7 years from the start of progression. During the study, 95.2% of patients had ≥ 1 hospitalisation for respiratory care and 34.3% were hospitalised in an intensive care unit. The median (interquartile range) total specific cost per patient during the follow-up period was €25,613 (10,622-54,287) and the median annual cost per patient was €18,362 (6856-52,026), of which €11,784 (3003-42,097) was related to hospitalisations. Limitations included the retrospective design and identification of cases through an algorithm in the absence of chest high-resolution computed tomography scans and pulmonary function tests. CONCLUSIONS: This large, real-world, longitudinal study provides important insights into the characteristics, epidemiology and healthcare resource utilisation and costs associated with PF-ILD in France using a comprehensive and exhaustive database, and provides vital evidence that PF-ILD represents a high burden on both patients and healthcare services. Trial registration ClinicalTrials.gov, NCT03858842. ISRCTN, ISRCTN12345678. Registered 3 January 2019-Retrospectively registered, https://clinicaltrials.gov/ct2/show/NCT03858842.

Dynamic assessment of lung injury by ultrasound in patients with acute paraquat poisoning.

OBJECTIVE: To investigate the value of ultrasound in the dynamic assessment of lung injury after acute paraquat poisoning. METHODS: A prospective observational study was performed on patients with paraquat poisoning from admission to day 28 or discharge. Ultrasound assessment of the lungs was performtyed every 48 hours. The correlation of the lung ultrasound score (LUS) with other indicators was analyzed. RESULTS: Twenty-six patients were enrolled, with an average age of 46 ± 16 years. The average toxic dose was 95 ± 51 mL. The intensive care unit (ICU) stay averaged 9 ± 8 days, and the 28-day mortality was 88.5%. There was a significant negative correlation between LUS and oxygenation index (rho = -0.896) and a significant positive correlation between LUS and carbon dioxide concentration (rho = 0.567). Lung ultrasound and computed tomography imaging correlated closely. CONCLUSION: Lung ultrasound can reflect changes in lung status in patients with paraquat poisoning and can be used to evaluate lung injury in these patients. Trial registration: ChiCTR, ChiCTR-DDD-16010211. Registered 21 December 2016, http://www.chictr.org.cn/listbycreater.aspx .

The supportive care needs of people living with pulmonary fibrosis and their caregivers: a systematic review.

BACKGROUND: People with pulmonary fibrosis often experience a protracted time to diagnosis, high symptom burden and limited disease information. This review aimed to identify the supportive care needs reported by people with pulmonary fibrosis and their caregivers. METHODS: A systematic review was conducted according to PRISMA guidelines. Studies that investigated the supportive care needs of people with pulmonary fibrosis or their caregivers were included. Supportive care needs were extracted and mapped to eight pre-specified domains using a framework synthesis method. RESULTS: A total of 35 studies were included. The most frequently reported needs were in the domain of information/education, including information on supplemental oxygen, disease progression and prognosis, pharmacological treatments and end-of-life planning. Psychosocial/emotional needs were also frequently reported, including management of anxiety, anger, sadness and fear. An additional domain of "access to care" was identified that had not been specified a priori; this included access to peer support, psychological support, specialist centres and support for families of people with pulmonary fibrosis. CONCLUSION: People with pulmonary fibrosis report many unmet needs for supportive care, particularly related to insufficient information and lack of psychosocial support. These data can inform the development of comprehensive care models for people with pulmonary fibrosis and their loved ones.

Opportunities to diagnose fibrotic lung diseases in routine care: A primary care cohort study.

BACKGROUND AND OBJECTIVE: Temporal trends of healthcare use in the period before a diagnosis of pulmonary fibrosis are poorly understood. We investigated trends in respiratory symptoms and LR HRU in the 10 years prior to diagnosis. METHODS: We analysed a primary care clinical cohort database (UK OPCRD) and assessed patients aged ≥40 years who had an electronically coded diagnosis of pulmonary fibrosis between 2005 and 2015 and a minimum 2 years of continuous medical records prior to diagnosis. Exclusion criteria consisted of electronic codes for recognized causes of pulmonary fibrosis such as CTD, sarcoidosis or EAA. RESULTS: Data for 2223 patients were assessed. Over the 10 years prior to diagnosis of pulmonary fibrosis, there was a progressive increase in HRU across multiple LR-related domains. Five years before diagnosis, 18% of patients had multiple healthcare contacts for LR complaints; this increased to 79% in the year before diagnosis, with 38% of patients having five or more healthcare contacts. CONCLUSION: There are opportunities to diagnose pulmonary fibrosis at an earlier stage; research into case-finding algorithms and strategies to educate primary care physicians is required.

21st Century Tools for Nanotoxicology: Transcriptomic Biomarker Panel and Precision-Cut Lung Slice Organ Mimic System for the Assessment of Nanomaterial-Induced Lung Fibrosis.

There is an urgent need for reliable toxicity assays to support the human health risk assessment of an ever increasing number of engineered nanomaterials (ENMs). Animal testing is not a suitable option for ENMs. Sensitive in vitro models and mechanism-based targeted in vitro assays that enable accurate prediction of in vivo responses are not yet available. In this proof-of-principle study, publicly available mouse lung transcriptomics data from studies investigating xenobiotic-induced lung diseases are used and a 17-gene biomarker panel (PFS17) applicable to the assessment of lung fibrosis is developed. The PFS17 is validated using a limited number of in vivo mouse lung transcriptomics datasets from studies investigating ENM-induced responses. In addition, an ex vivo precision-cut lung slice (PCLS) model is optimized for screening of potentially inflammogenic and pro-fibrotic ENMs. Using bleomycin and a multiwalled carbon nanotube, the practical application of the PCLS method as a sensitive alternative to whole animal tests to screen ENMs that may potentially induce inhalation toxicity is shown. Conditional to further optimization and validation, it is established that a combination of PFS17 and the ex vivo PCLS method will serve as a robust and sensitive approach to assess lung inflammation and fibrosis induced by ENMs.

Computed Tomography Honeycombing Identifies a Progressive Fibrotic Phenotype with Increased Mortality across Diverse Interstitial Lung Diseases.

Rationale: Honeycombing on chest computed tomography (CT) has been described in diverse forms of interstitial lung disease (ILD); however, its prevalence and association with mortality across the spectrum of ILD remains unclear. Objective: To determine the prevalence and prognostic value of CT honeycombing and characterize associated mortality patterns across diverse ILD subtypes in a multicenter cohort. Methods: This was an observational cohort study of adult participants with multidisciplinary or adjudicated ILD diagnosis and documentation of chest CT imaging at index diagnosis across five U.S. hospitals (one tertiary and four nontertiary medical centers). Participants were stratified based on presence or absence of CT honeycombing. Vital status was determined from review of medical records and social security death index. Transplant-free survival was analyzed using univariate and multivariable Cox regression. Results: The sample comprised 1,330 participants (mean age, 66.8 yr; 50% men) with 4,831 person-years of follow-up. The prevalences of CT honeycombing were 42.0%, 41.9%, 37.6%, and 28.6% in chronic hypersensitivity pneumonitis, connective tissue disease-related ILD (CTD-ILD), idiopathic pulmonary fibrosis (IPF), and unclassifiable/other ILDs, respectively. Among those with CT honeycombing, cumulative mortality hazards were similar across ILD subtypes, except for CTD-ILD, which had a lower mortality hazard. Overall, the mean survival time was shorter among those with CT honeycombing (107 mo; 95% confidence interval [CI], 92-122 mo) than those without CT honeycombing (161 mo; 95% CI, 147-174 mo). CT honeycombing was associated with an increased mortality rate (hazard ratio, 1.72; 95% CI, 1.38-2.14) even after adjustment for center, sex, age, forced vital capacity, diffusing capacity, ILD subtype, and use of immunosuppressive therapy (hazard ratio, 1.62; 95% CI, 1.29-2.02). CT honeycombing was associated with an increased mortality rate within non-IPF ILD subgroups (chronic hypersensitivity pneumonitis, CTD-ILD, and unclassifiable/other ILD). In IPF, however, mortality rates were similar between those with and without CT honeycombing. Conclusions: CT honeycombing is prevalent in diverse forms of ILD and uniquely identifies a progressive fibrotic ILD phenotype with a high mortality rate similar to IPF. CT honeycombing did not confer additional risk in IPF, which is already known to be a progressive fibrotic ILD phenotype regardless of the presence of CT honeycombing.

Patients' perceptions and patient-reported outcomes in progressive-fibrosing interstitial lung diseases.

The effects of interstitial lung disease (ILD) create a significant burden on patients, unsettling almost every domain of their lives, disrupting their physical and emotional well-being and impairing their quality of life (QoL). Because many ILDs are incurable, and there are limited reliably-effective, life-prolonging treatment options available, the focus of many therapeutic interventions has been on improving or maintaining how patients with ILD feel and function, and by extension, their QoL. Such patient-centred outcomes are best assessed by patients themselves through tools that capture their perceptions, which inherently incorporate their values and judgements. These patient-reported outcome measures (PROs) can be used to assess an array of constructs affected by a disease or the interventions implemented to treat it. Here, we review the impact of ILD that may present with a progressive-fibrosing phenotype on patients' lives and examine how PROs have been used to measure that impact and the effectiveness of therapeutic interventions.

Healthcare utilisation and costs in the diagnosis and treatment of progressive-fibrosing interstitial lung diseases.

There are over 200 interstitial lung diseases (ILDs). In addition to patients with idiopathic pulmonary fibrosis (IPF), a percentage of patients with other ILDs also develop progressive fibrosis of the lung during their disease course. Patients with progressive-fibrosing ILDs may show limited response to immunomodulatory therapy, worsening symptoms and lung function and, ultimately, early mortality. There are few data for ILDs that may present a progressive fibrosing phenotype specifically, but we believe the burden and healthcare costs associated with these conditions may be comparable to those reported in IPF. This review discusses the burden of ILDs that may present a progressive fibrosing phenotype and the factors impacting healthcare utilisation.

Assessment of quantitative polymerase chain reaction for equine herpesvirus-5 in blood, nasal secretions and bronchoalveolar lavage fluid for the laboratory diagnosis of equine multinodular pulmonary fibrosis.

REASONS FOR PERFORMING STUDY: The ante mortem diagnosis of equine multinodular pulmonary fibrosis (EMPF) relies on histopathological results and polymerase chain reaction (PCR)-positive equine herpesvirus (EHV)-5 testing of lung tissue. Polymerase chain reaction detection of EHV-5 in bronchoalveolar lavage fluid (BALF) is commonly used to support a diagnosis of EMPF. However, the diagnostic power of EHV-5 testing on BALF and other biological samples such as blood and nasal secretions has yet to be shown to support a diagnosis of EMPF. OBJECTIVES: To determine the frequency of detection and the viral loads of EHV-5 by quantitative PCR (qPCR) in blood, nasal secretions and BALF from horses confirmed with EMPF, healthy horses and horses with non-EMPF pulmonary diseases. STUDY DESIGN: Prospective study. METHODS: The study population consisted of 70 adult horses divided into 4 groups based on a combination of clinical findings, cytology of BALF, imaging studies of the thoracic cavity and histopathology of pulmonary tissue: control group (n = 14), EMPF group (n = 11); inflammatory airway disease group (n = 32); and non-EMPF interstitial lung disease group (n = 13). For each horse, whole blood, nasal secretions and BALF were available for EHV-5 qPCR testing. Sensitivities, specificities and their respective 95% confidence intervals were calculated for viral loads from blood, nasal secretions and BALF. In addition, these measures were calculated for combined use of blood and nasal secretions. RESULTS: The detection of EHV-5 in BALF was strongly associated with EMPF (sensitivity 91%, specificity 98.3%). Detection of EHV-5 in blood was, independent of the viral loads, strongly associated with EMPF with a sensitivity of 91% and specificity of 83.1%. The detection of EHV-5 in nasal secretions displayed the highest sensitivity (72.7%) and specificity (83.1%) at a level of >245,890 glycoprotein B target genes/million cells to support a diagnosis of EMPF. Dually positive blood and nasal secretions at any viral loads in support of EMPF yielded a sensitivity and specificity of 90% and 89.8%, respectively. CONCLUSIONS: Although histopathological confirmation (lung biopsy) is considered the gold standard for EMPF diagnosis, results of qPCR testing of BALF or a combination of whole blood and nasal secretions should be regarded as clinically useful in support of this diagnosis. The latter testing may be relevant when dealing with horses in respiratory distress, for which invasive procedures such as BALF collection or lung biopsies may be detrimental to their health.

Patient factors associated with lung transplant referral and waitlist for patients with cystic fibrosis and pulmonary fibrosis.

BACKGROUND: Since 2005, the Lung Allocation Score (LAS) has prioritized patient benefit and post-transplant survival, reducing waitlist to transplant time to <200 days and decreasing mortality on the waitlist. A current challenge is the wait for the waitlist-the time between the patient's transplant-eligible diagnosis and waitlist registration. METHODS: We investigated whether sociodemographic (age, sex, race, insurance, marital status, median household income) and clinical (forced expiratory volume in 1 second [FEV1] percent of predicted, body mass index, depression/anxiety, alcohol/substance misuse, absolute/relative contraindications) factors influenced referral and waitlist registration. We conducted a retrospective cohort study through chart review of hospitalized patients on the University of Chicago general medicine service from 2006 to 2014 who met transplant-eligible criteria and ICD-9 billing codes for cystic fibrosis (CF) and pulmonary fibrosis (PF). We analyzed the times from transplant eligibility to referral, work-up and waitlisting using Kaplan-Meier curves and log-rank tests. RESULTS: Overall, the referral rate for transplant-eligible patients was 64%. Of those referred, approximately 36% reach the lung transplant waitlist. Referred CF patients were significantly more likely to reach the transplant waitlist than PF patients (CF 60% vs PF 22%, p < 0.05). In addition, CF patients had a shorter wait from transplant eligibility to waitlist than PF patients (329 vs 2,369 days, respectively [25th percentile], p < 0.05). Patients with PF and CF both faced delays from eligibility to referral and waitlist. CONCLUSIONS: Quality improvement efforts are needed to better identify and refer appropriate patients for lung transplant evaluation. Targeted interventions may facilitate more efficient evaluation completion and waitlist appearance.

Validity and Reliability of the Chronic Respiratory Disease Questionnaire in Elderly Individuals with Mild to Moderate Non-Cystic Fibrosis Bronchiectasis.

BACKGROUND: The chronic respiratory disease questionnaire (CRDQ) is designed to assess health-related quality of life (HRQOL) in chronic respiratory conditions, but its reliability, validity and responsiveness in individuals with mild to moderate non-cystic fibrosis (CF) bronchiectasis are unclear. OBJECTIVES: This study aimed to determine measurement properties of the CRDQ in non-CF bronchiectasis. METHODS: Participants with non-CF bronchiectasis involved in a randomised controlled trial of exercise training were recruited. Internal consistency was assessed using Cronbach's α. Over 8 weeks, reliability was evaluated using intra-class correlation coefficients and Bland-Altman analysis for measures of agreement. Convergent and divergent validity was assessed by correlations with the other HRQOL questionnaires and the Hospital Anxiety and Depression Scale (HADS). The responsiveness to exercise training was assessed using effect sizes and standardised response means. RESULTS: Eighty-five participants were included (mean age ± SD, 64 ± 13 years). Internal consistency was adequate (>0.7) for all CRDQ domains and the total score. Test-retest reliability ranged from 0.69 to 0.85 for each CRDQ domain and was 0.82 for the total score. Dyspnoea (CRDQ) was related to St George's respiratory questionnaire (SGRQ) symptoms only (r = 0.38), with no relationship to the Leicester cough questionnaire (LCQ) or HADS. Moderate correlations were found between the total score of the CRDQ, the SGRQ (rs = -0.49) and the LCQ score (rs = 0.51). Lower CRDQ scores were associated with higher anxiety and depression (rs = -0.46 to -0.56). The responsiveness of the CRDQ was small (effect size 0.1-0.24). CONCLUSIONS: The CRDQ is a valid and reliable measure of HRQOL in mild to moderate non-CF bronchiectasis, but responsiveness was limited.

Magnetic resonance imaging for noninvasive assessment of lung fibrosis onset and progression: cross-validation and comparison of different magnetic resonance imaging protocols with micro-computed tomography and histology in the bleomycin-induced mouse model.

OBJECTIVES: Bleomycin instillation is frequently used to model lung fibrosis, although the onset and severity of pathology varies highly between mice. This makes non-invasive fibrosis detection and quantification essential to obtain a comprehensive analysis of the disease course and to validate novel therapies. Magnetic resonance imaging (MRI) of lung disease progression and therapy may provide such a sensitive in vivo readout of lung fibrosis, bypassing radiotoxicity concerns (when using micro-CT [µCT]) and elaborate invasive end point measurements (histology). We aimed to optimize and evaluate 3 different lung MRI contrast and acquisition methods to visualize disease onset and progression in the bleomycin-induced mouse model of lung fibrosis using a small-animal MRI scanner. For validation, we compared the MRI results with established µCT and histological measures of lung fibrosis. MATERIALS AND METHODS: Free-breathing bleomycin-instilled and control mice were scanned in vivo with respiration-triggered conventional, ultrashort echo time and self-gated MRI pulse sequences (9.4 T) and µCT at baseline and weekly at days 7, 14, 21, and 28 after bleomycin instillation. After the last imaging time point, the mice were killed and the lungs were isolated for criterion standard histological analysis of lung fibrosis and quantification of lung collagen content for validation of the imaging results. The agreement between quantitative MRI and µCT data and standard measurements was analyzed by linear regression. RESULTS: All 3 MRI protocols were able to visualize and quantify lung pathology onset and progression in individual bleomycin-instilled mice. In vivo MRI results were in excellent agreement with in vivo µCT and criterion standard histological measures of lung fibrosis. Ultrashort echo time MRI appeared particularly useful for detecting early disease; self-gated MRI, for improved breathing motion handling. DISCUSSION: Magnetic resonance imaging sensitively visualizes and quantifies lung fibrosis in vivo, which makes it a noninvasive, translatable, safe, and potentially more versatile alternative to invasive methods or µCT, thereby stimulating pathogenesis and preclinical research.

Ultrasound in the assessment of pulmonary fibrosis in connective tissue disorders: correlation with high-resolution computed tomography.

OBJECTIVE: To investigate the correlation between ultrasound (US) B-lines and high-resolution computed tomography (HRCT) findings in the assessment of pulmonary fibrosis (PF) in patients with connective tissue disorders (CTD). METHODS: Thirty-four patients with a diagnosis of CTD were included. Each patient underwent clinical examination, pulmonary function test (PFT), chest HRCT, and lung US by an experienced radiologist or rheumatologist. A second rheumatologist carried out US examinations to assess interobserver agreement. In each patient, US B-line lung assessment including 50 intercostal spaces (IS) was performed. For the anterior and lateral chest, the IS were the second to the fifth along the parasternal, mid-clavicular, anterior axillary, and medial axillary lines (the left fifth IS of the anterior and lateral chest was not performed because of the presence of the heart, which limits lung visualization). For the posterior chest, the IS assessed were the seventh to the eighth along the posterior-axillary and subscapular lines. The second to eighth IS were assessed in the paravertebral line. In each IS, the number of US B-lines under the transducer was recorded, summed, and graded according to the following semiquantitative scoring: grade 0 = normal (< 10 B-lines); grade 1 = mild (11 to 20 B-lines); grade 2 = moderate (21 to 50 B-lines); and grade 3 = marked (> 50 B-lines). RESULTS: A total of 1700 IS in 34 patients were assessed. A significant linear correlation was found between the US score and the HRCT score (p < 0.001; correlation coefficient ρ = 0.875). A positive correlation was found between US B-line assessments and values of DLCO (p = 0.014). Both κ values and overall percentages of interobserver agreement showed excellent agreement. CONCLUSION: Our study demonstrates that US B-line assessment may be a useful and reliable additional imaging method in the evaluation of PF in patients with CTD.

A systematic review of validated methods for identifying pulmonary fibrosis and interstitial lung disease using administrative and claims data.

PURPOSE: The Food and Drug Administration's Mini-Sentinel pilot program initially aimed to conduct active surveillance to refine safety signals that emerge for marketed medical products. A key facet of this surveillance is to develop and understand the validity of algorithms for identifying health outcomes of interest (HOIs) from administrative and claims data. This paper summarizes the process and findings of the algorithm review of pulmonary fibrosis and interstitial lung disease. METHODS: PubMed and Iowa Drug Information Service Web searches were conducted to identify citations applicable to the pulmonary fibrosis/interstitial lung disease HOI. Level 1 abstract reviews and Level 2 full-text reviews were conducted to find articles using administrative and claims data to identify pulmonary fibrosis and interstitial lung disease, including validation estimates of the coding algorithms. RESULTS: Our search revealed a deficiency of literature focusing on pulmonary fibrosis and interstitial lung disease algorithms and validation estimates. Only five studies provided codes; none provided validation estimates. Because interstitial lung disease includes a broad spectrum of diseases, including pulmonary fibrosis, the scope of these studies varied, as did the corresponding diagnostic codes used. CONCLUSIONS: Research needs to be conducted on designing validation studies to test pulmonary fibrosis and interstitial lung disease algorithms and estimating their predictive power, sensitivity, and specificity.

[Diagnosing and expertizing asbestos-induced occupational diseases]. / Diagnostik und Begutachtung asbestbedingter Berufskrankheiten.

Due to latency periods that can last for decades, asbestos-related diseases show 18 years after the enforcement of the prohibition of asbestos application in Germany their highest numbers. In the centre of attention are asbestos-induced pleural fibroses, mesotheliomas, asbestoses, lung and laryngeal cancer. Diagnosing and expertizing these diseases causes difficulties, is hitherto non-uniform and does frequently not correspond to the current medico-scientific expertise. This induced the German Respiratory Society as well as the German Society of Occupational and Environmental Medicine in cooperation with the German Society of Pathology, the German Radiology Society and the German Society of Otorhinolaryngology, Head and Cervical Surgery, to develop the above mentioned guideline during seven meetings moderated by AWMF. The required thorough diagnosis is based on the detailed recording of a qualified occupational history. Since the sole radiological and pathological-anatomical findings cannot sufficiently contribute to the causal relationship the occupational history recorded by a general physician and a specialist is of decisive importance. These physicians have to report suspected occupational diseases and to advise patients on social and medical questions. Frequently, problems occur if the recognition of an occupational disease is neglected due to a supposedly too low exposure or too few ferruginous bodies or low fibre concentrations in lung tissue. The new S2k directive summarizing the current medico-scientific knowledge is for this reason, for diagnoses and expert opinions as well as for the determination of a reduced capacity for work a very important source of information.

Sarcoidosis-associated pulmonary hypertension: assessment and management.

Pulmonary hypertension (PH) is a recognized complication of sarcoidosis, with increased morbidity and poor prognosis. Sarcoidosis-associated pulmonary hypertension (SAPH) is typically seen in advanced cases, with pulmonary fibrosis, destruction and obliteration of the pulmonary vasculature, and chronic hypoxemia. PH can, however, occur in the absence of pulmonary fibrosis, suggesting alternative pathophysiological mechanisms. Diverse processes may coexist in the pathogenesis of SAPH, and there is an overlap with mechanisms of pulmonary arterial hypertension (PAH). This has encouraged the study of PAH-specific therapeutic agents in the treatment of SAPH. In small series, prostacyclin analogues, endothelin receptor antagonists, and phosphodiesterase-5 inhibitors have been shown to improve hemodynamics, functional status, and outcomes. This article reviews the most recent data available in the epidemiology, pathophysiology, diagnosis, and treatment of SAPH.

Assessment of chest high-field magnetic resonance imaging in children and young adults with noncystic fibrosis chronic lung disease: comparison to high-resolution computed tomography and correlation with pulmonary function.

OBJECTIVES: Magnetic resonance imaging (MRI) has been proposed as a radiation-free alternative to high resolution computed tomography (HRCT) for the assessment and follow-up of chest disorders. Thus far, no study has compared the efficacy of high-field MRI and HRCT in children and adults with noncystic fibrosis (CF) chronic lung disease. THE AIMS OF OUR STUDY WERE: (1) to assess whether chest high-field MRI is as effective as chest HRCT in identifying pulmonary abnormalities; and (2) to investigate the relationships between the severity and extent of lung disease, and functional data in patients with non-CF chronic lung disease. MATERIALS AND METHODS: Forty-one subjects (median age, 13.8 years; range, 5.9-29.3 years; 30 children/11 adults) with primary ciliary dyskinesia (n = 14), primary immunodeficiency (n = 14), or recurrent pneumonia (n = 13) underwent pulmonary function tests, chest HRCT (120 kV, dose-modulated mAs) and high-field 3.0-T MRI (HASTE; transversal orientation; repetition time/echo time/flip angle/acquisition time, infinite/92 milliseconds/150 degrees/approximately 90 seconds). HRCT and MRI images were scored in consensus by 2 raters using a modified version of the Helbich scoring system. The maximal score was 25. RESULTS: HRCT and high-field MRI total scores were 11 (range: 1-20) and 11 (range: 1-17), respectively. There was good agreement between the 2 techniques for all scores (r > 0.8). HRCT and MRI total scores, and extent of bronchiectasis scores were significantly related to pulmonary function tests (r = -0.4, P < 0.05). The MRI mucous plugging score was significantly related to pulmonary function tests (r = -0.4, P < 0.05). CONCLUSIONS: Chest high-field 3.0-T MRI appears to be as effective as HRCT in assessing the extent and severity of lung abnormalities in non-CF chronic lung diseases, and might be a reliable radiation-free option to HRCT.

The 6-min walk test: clinical and research role, technique, coding, and reimbursement.

FEV(1) is recommended for rating the severity of obstructive and restrictive pulmonary diseases, but it only moderately correlates with quality of life, mortality, and functional status. The 6-min walk test (6MWT) has been increasingly used in clinical practice and research studies as an objective measurement of functional status in patients with moderate-to-severe impairment. This low complexity test measures the distance a patient can quickly walk back and forth in a 30-m (100-foot) corridor in a period of 6 min, referred to as the 6-min walk distance (6MWD). The 6MWD, and in some circumstances oxygen desaturation during the 6MWT, are useful to assess response to medical interventions, to assess prognosis in various conditions, and as a single measurement of functional status. Strictly scripted test instructions and encouragement at baseline and at each minute of exercise is vital to obtain reproducible results. The 6MWT is reported using Current Procedural Terminology code 94620 (simple pulmonary stress test). This code is also appropriate for other simple exercise tests, including oxygen titration (if additional parameters are measured), exercise-induced bronchospasm evaluation using pre- and postexercise spirometry, and exercise prescription for pulmonary rehabilitation. Use of code 94620 to bill for services must be supported by significant documentation.

The Medical Research Council chronic dyspnea score predicts the survival of patients with idiopathic pulmonary fibrosis.

BACKGROUND: The Medical Research Council (MRC) chronic dyspnea scale (6-point) is used in different clinical conditions to grade breathlessness on daily activities. We have previously shown that in patients with histologically documented usual interstitial pneumonia/idiopathic pulmonary fibrosis (UIP/IPF), the MRC dyspnea scale is useful in estimating disease severity. The aim of this study was to further investigate the usefulness of the MRC scale in IPF as a marker of survival. METHODS: The records of 25 patients with histologically documented UIP/IPF were retrospectively reviewed. Clinical parameters, pulmonary function tests, and arterial blood gases at the time of diagnosis, as well as survival time were retrieved and recorded for each patient. The impact of the different variables determined at diagnosis on survival was examined using the Kaplan-Meier and uni- and multi-variate Cox-regression analyses. RESULTS: Among the baseline clinical and physiologic parameters determined at the time of IPF diagnosis, the MRC score, the Tiffeneau index, and the total lung capacity were the only significant and independent predictors of survival. In specific, a high MRC score, a high Tiffeneau index, and a low total lung capacity at presentation were associated with shorter survival. CONCLUSION: In accordance with the previous work, our results indicate that the Tiffeneau index and total lung capacity (TLC) are the important determinants of survival in patients with IPF. In addition, we show that the simple MRC chronic dyspnea score estimated at the time of diagnosis is equally predictive of survival and may aid clinicians in assessing the prognosis of new cases of IPF.

A critical assessment of treatment options for idiopathic pulmonary fibrosis.

BACKGROUND: To date, no management approach has proven to be efficacious for the treatment of idiopathic pulmonary fibrosis (IPF). Consequently, therapeutic options remain controversial and confusing for many clinicians. We sought to formally review available evidence on treatment options for IPF and to have a diverse panel of physicians rate the "appropriateness," "inappropriateness," or "uncertainty" of some of the available therapeutic options. METHODS: The RAND/UCLA Appropriateness Method was used to review and rate multiple clinical scenarios for the treatment of IPF. The panel was composed of nine physicians from geographically diverse areas who received a systematic review on the risks and benefits of commonly used treatments for IPF as background. RESULTS: A total of 324 clinical scenarios were rated: 25% as appropriate; 39%, uncertain; and 36%, inappropriate. The panel disagreed about 12% of the therapy indications in the final ratings, falling from 26% in the first-round ratings. CONCLUSIONS: Key themes emerged from the consensus process. Lacking evidence for a definitive therapy, it was considered most appropriate to enroll eligible patients in clinical trials and refer eligible patients for transplant evaluation. For patients without access to clinical trials, the committee was not unanimous regarding treatment recommendations. It was considered inappropriate for patients with a confident diagnosis of IPF to be treated with corticosteroids as the sole agent: corticosteroids should be used in conjunction with azathioprine. With progressive disease despite such combination use, there was agreement for the use of interferon gamma-1b in patients unwilling or unable to participate in available clinical trials.