Showing papers on "Interstitial lung disease published in 2010"
TL;DR: Small airways obstruction, mediated by an influx of inflammatory cells, often characterises pneumothorax and may become manifest in the smaller airways at an earlier stage with ‘emphysema-like changes’ (ELCs).
Abstract: The term ‘pneumothorax’ was first coined by Itard and then Laennec in 1803 and 1819 respectively,1 and refers to air in the pleural cavity (ie, interspersed between the lung and the chest wall). At that time, most cases of pneumothorax were secondary to tuberculosis, although some were recognised as occurring in otherwise healthy patients (‘pneumothorax simple’). This classification has endured subsequently, with the first modern description of pneumothorax occurring in healthy people (primary spontaneous pneumothorax, PSP) being that of Kjaergaard2 in 1932. It is a significant global health problem, with a reported incidence of 18–28/100 000 cases per annum for men and 1.2–6/100 000 for women.3
Secondary pneumothorax (SSP) is associated with underlying lung disease, in distinction to PSP, although tuberculosis is no longer the commonest underlying lung disease in the developed world. The consequences of a pneumothorax in patients with pre-existing lung disease are significantly greater, and the management is potentially more difficult. Combined hospital admission rates for PSP and SSP in the UK have been reported as 16.7/100 000 for men and 5.8/100 000 for women, with corresponding mortality rates of 1.26/million and 0.62/million per annum between 1991 and 1995.4
With regard to the aetiology of pneumothorax, anatomical abnormalities have been demonstrated, even in the absence of overt underlying lung disease. Subpleural blebs and bullae are found at the lung apices at thoracoscopy and on CT scanning in up to 90% of cases of PSP,5 6 and are thought to play a role. More recent autofluorescence studies7 have revealed pleural porosities in adjacent areas that were invisible with white light. Small airways obstruction, mediated by an influx of inflammatory cells, often characterises pneumothorax and may become manifest in the smaller airways at an earlier stage with ‘emphysema-like changes’ (ELCs).8
Smoking has been implicated in this …
1,066 citations
TL;DR: A definite usual interstitial pneumonia pattern on high-resolution computed tomography (HRCT) has important prognostic implications in rheumatoid arthritis-associated interstitial lung disease (RA-ILD).
Abstract: Interstitial lung disease is a common manifestation of rheumatoid arthritis; however, little is known about factors that influence its prognosis. The aim of the present study was to determine whether or not the usual interstitial pneumonia pattern found on high-resolution computed tomography (HRCT) is of prognostic significance in rheumatoid arthritis-associated interstitial lung disease (RA-ILD). Patients with RA-ILD were identified retrospectively (n = 82). The relationship of a definite usual interstitial pneumonia pattern on HRCT to survival was determined and compared to that in a cohort of patients with radiologically diagnosed idiopathic pulmonary fibrosis (n = 51). A definite usual interstitial pneumonia pattern was seen in 20 (24%) out of 82 patients with RA-ILD. These patients showed worse survival than those without this pattern (median survival 3.2 versus 6.6 yrs), and a similar survival to those with idiopathic pulmonary fibrosis. On multivariate analysis, a definite usual interstitial pneumonia pattern on HRCT was associated with worse survival (hazard ratio of 2.3). Analysis of specific HRCT features demonstrated that traction bronchiectasis and honeycomb fibrosis were associated with worse survival (hazard ratio of 2.6 and 2.1, respectively). Female sex (hazard ratio of 0.30) and a higher baseline diffusing capacity of the lung for carbon monoxide (hazard ratio of 0.96) were associated with better survival. A definite usual interstitial pneumonia pattern on HRCT has important prognostic implications in RA-ILD.
444 citations
TL;DR: Abnormalities in the surfactant system and disruption of type II cell homeostasis underlie the pathogenesis of pulmonary disorders previously considered idiopathic, providing the basis for improved diagnosis and therapies of these rare lung diseases.
Abstract: The alveolar region of the lung creates an extensive epithelial surface that mediates the transfer of oxygen and carbon dioxide required for respiration after birth. Maintenance of pulmonary function depends on the function of type II epithelial cells that synthesize and secrete pulmonary surfactant lipids and proteins, reducing the collapsing forces created at the air-liquid interface in the alveoli. Genetic and acquired disorders associated with the surfactant system cause both acute and chronic lung disease. Mutations in the ABCA3, SFTPA, SFTPB, SFTPC, SCL34A2, and TERT genes disrupt type II cell function and/or surfactant homeostasis, causing neonatal respiratory failure and chronic interstitial lung disease. Defects in GM-CSF receptor function disrupt surfactant clearance, causing pulmonary alveolar proteinosis. Abnormalities in the surfactant system and disruption of type II cell homeostasis underlie the pathogenesis of pulmonary disorders previously considered idiopathic, providing the basis for improved diagnosis and therapies of these rare lung diseases.
370 citations
TL;DR: To achieve a timely diagnosis and help reduce associated morbidity and mortality, it is essential to recognize both the typical and the atypical radiologic manifestations of the disease, take note of features that may be suggestive of diseases other than sarcoidosis, and correlate imaging features with pathologic findings to help narrow the differential diagnosis.
Abstract: Sarcoidosis is a multisystem disorder that is characterized by noncaseous epithelioid cell granulomas, which may affect almost any organ. Thoracic involvement is common and accounts for most of the morbidity and mortality associated with the disease. Thoracic radiologic abnormalities are seen at some stage in approximately 90% of patients with sarcoidosis, and an estimated 20% develop chronic lung disease leading to pulmonary fibrosis. Although chest radiography is often the first diagnostic imaging study in patients with pulmonary involvement, computed tomography (CT) is more sensitive for the detection of adenopathy and subtle parenchymal disease. Pulmonary sarcoidosis may manifest with various radiologic patterns: Bilateral hilar lymph node enlargement is the most common finding, followed by interstitial lung disease. At high-resolution CT, the most typical findings of pulmonary involvement are micronodules with a perilymphatic distribution, fibrotic changes, and bilateral perihilar opacities. Atypical manifestations, such as masslike or alveolar opacities, honeycomb-like cysts, miliary opacities, mosaic attenuation, tracheobronchial involvement, and pleural disease, and complications such as aspergillomas, also may be seen. To achieve a timely diagnosis and help reduce associated morbidity and mortality, it is essential to recognize both the typical and the atypical radiologic manifestations of the disease, take note of features that may be suggestive of diseases other than sarcoidosis, and correlate imaging features with pathologic findings to help narrow the differential diagnosis.
339 citations
TL;DR: In the 35 years since the association between inflammatory myopathy and interstitial lung disease was initially described, there has been progress in diagnosing and treating this disorder, however, there remains much about pathogenesis and therapeutics to be learned.
323 citations
TL;DR: The induced autoimmune diseases with the poorest outcomes were interstitial lung disease, inflammatory ocular disease and central nervous system demyelinating diseases.
280 citations
TL;DR: This commentary illustrates what it perceive to be the limitations in the present approach to the classification of this group of patients and discusses problems with redefining the diagnosis of undifferentiated connective tissue disease to encompass patients with interstitial pneumonia.
270 citations
255 citations
TL;DR: RA-ILD is an important and early feature of RA, related to disease activity and has a poor prognosis, and further studies are required to determine whether screening for pulmonary disease would identify patients at an earlier stage.
Abstract: Objectives Pulmonary complications of RA are well described. Although some are benign, interstitial lung disease (ILD) has a poor prognosis. Few RA inception cohorts have reported the natural history of ILD related to RA (RA-ILD). We examine its incidence, outcome and prognostic indicators. Methods Extra-articular features and comorbidity have been recorded yearly in a well-established inception cohort of RA with a 20-year follow-up. Standard clinical, laboratory and radiological measures of RA were recorded at baseline and yearly. Details of deaths were provided by a national central register. Results Out of 1460 patients, 52 developed RA-ILD, half either at baseline or within 3 years of onset. The annualized incidence was 4.1/1000 (95% CI 3.0, 5.4) and the 15-year cumulative incidence 62.9/1000 (95% CI 43.0, 91.7). Incidence of RA-ILD was associated with older age, raised baseline ESR and HAQ. Evidence to implicate any drug effect (e.g. MTX) was lacking. Of these patients, 39 died, attributed to RA-ILD in 28. Median survival following diagnosis of RA-ILD was 3 years. Conclusions RA-ILD is an important and early feature of RA. It is related to disease activity and has a poor prognosis. Further studies are required to determine whether screening for pulmonary disease would identify these patients at an earlier stage.
250 citations
01 May 2010
TL;DR: Viral infection was not detected in most cases of acute exacerbation of idiopathic pulmonary fibrosis, but TTV was present in a significant minority of cases, and Cases of acute lung injury; the clinical significance of this finding remains to be determined.
Abstract: Rationale: Idiopathic pulmonary fibrosis is a progressive, uniformly fatal interstitial lung disease. An acute exacerbation of idiopathic pulmonaryfibrosisisanepisodeofacuterespiratoryworseningwithoutanidentifiableetiology.Occultviralinfectionhasbeenproposed as a possible cause of acute exacerbation. Objectives: To use unbiased genomics-based discovery methods to define the role of viruses in acute exacerbation of idiopathic pulmonary fibrosis. Methods: Bronchoalveolar lavage and serum from patients with acute exacerbation of idiopathic pulmonary fibrosis, stable disease, and acute lung injury were tested for viral nucleic acid using multiplex polymerase chain reaction, pan-viral microarray, and highthroughput cDNA sequencing. Measurements and Main Results: Four of forty-three patients with acute exacerbation of idiopathic pulmonary fibrosis had evidence of common respiratory viral infection (parainfluenza [n ¼ 1], rhinovirus [n ¼ 2], coronavirus [n ¼ 1]); no viruses were detected in the bronchoalveolar lavage from stable patients. Pan-viral microarrays revealed additional evidence of viral infection (herpes simplex virus [n ¼ 1], Epstein-Barr virus [n ¼ 2], and torque teno virus [TTV] [n ¼ 12]) in patients with acute exacerbation. TTV infection was significantlymorecommoninpatientswithacuteexacerbationthanstable controls (P ¼ 0.0003), but present in a similar percentage of acute lunginjury controls. Deepsequencingof a subset of acuteexacerbation cases confirmed the presence of TTV but did not identify additional viruses. Conclusions: Viral infection was not detected in most cases of acute exacerbation of idiopathic pulmonary fibrosis. TTV was present in a significant minority of cases, and cases of acute lung injury; the clinical significance of this finding remains to be determined.
236 citations
TL;DR: It is important that smoking-related interstitial fibrosis be distinguished from specific forms of fibrosing lung disease that may be associated with poor prognoses, especially usual interstitial pneumonia.
TL;DR: Utilizing oxidative enzymes, antioxidants, or the matrix as therapeutic targets to control oxidative stress in IPF will continue be an area of active research and innovative discoveries in the coming years.
TL;DR: A sequential CT interpretation scheme is an efficient method for the visual interpretation of CT data and tends to have greater tobacco smoke exposure than subjects without early ILD.
TL;DR: This study clarifies the clinical characteristics of CPFE patients and suggests that these patients have some different clinical characteristics from COPD patients.
Abstract: UNLABELLED This study describes the clinical characteristics of patients with CPFE and compares these with patients with COPD. It should therefore be noteworthy that the prevalence of lung cancer might be high in CPFE patients. BACKGROUND AND OBJECTIVE Patients with combined pulmonary fibrosis and emphysema (CPFE) are sometimes seen, and we speculate that these patients have some different clinical characteristics from COPD patients. This study clarifies the clinical characteristics of CPFE patients. METHODS This was a retrospective study of 47 stable patients with concurrent emphysema and diffuse parenchymal lung disease with fibrosis, based on the findings of chest CT (CPFE patients). The clinical characteristics of CPFE patients were compared with those of emphysema-dominant COPD patients without parenchymal lung disease (COPD patients). RESULTS Forty-six of the 47 CPFE patients were male. Paraseptal emphysema was particularly common in the CPFE group. Honeycombing, ground-glass opacities and reticular opacities were present in 75.6%, 62.2% and 84.4% of CPFE patients, respectively. Twenty-two of the 47 CPFE patients (46.8%) had lung cancer. Pulmonary function tests showed that the CPFE group had milder airflow limitation and lower diffusing capacity than the COPD group. Desaturation during 6-min walking test in CPFE patients tended to be more severe than in COPD patients, if the level of FEV1 or 6MWD was equal. CONCLUSIONS CPFE patients had some different clinical characteristics in comparison with COPD patients and may also have a high prevalence of lung cancer.
TL;DR: Serum biomarkers may provide new insights as risk factors for pulmonary fibrosis and as measures of disease progression, and further studies are needed to better understand these conditions, and to develop effective therapeutic interventions.
Abstract: Interstitial lung disease (ILD) is a challenging clinical entity associated with multiple connective tissue diseases, and is a significant cause of morbidity and mortality. Effective therapies for connective tissue disease-associated interstitial lung disease (CTD-ILD) are still lacking. Multidisciplinary clinics dedicated to the early diagnosis and improved management of patients with CTD-ILD are now being established. There is rapid progress in understanding and identifying the effector cells, the proinflammatory and profibrotic mediators, and the pathways involved in the pathogenesis of CTD-ILD. Serum biomarkers may provide new insights as risk factors for pulmonary fibrosis and as measures of disease progression. Despite these recent advances, the management of patients with CTD-ILD remains suboptimal. Further studies are therefore urgently needed to better understand these conditions, and to develop effective therapeutic interventions.
TL;DR: Serum ferritin can be useful as a predictor of the occurrence of A/SIP and correlates with the prognosis of A-SIP in DM, and the intensive treatment using combination therapy with various immunosuppressant agents should be chosen for patients with ILD with DM showing hyperferritinaemia, especially levels >1500 ng/ml.
Abstract: Objectives. Acute/subacute interstitial pneumonia (A/SIP) is an intractable and fatal complication of DM. Since a useful indicator predicting the complication of A/SIP has not been found, the aim of this study was to determine whether serum ferritin is a potential predictive indicator of the occurrence of A/SIP in 64 patients with DM. Methods. Of the total patients enrolled, 19 had A/SIP, 24 had chronic interstitial pneumonia and 21 were without interstitial lung disease (ILD). Clinical manifestations and laboratory data were obtained from medical records on admission. Results. Serum ferritin levels were extremely high in patients with DM with A/SIP. It was significantly higher in DM with A/SIP than that in DM without A/SIP (median 790 vs 186 ng/ml; P 1500 ng/ml.
TL;DR: In this article, a review discusses in detail pathologic and clinical features of GLILD and its proposed pathogenesis with a particular attention to potential role of human herpesvirus 8 (HHV-8).
TL;DR: The association of GER with SSc-ILD is strongly supported by several studies, and an aggressive treatment for reflux is recommended in all SSc patients with ILD; however, future studies need to be performed to prove a long-term benefit.
TL;DR: The use of mTOR inhibitors such as rapamycin and everolimus is discussed and recent clinical trials of these drugs in TSC are reviewed.
Abstract: Tuberous sclerosis complex (TSC) is an important cause of epilepsy and autism, as well as renal and pulmonary disease in adults and children. Affected individuals are subject to hamartomas in various organ systems which result from constitutive activation of the protein kinase mTOR (mammalian target of rapamycin). The clinical course, prognosis and appropriate therapy for TSC patients are often different from that for individuals with epilepsy, renal tumors, or interstitial lung disease, from other causes. Additionally, TSC serves as a model for other conditions in which the mTOR pathways are also up-regulated. This article reviews the molecular pathophysiology and management of neurological, renal and pulmonary manifestations of the disorder. The use of mTOR inhibitors such as rapamycin and everolimus is discussed and recent clinical trials of these drugs in TSC are reviewed.
TL;DR: Current concepts of pathophysiological mechanisms, etiology and diagnostic approaches, as well as therapeutic strategies are reviewed, to discuss the various causes of pediatric ILD specific to infancy.
Abstract: Interstitial lung disease (ILD) in infants and children comprises a large spectrum of rare respiratory disorders that are mostly chronic and associated with high morbidity and mortality. These disorders are characterized by inflammatory and fibrotic changes that affect alveolar walls. Typical features of ILD include dyspnea, diffuse infiltrates on chest radiographs, and abnormal pulmonary function tests with restrictive ventilatory defect and/or impaired gas exchange. Many pathological situations can impair gas exchange and, therefore, may contribute to progressive lung damage and ILD. Consequently, diagnosis approach needs to be structured with a clinical evaluation requiring a careful history paying attention to exposures and systemic diseases. Several classifications for ILD have been proposed but none is entirely satisfactory especially in children. The present article reviews current concepts of pathophysiological mechanisms, etiology and diagnostic approaches, as well as therapeutic strategies. The following diagnostic grouping is used to discuss the various causes of pediatric ILD: 1) exposure-related ILD; 2) systemic disease-associated ILD; 3) alveolar structure disorder-associated ILD; and 4) ILD specific to infancy. Therapeutic options include mainly anti-inflammatory, immunosuppressive, and/or anti-fibrotic drugs. The outcome is highly variable with a mortality rate around 15%. An overall favorable response to corticosteroid therapy is observed in around 50% of cases, often associated with sequelae such as limited exercise tolerance or the need for long-term oxygen therapy.
TL;DR: To report the experience using mycophenolate mofetil as first‐line treatment for dermatomyositis‐associated interstitial lung disease.
Abstract: Objective
To report our experience using mycophenolate mofetil as first-line treatment for dermatomyositis-associated interstitial lung disease.
Methods
We examined the medical records of all 16 dermatomyositis patients with interstitial lung disease seen in our outpatient university hospital dermatology clinic between May 26, 2006, and May 25, 2009. In this retrospective case series, we describe the clinical course of the 4 patients with definitive evidence of interstitial lung disease on radiologic imaging who were treated with mycophenolate mofetil and had pulmonary data available to document their outcome. All of the patients also received prednisone.
Results
All 3 patients with at least 1 year of followup receiving mycophenolate mofetil experienced complete normalization of pulmonary function tests (including diffusing capacity for carbon monoxide) and resolution of dyspnea. They were also able to reduce their prednisone doses. The only patient with pre- and posttreatment chest computed tomography imaging had total resolution of her interstitial opacities. The patient with only 5 months of posttreatment followup experienced an improvement in diffusing capacity for carbon monoxide from 44% to 77% predicted, but no change in dyspnea.
Conclusion
These promising data indicate that mycophenolate mofetil may be a useful therapy for interstitial lung disease in patients with dermatomyositis, but larger studies are needed to more definitively evaluate the role of this medication in therapy.
TL;DR: It is indicated that ILD patients with anti-ARS antibodies usually have a chronic clinical course, lung-base predominant GGO with volume loss, NSIP and/or OP patterns, and a good response to corticosteroid treatment; however, some have a rapidly worsening course and recurrence, despite therapy.
Abstract: Objective The aim of this study was to investigate the clinicopathological characteristics of interstitial lung disease (ILD) patients with anti-aminoacyl-tRNA synthetase (anti-ARS) autoantibodies. Patients and Methods We examined 14 ILD patients with anti-ARS autoantibodies between 2004 and 2007 and retrospectively investigated their clinical, radiographic, and pathological findings. Results Anti-Jo-1 antibodies were the most common (10 of 14), followed by anti-OJ, anti-KS, and anti-EJ (1 each for 3 patients); 1 patient with polymyositis had both anti-Jo-1 and anti-PL-12 antibodies. Ten patients had a chronic clinical course, whereas 4 presented with subacute deterioration. Of 8 patients with myositis, 1 (12.5%) had myositis-preceding ILD, 3 (37.5%) had ILD-preceding myositis, and 4 (50%) had simultaneous onset. Chest high-resolution computed tomography frequently showed lung-base predominant ground glass opacities (GGO) with volume loss. The results of surgical lung biopsies indicated that 4 patients had nonspecific interstitial pneumonia (NSIP) and/or organizing pneumonia (OP) patterns. All but 1 received corticosteroid therapy, and 6 patients were also given cyclosporin. The mean duration of follow-up was 22 months (range, 5-47 months). ILD improved in 9 patients and stabilized in 3; however, in 1 patient, it initially improved during 6 months, then progressively worsened despite treatment, and finally resulted in death. Conclusion These results indicate that ILD patients with anti-ARS antibodies usually have a chronic clinical course, lung-base predominant GGO with volume loss, NSIP and/or OP patterns, and a good response to corticosteroid treatment; however, some have a rapidly worsening course and recurrence, despite therapy.
TL;DR: The prevalence, lung function and prognosis of IPF combined with emphysema were evaluated and the prognosis was good for both the smoker and the sufferer.
Abstract: Background and objective: In this study the prevalence, lung function and prognosis of IPF combined with emphysema were evaluated.
Methods: Consecutive patients with usual interstitial pneumonia (UIP) on high-resolution computed tomography (HRCT), with or without emphysema, were assessed retrospectively. The area of fibrosis in the base of the lungs was assessed by HRCT as minimal (<2 cm from the subpleura), moderate (≥2 cm from the subpleura, <1/3 of the area of the base of the lungs) or severe (≥1/3 of the area of the base of the lungs).
Results: Among 660 patients with UIP on HRCT, 221 showed upper-lobe emphysema. Pulmonary function results for patients with UIP and UIP/emphysema, respectively, were: FVC, 71.8% and 87.1%; FEV1%, 86.7% and 87.9%; and DLCO, 74.3% and 65.2% of predicted. The relationship between FVC, the extent of fibrosis and survival was investigated in 362 patients with records of pulmonary function tests and no lung cancer at the time of entry into the study. Although the extent of fibrosis was similar between the groups, 71.3% of UIP patients met the lung volume criteria for IPF (FVC <80% of predicted), whereas only 26.5% of UIP/emphysema patients met the lung volume criteria for IPF. Median survival was 7.5 years in the UIP group and 8.5 years in the UIP/emphysema group.
Conclusions: Emphysema was a common finding in patients with UIP. Patients with UIP and emphysema had greater lung volumes and better survival compared with those with UIP alone.
TL;DR: Assessment of exercise capacity may provide useful information for both clinicians and patients when evaluating the risks and benefits of new treatments, and oxygen administration should be standardized on repeat testing.
Abstract: Exercise limitation is a cardinal feature of the interstitial lung diseases and is frequently associated with marked dyspnoea on exertion. People with interstitial lung disease exhibit a rapid, shallow breathing pattern during exercise that worsens as disease progresses. Despite this, ventilatory mechanics are not the major limitation to exercise in most patients, with impaired gas exchange and circulatory limitation playing a more important role. Peripheral and respiratory muscle dysfunction may also contribute to impaired exercise tolerance, either due to systemic manifestations of the underlying disease, treatment side-effects or deconditioning. Measures of exercise capacity or desaturation obtained from maximal and submaximal exercise tests are good predictors of survival in patients with idiopathic pulmonary fibrosis. However, to date few pharmaceutical treatments have affected exercise outcomes despite improvements in other important clinical markers. Supplemental oxygen acutely improves exercise ca...
TL;DR: The direct suppression of PAi-1 in the lung by the intrapulmonary administration of PAI-1-siRNA attenuated the development and progression of pulmonary fibrosis.
Abstract: Background and aim There is a growing body of evidence demonstrating that plasminogen activator inhibitor-1 (PAI-1) is involved in the progression of pulmonary fibrosis. In fact, PAI-1 knockout mice are protected from bleomycin-induced pulmonary fibrosis. This study was conducted to determine whether the intrapulmonary administration of small interfering RNA (siRNA) targeting PAI-1 (PAI-1-siRNA) limits the development of bleomycin-induced pulmonary fibrosis. Methods Lung biopsies from patients with idiopathic pulmonary fibrosis (IPF) were stained for PAI-1. The distribution of siRNA in the lung, the PAI-1 level in bronchoalveolar (BAL) fluid and the extent of fibrotic changes in the lung were evaluated following the intranasal administration of PAI-1-siRNA in a mouse model of bleomycin-induced pulmonary fibrosis. The effect of PAI-1-siRNA on the epithelial to mesenchymal transition (EMT) was also evaluated using a mouse lung epithelial cell line, LA-4. Results PAI-1 was overexpressed in the hyperplastic type 2 pneumocytes lining the honeycomb lesions of patients with IPF. The single intranasal instillation of PAI1-siRNA resulted in the diffuse uptake of siRNA into the epithelial cells lining the dense fibrotic lesions. The repeated administration of PAI-1-siRNA initiated during either the inflammatory or the fibrotic phase into bleomycin-injured mice reduced the PAI-1 level in BAL fluid and limited the accumulation of collagen in the lungs. EMT induced by transforming growth factor b (TGFb) in LA-4 cells was inhibited by transfection with PAI-1-siRNA. Conclusions The direct suppression of PAI-1 in the lung by the intrapulmonary administration of PAI-1-siRNA attenuated the development and progression of pulmonary fibrosis. The inhibition of EMT may be, at least in part, involved in this effect.
Journal Article•
TL;DR: The authors' technique exhibited good accuracy for detecting fibrosis at a threshold of both 1% (i.e. presence or absence of pulmonary fibrosis) and a clinically meaningful threshold of 25% extent of fibrosis in patients with SSc-ILD.
Abstract: Objectives. To evaluate an improved quantitative lung fibrosis score based on a computer-aided diagnosis (CAD) system that classifies CT pixels with the visual semi-quantitative pulmonary fibrosis score in patients with scleroderma-related interstitial lung disease (SSc-ILD). Methods. High-resolution, thin-section CT images were obtained and analysed on 129 subjects with SSc-ILD (36 men, 93 women; mean age 48.8±12.1 years) who underwent baseline CT in the prone position at full inspiration. The CAD system segmented each lung of each patient into 3 zones. A quantitative lung fibrosis (QLF) score was established via 5 steps: 1) images were denoised; 2) images were grid sampled; 3) the characteristics of grid intensities were converted into texture features; 4) texture features classified pixels as fibrotic or non-fibrotic, with fibrosis defined by a reticular pattern with architectural distortion; and 5) fibrotic pixels were reported as percentages. Quantitative scores were obtained from 709 zones with complete data and then compared with ordinal scores from two independent expert radiologists. ROC curve analyses were used to measure performance. Results. When the two radiologists agreed that fibrosis affected more than 1% or 25% of a zone or zones, the areas under the ROC curves for QLF score were 0.86 and 0.96, respectively. Conclusion. Our technique exhibited good accuracy for detecting fibrosis at a threshold of both 1% (i.e. presence or absence of pulmonary fibrosis) and a clinically meaningful threshold of 25% extent of fibrosis in patients with SSc-ILD.
TL;DR: A comprehensive diagnostic evaluation is recommended for all patients with fibrotic lung disease and acute respiratory decompensation and the effect on clinical outcomes of this evaluation remains unknown.
Abstract: Background and objective: A comprehensive diagnostic evaluation is recommended for all patients with fibrotic lung disease and acute respiratory decompensation. However, the effect on clinical outcomes of this evaluation remains unknown.
Methods: We evaluated 27 consecutive patients with fibrotic lung disease who were hospitalized for an acute respiratory decline between June 2006 and April 2009. An interstitial lung disease expert assisted with the acute care of each patient. A retrospective review of the patient charts was performed to obtain demographic and clinical data, and to assess outcomes.
Results: Using a strict definition of acute exacerbation (AE) of fibrotic lung disease derived from the IPF Network Pulmonary Perspective statement, 10 of the 27 patients were classified as definite AE and nine as suspected AE. In eight patients, infectious agents were identified as potential explanations for the respiratory decline. No patients with congestive heart failure or pulmonary embolism were identified. Overall survival to discharge was 37.0%. One-year survival was 14.8%. There were no differences in outcomes for patients with AE compared with those for whom potential infectious aetiologies were identified (log rank, P = 0.932). Patients with IPF showed a decreased rate of survival compared with patients with non-IPF fibrotic disease (1-year survival 0% vs 28.6%, log rank, P = 0.045).
Conclusions: In patients with fibrotic lung disease and an acute respiratory decline, a detailed diagnostic evaluation revealed a potential infectious aetiology in up to one-third of cases. However, there was no association between this finding and outcomes in these patients. One-year survival was dismal in patients who suffered an acute respiratory decompensation.
TL;DR: Auto-antibody profile seems to determine the frequency, course and severity of lung disease and new biological therapies such as rituximab show promising results in resistant and acute lung disease but controlled trials are needed.
Abstract: Purpose of reviewTo present the latest findings regarding interstitial lung disease (ILD) in idiopathic inflammatory myopathies, focusing on the phenotype of ILD and auto-antibodies, pathogenesis and treatment.Recent findingsInterstitial lung disease is a common manifestation of myositis and differe
TL;DR: Systemic sclerosis is a multisystem disorder characterized by disturbance in fibroblast function, microvascular disease, and immune system activation, culminating in fibrosis of the skin and internal organs.
Abstract: Introduction Systemic sclerosis (SSc; scleroderma) is a multisystem disorder characterized by disturbance in fibroblast function, microvascular disease, and immune system activation, culminating in fibrosis of the skin and internal organs (1,2). SSc is associated with extensive morbidity, including disfiguring skin thickening, finger ulcers, joint contractures, pulmonary hypertension, interstitial lung disease, chronic diarrhea, and renal failure (1,2). The rate of disease onset is highest between 30 and 50 years of age, with the risk for women being 4 to 5 times higher than for men (3,4). Median survival time from diagnosis is 11 years, and patients are 3.7 times more likely to die within 10 years of diagnosis (44.9% mortality) than age-, sex-, and race-matched individuals without SSc (12.0% mortality) (3).