Germline variant of CTC1 gene in a patient with pulmonary fibrosis and myelodysplastic syndrome.

Introduction: Telomeropathies are associated with a wide range of diseases and less common combinations of various pulmonary and extrapulmonary disorders. Case presentation: In proband with high-risk myelodysplastic syndrome and interstitial pulmonary fibrosis, whole exome sequencing revealed a germline heterozygous variant of CTC1 gene (c.1360delG). This "frameshift" variant results in a premature stop codon and is classified as likely pathogenic/pathogenic. So far, this gene variant has been described in a heterozygous state in adult patients with hematological diseases such as idiopathic aplastic anemia or paroxysmal nocturnal hemoglobinuria, but also in interstitial pulmonary fibrosis. Described CTC1 gene variant affects telomere length and leads to telomeropathies. Conclusions: In our case report, we describe a rare case of coincidence of pulmonary fibrosis and hematological malignancy caused by a germline gene mutation in CTC1. Lung diseases and hematologic malignancies associated with short telomeres do not respond well to standard treatment.

Research Progress of Respiratory Disease and Idiopathic Pulmonary Fibrosis Based on Artificial Intelligence.

Machine Learning (ML) is an algorithm based on big data, which learns patterns from the previously observed data through classifying, predicting, and optimizing to accomplish specific tasks. In recent years, there has been rapid development in the field of ML in medicine, including lung imaging analysis, intensive medical monitoring, mechanical ventilation, and there is need for intubation etiology prediction evaluation, pulmonary function evaluation and prediction, obstructive sleep apnea, such as biological information monitoring and so on. ML can have good performance and is a great potential tool, especially in the imaging diagnosis of interstitial lung disease. Idiopathic pulmonary fibrosis (IPF) is a major problem in the treatment of respiratory diseases, due to the abnormal proliferation of fibroblasts, leading to lung tissue destruction. The diagnosis mainly depends on the early detection of imaging and early treatment, which can effectively prolong the life of patients. If the computer can be used to assist the examination results related to the effects of fibrosis, a timely diagnosis of such diseases will be of great value to both doctors and patients. We also previously proposed a machine learning algorithm model that can play a good clinical guiding role in early imaging prediction of idiopathic pulmonary fibrosis. At present, AI and machine learning have great potential and ability to transform many aspects of respiratory medicine and are the focus and hotspot of research. AI needs to become an invisible, seamless, and impartial auxiliary tool to help patients and doctors make better decisions in an efficient, effective, and acceptable way. The purpose of this paper is to review the current application of machine learning in various aspects of respiratory diseases, with the hope to provide some help and guidance for clinicians when applying algorithm models.

Anti-vinculin antibodies as a novel biomarker in Egyptian patients with systemic sclerosis.

INTRODUCTION: Systemic sclerosis (SSc) is an autoimmune disorder that causes vasculopathy and scarring, most commonly in the lungs and skin, but it can also affect other organs. Endothelial vinculin plays a critical role in angiogenesis regulation. Therefore, vinculin overexpression in SSc may give rise to anti-vinculin antibodies, which may contribute to the development of SSc vasculopathy. The current research aims to (1) determine whether anti-vinculin autoantibodies play a significant role in the diagnosis of SSc and (2) compare anti-vinculin serum levels between two scleroderma patient populations, namely, pulmonary artery hypertension (PAH)-predominant and interstitial pulmonary fibrosis (IPF)-predominant groups. METHODS: This research included 140 participants categorized into three groups: group I-patients with PAH-predominant; group II-patients with ILD-predominant; group III-the control group. Anti-vinculin antibodies were detected in serum samples collected from all participants using ELISA. All subjects underwent high-resolution computed tomography (CT), diffusing capacity for carbon monoxide, and pulmonary function tests. RESULTS: Patients in group I (PAH-predominant group, N = 35) were 41.3 [± 11.4] years old, with 80% being women. Patients in group II (ILD-predominant group, N = 35) were 41.0 [± 11.5] years old. The SSc group showed significantly higher anti-vinculin antibody levels than the control group (P < 0.001). The PAH-predominant group demonstrated significantly higher anti-vinculin antibody levels and anti-vinculin positivity than the ILD-predominant group. CONCLUSION: Anti-vinculin antibodies in the blood appear to be diagnostic biomarkers for scleroderma. Furthermore, they shed light on some novel perspectives on the pathophysiology of specific lung fibrotic changes. Key Points • This study included two groups of systemic sclerosis patients (PAH-predominant group, ILD-predominant group) as well as a control group to investigate the significance of anti-vinculin antibodies in such cases. • Our results have demonstrated that anti-vinculin antibodies can play a significant role in diagnosing and monitoring systemic sclerosis disease.

Lung Damage in Rheumatoid Arthritis-A Retrospective Study.

The current study aimed to evaluate rheumatoid arthritis (RA) patients with interstitial lung disease (ILD) in clinical practice and whether disease characteristics are associated with X-ray and high-resolution computed tomography (HR-CT) findings. Medical history of RA patients from a tertiary rheumatology clinic was retrieved from its electronic database starting from 1 January 2019 until the study date (8 August 2022) using International Classification of Disease version 10 codes for RA, ILD and exclusion criteria. The study included 78 RA patients (75.6% women, 15.4% active smokers), with average time from RA to ILD of 5.6 years. Regarding chest X-ray findings, men had a higher prevalence of nodules, combined fibrosis and nodules and combined bronchiectasis and nodules, rheumatoid factor (RF)-positive patients had a higher prevalence of fibrosis and anti-cyclic citrullinated peptide antibodies (ACPA)-positive patients had a higher prevalence of bronchiectasis. Regarding HR-CT findings, patients actively treated with methotrexate had a higher prevalence of nodules; a combination of fibrosis and nodules; combination of emphysema and nodules; and combination of fibrosis, emphysema and nodules. ILD develops within approximately 5 years from RA diagnosis, and ILD-associated imaging findings on chest X-rays and HR-CT are more prevalent among men with RA, among patients with positive RA serology (RF and/or ACPA) and RA patients on methotrexate.

Implementation of guideline recommendations and outcomes in patients with idiopathic pulmonary fibrosis: Data from the IPF-PRO registry.

BACKGROUND: Few data are available on the extent to which clinical practice is aligned with international guidelines for the management of idiopathic pulmonary fibrosis (IPF). We investigated the extent to which management guidelines for IPF have been implemented in the US IPF-PRO Registry and associations between implementation of guidelines and clinical outcomes. METHODS: We assessed the implementation of eight recommendations in clinical practice guidelines within the 6 months after enrollment: visit to a specialized clinic; pulmonary function testing; use of oxygen in patients with resting hypoxemia and exercise-induced hypoxemia; referral for pulmonary rehabilitation; treatment of gastro-esophageal reflux disease; initiation of anti-fibrotic therapy; referral for lung transplant evaluation. An implementation score was calculated as the number of recommendations achieved divided by the number for which the patient was eligible. Associations between implementation score and outcomes were analyzed using logistic regression and Cox proportional hazards models. RESULTS: Among 727 patients, median (Q1, Q3) implementation score was 0.6 (0.5, 0.8). Patients with an implementation score >0.6 had greater disease severity than those with a lower score. Implementation was lowest for referral for pulmonary rehabilitation (19.5%) and lung transplant evaluation (22.3%). In unadjusted models, patients with higher implementation scores had a greater risk of death, death or lung transplant, and hospitalization, but no significant associations were observed in adjusted models. CONCLUSIONS: Management guidelines were more likely to be implemented in patients with IPF with greater disease severity. When adjusted for disease severity, no association was found between implementation of management guidelines and clinical outcomes.

NEDD4L-induced ß-catenin ubiquitination suppresses the formation and progression of interstitial pulmonary fibrosis via inhibiting the CTHRC1/HIF-1α axis.

Interstitial pulmonary fibrosis (IPF) is a severe progressive lung disease with limited therapeutic options and poor prognosis. Initially, we found the downregulated level of neural precursor cell expressed developmentally down-regulated 4-like protein (NEDD4L) in IPF-related expression microarray dataset, and this study was thus performed to explore the molecular mechanism of NEDD4L in IPF. The expression of NEDD4L was subsequently validated in lung tissues of IPF patients and mouse models. Then, mouse primary lung fibroblasts (LFs) were collected for in vitro functional experiments, with CCK-8, Transwell, and immunofluorescence assays used to examine the viability, migration, and differentiation of LFs. The in vitro findings were further assessed using in vivo mouse models. The expression of NEDD4L was down-regulated in lung tissues of IPF patients and mouse models. Overexpression of NEDD4L restricted the formation and progression of IPF in mice and attenuated the proliferative, invasive and differentiative abilities of LFs. Further, NEDD4L halted LFs activity by enhancing ß-catenin ubiquitination and down-regulating the CTHRC1/HIF-1α axis. Also, in vivo experiments then validated that NEDD4L silencing repressed ß-catenin ubiquitination and activated the CTHRC1/HIF-1α axis, thereby aggravating IPF in mice. NEDD4L may suppress the formation and progression of IPF through augmenting ß-catenin ubiquitination and inhibiting the CTHRC1/HIF-1α axis.

Human-Based Advanced in vitro Approaches to Investigate Lung Fibrosis and Pulmonary Effects of COVID-19.

The coronavirus disease 2019 (COVID-19) pandemic has caused considerable socio-economic burden, which fueled the development of treatment strategies and vaccines at an unprecedented speed. However, our knowledge on disease recovery is sparse and concerns about long-term pulmonary impairments are increasing. Causing a broad spectrum of symptoms, COVID-19 can manifest as acute respiratory distress syndrome (ARDS) in the most severely affected patients. Notably, pulmonary infection with Severe Acute Respiratory Syndrome coronavirus 2 (SARS-CoV-2), the causing agent of COVID-19, induces diffuse alveolar damage (DAD) followed by fibrotic remodeling and persistent reduced oxygenation in some patients. It is currently not known whether tissue scaring fully resolves or progresses to interstitial pulmonary fibrosis. The most aggressive form of pulmonary fibrosis is idiopathic pulmonary fibrosis (IPF). IPF is a fatal disease that progressively destroys alveolar architecture by uncontrolled fibroblast proliferation and the deposition of collagen and extracellular matrix (ECM) proteins. It is assumed that micro-injuries to the alveolar epithelium may be induced by inhalation of micro-particles, pathophysiological mechanical stress or viral infections, which can result in abnormal wound healing response. However, the exact underlying causes and molecular mechanisms of lung fibrosis are poorly understood due to the limited availability of clinically relevant models. Recently, the emergence of SARS-CoV-2 with the urgent need to investigate its pathogenesis and address drug options, has led to the broad application of in vivo and in vitro models to study lung diseases. In particular, advanced in vitro models including precision-cut lung slices (PCLS), lung organoids, 3D in vitro tissues and lung-on-chip (LOC) models have been successfully employed for drug screens. In order to gain a deeper understanding of SARS-CoV-2 infection and ultimately alveolar tissue regeneration, it will be crucial to optimize the available models for SARS-CoV-2 infection in multicellular systems that recapitulate tissue regeneration and fibrotic remodeling. Current evidence for SARS-CoV-2 mediated pulmonary fibrosis and a selection of classical and novel lung models will be discussed in this review.

Health Effects of Ozone on Respiratory Diseases.

Ozone is known to cause bronchial inflammation and airway hyper-responsiveness via oxidative injury and inflammation. While other ambient air pollutants such as particulate matter (PM) and nitrogen dioxide showed decreasing trends in mean annual concentrations, ozone concentrations have not declined recently in most countries across the world. Short-term exposure to high concentrations of ozone has been associated with increased mortality and cardiovascular and respiratory morbidity in many regions of the world. However, the long-term effects of ozone have been less investigated than the short-term exposure due to the difficulty in modeling ozone exposure and linking between individual exposures and health outcome data. A recently developed model of ozone exposure enabled the investigation of long-term ozone effects on health outcomes. Recent findings suggested that long-term exposure to ozone was associated with an increased risk of cardiovascular and respiratory mortality. Longitudinal studies using large cohorts also revealed that long-term exposure to ozone was associated with a greater decline in lung function and the progression of emphysema. The development of long-term standards for ozone as well as PM should be considered to protect the respiratory health of the general population and people with chronic respiratory diseases.

Interstitial lung involvement in systemic sclerosis / Compromiso intersticial pulmonar en la esclerosis sistémica

ABSTRACT Introduction: Systemic sclerosis can involve the lung parenchyma leading to serious complications and even death. Objectives: To describe the most relevant aspects of interstitial lung disease related to systemic sclerosis emphasizing diagnosis and treatment. Materials and methods: A literature review was performed searching in the databases Medline and EMBASE using the MeSH terms «Scleroderma, Systemic", «Lung Diseases, Interstitial¼ and «Pulmonary Fibrosis¼ Results and conclusions: Interstitial lung disease is a common clinical manifestation of systemic sclerosis and one of the main causes of death. Treatment options are limited and have a modest effect in most of the cases.

RESUMEN Introducción: La esclerosis sistémica puede potencialmente comprometer el parénquima pulmonar, llevando a serias complicaciones e incluso a la muerte. Objetivos: Describir los aspectos más relevantes en cuanto a las generalidades de la enfermedad pulmonar intersticial en esclerosis sistémica, su diagnóstico y su tratamiento. Materiales y métodos: Se realizó una búsqueda de literatura en las bases de datos Medline y EMBASE utilizando los términos MeSH «Scleroderma, Systemic¼, «Lung Diseases, Interstitial¼ y «Pulmonary Fibrosis¼. Resultados y conclusiones: La enfermedad pulmonar intersticial es una manifestación frecuente de la esclerosis sistémica y una de las principales causas de muerte en los pacientes que la padecen. Las opciones terapéuticas son limitadas y su efecto es, en muchos casos, modesto.

Transcription factor YY1 inhibits the expression of THY1 to promote interstitial pulmonary fibrosis by activating the HSF1/miR-214 axis.

Interstitial pulmonary fibrosis (IPF) is a progressive disease of diverse etiology manifesting with proliferation of lung fibroblasts and accumulation of extracellular matrix deposition in pulmonary interstitium. Recent studies show aberrant expression of mRNAs and microRNAs (miRNAs) in human embryonic pulmonary fibroblasts (HEPFs). In this study, we investigated effects of the YY1/HSF1/miR-214/THY1 axis on the functions of HEPFs and IPF. Loss- and gain-of-function tests were conducted to identify roles of YY1, HSF1, miR-214, and THY1 in IPF. As determined by RT-qPCR or western blot assay, silencing YY1 down-regulated HSF1 expression and attenuated the expression of pro-proliferative and fibrosis markers in HEPFs. Meanwhile, viability of HEPFs was impeded by YY1 knockdown. The binding relationship between miR-214 and THY1 was verified using dual-luciferase reporter assay. In HEPFs, down-regulation of HSF1 reduced miR-214 expression to repress proliferation and fibrogenic transformation of HEPFs, while inhibition of miR-214 expression could restrain the fibrogenic transformation property of HEPFs by up-regulating THY1. Subsequently, IPF model in mice was induced by bleomycin treatment. These animal experiments validated the protective effects of YY1 knockdown against IPF-induced lung pathological manifestations, which could be reversed by THY1 knockdown. Our study demonstrates the important involvement of YY1/HSF1/miR-214/THY1 axis in the development of IPF.

Aging and Lung Disease.

People worldwide are living longer, and it is estimated that by 2050, the proportion of the world's population over 60 years of age will nearly double. Natural lung aging is associated with molecular and physiological changes that cause alterations in lung function, diminished pulmonary remodeling and regenerative capacity, and increased susceptibility to acute and chronic lung diseases. As the aging population rapidly grows, it is essential to examine how alterations in cellular function and cell-to-cell interactions of pulmonary resident cells and systemic immune cells contribute to a higher risk of increased susceptibility to infection and development of chronic diseases, such as chronic obstructive pulmonary disease and interstitial pulmonary fibrosis. This review provides an overview of physiological, structural, and cellular changes in the aging lung and immune system that facilitate the development and progression of disease.

Pilot experience of multidisciplinary team discussion dedicated to inherited pulmonary fibrosis.

BACKGROUND: Genetic testing is proposed for suspected cases of monogenic pulmonary fibrosis, but clinicians and patients need specific information and recommendation about the related diagnosis and management issues. Because multidisciplinary discussion (MDD) has been shown to improve accuracy of interstitial lung disease (ILD) diagnosis, we evaluated the feasibility of a genetic MDD (geneMDD) dedicated to the indication for and interpretation of genetic testing. The geneMDD group met monthly and included pediatric and adult lung specialists with ILD expertise, molecular and clinical geneticists, and one radiologist. Hematologists, rheumatologists, dermatologists, hepatologists, and pathologists were also invited to attend. RESULTS: Since 2016, physicians from 34 different centers in 7 countries have participated in the geneMDD. The medical files of 95 patients (53 males) have been discussed. The median age of patients was 43 years [range 0-77], 10 were ≤ 15 years old, and 6 were deceased at the time of the discussion. Among 85 analyses available, the geneMDD considered the rare gene variants pathogenic for 61: 37 variants in telomere-related genes, 23 variants in surfactant-related genes and 1 variant in MARS. Genetic counseling was offered for relatives of these patients. The geneMDD therapeutic proposals were as follows: antifibrotic drugs (n = 25), steroids or immunomodulatory therapy (n = 18), organ transplantation (n = 21), watch and wait (n = 21), or best supportive care (n = 4). CONCLUSION: Our experience shows that a dedicated geneMDD is feasible regardless of a patient's age and provides a unique opportunity to adapt patient management and therapy in this very rare condition.

Detection of mitochondrial transfer RNA (mt-tRNA) gene mutations in patients with idiopathic pulmonary fibrosis and sarcoidosis.

Mitochondrial reactive oxygen species production may lead to tissue injury associated with two respiratory disorders of unknown origin which are shared by common tissue fibrosis, IPF and sarcoidosis. Sequence analysis of 22 mt-tRNA genes and parts of their flanking genes revealed 32 and 45 mutations in 38/40 IPF and 69/85 sarcoidosis patients respectively. 4 novel mutations were identified. 15/32 and 25/45 mutations were exclusively expressed while 12/32 and 17/45 mutations predominantly occurred in IPF and sarcoidosis group respectively, compared to healthy controls. Novel mutation combinations were solely expressed in disease. Hence, a mitochondrial-mediated pathogenic pathway seems to underlie both entities.

Neumonitis intersticial descamativa en el paciente pediátrico / Desquamative interstitial penumonitis in a pediatric patient

Introducción: la neumonitis intersticial descamativa es una entidad caracterizada en la clínica por mostrar tos, disnea, cianosis e hipercapnia, con un patrón restrictivo de las pruebas funcionales respiratorias, debido a la presencia de fibrosis pulmonar, cuya frecuencia es inusual en Pediatría. Presentación del caso: adolescente que fue remitida al Hospital Docente Pediátrico del Cerro por sospecha de dengue, al referir fiebre de 38 °C de 3 días de evolución, acompañada de dolores musculares en miembros inferiores, escalofríos y cefalea, por lo cual fue internada en la sala de misceláneas. Durante su evolución mostró dificultad respiratoria, tos seca, taquipnea, taquicardia y disminución del murmullo vesicular en la base del pulmón izquierdo. Se observó en la radiografía de tórax una opacidad en dicha zona y fue tratada con antibióticos. En etapa posterior se trasladó a la Unidad de Cuidados Intensivos por ocurrir un incremento de las lesiones pulmonares e insuficiencia respiratoria; por ello, se indicó ventilación mecánica, variedad presión controlada. Posteriormente se aisló en hemocultivo y secreciones bronquiales, Pseudomona Stutzeri, evento considerado como una sepsis asociada a cuidados sanitarios. Se planteó un distrés respiratorio del adulto en niños que no involucionó, y falleció en un cuadro de insuficiencia respiratoria a los 19 días de estadía. Conclusiones: esta paciente mostró síntomas y signos sugestivos de una infección pulmonar bacteriana de evolución tórpida. Los hallazgos necrópsicos describen la presencia de una bronconeumonía bacteriana como causa directa, y una neumonitis intersticial descamativa, como entidad básica del fallecimiento(AU)

Introduction: desquamative interstitial pneumonitis is a characterized condition in the clinical field since it shows cough, dysnea, cyanosis and hypercapnia, with a restrictive pattern of the functional respiratory tests due to the presence of pulmonary fibrosis that is unusual in pediatrics. Case presentation: a female adolescent was referred to the pediatric teaching hospital of Cerro on suspicion of dengue since she presented with 38 °C for three days, accompanied with muscle aches in lower limbs, chills and headache. She was admitted to a general ward. During her progression, she showed respiratory distress, unproductive cough, tachypnea, tachycardia and reduction of vesicular murmur in the left lung basis. The thoracic X ray showed opacity in the area and was treated with antibiotics. In a later phase, she was moced to the intensive care unit due to increase in pulmonary lesions and respiratory failure. She was also under mechanical ventilation with controlled pressure. Later, Pseudomona Stutzeri was isolated in blood culture and bronchial secretions, an event considered to be health care-associated sepsis. It was stated that this case was a respiratory distress of adult in a child that evolved and finally the adolescent died of respiratory failure 19 days after her hospitalization. Conclusions: this patient showed symptoms and signs suggestive of bacterial pulmonary infection of torpid progression. The necropsis finding describe the presence of bacterial bronchopneumonia as a direct cause and desquamative interstitial pneumonitis as the basic condition for death(AU)

Neumonitis intersticial descamativa en el paciente pediátrico / Desquamative interstitial penumonitis in a pediatric patient

Introducción: la neumonitis intersticial descamativa es una entidad caracterizada en la clínica por mostrar tos, disnea, cianosis e hipercapnia, con un patrón restrictivo de las pruebas funcionales respiratorias, debido a la presencia de fibrosis pulmonar, cuya frecuencia es inusual en Pediatría.Presentación del caso: adolescente que fue remitida al Hospital Docente Pediátrico del Cerro por sospecha de dengue, al referir fiebre de 38 °C de 3 días de evolución, acompañada de dolores musculares en miembros inferiores, escalofríos y cefalea, por lo cual fue internada en la sala de misceláneas. Durante su evolución mostró dificultad respiratoria, tos seca, taquipnea, taquicardia y disminución del murmullo vesicular en la base del pulmón izquierdo. Se observó en la radiografía de tórax una opacidad en dicha zona y fue tratada con antibióticos. En etapa posterior se trasladó a la Unidad de Cuidados Intensivos por ocurrir un incremento de las lesiones pulmonares e insuficiencia respiratoria; por ello, se indicó ventilación mecánica, variedad presión controlada. Posteriormente se aisló en hemocultivo y secreciones bronquiales, Pseudomona Stutzeri, evento considerado como una sepsis asociada a cuidados sanitarios. Se planteó un distrés respiratorio del adulto en niños que no involucionó, y falleció en un cuadro de insuficiencia respiratoria a los 19 días de estadía.Conclusiones: esta paciente mostró síntomas y signos sugestivos de una infección pulmonar bacteriana de evolución tórpida. Los hallazgos necrópsicos describen la presencia de una bronconeumonía bacteriana como causa directa, y una neumonitis intersticial descamativa, como entidad básica del fallecimiento(AU)

Mixed connective tissue disease.

The concept of mixed connective tissue disease (MCTD) as a separate connective tissue disease (CTD) has persisted for more than four decades. High titers of antibodies targeting the U1 small nuclear ribonucleoprotein particle (U1 snRNP) in peripheral blood are a sine qua non for the diagnosis of MCTD, in addition to distinct clinical features including Raynaud's phenomenon (RP), "puffy hands," arthritis, myositis, pleuritis, pericarditis, interstitial lung disease (ILD), and pulmonary hypertension (PH). Recently, population-based epidemiology data from Norway estimated the point prevalence of adult-onset MCTD to be 3.8 per 100,000 and the mean annual incidence to be 2.1 per million per year, supporting the notion that MCTD is the least common CTD. Little is known about the etiology of MCTD, but recent genetic studies have confirmed that MCTD is a strongly HLA (​human leukocyte antigen)-linked disease, as the HLA profiles of MCTD differ distinctly from the corresponding profiles of ethnically matched healthy controls and other CTDs. In the first section of this review, we provide an update on the clinical, immunological, and genetic features of MCTD and discuss the relationship between MCTD and the other CTDs. Then we proceed to discuss the recent advances in therapy and our current understanding of prognosis and prognostic factors, especially those that are associated with the more serious pulmonary and cardiovascular complications of the disease. In the final section, we discuss some of the key, unresolved questions related to anti-RNP-associated diseases and indicate how these questions may be approached in future studies.

Ultrasound in the interstitial pulmonary fibrosis. Can it facilitate a best routine assessment in rheumatic disorders?

Ultrasound (US) is increasing its potential in the assessment of several rheumatic disorders. Recently, different applications of this imaging technique have emerged. Interesting data supporting its utility and validity in the assessment of the lung to detect and quantify interstitial pulmonary fibrosis in rheumatic diseases, even in subclinical phases, have been reported. The main purpose of this review is to provide an overview of the role of US in the assessment of interstitial pulmonary fibrosis in rheumatic disorders and to discuss the current evidence supporting its clinical relevance in daily clinical practice.

The comparison of high-resolution computed tomography findings in asbestosis and idiopathic pulmonary fibrosis.

BACKGROUND: To determine whether the HRCT findings are useful to differentiate asbestosis from idiopathic pulmonary fibrosis (IPF). METHODS: We assessed HRCT scans from patients with asbestosis (n = 96) and IPF (n = 65). The frequencies and extent of parenchymal abnormalities and the frequencies of pleural changes were evaluated by consensus of two chest radiologists. RESULTS: There was a significant difference between IPF and asbestosis in pleural changes. In addition, there were significant differences between IPF and asbestosis in several parenchymal abnormalities on CT, especially in the less advanced stage of both diseases. On multivariate analysis, HRCT features that distinguished asbestosis from IPF were subpleural lines at a distance of less than 5 mm from the inner chest wall, subpleural dots and parenchymal bands. CONCLUSIONS: There are significant differences between IPF and asbestosis in the parenchymal and pleural abnormalities on CT.

Associations between anti-Ro52 antibodies and lung fibrosis in mixed connective tissue disease.

OBJECTIVE: MCTD is a chronic, immune-mediated disorder defined by the combined presence of serum anti-RNP antibodies and distinct clinical features, including progressive lung fibrosis. The aim of the study was to evaluate the potential impact of anti-SSA (i.e. Ro52 and Ro60) and anti-SSB autoantibodies as markers for disease outcomes in MCTD. METHODS: Stored serum samples from 113 patients included in the cross-sectional, nationwide Norwegian MCTD cohort were screened for the presence of anti-Ro52, anti-Ro60 and anti-SSB by a commercial line immunoassay. Correlation analyses were carried out with clinical parameters, including quantitative lung fibrosis scores by high-resolution CT. Lung fibrosis was defined by reticular pattern changes according to the Fleischner Society CT criteria for interstitial lung disease. RESULTS: Anti-Ro52 antibodies were present in 29%, anti-Ro60 in 19% and anti-SSB in 6% of the MCTD sera. High-resolution CT scoring identified lung fibrosis in 38 of 113 (34%) MCTD patients. Anti-Ro52 antibodies were detected in 50% (19 of 38) of the MCTD patients with lung fibrosis and in 19% (14 of 75) without lung fibrosis (P < 0.001). The odds ratio for the presence of anti-Ro52 antibodies in lung fibrosis was 4.4 (95% CI 1.8, 10.3). Anti-Ro52 antibodies were equally frequent in patients with mild to moderate (eight of 17; 44%) and severe fibrosis (11 of 21; 52%). Anti-Ro52 was not associated with any of the other clinical parameters assessed, nor was anti-Ro60 or anti-SSB. CONCLUSION: Our cross-sectional data suggest that anti-Ro52 antibodies may serve as a potential marker for lung fibrosis in MCTD.