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Experimental models of sarcoidosis: where are we now?
Purpose of review Sarcoidosis remains a mysterious disease that presents many challenges both in pathogenetic understanding and in the management of patients. This review presents experimental models for sarcoidosis developed since 2016 and discusses their strengths and weaknesses and how they have contributed to the understanding and therapeutic approaches in this disease. In addition, future directions are proposed to overcome the limitations of current models. Recent findings New cellular models using infectious antigen as trigger, and transgenic models in mice have recently been developed to study signaling pathwa...
Source: Current Opinion in Pulmonary Medicine - August 13, 2020 Category: Respiratory Medicine Tags: SARCOIDOSIS: Edited by Daniel A. Culver and Hilario Nunes Source Type: research

Recent advances in sarcoidosis genomics: epigenetics, gene expression, and gene by environment (G × E) interaction studies
Purpose of review We aim to review the most recent findings in genomics of sarcoidosis and highlight the gaps in the field. Recent findings Original explorations of sarcoidosis subphenotypes, including cases associated with the World Trade Center and ocular sarcoidosis, have identified novel risk loci. Innovative gene--environment interaction studies utilizing modern analytical techniques have discovered risk loci associated with smoking and insecticide exposure. The application of whole-exome sequencing has identified genetic variants associated with persistent sarcoidosis and rare functional variations. A single epi...
Source: Current Opinion in Pulmonary Medicine - August 13, 2020 Category: Respiratory Medicine Tags: SARCOIDOSIS: Edited by Daniel A. Culver and Hilario Nunes Source Type: research

Immunology of sarcoidosis: old companions, new relationships
Purpose of review The immune determinants of granuloma formation and disease progression in sarcoidosis have not been completely disclosed, and the role of both innate and the adaptive immunity is still under investigation. Recent findings M2 macrophage polarization, previously thought to be a specific feature of a progressing and fibrosing disease, has been related to the initial steps of granuloma formation both in animal and in-vitro models. The dysregulation of specific metabolic pathways and autophagy has been associated with disease activity and progression. T cells have been reported to be strongly influenced b...
Source: Current Opinion in Pulmonary Medicine - August 13, 2020 Category: Respiratory Medicine Tags: SARCOIDOSIS: Edited by Daniel A. Culver and Hilario Nunes Source Type: research

Sarcoidosis epidemiology: recent estimates of incidence, prevalence and risk factors
Purpose of review The aim of this review is to describe the latest studies on sarcoidosis incidence, prevalence and risk factors with a special focus on reports in the last 2 years. The potential biases affecting these studies are discussed. Recent findings The prevalence and incidence of sarcoidosis vary greatly depending on region of the world. Variations in data sources and settings can affect estimates of the burden of sarcoidosis, sometimes making them difficult to compare across countries. It is not well understood how the distribution of sarcoidosis phenotypes differs across populations. Age, sex and race are t...
Source: Current Opinion in Pulmonary Medicine - August 13, 2020 Category: Respiratory Medicine Tags: SARCOIDOSIS: Edited by Daniel A. Culver and Hilario Nunes Source Type: research

The future is now: a generation of sarcoidologists emerges
No abstract available (Source: Current Opinion in Pulmonary Medicine)
Source: Current Opinion in Pulmonary Medicine - August 13, 2020 Category: Respiratory Medicine Tags: SARCOIDOSIS: Edited by Daniel A. Culver and Hilario Nunes Source Type: research

Advances in lung transplantation for interstitial lung diseases
Purpose of review Lung transplantation (LTx) is increasingly used as ultimate treatment modality in end-stage interstitial lung diseases (ILDs). This review aims to give an overview of the latest evolutions in this field. Recent findings In the last two years, important new findings regarding LTx outcomes in specific ILD entities have been reported. More data are available on optimization of pre-LTx management of ILD patients especially with regard to pretransplant antifibrotic treatment. Summary LTx is the only treatment option with curative intent for ILDs and is increasingly used for this indication. Several stu...
Source: Current Opinion in Pulmonary Medicine - August 13, 2020 Category: Respiratory Medicine Tags: INTERSTITIAL LUNG DISEASE: Edited by Bruno Crestani and Martin R.J. Kolb Source Type: research

Insights into pathogenesis and clinical implications in myositis-associated interstitial lung diseases
Purpose of review Interstitial lung diseases (ILDs) have been reported to be associated with myositis (including polymyositis and dermatomyositis). These myositis-associated ILDs carry significant morbidity and mortality. This review summarizes recent findings on myositis-associated ILD with a focus on pathogenesis and emerging treatment. Recent findings Recent advances in genetics have revealed 22 myositis-associated genome-wide loci, which were significantly enriched in regulatory regions in immune cells. An analysis of such disease-associated loci elucidated potential drug targets (e.g., TYK2 targeted by tofacitini...
Source: Current Opinion in Pulmonary Medicine - August 13, 2020 Category: Respiratory Medicine Tags: INTERSTITIAL LUNG DISEASE: Edited by Bruno Crestani and Martin R.J. Kolb Source Type: research

New therapeutic strategies in lung vasculitis
Purpose of review To summarize the latest publications and provide a practical overview of treatment strategies for lung vasculitis associated with antineutrophil cytoplasmic antibodies (ANCAs). Recent findings In patients with severe ANCA-associated vasculitis, plasma exchange, as adjunctive therapy to standard treatment, is not associated with improved survival or reduced risk of end-stage kidney disease. A regimen with reduced dose of glucocorticoids is equally effective to induce remission as a standard regimen. In patients without organ or life-threatening disease, mycophenolate mofetil can be used in combination...
Source: Current Opinion in Pulmonary Medicine - August 13, 2020 Category: Respiratory Medicine Tags: INTERSTITIAL LUNG DISEASE: Edited by Bruno Crestani and Martin R.J. Kolb Source Type: research

Systemic sclerosis-associated interstitial lung disease
Purpose of review In this review, the authors discuss systemic sclerosis-associated interstitial lung disease (SSc-ILD) with a focus on recent developments in diagnosis, surveillance, and management. Recent findings With advances in the management of SSc, the importance of ILD has been increasingly recognized and is the leading cause of mortality. Early detection is essential, and a combination of lung function testing and chest imaging are key tools in diagnosis and surveillance. The foundation of treatment is immunomodulation with recent studies identifying several potential new agents. The use of therapies targetin...
Source: Current Opinion in Pulmonary Medicine - August 13, 2020 Category: Respiratory Medicine Tags: INTERSTITIAL LUNG DISEASE: Edited by Bruno Crestani and Martin R.J. Kolb Source Type: research

Recent advances in rheumatoid arthritis-associated interstitial lung disease
Purpose of review To provide an overview of recent studies that could be helpful in a better understanding of rheumatoid arthritis-associated interstitial lung disease (RA-ILD) and to facilitate the clinical management of this severe complication of RA. Recent findings The advances in deciphering the genetic architecture of RA-ILD support the hypothesis of RA-ILD as a complex disease with a complex phenotype encompassing at least the usual interstitial pneumonia (UIP) high-resolution CT pattern and non-UIP. Genetics studies have provided evidence for a shared genetic background in idiopathic pulmonary fibrosis (IPF) a...
Source: Current Opinion in Pulmonary Medicine - August 13, 2020 Category: Respiratory Medicine Tags: INTERSTITIAL LUNG DISEASE: Edited by Bruno Crestani and Martin R.J. Kolb Source Type: research

Pulmonary rehabilitation in interstitial lung diseases
Purpose of review This review will provide an overview of the components and current evidence to support pulmonary rehabilitation in patients with interstitial lung diseases (ILD), focusing on the latest findings in enhancing quality of life and functional capacity using exercise training and education. Recent findings Recent literature demonstrates a longer duration of pulmonary rehabilitation results in sustained improvements in functional capacity and quality of life in patients with ILD. Educational components tailored to ILD provide added value to supervised exercise training. Enhancing exercise training with hyp...
Source: Current Opinion in Pulmonary Medicine - August 13, 2020 Category: Respiratory Medicine Tags: INTERSTITIAL LUNG DISEASE: Edited by Bruno Crestani and Martin R.J. Kolb Source Type: research

Oxygen for interstitial lung diseases
Purpose of review Supplemental oxygen therapy is prescribed for management of hypoxaemia in patients with interstitial lung disease (ILD). This review summarizes current evidence and implications of the use of supplemental oxygen therapy at home and during exercise training in ILD. Recent findings Despite the significance of hypoxaemia in patients with ILD, there is a lack of high-quality evidence to guide the use of oxygen therapy in this population. Recent studies suggest that ambulatory oxygen may improve symptoms and health-related quality of life in patients with ILD. Long-term oxygen therapy for resting hypoxaem...
Source: Current Opinion in Pulmonary Medicine - August 13, 2020 Category: Respiratory Medicine Tags: INTERSTITIAL LUNG DISEASE: Edited by Bruno Crestani and Martin R.J. Kolb Source Type: research

Burden of idiopathic pulmonary fibrosis on patients’ emotional well being and quality of life: a literature review
Purpose of review To present an overview of the impact of idiopathic pulmonary fibrosis (IPF) on patients’ emotional well being and quality of life (QoL). Recent findings IPF is an interstitial lung disease which causes irreversible, progressive lung scarring in a pathological pattern of usual interstitial pneumonia. The incidence of IPF is increasing at a global level, subjecting an increasing number of people to its high morbidity and risk of mortality. Diagnosis is based on a multidisciplinary team approach and the exclusion of other interstitial lung diseases. Two novel antifibrotic treatments, pirfenidone and n...
Source: Current Opinion in Pulmonary Medicine - August 13, 2020 Category: Respiratory Medicine Tags: INTERSTITIAL LUNG DISEASE: Edited by Bruno Crestani and Martin R.J. Kolb Source Type: research

Frailty in patients with interstitial lung disease
Purpose of review The incidence of age-related diseases such as interstitial lung disease (ILD) is rising, and the importance of multimorbidity and accumulation of health deficits in patients with chronic lung diseases is increasingly recognized. There are multiple relationships between aging and ILD on a demographic and a biological level. Frailty conceptualizes the decline of a patient's physiological reserves and complements the chronological and biological aspects of aging. Recent findings Frailty affects more than 50% of patients with ILD, with respiratory impairment, accelerated biological aging, comorbidities, ...
Source: Current Opinion in Pulmonary Medicine - August 13, 2020 Category: Respiratory Medicine Tags: INTERSTITIAL LUNG DISEASE: Edited by Bruno Crestani and Martin R.J. Kolb Source Type: research

Obstructive sleep apnea in pulmonary fibrosis
Purpose of review In previous years, there was limited research related to the role of sleep in interstitial lung diseases (ILDs). Physicians treating ILD patients tended to focus mainly on the daily disabling symptoms overlooking the possible significant role of coexisting sleep disorders, such as obstructive sleep apnea (OSA). However, recently, there has been a growing interest in OSA in ILDs, as well as OSA effect on sleep, life quality and outcome in these patients with emphasis on idiopathic pulmonary fibrosis (IPF). Recent findings OSA has been recognized as an important, high-prevalence comorbidity for the dia...
Source: Current Opinion in Pulmonary Medicine - August 13, 2020 Category: Respiratory Medicine Tags: INTERSTITIAL LUNG DISEASE: Edited by Bruno Crestani and Martin R.J. Kolb Source Type: research