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Sarcoidosis and maligancy: the chicken and the egg?
Purpose of review There is a complex interaction between sarcoidosis and malignancy. Since tumors can elicit a granulomatous reaction, the presence of granulomas alone is insufficient to diagnose sarcoidosis in a patient with cancer. In addition, check point inhibitors can also lead to a granulomatous reaction which can be misdiagnosed as sarcoidosis. These issues need to be considered when exploring the relationship between sarcoidosis and malignancy. Despite these limitations, a growing amount of evidence supports the potential interaction of sarcoidosis and malignancy. Recent findings Several large epidemiolo...
Source: Current Opinion in Pulmonary Medicine - August 11, 2021 Category: Respiratory Medicine Tags: SARCOIDOSIS: Edited by Marlies S. Wijsenbeek and Nadera J. Sweiss Source Type: research

Sarcoidosis and autoimmunity
Purpose of review Sarcoidosis is a poorly understood multisystem granulomatous disease that frequently involves the lungs but can affect any organ system. In this review, we summarize recent developments in the understanding of the immune dysregulation seen in sarcoidosis and propose a new expanded definition of human autoimmunity in sarcoidosis, and the implications it would have on treating sarcoidosis with targeted immunotherapy regimens in the future. Recent findings Sarcoidosis has been linked to infectious organisms like Mycobacterium and Cutibacterium, and certain manifestations of sarcoidosis have been l...
Source: Current Opinion in Pulmonary Medicine - August 11, 2021 Category: Respiratory Medicine Tags: SARCOIDOSIS: Edited by Marlies S. Wijsenbeek and Nadera J. Sweiss Source Type: research

Drug-induced sarcoidosis-like reactions
Purpose of review Sarcoidosis is a complex granulomatous disease of unknown cause. Several drug categories are able to induce a systemic granulomatous indistinguishable from sarcoidosis, known as drug-induced sarcoidosis-like reaction (DISR). This granulomatous inflammation can resolve if the medication is discontinued. In this review, we discuss recent literature on medication associated with DISR, possible pathophysiology, clinical features, and treatment. Recent findings Recently, increasing reports on DISR have expanded the list of drugs associated with the systemic granulomatous eruption. Most reported drug...
Source: Current Opinion in Pulmonary Medicine - August 11, 2021 Category: Respiratory Medicine Tags: SARCOIDOSIS: Edited by Marlies S. Wijsenbeek and Nadera J. Sweiss Source Type: research

The emerging role of inorganic elements as potential antigens in sarcoidosis
Purpose of review Previous studies mainly described a role for organic agents as possible triggers for sarcoidosis. In this review, we address recent studies suggesting a possible role for inorganic elements, such as metals or silica in sarcoidosis pathogenesis. Recent findings Several epidemiological papers suggest that inorganic agents, either by environmental exposures or occupational activities, could trigger sarcoidosis. Association between inorganics and sarcoidosis is also described in several recently published case reports and studies demonstrating immunological sensitization to inorganic agents in sarc...
Source: Current Opinion in Pulmonary Medicine - August 11, 2021 Category: Respiratory Medicine Tags: SARCOIDOSIS: Edited by Marlies S. Wijsenbeek and Nadera J. Sweiss Source Type: research

Genetics in sarcoidosis
Purpose of review Epidemiological and clinical observations as well as familial clustering support the existence of a genetic predisposition to sarcoidosis. In this article, we review the most recent findings in genetics of sarcoidosis and discuss how the identification of risk alleles may help advancing our understanding of disease etiology and development. Recent findings Genetic studies of sarcoidosis phenotypes have identified novel and ancestry-specific associations. Gene-environment interaction studies highlighted the importance of integrating genetic information when assessing the relationship between sar...
Source: Current Opinion in Pulmonary Medicine - August 11, 2021 Category: Respiratory Medicine Tags: SARCOIDOSIS: Edited by Marlies S. Wijsenbeek and Nadera J. Sweiss Source Type: research

Editorial: The game is changing in sarcoidosis
No abstract available (Source: Current Opinion in Pulmonary Medicine)
Source: Current Opinion in Pulmonary Medicine - August 11, 2021 Category: Respiratory Medicine Tags: SARCOIDOSIS: Edited by Marlies S. Wijsenbeek and Nadera J. Sweiss Source Type: research

Chronic hypersensitivity pneumonitis: real world diagnostic criteria
Purpose of review Establishing a diagnosis of hypersensitivity pneumonitis (HP) and distinguishing it from other forms of interstitial lung diseases represents a common challenge in clinical practice. This review summarizes the latest literature and guidelines on HP while integrating some real-life conundrums. Recent findings Advances in the understanding of the pathobiology of fibrotic HP and other progressive pulmonary fibrosis have changed how we approach the diagnosis and treatment of interstitial lung disease. Classifications now embrace distinguishing two clinical phenotypes: nonfibrotic and fibrotic HP be...
Source: Current Opinion in Pulmonary Medicine - August 11, 2021 Category: Respiratory Medicine Tags: INTERSTITIAL LUNG DISEASE: Edited by Francesco Bonella, Athol U. Wells and Justin Oldham Source Type: research

Unclassifiable, or simply unclassified interstitial lung disease?
Purpose of review Unclassifiable interstitial lung disease (ILD) comprises a subset of ILDs which cannot be classified according to the current diagnostic framework. This is a likely a heterogeneous group of diseases rather than a single entity and it is poorly defined and hence problematic for prognosis and therapy. Recent findings With increased treatment options for progressive fibrosing ILD it is increasingly relevant to correctly categorise ILD. Summary This review article will summarise the definition and reasons for a diagnosis of unclassifiable ILD, the current management options and possible futu...
Source: Current Opinion in Pulmonary Medicine - August 11, 2021 Category: Respiratory Medicine Tags: INTERSTITIAL LUNG DISEASE: Edited by Francesco Bonella, Athol U. Wells and Justin Oldham Source Type: research

Pulmonary hypertension in interstitial lung disease: screening, diagnosis and treatment
Purpose of review Pulmonary vascular disease resulting in pulmonary hypertension in the context of interstitial lung disease (PH-ILD) is a common complication that presents many challenges in clinical practice. Despite recent advances, the pathogenetic interplay between parenchymal and vascular disease in ILD is not fully understood. This review provides an overview of the current knowledge and recent advances in the field. Recent findings Clinical trials employing the phosphodiesterase-5-inhibitor sildenafil delivered negative results whereas riociguat showed harmful effects in the PH-ILD population. More recen...
Source: Current Opinion in Pulmonary Medicine - August 11, 2021 Category: Respiratory Medicine Tags: INTERSTITIAL LUNG DISEASE: Edited by Francesco Bonella, Athol U. Wells and Justin Oldham Source Type: research

The other connective tissue disease-associated interstitial lung diseases: Sjogren's syndrome, mixed connective tissue disease, and systemic lupus erythematosus
Purpose of review We review the clinical manifestations of three less common connective tissue disease (CTD)-associated interstitial lung diseases (ILDs): Sjogren's syndrome (SjS), mixed CTD (MCTD), and systemic lupus erythematosus (SLE). Recent findings SjS is classically associated with lymphocytic interstitial pneumonia and cystic lung disease, but the most common type of ILD in Sjogren's patients is nonspecific interstitial pneumonia. ILD is prevalent in MCTD and associated with worse survival. SLE-associated ILD, while rare, is more common in those with CTD overlap syndromes. Regardless of underlying cause,...
Source: Current Opinion in Pulmonary Medicine - August 11, 2021 Category: Respiratory Medicine Tags: INTERSTITIAL LUNG DISEASE: Edited by Francesco Bonella, Athol U. Wells and Justin Oldham Source Type: research

Interstitial pneumonia with autoimmune features: from research classification to diagnosis
Purpose of review The term interstitial pneumonia with autoimmune features (IPAF) was first proposed by an international task force in 2015 as a research classification to standardise nomenclature regarding patients with idiopathic interstitial pneumonia and features of connective tissue disease. However, how the use of this term and its proposed definition translates to clinical practice remains uncertain. This review will provide a comprehensive overview of studies of IPAF cohorts to date, discuss the consideration of IPAF as a distinct diagnostic entity and outline a suggested approach to patient management. Rece...
Source: Current Opinion in Pulmonary Medicine - August 11, 2021 Category: Respiratory Medicine Tags: INTERSTITIAL LUNG DISEASE: Edited by Francesco Bonella, Athol U. Wells and Justin Oldham Source Type: research

The progressive fibrotic phenotype in current clinical practice
Purpose of review The progressive fibrotic phenotype (PFP), a term that covers large sub-groups of patients with fibrotic lung diseases that clinically progress despite appropriate usual management, is now an everyday problem for patients and clinicians alike. This review covers recent data that are relevant to major clinical uncertainties. Recent findings The clinical relevance of the PFP is covered by a brief review of data from which this entity was constructed. Estimates of the prevalence of the PFP are cited. The importance of an accurate initial diagnosis is emphasized – with refutation of the belief tha...
Source: Current Opinion in Pulmonary Medicine - August 11, 2021 Category: Respiratory Medicine Tags: INTERSTITIAL LUNG DISEASE: Edited by Francesco Bonella, Athol U. Wells and Justin Oldham Source Type: research

The justification for the progressive fibrotic phenotype
Purpose of review Describe the concept and recent data for the concept of progressive fibrotic interstitial lung disease (ILD). Recent findings Making an accurate diagnosis is critical to help determine appropriate therapy and predict prognosis. This is certainly true in the field of ILD where a diagnosis of idiopathic pulmonary fibrosis (IPF) leads a clinician to consider initiation of antifibrotic therapy, and avoidance of immunosuppression due to possible harm, at the time of diagnosis due to the high probability of disease progression. In other types of ILD immunosuppression may be helpful such as those asso...
Source: Current Opinion in Pulmonary Medicine - August 11, 2021 Category: Respiratory Medicine Tags: INTERSTITIAL LUNG DISEASE: Edited by Francesco Bonella, Athol U. Wells and Justin Oldham Source Type: research

Biopsy in interstitial lung disease: specific diagnosis and the identification of the progressive fibrotic phenotype
Purpose of review The evaluation of progression in fibrotic interstitial lung diseases (ILDs) may require a multidimensional approach. This review will cover the role and usefulness of lung biopsy in diagnosis and assessment of the progressive fibrotic phenotype. Recent findings The identification of specific findings and the balance between inflammation and fibrosis on lung biopsy may help distinguishing different disease entities and may likely determine the effect of treatment and possibly prognosis. The fibrotic morphological patterns potentially associated with a progressive phenotype include usual intersti...
Source: Current Opinion in Pulmonary Medicine - August 11, 2021 Category: Respiratory Medicine Tags: INTERSTITIAL LUNG DISEASE: Edited by Francesco Bonella, Athol U. Wells and Justin Oldham Source Type: research

Thoracic computed tomography in the progressive fibrotic phenotype
Purpose of review To discuss rationale and methods for determining progressive lung fibrosis on thoracic computed tomography (CT) and describe limitations and challenges Recent findings Identifying patients with progressive lung fibrosis is important to determine optimal treatment. Serial high-resolution computed tomography is a method of determining disease progression. A number of studies are reviewed in this article, that have explored various parameters (both visual and automated) that signify progressive fibrosis on CT. Summary To reliably use serial CT as a marker of disease progression in fibrotic ...
Source: Current Opinion in Pulmonary Medicine - August 11, 2021 Category: Respiratory Medicine Tags: INTERSTITIAL LUNG DISEASE: Edited by Francesco Bonella, Athol U. Wells and Justin Oldham Source Type: research