scholarly journals Nocturnal hypoxaemia is associated with adverse outcomes in interstitial lung disease

Respirology ◽  
2019 ◽  
Vol 24 (10) ◽  
pp. 996-1004 ◽  
Author(s):  
Lauren K. Troy ◽  
Iven H. Young ◽  
Edmund M.T. Lau ◽  
Keith K.H. Wong ◽  
Brendon J. Yee ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Adverse Outcomes ◽  
Nocturnal Hypoxaemia

scholarly journals Histology of Interstitial Lung Disease in Common Variable Immune Deficiency

2020 ◽  
Vol 11 ◽  
Author(s):  
Fatima Dhalla ◽  
Dylan J. Mac Lochlainn ◽  
Helen Chapel ◽  
Smita Y. Patel
Keyword(s):  
Immune Deficiency ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Interstitial Fibrosis ◽  
Granulomatous Inflammation ◽  
Lymphoid Hyperplasia ◽  
Adverse Outcomes ◽  
Common Variable Immune Deficiency ◽  
Immune Deficiencies ◽  
Common Variable

Interstitial lung disease (ILD) is an important non-infectious complication in several primary immune deficiencies. In common variable immune deficiency (CVID) it is associated with complex clinical phenotypes and adverse outcomes. The histology of ILD in CVID is heterogeneous and mixed patterns are frequently observed within a single biopsy, including non-necrotising granulomatous inflammation, lymphoid interstitial pneumonitis, lymphoid hyperplasia, follicular bronchiolitis, organizing pneumonia, and interstitial fibrosis; ILD has to be differentiated from lymphoma. The term granulomatous-lymphocytic interstitial lung disease (GLILD), coined to describe the histopathological findings within the lungs of patients with CVID with or without multisystem granulomata, is somewhat controversial as pulmonary granulomata are not always present on histology and the nature of infiltrating lymphocytes is variable. In this mini review we summarize the literature on the histology of CVID-related ILD and discuss some of the factors that may contribute to the inter- and intra- patient variability in the histological patterns reported. Finally, we highlight areas for future development. In particular, there is a need for standardization of histological assessments and reporting, together with a better understanding of the immunopathogenesis of CVID-related ILD to resolve the apparent heterogeneity of ILD in this setting and guide the selection of rational targeted therapies in different patients.

scholarly journals Natural history and screening of interstitial lung disease in systemic autoimmune rheumatic disorders

2021 ◽  
Vol 13 ◽  
pp. 1759720X2110375
Author(s):  
Panagiotis Panagopoulos ◽  
Andreas Goules ◽  
Anna Maria Hoffmann-Vold ◽  
Eric L. Matteson ◽  
Athanasios Tzioufas
Keyword(s):  
Interstitial Lung Disease ◽  
Natural History ◽  
Pulmonary Function ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Lupus Erythematosus ◽  
Pulmonary Function Tests ◽  
Usual Interstitial Pneumonia ◽  
Adverse Outcomes ◽  
Rapid Progression

Interstitial lung disease (ILD) is a relatively frequent manifestation of systemic autoimmune rheumatic disorders (SARDs), including systemic sclerosis (SSc), rheumatoid arthritis (RA), idiopathic inflammatory myopathies (IIM), systemic lupus erythematosus (SLE), primary Sjögren’s syndrome (pSS), and anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis. Interstitial pneumonia with autoimmune features (IPAF) has been proposed to describe patients with ILD who have clinical or serological findings compatible with SARDs but they are not sufficient for a definite diagnosis. ILD may present with different patterns among patients with SARDs, but most commonly as nonspecific interstitial pneumonia (NSIP), with the exception of RA and ANCA vasculitis that more often present with usual interstitial pneumonia (UIP). The natural history of ILD is quite variable, even among patients with the same SARD. It may present with subclinical features following a slow progressively course or with acute manifestations and clinically significant rapid progression leading to severe deterioration of pulmonary function and respiratory failure. The radiographic pattern of ILD, the extent of the disease, the baseline pulmonary function, the pulmonary function deterioration rate over time and clinical variables related to the primary SARD, such as age, sex and the clinical phenotype, are considered prognostic factors for SARDs-ILD associated with adverse outcomes and increased mortality. Different modalities can be employed for ILD detection including clinical evaluation, pulmonary function tests, high resolution computed tomography and novel techniques such as lung ultrasound and serum biomarkers. ILD may determine the clinical outcome of SARDs, since it is associated with significant morbidity and mortality and therefore screening of patients with SARDs for ILD is of great clinical importance.

scholarly journals Nocturnal hypoxaemia in interstitial lung disease: An easy target to treat?

Respirology ◽  
2019 ◽  
Vol 24 (10) ◽  
pp. 930-932 ◽  
Author(s):  
Christopher J. Ryerson ◽  
Sana Vahidy
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Nocturnal Hypoxaemia

scholarly journals Oxygen therapy in COPD and interstitial lung disease: navigating the knowns and unknowns

2019 ◽  
Vol 5 (3) ◽  
pp. 00118-2019 ◽  
Author(s):  
Yet H. Khor ◽  
Elisabetta A. Renzoni ◽  
Dina Visca ◽  
Christine F. McDonald ◽  
Nicole S. L. Goh
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Oxygen Therapy ◽  
Current Evidence ◽  
Chronic Obstructive ◽  
Obstructive Pulmonary Disease ◽  
Nocturnal Hypoxaemia ◽  
Long Term Oxygen Therapy

Domiciliary oxygen therapy is often prescribed for patients with hypoxaemia due to advanced lung disease, most commonly chronic obstructive pulmonary disease (COPD) and interstitial lung disease (ILD). Long-term oxygen therapy (LTOT) trials conducted in patients with COPD in the 1980s remain the basis for clinical decisions and guideline recommendations regarding LTOT for patients with non-COPD conditions as there is a lack of high-quality evidence concerning its use in the non-COPD population. There is also a lack of evidence for the use of ambulatory and nocturnal oxygen therapy in patients with isolated exertional and nocturnal hypoxaemia. These deficiencies pose significant challenges in patient care, with consequent discrepancies in guideline recommendations and clinical approaches. In recent years, new studies have been and are currently being conducted to fill the gaps in our understanding and use of domiciliary oxygen therapy for other indications, including ILD. This article provides a comparison of the epidemiology and significance of hypoxaemia in patients with COPD and ILD, with an up-to-date review of current evidence regarding the role of different types of domiciliary oxygen therapy in these conditions.

Nocturnal hypoxaemia in interstitial lung disease: a systematic review

Thorax ◽  
2021 ◽  
pp. thoraxjnl-2020-216749
Author(s):  
Yet Hong Khor ◽  
Yvonne Ng ◽  
Duncan Sweeney ◽  
Christopher J Ryerson
Keyword(s):  
Systematic Review ◽  
Pulmonary Hypertension ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Supplemental Oxygen ◽  
Consensus Definition ◽  
Pooled Prevalence ◽  
Obstructive Sleep ◽  
Ventilatory Drive ◽  
Nocturnal Hypoxaemia

BackgroundPatients with interstitial lung disease (ILD) are at risk of developing nocturnal hypoxaemia due to ventilatory restriction and impaired gas exchange that worsen with supine posture and reduced ventilatory drive during sleep. This systematic review synthesised literature on the diagnostic evaluation, epidemiology, associations, management and prognosis of nocturnal hypoxaemia in ILD.MethodsOvid MEDLINE, Embase and CENTRAL databases were searched for eligible studies. Meta-analyses with subgroup analyses were conducted, where possible.ResultsFifty-three studies were included (total participant number=2590). The most common definition for clinically significant nocturnal hypoxaemia was ≥10% of total sleep time with oxyhaemoglobin saturation <90%, with pooled prevalence of 37%. There were no significant differences in pooled prevalence according to ILD subtype and comorbid obstructive sleep apnoea status. Study heterogeneity precluded meta-analysis of associations and prognosis. Diffusing capacity for carbon monoxide (DLCO) and echocardiographic features for pulmonary hypertension were consistently associated with nocturnal hypoxaemia. There were inconsistent associations between nocturnal hypoxaemia with ILD subtype and severity. Multivariable analyses in most studies demonstrated significant associations of nocturnal hypoxaemia with survival. Two small short-term intervention studies demonstrated that supplemental oxygen of 1–3 L/min corrected nocturnal hypoxaemia, with improved heart rate control during in-laboratory observation and increased serum antioxidant levels after 1 month of therapy.ConclusionNocturnal hypoxaemia is common, associated with DLCO impairment and markers suggestive of pulmonary hypertension, and a potential prognostic factor in patients in ILD. There is a need to establish a consensus definition of nocturnal hypoxaemia and evaluate long-term effects of nocturnal supplemental oxygen in ILD.

Cellular mechanisms of interstitial lung disease in children caused by mutations of the ABCA3-transporter

Pneumologie ◽  
2010 ◽  
Vol 64 (01) ◽  
Author(s):  
N Weichert ◽  
E Kaltenborn ◽  
A Hector ◽  
M Woischnik ◽  
S Moslavac ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Cellular Mechanisms
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