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BackgroundCommon Variable Immunodeficiency Disorders (CVID) encompass a spectrum of immunodeficiency characterised by recurrent infections and diverse non-infectious complications (NICs). This study aimed to describe the clinical features and variation in NICs in CVID with and without interstitial lung disease (ILD) from a large UK national registry population.MethodsRetrospective, cross-sectional data from a UK multicentre database (previously known as UKPIN), categorising patients into those with CVID-ILD and those with NICs related to CVID but without pulmonary involvement (CVID-EP; EP= extra-pulmonary involvement only).Results129 patients were included. Chronic lung diseases, especially CVID-ILD, are prominent complications in complex CVID, occurring in 62% of the cohort. Bronchiectasis was common (64% of the cohort) and associated with greater pulmonary function impairment in patients with CVID-ILD compared to those without bronchiectasis. Lymphadenopathy and the absence of gastrointestinal diseases were significant predictors of ILD in complex CVID. Although the presence of liver disease did not differ significantly between the groups, nearly half of the CVID-ILD patients were found to have liver disease. Patients with CVID-ILD were more likely to receive immunosuppressive treatments such as rituximab and mycophenolate mofetil than the CVID-EP group, indicating greater need for treatment and risk of complications.ConclusionThis study highlights the significant burden of CVID-ILD within the CVID population with NICs only. The lungs emerged as the most frequently affected organ, with ILD and bronchiectasis both highly prevalent. These findings emphasise the necessity of a comprehensive and multidisciplinary approach in managing CVID patients, considering their susceptibility to various comorbidities and complications.

BackgroundPulmonary hypertension due to chronic obstructive pulmonary disease (COPD) and interstitial lung disease (ILD) is classified as group 3 pulmonary hypertension. Inhaled treprostinil, a prostaglandin I2 analogue also known as prostacyclin, has recently been approved as a first drug for patients with pulmonary hypertension secondary to ILD. However, due to a lack of evidence, no therapies are currently approved for those with COPD-associated pulmonary hypertension. Thus, this systematic review aims to summarise the current evidence to assess the impact of inhaled prostaglandin I2 analogue use on the pulmonary hemodynamics, exercise function, lung function, and gas exchange in patients with pulmonary hypertension due to COPD.MethodsWe systematically searched the electronic databases of Medline, Embase, Scopus and Cochrane from inception to 1 February 2023. Studies of adult patients with a confirmed diagnosis of COPD-associated pulmonary hypertension who received inhaled drugs targeting the prostacyclin pathway were included in the systematic review. Case reports, systematic reviews, conference abstracts with no full text, non-full-text articles, non-English manuscripts and book chapters were excluded from this systematic review. A risk-of-bias assessment was carried out for the studies included in this review, using two different Cochrane risk-of-bias tools for randomised and non-randomised clinical trials.ResultsA total of four studies met our inclusion criteria and were included in this systematic review. The results of one prospective clinical trial showed an improvement in the pulmonary hemodynamics (e.g., cardiac index, cardiac output and mean pulmonary artery pressure) in response to inhaled prostacyclin use in patients with pulmonary hypertension secondary to COPD. However, the severity of dyspnoea, lung function, exercise capacity and gas exchange were not affected when inhaled prostacyclin was used for patients with COPD-related pulmonary hypertension.ConclusionThis systematic review demonstrated that although inhaled prostacyclin does not seem to improve COPD-related outcomes (e.g., lung function and exercise capacity), short-term use of inhaled prostacyclin has the potential to reduce mean pulmonary artery pressure and pulmonary vascular resistance without impairing ventilation-perfusion mismatch. Further studies with larger sample sizes are warranted.Systematic review registrationCRD42022372803, https://www.crd.york.ac.uk/prospero/display_record.php?RecordID=372803.

IntroductionCommon variable immunodeficiency related interstitial lung disease (CVID-ILD, also referred to as GLILD) is generally considered a manifestation of systemic immune dysregulation occurring in up to 20% of people with CVID. There is a lack of evidence-based guidelines for the diagnosis and management of CVID-ILD.AimTo systematically review use of diagnostic tests for assessing patients with CVID for possible ILD, and to evaluate their utility and risks.MethodsEMBASE, MEDLINE, PubMed and Cochrane databases were searched. Papers reporting information on the diagnosis of ILD in patients with CVID were included.Results58 studies were included. Radiology was the investigation modality most commonly used. HRCT was the most reported test, as abnormal radiology often first raised suspicion of CVID-ILD. Lung biopsy was used in 42 (72%) of studies, and surgical lung biopsy had more conclusive results compared to trans-bronchial biopsy (TBB). Analysis of broncho-alveolar lavage was reported in 24 (41%) studies, primarily to exclude infection. Pulmonary function tests, most commonly gas transfer, were widely used. However, results varied from normal to severely impaired, typically with a restrictive pattern and reduced gas transfer.ConclusionConsensus diagnostic criteria are urgently required to support accurate assessment and monitoring in CVID-ILD. ESID and the ERS e-GLILDnet CRC have initiated a diagnostic and management guideline through international collaboration.Systematic review registrationhttps://www.crd.york.ac.uk/prospero/, identifier CRD42022276337.

Aim The aim of the study was to evaluate the response in lung function to different treatment regimens for common variable immunodeficiency patients with granulomatous lymphocytic interstitial lung disease (GLILD). Method A longitudinal retrospective cohort study was carried out. Patients were divided into three groups. To assess the response to different treatments, we compared baseline lung function with post-treatment tests. Results 14 patients with GLILD were included, seven of whom were treated with acute corticosteroids for a mean duration of 132±65 days. Spirometry results were unchanged, but there was a significant improvement in diffusing capacity of the lung for carbon monoxide (DLCO)% and transfer coefficient of the lung for carbon monoxide (KCO)% (median change in DLCO%=7%, p=0.04, and KCO%=13%, p=0.02). Relapse occurred in three out of seven patients. Five patients were treated with long-term mycophenolate mofetil (MMF) with/without corticosteroids for a mean duration of 1277±917 days. No changes were found in spirometry; however, there was a significant increase in DLCO% and KCO% (median change in each of DLCO% and KCO%=10%, p=0.04). Four patients on steroids with MMF successfully weaned the prednisone dose over 12 months. Four patients never received immunosuppression therapy. A significant decline was found in their lung function assessed over 7.5 years. The median reduction in the forced vital capacity (FVC)%, forced expiratory volume in 1 s (FEV1)% and DLCO% was 15%, 7% and 15%, equivalent to 2%, 1% and 2% per year, respectively. Conclusion Corticosteroids improve gas transfer in GLILD, but patients often relapse. The use of MMF was associated with long-term effectiveness in GLILD and permits weaning of corticosteroids. A delay in initiating and continuing maintenance treatment could lead to disease progression.

Abstract Background Long-COVID research to date focuses on outcomes in non-hospitalised vs. hospitalised survivors. However Emergency Department attendees (post-ED) presenting with acute COVID-19 may experience less supported recovery compared to people admitted and discharged from hospital (post-hospitalised group, PH). Objective We evaluated outcomes and predictors of specialty care referrals (SCR) in those with ongoing symptomatic Long-COVID, comparing post-ED and PH adults. Methods This prospective observational cohort study evaluates 800 PH and 484 post-ED adults from a single hospital in London, United Kingdom. Participants had either confirmed laboratory-positive SARS-CoV-2 infection or clinically suspected acute COVID-19 and were offered post-COVID clinical follow-up at approximately six weeks after their ED attendance or inpatient discharge, to assess ongoing symptoms and support recovery. Multiple logistic regression determined associations with specialist care referrals (SCR) to respiratory, cardiology, physiotherapy (including chest physiotherapy), and mental health services. Results Presence of at least one Long-COVID symptom was lower in adults attending ED services with acute COVID-19 compared to those hospitalised (70.1% post-ED vs. 79.5% PH adults, p

Objectives COVID-19 studies report on hospital admission outcomes across SARS-CoV-2 waves of infection but knowledge of the impact of SARS-CoV-2 variants on the development of Long COVID in hospital survivors is limited. We sought to investigate Long COVID outcomes, aiming to compare outcomes in adult hospitalised survivors with known variants of concern during our first and second UK COVID-19 waves, prior to widespread vaccination.Design Prospective observational cross-sectional study.Setting Secondary care tertiary hospital in the UK.Participants This study investigated Long COVID in 673 adults with laboratory-positive SARS-CoV-2 infection or clinically suspected COVID-19, 6 weeks after hospital discharge. We compared adults with wave 1 (wildtype variant, admitted from February to April 2020) and wave 2 patients (confirmed Alpha variant on viral sequencing (B.1.1.7), admitted from December 2020 to February 2021).Outcome measures Associations of Long COVID presence (one or more of 14 symptoms) and total number of Long COVID symptoms with SARS-CoV-2 variant were analysed using multiple logistic and Poisson regression, respectively.Results 322/400 (wave 1) and 248/273 (wave 2) patients completed follow-up. Predictors of increased total number of Long COVID symptoms included: pre-existing lung disease (adjusted count ratio (aCR)=1.26, 95% CI 1.07, 1.48) and more COVID-19 admission symptoms (aCR=1.07, 95% CI 1.02, 1.12). Weaker associations included increased length of inpatient stay (aCR=1.02, 95% CI 1.00, 1.03) and later review after discharge (aCR=1.00, 95% CI 1.00, 1.01). SARS-CoV-2 variant was not associated with Long COVID presence (OR=0.99, 95% CI 0.24, 4.20) or total number of symptoms (aCR=1.09, 95% CI 0.82, 1.44).Conclusions Patients with chronic lung disease or greater COVID-19 admission symptoms have higher Long COVID risk. SARS-CoV-2 variant was not predictive of Long COVID though in wave 2 we identified fewer admission symptoms, improved clinical trajectory and outcomes. Addressing modifiable factors such as length of stay and timepoint of clinical review following discharge may enable clinicians to move from Long COVID risk stratification towards improving its outcome.