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Hypersensitivity pneumonitis (HP) is an immune‐mediated interstitial lung disease (ILD) relating to specific occupational, environmental or medication exposures. Disease behaviour is influenced by the nature of exposure and the host response, with varying degrees of lung inflammation and fibrosis seen within individuals. The differentiation of HP from other ILDs is important due to distinct causes, pathophysiology, prognosis and management implications. This Thoracic Society of Australia and New Zealand (TSANZ) position statement aims to provide an up‐to‐date summary of the evidence for clinicians relating to the diagnosis and management of HP in adults, in the Australian and New Zealand context. This document highlights recent relevant findings and gaps in the literature for which further research is required. [ABSTRACT FROM AUTHOR]

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SUMMARY OF KEY POINTS: Air pollutants have various effects on human health in environmental and occupational settings.Air pollutants can be a risk factor for incidence, exacerbation/aggravation and death due to various lung diseases, including asthma, chronic obstructive pulmonary disease (COPD), hypersensitivity pneumonitis or pneumonia (HP), pulmonary fibrosis such as pneumoconiosis and malignant respiratory diseases such as lung cancer and malignant pleural mesothelioma.Environmental and occupational respiratory diseases are crucial clinical and social issues worldwide, although the burden of respiratory disease due to environmental and occupational causes varies depending on country/region, demographic variables, geographical location, industrial structure and socioeconomic situation.The correct recognition of environmental and occupational lung diseases and taking appropriate measures are essential to their effective prevention. [ABSTRACT FROM AUTHOR]

This article discusses the impact of hiatus hernia (HH) in patients with hypersensitivity pneumonitis (HP). The study found that about one-third of HP patients had HH, which is higher than the general population. However, the causal relationship between HH and lung fibrosis has not been established. The study also found that the presence and size of HH were not associated with body mass index (BMI) or the extent of fibrosis on CT scans. Additionally, there was no relationship between HH and baseline lung function. In patients with a usual interstitial pneumonia (UIP) pattern on CT, there was a trend towards poorer survival and lung function decline in those with HH. Consolidation, a CT marker of aspiration, was more prevalent in HP patients with larger HH. Further research is needed to understand the potential role of HH in the development of HP. [Extracted from the article]

Interstitial lung diseases (ILDs) are chronic conditions that significantly impact patients' quality of life, exercise capacity, and lifespan. Managing ILDs poses challenges across low-income, middle-income, and high-income countries due to various factors such as weather, air pollution, cultural beliefs, and income levels. High-income countries are perceived to have better resources for ILD care, but they still face challenges such as delays in access to healthcare and a lack of trained personnel. Middle-income and low-income countries struggle with limited healthcare infrastructure, high costs of diagnostic tests, delays in diagnosis, and inadequate rehabilitation and palliative care facilities. Disparities in health insurance coverage and access to medications also exist. Collaborative efforts, education, and research are needed to address these challenges and improve ILD management globally. [Extracted from the article]

Background and Objective: The acute‐phase protein C‐reactive protein (CRP) is known to be associated with poor outcomes in cancer and cardiovascular disease, but there is limited evidence of its prognostic implications in interstitial lung diseases (ILDs). We therefore set out to test whether baseline serum CRP levels are associated with mortality in four different ILDs. Methods: In this retrospective study, clinically measured CRP levels, as well as baseline demographics and lung function measures, were collected for ILD patients first presenting to the Royal Brompton Hospital between January 2010 and December 2019. Cox regression analysis was used to determine the relationship with 5‐year mortality. Results: Patients included in the study were: idiopathic pulmonary fibrosis (IPF) n = 422, fibrotic hypersensitivity pneumonitis (fHP) n = 233, rheumatoid arthritis associated ILD (RA‐ILD) n = 111 and Systemic Sclerosis associated ILD (SSc‐ILD) n = 86. Patients with a recent history of infection were excluded. Higher CRP levels were associated with shorter 5‐year survival in all four disease groups on both univariable analyses, and after adjusting for age, gender, smoking history, immunosuppressive therapy and baseline disease severity (IPF: HR (95% CI): 1.3 (1.1–1.5), p = 0.003, fHP: 1.5 (1.2–1.9), p = 0.001, RA‐ILD: 1.4 (1.1–1.84), p = 0.01 and SSc‐ILD: 2.7 (1.6–4.5), p < 0.001). Conclusion: Higher CRP levels are independently associated with reduced 5‐year survival in IPF, fHP, RA‐ILD and SSc‐ILD. [ABSTRACT FROM AUTHOR]

Histopathologic features typical for fHP are (1) chronic fibrosing interstitial pneumonia with fibrotic NSIP or UIP-like pattern, (2) airway-centred fibrosis with or without peribronchiolar metaplasia, (3) poorly formed non-necrotizing granulomas and (4) absence of features to suggest alternative diagnosis. Diagnosis of hypersensitivity pneumonitis, interstitial lung disease, pulmonary fibrosis, hypersensitivity pneumonitis In patients in whom a confident diagnosis of HP cannot be made despite multidisciplinary review of clinical, radiographic and BAL findings (typically in patients with indeterminate imaging pattern for HP), the diagnosis of HP can only be ascertained with histopathology features found on lung biopsy (Figure 1A). Keywords: diagnosis of hypersensitivity pneumonitis; hypersensitivity pneumonitis; interstitial lung disease; pulmonary fibrosis EN diagnosis of hypersensitivity pneumonitis hypersensitivity pneumonitis interstitial lung disease pulmonary fibrosis 599 602 4 06/19/23 20230701 NES 230701 Hypersensitivity pneumonitis (HP) is a complex form of interstitial lung disease (ILD) characterized by immune-mediated inflammation following an inciting antigen exposure in a susceptible individual.[1] Chronic exposure leads to progression of inflammation with evolution of fibrotic response in the lung and progressive pulmonary fibrosis (PPF). [Extracted from the article]

The article discusses the significance of pets in the lives of individuals, particularly those with respiratory illnesses. It highlights the emotional support and companionship that animals provide, emphasizing the importance of considering pets as beloved family members and vital social supports. The author shares personal experiences with their dog, Bertie, and recounts stories of patients whose pets played a crucial role in their lives, showcasing the need for holistic care that includes acknowledging the presence of pets in patients' lives. The article encourages healthcare providers to inquire about pets during patient assessments to enhance communication and strengthen therapeutic relationships. [Extracted from the article]

Background and Objective: Identification of an exposure is integral to the diagnosis, management, and prognostication of chronic hypersensitivity pneumonitis (CHP). Standardized questionnaires may aid in the identification of exposures, however, there currently are no evidence‐based patient‐validated questionnaires available. Key qualifiers (including duration and frequency) which indicate exposure relevance are also poorly defined. This study assessed the use of a standardized CHP exposure questionnaire in the identification of exposures and diagnostic confidence of CHP. Methods: People with a multi‐disciplinary meeting (MDM) diagnosis from five Australian interstitial lung disease (ILD) expert centres who provided informed consent were included. Participants completed a previously developed standardized CHP Exposure Questionnaire. Responses were collected with the participant's MDM data, including diagnosis, diagnostic confidence, and clinician‐elicited exposures. Results: One hundred thirty participants (IPF = 58, CHP = 24, CTD‐ILD = 17, unclassifiable = 19, other = 12) were included. In 33% of CHP participants, a standardized questionnaire elicited an exposure where the clinician did not. 63% of these had provisional low confidence CHP; and an exposure history would have increased the diagnostic confidence in these cases. Using the standardized questionnaire, 96% of CHP participants reporting any exposure, compared with 75% of non‐HP ILD participants. CHP participants were 3.5 times more likely (p = 0.004) to report their symptoms improved on avoidance, and 2.3 times more likely (p = 0.018) to report daily frequent exposure, compared with non‐HP ILDs. Conclusion: A standardized questionnaire which elicits exposure characteristics in addition to presence or absence of relevant exposures can increase the diagnostic confidence of CHP and reduce the proportion of antigen‐indeterminate CHP. This case–control study found that a standardized evidence‐based chronic hypersensitivity pneumonitis (CHP) exposure questionnaire increased the diagnostic confidence of CHP and reduced the proportion of antigen‐indeterminate CHP. See relatedEditorial [ABSTRACT FROM AUTHOR]

Background and Objective: Pulmonary hypertension is a life‐limiting complication of interstitial lung disease (ILD‐PH). We investigated whether treatment with phosphodiesterase 5 inhibitors (PDE5i) in patients with ILD‐PH was associated with improved survival. Methods: Consecutive incident patients with ILD‐PH and right heart catheterisation, echocardiography and spirometry data were followed from diagnosis to death, transplantation or censoring with all follow‐up and survival data modelled by Bayesian methods. Results: The diagnoses in 128 patients were idiopathic pulmonary fibrosis (n = 74, 58%), hypersensitivity pneumonitis (n = 17, 13%), non‐specific interstitial pneumonia (n = 12, 9%), undifferentiated ILD (n = 8, 6%) and other lung diseases (n = 17, 13%). Final outcomes were death (n = 106, 83%), transplantation (n = 9, 7%) and censoring (n = 13, 10%). Patients treated with PDE5i (n = 50, 39%) had higher mean pulmonary artery pressure (median 38 mm Hg [interquartile range, IQR: 34, 43] vs. 35 mm Hg [IQR: 31, 38], p = 0.07) and percentage predicted forced vital capacity (FVC; median 57% [IQR: 51, 73] vs. 52% [IQR: 45, 66], p=0.08) though differences did not reach significance. Patients treated with PDE5i survived longer than untreated patients (median 2.18 years [95% CI: 1.43, 3.04] vs. 0.94 years [0.69, 1.51], p = 0.003) independent of all other prognostic markers by Bayesian joint‐modelling (HR 0.39, 95% CI: 0.23, 0.59, p < 0.001) and propensity‐matched analyses (HR 0.38, 95% CI: 0.22, 0.58, p < 0.001). Survival difference with treatment was significantly larger if right ventricular function was normal, rather than abnormal, at presentation (+2.55 years, 95% CI: −0.03, +3.97 vs. +0.98 years, 95% CI: +0.47, +2.00, p = 0.04). Conclusion: PDE5i treatment in ILD‐PH should be investigated by a prospective randomized trial. [ABSTRACT FROM AUTHOR]

Background and objective: Rheumatoid arthritis (RA) is a frequent cause of interstitial lung disease (ILD); however, the impact of rheumatoid factor and anti‐citrullinated peptide antibody seropositivity in ILD without connective tissue disease (CTD) is unclear. We examined the association of seropositivity with ILD progression, mortality and response to immunosuppression in non‐CTD ILD. Methods: A total of 1570 non‐CTD patients (with idiopathic pulmonary fibrosis, hypersensitivity pneumonitis, interstitial pneumonia with autoimmune features or unclassifiable ILD) and 181 RA‐ILD patients were included from a prospective registry. Longitudinal forced vital capacity (FVC), transplant‐free survival and incidence of progressive fibrosing‐ILD (PF‐ILD) were compared between seronegative non‐CTD ILD (reference group), seropositive non‐CTD ILD and RA‐ILD using linear mixed‐effect and Cox proportional hazards models adjusted for age, sex, smoking pack‐years and baseline FVC. Interaction between seropositivity and immunosuppression on FVC decline was assessed in patients with ≥6 months of follow‐up before and after the treatment. Results: Two hundred and seventeen (13.8%) patients with seropositive non‐CTD ILD had similar rates of FVC decline and transplant‐free survival compared to seronegative non‐CTD ILD, but more frequently met the criteria for PF‐ILD (hazard ratio [HR] = 1.35, p = 0.004). RA‐ILD had slower FVC decline (p = 0.03), less PF‐ILD (HR = 0.75, p = 0.03) and lower likelihood of lung transplant or death (HR = 0.66, p = 0.01) compared to seronegative non‐CTD ILD. No interaction was found between seropositivity and treatment on FVC decline in non‐CTD ILD. Conclusion: Seropositivity in non‐CTD ILD was not associated with improved outcomes or treatment response, highlighting the importance of other disease features in determining prognosis and predicting response to immunosuppression. We show that rheumatoid factor and anti‐citrullinated peptide antibody are not associated with outcomes or response to immunosuppression in patients with interstitial lung diseases not due to connective tissue disease, indicating the need for future studies to test the prognostic impact of other clinical and radiological features that may predict response to therapy. See relatedEditorial [ABSTRACT FROM AUTHOR]

Keywords: clinical teaching; continued medical education; professional development; training programme EN clinical teaching continued medical education professional development training programme 6 8 3 12/19/22 20230101 NES 230101 GLO:FS9/01jan23:resp14428-gra-0001.jpg PHOTO (COLOR): . gl This quarterly editorial discussing I Respirology i articles of continuing educational value is the fourth in a series and the final covering publications from 2022. The intriguing study published by Fitzgerald et al. in the October edition of I Respirology i explores the dynamics of both the ipsilateral and contralateral hemidiaphragm in patients with unilateral effusions.[7] This is the first study to evaluate the contralateral hemidiaphragm in patients with pleural effusion requiring drainage. Https://onlinelibrary.wiley.com/doi/10.1111/resp.14316 Many patients presenting with pleural effusions related to acute disease such as pneumonia, pleural space infection and cardiac failure improve after appropriate treatment of the underlying condition.[4] However, some patients develop a recurrent or persistent fluid collection, especially in patients with underlying malignancy. [Extracted from the article]

Background and objective: A proportion of patients with fibrotic hypersensitivity pneumonitis (fHP) follow a progressive disease course despite immunosuppressive treatment. Little is known about predictors of mortality in fHP. We aimed to investigate the impact of short‐term lung function changes in fHP on mortality. Methods: Baseline demographics for 145 consecutive patients with a multi‐disciplinary team diagnosis of fHP, as well as baseline and 1‐year follow‐up of lung function, baseline echocardiographic findings, bronchoalveolar lavage (BAL) cellularity and all‐cause mortality were recorded. Changes in forced vital capacity (FVC) ≥ 5% and ≥10%, and diffusion capacity of the lung for carbon monoxide (DLCO) ≥ 10% and ≥15% at 1 year were calculated. Cox proportional hazards analysis was performed to test for associations with mortality. Results: Baseline lung function severity, age, presence of honeycombing on computed tomography (CT) and echocardiographic pulmonary arterial systolic pressure (PASP) ≥ 40 mm Hg were associated with early mortality, while BAL lymphocytosis was associated with improved survival. A decline in FVC ≥ 5% (hazard ratio [HR]: 3.10, 95% CI: 2.00–4.81, p < 0.001), FVC ≥ 10% (HR: 3.11, 95% CI: 1.94–4.99, p < 0.001), DLCO ≥ 10% (HR: 2.80, 95% CI: 1.78–4.42, p < 0.001) and DLCO ≥ 15% (HR: 2.92, 95% CI: 1.18–4.72, p < 0.001) at 1 year was associated with markedly reduced survival on univariable and multivariable analyses after correcting for demographic variables, disease severity, honeycombing on CT and treatment, as well as BAL lymphocytosis and PASP ≥ 40 mm Hg on echocardiography, in separate models. Conclusion: Worsening in FVC and DLCO at 1 year, including a marginal decline in FVC ≥ 5% and DLCO ≥ 10%, is predictive of markedly reduced survival in fHP. [ABSTRACT FROM AUTHOR]

Keywords: COLDICE; cryobiopsy; idiopathic pulmonary fibrosis; interstitial lung disease; telomeres EN COLDICE cryobiopsy idiopathic pulmonary fibrosis interstitial lung disease telomeres 756 763 8 06/22/20 20200701 NES 200701 Abbreviations CT computed tomography DL CO diffusion capacity of the lung for carbon monoxide FVC forced vital capacity ILA interstitial lung abnormality ILD interstitial lung disease IPF idiopathic pulmonary fibrosis MRCI Medication Regimen Complexity Index PrePF Preclinical pulmonary fibrosis SLB surgical lung biopsy TB tuberculosis TBLC transbronchial lung cryobiopsy Summary of key points Antifibrotic therapy has been proven to be effective in the treatment of progressive fibrosing lung diseases other than IPF. While the increased risk of sarcoidosis in patients with TB persisted throughout the 15-year follow-up period, the risk of developing TB in sarcoid patients was increased only in the first year after diagnosis. Silicosis, a severe occupational lung disease, is sometimes misdiagnosed as sarcoidosis due to imaging similarities, including centrilobular nodularity, bilateral airspace opacification (albeit unlike sarcoid often lower predominant) and calcified lymphadenopathy.[23] There remain no effective treatment options for this entirely avoidable lung disease, now a source of growing international concern.[24] IMPACT OF ILD ON QUALITY OF LIFE Key Points Patients continue to report deficiencies in the quality of their care with specific reference to provision of information, drug side effect management and access to specialist care. Polypharmacy is a common burden in patients with ILD, with the greatest increase seen in IPF patients following initiation of antifibrotic therapy. [Extracted from the article]

Background and objective: Acute exacerbation (AE) is a severe complication of idiopathic pulmonary fibrosis (AE‐IPF). In 2016, an international working group revised its definition and diagnostic criteria; however, few studies have assessed the frequency and prognosis of AE in patients with other fibrotic interstitial lung diseases (FILD). Methods: We used data from 1019 consecutive interstitial lung disease (ILD) patients initially evaluated between January 2008 and July 2015. All subject diagnoses were made by multidisciplinary discussion in December 2018. ILD was categorized as IPF (n = 462) and other FILD which included non‐specific interstitial pneumonia (n = 22), chronic hypersensitivity pneumonitis (n = 29), connective tissue disease‐associated ILD (n = 205) and unclassifiable ILD (n = 209). Using the 2016 definition of AE‐IPF, we identified all subjects with an AE. Results: During the observational period, 193 patients experienced a first AE (AE‐FILD n = 69, AE‐IPF n = 124). The time to first AE was significantly longer in FILD than IPF (log‐rank test, P < 0.001). After adjusting for potentially influential confounders, FILD remained a significant predictor of longer time to first AE compared with IPF (hazard ratio: 0.453; 95% CI: 0.317–0.647, P = 0.006). In a multivariate Cox proportional analysis, baseline disease severity was closely associated with the incidence of AE‐ILD. Even after adjustment for other clinical variables, AE had a negative impact on overall survival. AE‐FILD and AE‐IPF showed similar poor short‐term outcomes. Conclusion: All forms of ILD are at risk of AE and have a similar outcome to AE‐IPF. [ABSTRACT FROM AUTHOR]

Background and objective: Probe‐based confocal laser endomicroscopy (pCLE) enables in vivo microimaging of the distal lung, during bronchoscopy. This study aims at identifying pCLE descriptors of chronic interstitial lung diseases (ILD), their correlations with chest HRCT and assessing inter‐observer agreement. Methods: pCLE was performed in 21 healthy volunteers (HV) and 59 non‐smoking ILD patients, including 19 patients with idiopathic pulmonary fibrosis (IPF) or asbestosis, 15 with connective tissue disease‐associated ILD (CTD‐ILD), 17 with sarcoidosis and 8 with hypersensitivity pneumonitis (HP). pCLE descriptors were identified in ILD on the basis of comparison with HV. Results: Nine pCLE descriptors were more frequent in ILD compared to HV, with good inter‐observer agreement, including fluorescent bronchiolar cells (sarcoidosis, CTD‐ILD and HP), fluorescent alveolar cells (CTD‐ILD and HP), small alveolar entrance rings (IPF or asbestosis and CTD‐ILD), enlarged axial elastic fibres (IPF or asbestosis), septal fibres (IPF or asbestosis, CTD‐ILD and HP), disorganized acinar network and rigid acinar network (IPF or asbestosis and CTD‐ILD), dense elastic network (IPF or asbestosis) and alveolar fluorescent nodular structures (in sarcoidosis) (P < 0.01, Fisher's exact test, all comparisons). The distribution of nodules on computed tomography (CT) appeared to correlate with pCLE alveolar nodular structures, rigid acinar network and septal fibres, while reticulations were associated with septal fibres and disorganized or dense acinar network; ground‐glass opacities on CT with small alveolar entrances, rigid elastic network and septal fibres; and honeycombing with septal fibres. Conclusion: In the four groups of ILD studied, 9 pCLE descriptors are described, which appear specific and reproducible, and correlate with chest HRCT patterns. [ABSTRACT FROM AUTHOR]

Background and objective: Patients with interstitial lung disease (ILD) are often prescribed disease‐targeted and symptomatic therapies, both of which can cause significant treatment burden due to polypharmacy and drug–disease interactions. This study aimed to evaluate medication regimen complexity before and after introduction of ILD‐specific therapies. Potential drug–disease interactions were evaluated for patients who were prescribed prednisolone. Methods: In this study, 214 patients with ILD were assessed for demographic information, co‐morbidities and medication use. Medication lists were reviewed prior to and after the introduction of ILD‐specific therapies. Complexity of treatment regimen was examined using the validated Medication Regimen Complexity Index (MRCI). Results: Of the 214 patients, 75 had idiopathic pulmonary fibrosis (IPF) while the rest had inflammatory ILD (chronic hypersensitivity pneumonitis: 45; connective tissue disease‐related ILD: 41). Polypharmacy was common at baseline (IPF: 51%, inflammatory ILD: 63%). Following introduction of ILD‐specific therapies, median total MRCI scores significantly increased from 8 (interquartile range (IQR) = 8–15) to 22.5 (17.5–27.5) and 14.5 (8.5–21) to 21.5 (16–30) for IPF and inflammatory ILD groups, respectively (P < 0.0001 for both). Complex dosing instructions contributed the most to total MRCI scores for ILD‐specific therapies. Among patients receiving prednisolone (n = 113), 88% had ≥1 co‐morbidity which may be impacted. Common co‐morbidities included gastrointestinal diseases (56%), obesity (37%), osteoporosis (24%) and diabetes mellitus (18%). Conclusion: Polypharmacy and complex medication regimen are common in patients with ILD of different aetiologies. There is a high frequency of potential drug–disease interactions among patients who are prescribed systemic corticosteroids. These findings highlight the need for careful evaluation of the impact of therapeutic complexity and burden in patients with ILD. [ABSTRACT FROM AUTHOR]

Keywords: home exposures; interstitial lung disease; video consultation EN home exposures interstitial lung disease video consultation 401 402 2 04/04/23 20230401 NES 230401 During the pandemic, video consultations were adopted as an alternative to hospital face-to-face consultations in the routine follow-up of patients across a variety of specialties, including interstitial lung diseases. One video consult was with a long-standing patient with fibrotic hypersensitivity pneumonitis (HP) without an identified exposure, whose breathlessness and lung function had been worsening despite treatment. Having the opportunity to perform a video consultation with the patient in their home or work environment may provide insights, however serendipitous, into potential exposures and allow informed discussions and patient education on antigen avoidance. [Extracted from the article]

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Background and objective: Although idiopathic pulmonary fibrosis (IPF) patients experience a worse survival compared with chronic hypersensitivity pneumonitis (CHP), organic dust exposure is a known risk factor for both IPF and CHP. Methods: We divided patients diagnosed with IPF, based on their exposure to moulds/birds (absent: group A; present: group B). We retrospectively compared pulmonary function and survival between groups A and B, and a separate CHP cohort (group C). Results: A total of 293 patients were included (group A: n = 171, group B: n = 73, group C: n = 49). Demographics and baseline pulmonary function did not differ between groups A and B, but significant differences were seen between groups B and C. Median survival of group B was 84 months, which was longer than group A (43 months, P = 0.002), but lower than group C (157 months, P = 0.04), in both univariate and multivariate analyses. Antifibrotic treatment resulted in a better outcome in group A (hazard ratio (HR): 0.44) and group B (HR: 0.12) without interaction between exposure and antifibrotic use (P = 0.20). Forced vital capacity (FVC) decline was not associated with mould/bird exposure in this cohort. Conclusion: Group B patients experienced a better outcome compared with (non‐exposed) IPF patients, although worse compared with CHP patients. Antifibrotic treatment in group B resulted in a similar beneficial effect compared with group A. Further research is needed to ascertain the diagnostic designation in this exposed usual interstitial pneumonia (UIP) patient group without other CHP features. Usual interstitial pneumonia (UIP) patients exposed to moulds or birds without other features of chronic hypersensitivity pneumonitis (CHP) experience a better survival compared with non‐exposed idiopathic pulmonary fibrosis (IPF) patients, although still worse compared with definite CHP patients. A similar beneficial effect from antifibrotics was seen irrespective of mould/bird exposure. [ABSTRACT FROM AUTHOR]

GLO:FS9/01dec22:resp14396-fig-0001.jpg PHOTO (COLOR): 1 The main ridge of the Low Tatras, Panska Hola (Source: Eva Tedlova) gl The World Health Organization (WHO) declared a global health emergency in connection with the increasing worldwide number of COVID-19 positive cases on 30 January 2020 and subsequently declared a pandemic on 11 March 2020. COVID-19, diffuse parenchymal diseases, tuberculosis, Slovakia Keywords: COVID-19; diffuse parenchymal diseases; Slovakia; tuberculosis EN COVID-19 diffuse parenchymal diseases Slovakia tuberculosis 1093 1094 2 11/21/22 20221201 NES 221201 Slovakia, officially the Slovak Republic, is a small country in central Europe with 5.5 million inhabitants. [Extracted from the article]

ABSTRACT Background and objective To determine whether computer-based quantification ( CALIPER software) is superior to visual computed tomography ( CT) scoring in the identification of CT patterns indicative of restrictive and obstructive functional indices in hypersensitivity pneumonitis ( HP). Methods A total of 135 consecutive HP patients had CT parenchymal patterns evaluated quantitatively by both visual scoring and CALIPER. Results were evaluated against: forced vital capacity ( FVC), total lung capacity ( TLC), diffusing capacity for carbon monoxide ( DLCO) and a composite physiological index ( CPI) to identify which CT scoring method better correlated with functional indices. Results CALIPER-derived scores of total interstitial lung disease extent correlated more strongly than visual scores: FVC ( CALIPER R = 0.73, visual R = 0.51); DLCO ( CALIPER R = 0.61, visual R = 0.48); and CPI ( CALIPER R = 0·70, visual R = 0·55). The CT variable that correlated most strongly with restrictive functional indices was CALIPER pulmonary vessel volume ( PVV): FVC R = 0.75, DLCO R = 0.68 and CPI R = 0.76. Ground-glass opacity quantified by CALIPER alone demonstrated strong associations with restrictive functional indices: CALIPER FVC R = 0.65; DLCO R = 0.59; CPI R = 0.64; and visual = not significant. Decreased attenuation lung quantified by CALIPER was a better morphological measure of obstructive lung disease than equivalent visual scores as judged by relationships with TLC ( CALIPER R = 0.63 and visual R = 0.12). All results were maintained on multivariate analysis. Conclusion CALIPER improved on visual scoring in HP as judged by restrictive and obstructive functional correlations. Decreased attenuation regions of the lung quantified by CALIPER demonstrated better linkages to obstructive lung physiology than visually quantified CT scores. A novel CALIPER variable, the PVV, demonstrated the strongest linkages with restrictive functional indices and could represent a new automated index of disease severity in HP. [ABSTRACT FROM AUTHOR]

ABSTRACT Background and objective Lung biopsies from patients with hypersensitivity pneumonitis ( HP) have demonstrated small airway ( SA) involvement, but there is no information concerning SA function in HP, and it is unknown whether pharmacological treatment could modify its function. SA function in patients with chronic HP using ultrasonic pneumography ( UPG) and impulse oscillometry ( IOS) was explored. We also compared initial results with those obtained after 4 weeks of standardized treatment with azathioprine and prednisone. Methods The study group consisted of adults with recent diagnoses of HP. All patients completed UPG, IOS, spirometry, body plethysmography, single-breath carbon monoxide diffusing capacity ( DLCO) and the 6-min walk test ( 6MWT). The fraction of exhaled nitric oxide ( FENO) was obtained to assess eosinophilic airway inflammation. Measurements were taken at diagnosis and after 4 weeks of treatment. Results A total of 20 consecutive patients (16 women) with chronic HP participated in the study. Median age was 50 years (interquartile range ( IQR): 42-54). At diagnosis, the UPG phase 3 slope was abnormally high, consistent with maldistribution of ventilation. For IOS, all patients had low reactance at 5 Hz ( X5) and elevated reactance area ( AX) reflecting low compliance, and only eight (40%) patients had elevated R5 (resistance at 5 Hz (total)) and R5-20 (resistance at 5 Hz-resistance at 20 Hz (peripheral)) attributed to SA resistance. In contrast, FENO parameters were within normal limits. After treatment, forced vital capacity ( FVC), the 6-min walk distance and the distribution of ventilation showed significant improvement, although DLCO did not. Conclusion Patients with chronic HP have SA abnormalities that are partially revealed by the UPG and IOS tests. Lung volumes, but not gas exchange, improved after treatment with azathioprine and prednisone. [ABSTRACT FROM AUTHOR]

ABSTRACT Background and objective The objectives of this study were to determine the prevalence and characteristics of cough in idiopathic pulmonary fibrosis ( IPF), chronic hypersensitivity pneumonitis ( HP) and systemic sclerosis-associated interstitial lung disease ( SSc-ILD). Methods Cough severity was measured in consecutive patients with IPF ( n = 77), HP ( n = 32) and SSc-ILD ( n = 67) using a 10-cm visual analogue scale ( VAS). Dyspnoea and quality of life were measured using established questionnaires. Cough severity was compared across ILD subtypes and predictors of cough severity were determined using multivariate analysis. Results Cough was more common in IPF and chronic HP compared to SSc-ILD (87% and 83% vs 68%, P = 0.02). The median (interquartile range) VAS score was 39 (17-65) in the IPF cohort, 29 (11-48) in HP and 18 (0-33) in SSc-ILD ( P < 0.0001). Cough was more often productive in chronic HP and IPF (63% and 43% vs 21%, P < 0.001). Cough severity was independently predicted only by ILD diagnosis and higher dyspnoea score. Cough severity was not associated with other common causes of cough. Cough was a significant predictor of quality of life in IPF and SSc-ILD with adjustment for age, sex, dyspnoea and ILD severity; however, cough was not associated with quality of life in chronic HP. Conclusion Cough is more frequent, more severe and more often productive in IPF and chronic HP compared to SSc-ILD, despite similar ILD severity in these cohorts. Cough severity is strongly and independently associated with dyspnoea and pulmonary function, and is a significant contributor to reduced quality of life in both IPF and SSc-ILD. [ABSTRACT FROM AUTHOR]

ABSTRACT Background and objective Lobar and temporal histological variability in chronic bird-related hypersensitivity pneumonitis ( BRHP) has not been clearly elucidated. This study was designed to evaluate the spatio-temporal histopathological variability in chronic BRHP. Methods Fifty-two patients with chronic BRHP who underwent a surgical lung biopsy ( SLB) between 1992 and 2008 were evaluated. The histopathological characteristics of the lung biopsy specimens were classified by the 2002 American Thoracic Society/European Respiratory Society ( ATS/ ERS) consensus classification of idiopathic interstitial pneumonias ( IIPs). Autopsy specimens from seven patients were also evaluated to examine the serial changes from SLB to autopsy. Results In a study of lobar histological variability based on the findings of SLB, 7 patients were diagnosed with cellular nonspecific interstitial pneumonia ( NSIP) pattern, 16 with fibrotic NSIP pattern, 20 with fibrotic NSIP pattern and usual interstitial pneumonia ( UIP) (discordant UIP) pattern and 9 with UIP (concordant UIP) pattern. In a study of sequential changes, specimens of SLBs with fibrotic NSIP pattern changed to a bronchiolocentric interstitial pneumonia ( BIP) pattern or UIP pattern. Conclusion Interlobar and intralobar histological variability is present in chronic BRHP. In several patients with chronic BRHP, a fibrotic NSIP pattern may be an early lesion that progresses to a UIP pattern. [ABSTRACT FROM AUTHOR]

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Idiopathic pulmonary fibrosis ( IPF) is an incurable condition that is characterized by progressive pulmonary fibrosis, architectural distortion of the lung and loss of gas exchange units. Until recently, there was no effective treatment for this condition. However, there were two landmark trials published earlier this year, which have changed the management of this condition. Pirfenidone ( Assessment of Pirfenidone to Confirm Efficacy and Safety in Idiopathic Pulmonary Fibrosis trial) and nintedanib ( Efficacy and Safety of Nintedanib in Idiopathic Pulmonary Fibrosis-1 and -2 trials) have both demonstrated positive outcomes in patients with IPF. In this perspective, we critically discuss the role of these agents in IPF and in the broader pulmonary fibrosis population. [ABSTRACT FROM AUTHOR]

The extents of fibrosis and GGA were scored between 0% and 100% with increments of 5% on HRCT separately on the right and left at 5 sections. Asymmetric ratio was calculated as | (Right (Fibrosis + GGA) - Left (Fibrosis + GGA) | / | (Right (Fibrosis + GGA) + Left (Fibrosis + GGA) |. 1 Tokyo Medical and Dental University, Japan, 2 National Hospital Organization Disaster Medic... B Background and Aims b : In patients with fibrotic hypersensitivity pneumonitis (FHP), high resolution computed tomography (HRCT) sometimes shows laterality of fibrosis and ground glass attenuation (GGA). [Extracted from the article]

Corticosteroids alone or with Immunosuppressant are administered for the treatment when antigen avoidance is not sufficient to control disease progression. Changes in pulmonary function and laboratory parameters, frequency of acute exacerbations, and adverse events were compared between the patients treated with corticosteroids combined with tacrolimus and those with corticosteroids alone. 1 Tokyo Medical Dental University, Japan B Background and Aims b : Fibrotic hypersensitivity pneumonitis (fHP) is an allergic interstitial lung disease characterized by progressive lung fibrosis with poor prognosis. [Extracted from the article]

Background and objective The aim of this study is to compare the inflammatory profile before and after specific inhalation challenge ( SIC) in induced sputum from patients with hypersensitivity pneumonitis ( HP) and to investigate whether different causal antigens define the resulting profile. Methods A prospective study was conducted in 27 patients with HP: 15 patients due to exposure to birds ( BHP) and 12 due to exposure to fungi ( FHP), confirmed by SIC. Induced sputum was obtained before and/or 24 h after SIC. Cell types were determined by differential cell count using optical microscopy. Interferon-γ, interleukin ( IL)-12p70, IL-2, IL-10, IL-8, IL-6, IL-4, IL-5, IL-1β, tumour necrosis factor ( TNF)-α and TNF-β levels were measured in the supernatants. Results Following SIC, higher sputum neutrophilia levels ( P = 0.048) and an increase in IL-8 levels ( P = 0.017) were found in patients with FHP than in those with BHP. FHP patients also showed increased IL-1β, IL12-p70 and IL5 levels ( P = 0.011, P = 0.036 and P = 0.018, respectively) after SIC. In BHP, a trend towards increases in sputum eosinophils and TH2 cytokines ( IL4, IL5) was seen following SIC ( P = 0.059, P = 0.068 and P = 0.075 respectively). Conclusions This study shows that bronchial inflammation is present in patients with HP evidenced by increases in sputum neutrophils and eosinophils following exposure to the offending antigen during SIC. [ABSTRACT FROM AUTHOR]

Background and objective Natural killer ( NK) cells appear to be involved in the development of interstitial lung diseases ( ILD). The purpose of this study was to investigate the involvement of NK and natural killer T ( NKT)-like cells in two recognized cytotoxic ILD with systemic character, hypersensitivity pneumonitis ( HP) and cryptogenic organizing pneumonia ( COP), compared with idiopathic pulmonary fibrosis ( IPF) and controls. Methods Bronchoalveolar lavage fluid ( BALF) and peripheral blood ( PBL) cells and lymphocyte subsets of 83 patients (26 with COP, 19 with HP and 38 with IPF) and 10 controls were prospectively studied by flow cytometry. Results The percentage of NK and NKT-like cells was lower in BALF than in PBL in all patient groups and controls. Patients with COP presented with statistically significantly higher NK and NKT-like cell counts in BALF compared with controls ( P = 0.044 and P = 0.05 respectively) and IPF ( P = 0.049 and P = 0.045 respectively). BALF NKT-like cell count correlated with PBL NKT-like cell count only in COP (r = 0.627, P = 0.002). In addition, a significant positive correlation between BALF NKT-like cell and PBL cytotoxic T CD8+ cell count was observed in COP (r = 0.562, P = 0.006) but not in HP, IPF or controls. Conclusions Our study provides for the first time evidence for the implication of NKT-like cells in the pathogenesis of COP, as part of both localized and systemic cytotoxicity. [ABSTRACT FROM AUTHOR]

Humidifier lung is a phenotype of hypersensitivity pneumonitis (HP) caused by the inhalation of humidifier vapours. Granulomas in histopathological findings were significantly less common in humidifier lung patients than in summer-type HP patients ( I n i = 0 (0.0%) vs I n i = 9 (56.3%), I P i = 0.045). Serum KL-6 levels were significantly lower in humidifier lung patients than in summer-type HP patients (median (IQR): 316 (303-587 U/mL) vs 1690 U/mL (1093-3385 U/mL), I P i < 0.001). [Extracted from the article]

The aim of the present study was to report the features of five patients with concurrent histopathological features of pulmonary alveolar proteinosis (PAP) and hypersensitivity pneumonitis (HP) and their high-resolution CT (HRCT) appearances. Patients with histopathological features of both HP and PAP on surgical lung biopsy referred for tertiary review were retrospectively identified. The pathology and HRCT images were semi-quantitatively scored to evaluate the relative contribution to HP and PAP. Five patients had histopathological features of HP and PAP but had varied HRCT appearances. All had imaging features of PAP to a varying degree with two patients also showing characteristics of HP but three patients had ill-defined thickened interlobular septa, not typical of either disease. We describe the coexistence of PAP and HP in five patients and discuss possible linkages between these two distinct pathologies. Alveolar proteinosis and hypersensitivity pneumonitis are two distinct diseases. We describe five patients with histopathogical features of both on surgical lung biopsy, their clinical information and the HRCT appearances. Possible associations between the two disorders are also discussed. [ABSTRACT FROM AUTHOR]

A 55-year-old man developed progressive cough and dyspnoea after regular attendance at a public steam bath. Hypoxaemia, diffuse pulmonary infiltrates and a predominance of lymphocytes with an increased percentage of CD8+ T cells in his bronchoalveolar lavage fluid suggested hypersensitivity pneumonitis. Microbial cultures from the steam bath room and tank identified Exophiala jeanselmei. Immunoblotting assays from the patient's serum confirmed the major antigenic stimulus. The patient recovered fully after systemic corticosteroid treatment and cessation of further exposure. [ABSTRACT FROM AUTHOR]

Background and objective: Obstructive bronchiolar disease or constrictive bronchiolitis, particularly in non-transplant patients, is poorly understood. This study identified the associated diseases, presenting features, and clinical course of obstructive bronchiolar disease identified by CT in the non-transplant adult population. Methods: Retrospective single-centre study of 29 consecutive patients clinically diagnosed to have an obstructive bronchiolar disease based on the presence of respiratory symptoms and abnormal CT findings consisting of mosaic perfusion pattern with air trapping. Results: The median age was 54 years (range, 25–80 years); 20 were women (69%) and four patients (14%) had a smoking history. All 29 patients presented with respiratory symptoms, predominantly dyspnoea. The most common cause of obstructive bronchiolar disease was rheumatoid arthritis (34%). Other causes included hypersensitivity pneumonitis, multiple carcinoid tumorlets, Sjögren's syndrome, paraneoplastic pemphigus, inflammatory bowel disease and Swyer–James syndrome. The underlying cause was not identifiable in nine patients (31%), that is, cryptogenic constrictive bronchiolitis. An obstructive pattern was seen on pulmonary function testing in most patients (86%) with the exception of those with hypersensitivity pneumonitis and extreme obesity. Management usually included corticosteroid therapy, inhaled and oral, and bronchodilator therapy. Additional medications included macrolides, cytotoxic agents and other immunomodulator therapy. Pharmacologic therapy did not provide improvement in pulmonary function in the majority of patients but the follow-up data were limited. Conclusions: Diverse causes and underlying diseases are associated with obstructive bronchiolar disease diagnosed radiologically in the non-transplant adult population. Rheumatoid arthritis-associated and cryptogenic constrictive bronchiolitis are found in over one-half of these patients. Most patients with obstructive bronchiolar disease do not appear to improve with currently available therapy. [ABSTRACT FROM AUTHOR]

Keywords: antifibrotic therapy; interstitial lung disease; pulmonary fibrosis EN antifibrotic therapy interstitial lung disease pulmonary fibrosis 1231 1232 2 11/18/20 20201201 NES 201201 Interstitial lung diseases (ILD) encompass a heterogeneous group of inflammatory and fibro-proliferative disorders of varying aetiologies.1 Amongst these, idiopathic pulmonary fibrosis (IPF) is the most studied and archetypal fibrosing ILD.2 It is now recognized that a significant proportion of patients with other fibrosing ILD manifest a progressive phenotype, commonly termed as progressive fibrosing ILD (PF-ILD), with a similar disease course and underlying pathophysiology to IPF.1 The emergence of antifibrotic therapies has transformed the landscape of IPF management, with established efficacy in slowing disease progression, reducing acute exacerbation rates and improving survival.2 Similar therapeutic benefits have been shown in recent clinical trials of antifibrotic therapies in PF-ILD populations.3,4 While there is increasing clinical and research interest in PF-ILD, many questions remain unanswered. Using a prospective ILD registry of 346 well-characterized patients with multidisciplinary diagnosis, we explored the patient characteristics and potential healthcare implications of extending antifibrotic therapies to the PF-ILD cohort. While there is currently minimal real-world experience on the utilization of antifibrotic therapies in PF-ILD, our local experience with IPF suggests that 32.3% ( I n i = 21/65) of eligible patients proceeded to antifibrotic therapies. [Extracted from the article]

Extrinsic allergic alveolitis (also known as hypersensitivity pneumonitis) is caused by repeated inhalation of mainly organic antigens by sensitized subjects. This induces a hypersensitivity response in the distal bronchioles and alveoli and subjects may present clinically with a variety of symptoms. The aims of this review are to describe the current concepts of the immunological response, the diverse clinical presentation of this disease, the relevant investigations and management, and areas for future studies. [ABSTRACT FROM AUTHOR]

A 51-year-old housewife with hypersensitivity pneumonitis caused by Humicola fuscoatra is reported. The diagnosis was made by an inhalation challenge with H. fuscoatra antigen. She was admitted for diagnosis and treatment of a fever and productive cough. Auscultation of her lungs revealed inspiratory fine crackles. Her chest CT showed diffuse miliary nodules in a centri-lobular distribution with patchy ground glass opacities. Findings of transbronchial lung biopsy and BAL fluid were compatible with a hypersensitivity pneumonitis. Her symptoms worsened on returning home, which suggested the existence of some aetiological agent in the subject's house. H. fuscoatra , Penicillium decumbens and Aspergillus versicolor were isolated from a number of rooms. High titres of serum anti H. fuscoatra , P. decumbens and A. versicolor were detected. Inhalation challenge tests with both P. decumbens and A. versicolor antigen were negative, in contrast to that with H. fuscoatra which was positive. Based on these results, we advised the patient to cleanse her entire house. Since cleaning, her symptoms have not worsened upon returning home. This is the first report of hypersensitivity pneumonitis caused by H. fuscoatra antigen. [ABSTRACT FROM AUTHOR]

Objective: Only limited information exists concerning the clinical and pathological features of chronic hypersensitivity pneumonitis (HP) in Japan and elsewhere. We present data on clinicopathological features of chronic HP obtained through a Japanese nationwide survey. Methodology: We studied the clinical and pathological findings in 10 patients with chronic HP who underwent surgical lung biopsy or postmortem examination. Results: There were three types of clinical course: six of the 10 patients had persistent symptoms followed by repeated acute episodes; two showed a subacute onset with persistent symptoms; and two exhibited an insidious onset. Five patients made no attempt to avoid antigen exposure and they all had progressive disease. Pathological findings indicated that lesions were mainly centrilobular with or without epithelioid cell granulomas in specimens obtained during the acute or subacute stage. In contrast, most patients in the chronic stage predominantly showed interstitial fibrosis with a usual interstitial pneumonia pattern. Conclusions: The pathological findings of chronic HP depend on the stage of the disease at tissue sampling. [ABSTRACT FROM AUTHOR]

1 Toho University Omori Medical Center, Japan, 2 Fukujuji Hospital, Japan, 3 Toho University,... B Background and Aims b : Humidifier lung is a rare form of hypersensitivity pneumonitis (HP), and its clinico-radiological and pathological features are unclear. We compared clinical features, laboratory data, CT images, bronchoalveolar lavage fluid (BALF) and histopathological findings of these patients with those of 19 patients with summer-type HP (mean age, 57.4 years). Transbronchial biopsy specimens from patients with humidifier lung revealed alveolar septal wall thickening with lymphocyte infiltration in all patients. [Extracted from the article]

In farmer's lung, the patients were identified in the northern part of Japan, but seasonality was not found. B Results b : In total, 3982 patients were hospitalized with HP for 8 years, including summer-type HP (SHP) (n = 506), bird fancier's lung (BFL) (n = 220), ventilation pneumonitis (n = 124), farmer's lung (n = 53), and unspecified HP (n = 3049). 1 Tokyo Medical and Dental University, Japan B Background b : Although several epidemiological studies have been shown in Japan, their sample sizes were small for showing actual clinical situation. [Extracted from the article]

The diagnoses included fibrotic hypersensitivity pneumonitis (4 cases), non-fibrotic hypersensitivity pneumonitis (4 cases), Idiopathic non-specific interstitial pneumonia (4 cases), connective tissue disease-interstitial lung disease (3 case), Idiopathic Pulmonary Fibrosis (2 cases), and the like. 1 Tokyo Medical and Dental University, Japan B Background and Aims b : Multidisciplinary discussion (MDD) is considered as the standard diagnostic tool for interstitial lung diseases (ILDs). P16-45: Efficacy of multidisciplinary discussion (MDD) with transbronchial lung cryobiopsy. [Extracted from the article]

A CASE OF SUMMER-TYPE HYPERSENSITIVITY PNEUMONITIS WITH SEVERE OBSTRUCTIVE IMPAIRMENT CAUSED BY MEMBRANOUS BRONCHIOLITIS Hypersensitivity pneumonitis (Hp) is an extrinsic allergic disease induced by organic dust, and generally shows restrictive impairment in lung function. His lung function test on the fourth day showed severe obstructive impairment (%FEV1 27.6%, FEV1% 49.6%) without the decrease in DLCO (%DLCO 80.6%). [Extracted from the article]