Pathological features of connective tissue disease-associated interstitial lung disease in transbronchial cryobiopsies.

Transbronchial cryobiopsies are increasingly used for the diagnosis of interstitial lung disease (ILD), but there is a lack of published information on the features of specific ILD in cryobiopsies. Here we attempt to provide pathological guidelines for separating usual interstitial pneumonia (UIP) of idiopathic pulmonary fibrosis (IPF), fibrotic hypersensitivity pneumonitis (FHP) and connective tissue disease-associated ILD (CTD-ILD) in cryobiopsies. We examined 120 cryobiopsies from patients with multidisciplinary discussion (MDD)-established CTD-ILD and compared them to a prior series of 121 biopsies from patients with MDD-established IPF or FHP. A non-specific interstitial pneumonia (NSIP) pattern alone was seen in 36 of 120 (30%) CTD-ILD, three of 83 (3.6%) FHP and two of 38 (5.2%) IPF cases, statistically favouring a diagnosis of CTD-ILD. The combination of NSIP + OP was present in 29 of 120 (24%) CTD-ILD, two of 83 (2.4%) FHP and none of 38 (0%) IPF cases, favouring a diagnosis of CTD-ILD. A UIP pattern, defined as fibroblast foci plus any of patchy old fibrosis/fibrosis with architectural distortion/honeycombing, was identified in 28 of 120 (23%) CTD-ILD, 45 of 83 (54%) FHP and 27 of 38 (71%) IPF cases and supported a diagnosis of FHP or IPF. The number of lymphoid aggregates/mm2 and fibroblast foci/mm2 was not different in IPF, CTD-ILD or FHP cases with a UIP pattern. Interstitial giant cells supported a diagnosis of FHP or CTD-ILD over IPF, but were infrequent. In the correct clinical/radiological context the pathological findings of NSIP, and particularly NSIP plus OP, favour a diagnosis of CTD-ILD in a cryobiopsy, but CTD-ILD with a UIP pattern, FHP with a UIP pattern and IPF generally cannot be distinguished.

Churg A ，Poletti V ，Ravaglia C ，Matej R ，Vasakova MK ，Hornychova H ，Stewart B ，Patel D ，Duarte E ，Gomez Manjarres DC ，Mehta HJ ，Vaszar LT ，Tazelaar H ，Wright JL ... -  《-》

Location of Fibroblastic Foci: Does the Lesion You Observe Really Suggest Usual Interstitial Pneumonia?

Fibroblastic foci (FF) are considered important findings of usual interstitial pneumonia (UIP); however, they are not only specific to UIP but also observed in various fibrotic interstitial lung diseases (ILDs). Previous studies have reported the significance of FF comparing UIP with nonspecific interstitial pneumonia (NSIP) or secondary interstitial pneumonia, such as collagen vascular disease-related ILD (CVD-ILD) or fibrotic hypersensitivity pneumonitis (FHP). However, only few studies have mentioned their location, and no reports have shown significant results regarding their location. This study aimed to compare the spatial distribution of FF across various forms of ILDs, based on anatomical location. Among patients who underwent lung transplantation at Kyoto University Hospital between April 1, 2008, and March 31, 2023, those diagnosed with idiopathic pulmonary fibrosis (IPF) (n = 24), idiopathic NSIP (n = 11), CVD-ILD (n = 36), and FHP (n = 12) were included, and 744 slides were obtained. FF were classified into 4 categories: peripheral, such as subpleural/paraseptal; intralobular, along the alveolar wall (aFF); centrilobular (cFF); and distorted or dense fibrotic lesions. The number of total and each location's FF/cm2 were counted, and the percentage of each location's FF was calculated. IPF showed more total FF and peripheral FF than NSIP. FHP had more cFF than CVD (P = .026) and NSIP (P = .018). The dFF was higher in IPF than that in CVD (P = .018) and NSIP (P = .039). The aFF/total FF ratio was higher in CVD than that in FHP (P = .021) and IPF (P < .001). A high cFF/total FF ratio was correlated with FHP versus IPF (P = .032). In conclusion, FF with existing peripheral and distorted/dense fibrosis were more closely related to IPF, whereas cFF were highly correlated with FHP. Moreover, a high aFF/total FF ratio was suggestive of CVD.

Katsuragawa H ，Ito H ，Handa T ，Hamaji M ，Menju T ，Sakamoto R ，Date H ，Haga H ，Yoshizawa A ... -  《-》

A Low Forced Vital Capacity (FVC)/Diffusing Capacity of the Lung for Carbon Monoxide (DLCO) Ratio Increases Clinical Suspicion for Fibrotic Hypersensitivity Pneumonitis (FHP) Over Idiopathic Pulmonary Fibrosis (IPF).

Background and objective Fibrotic Hypersensitivity Pneumonitis (FHP) and idiopathic pulmonary fibrosis (IPF) are interstitial lung diseases (ILDs) that are challenging to differentiate with prognostic and therapeutic implications. Clinical observations suggest that patients with FHP may have a lower baseline ratio of forced vital capacity (FVC) to the diffusing capacity of the lung for carbon monoxide (DLCO), or FVC/DLCO (F/D) ratio, than patients with IPF. In light of this, we aimed to determine whether patients with FHP have a significantly lower baseline F/D ratio than patients with IPF. Methods A retrospective chart review was performed at a single academic ILD center. Patients with a probable or definite diagnosis of FHP or IPF were considered for inclusion, while patients with poor-quality pulmonary function tests (PFTs) were excluded. The data collected included demographics, diagnosis modality, FVC and DLCO values within six months of diagnosis, as well as hemoglobin levels within three months of PFTs. Baseline F/D ratios were calculated using each patient's FVC percentage of predicted value divided by the DLCO percentage of predicted value adjusted for hemoglobin when available. One-tailed independent two-sample T-tests were performed. Results Eighty-nine patients met the inclusion criteria: 39 (44%) with FHP and 50 (56%) with IPF. The mean baseline F/D ratio was significantly lower for patients with FHP (M = 1.24, 95% CI: 1.14, 1.33) than for patients with IPF (M = 1.44, 95% CI: 1.31, 1.57, T(87) = 2.23, p = 0.014). A secondary analysis excluding patients with pulmonary hypertension and resting hypoxemia was performed, yielding 72 patients: 32 (44%) with FHP and 40 (56%) with IPF. The mean baseline F/D ratio was significantly lower for patients with FHP (M = 1.22, 95% CI: 1.12, 1.31) compared to patients with IPF (M = 1.37, 95% CI: 1.27, 1.46, T (70) = 2.37, p = 0.01). Conclusions In patients with probable to definite FHP versus IPF, the baseline F/D ratio was significantly lower in patients with FHP, even after excluding patients with coexisting pulmonary hypertension and resting hypoxemia. A lower baseline F/D ratio may be a novel, clinic-ready index to heighten clinical suspicion for FHP compared to IPF. Further larger prospective studies are needed to validate our findings.

Vongvivitpatana TS ，Nambiar AM 《-》

Impact of preexisting interstitial lung disease on outcomes of lung cancer surgery: A monocentric retrospective study.

Interstitial lung disease (ILD) is a known risk factor for lung cancer (LC). However, the surgical risk of LC in patients with ILD remains unclear. Therefore, we conducted a single-center retrospective study to assess clinical features and outcomes of LC population who underwent surgery with or without ILD. Patients who underwent surgery for LC between January 2006 and June 2023 in our center were assessed using data extracted from the nationwide EPITHOR thoracic surgery database. Suspicion of ILD was based on patients' records. Confirmation of ILD was then made on the patient's medical and radiological history. Patients were classified according to the pattern of ILD. The study aimed to describe the outcomes after lung cancer resection in patients with confirmed LC-ILD group compared to those without ILD (LC-non-ILD): post-operative complications, disease-free survival (DFS) and overall survival (OS). A subgroup analysis was also performed on patients with idiopathic pulmonary fibrosis and lung cancer (LC-IPF). 4073 patients underwent surgery for LC at Assistance Publique des Hôpitaux de Marseille between January 2006 and June 2023. Of these, 4030 were in the LC-non-ILD group and 30 were LC-ILD patients. In the LC-ILD group, the predominant CT scan pattern was probable UIP (50 %). OS was not significantly lower in the LC-ILD group (45 months versus 84 months, p = 0.068). Dyspnea and tumor size were identified as potential univariate predictors of OS. No significant differences were observed on post-operative complications or their severity. The most common post-operative complications in the LC-ILD group were prolonged air leak, respiratory failure, or pneumonia. 13 patients had cancer recurrence in the LC-ILD group. Our study provides a comprehensive analysis of a LC-ILD population features and outcome when undergoing surgery for LC. Patients with LC-ILD appeared to have a reduced OS compared with LC-non-ILD. Further investigations with larger prospective studies could be useful to confirm and develop these preliminary findings.

Goga A ，Fourdrain A ，Habert P ，Nguyen Ngoc AT ，Bermudez J ，Mogenet A ，Simon E ，Gouton E ，Tomasini P ，Thomas PA ，Greillier L ，Pluvy J ... -  《Respiratory Medicine and Research》

Obstructive sleep apnea in patients with fibrotic interstitial lung disease (non-idiopathic pulmonary fibrosis): what should be offered?

Cardoso CG ，Valente C ，Serino M ，Rodrigues I ，Carvalho A ，Coelho DB ，Bastos HN ，Mota PC ，Morais A ，Drummond M ... -  《-》