Diagnosis Of Interstitial Lung Disease Research Articles

Application and Interpretation of High-Resolution Computed Tomography for the Diagnosis of Interstitial Lung Disease (ILD): ILD Protocol

Interstitial lung disease (ILD) encompasses a heterogeneous group of over 200 diffuse parenchymal lung diseases. Accurate diagnosis of ILD is essential for appropriate treatment but can be challenging to achieve. For patients with suspected ILD, high-resolution computed tomography (HRCT) is a minimally invasive approach that allows for convenient repeat imaging. Compared to a conventional computed tomography (CT) protocol, the HRCT protocol for ILD patients involves two additional series with different technical requirements: a prone inspiration scan, and a supine expiration scan. The prone scan is useful when dependent opacification is observed on a supine chest CT scan. It facilitates the diagnosis of honeycombing, reducing observer variation in diagnosing idiopathic pulmonary fibrosis. The expiratory scan is useful for identifying air trapping, which is essential for the diagnosis of hypersensitivity pneumonitis. It is used to assess the three-density sign, which is specific for hypersensitivity pneumonitis and characterized by patchy distribution of normal-appearing lobules, ground glass opacities, and lobules with reduced lung density and vessel size. The HRCT ILD protocol should be performed regularly to assess changes in the extent of fibrosis and to diagnose lung cancer or acute exacerbation when clinically indicated.

Lung transplantation for emphysema: only emphysema or something else?

Some studies have reviewed lung explants histology to determine the frequency of pretransplant non-identified neoplasms or explore its diagnostic correlation with a previous diagnosis of interstitial lung disease (ILD). This study aims to review the histopathology of explants from patients who underwent lung transplantation (LT). A retrospective, single-center study that included patients who underwent LT for emphysema between 01 January 2011 and 31 October 2021. The control group was composed of patients with lung cancer who underwent a lung resection between 01 November 2011 and 31 December 2019 and had a previous diagnosis of chronic obstructive pulmonary disease (COPD) prior to lung resection surgery. A systematic review was performed of histological findings to compare the frequency of additional histological diagnoses. The study sample included 160 patients (43.8%) who received a lung transplant for emphysema and 205 patients with COPD and lung cancer treated surgically. Although the patients in the cancer group were significantly older and had more comorbidities and higher cumulative tobacco consumption, transplant recipients received an additional significative histologic diagnosis more frequently (58.1% vs. 12.7%; P<0.001) including ILD, pneumoconiosis and others. Significant additional histological findings were more frequent in the group of lung transplant recipients with emphysema. Notably, these findings were not explained by tobacco use, and they were significantly more frequent in transplant recipients than in patients with a previous diagnosis of COPD and higher cumulative tobacco consumption but with a better respiratory functional status.

Connective tissue disease related interstitial lung disease: a single center experience

Objectives: We aimed to determine the clinical features of the patients followed by our hospital with the diagnosis of connective tissue disease related interstitial lung disease (CTD-ILD). Methods: The study included 113 patients who were followed up with the diagnosis of CTD-ILD, admitted to Kahramanmaraş Sütçü İmam University, Faculty of Medicine, Rheumatology Department between January 2019 and December 2020. Demographic characteristics, laboratory data and high-resolution computed tomography (HRCT) patterns of the patients were recorded retrospectively. Results: Of 113 patients diagnosed with CTD-ILD; 90 were female, 23 were male. When the distribution of connective tissue disease (CTD) evaluated; 50 were rheumatoid arthritis, 41 were systemic sclerosis, 8 were sjogren's syndrome, 4 were systemic lupus erythematosus, 7 were overlap syndrome and 3 were undifferentiated connective tissue disease. There was no statistically significant difference between laboratory parameters. In the HRCT evaluation, 86 patients had nonspecific interstitial pneumonia (NSIP), 25 patients had usual interstitial pneumonia (UIP), and 2 patients had lymphocytic interstitial pneumonia (LIP) pattern. The most common pattern, including rheumatoid arthritis, was NSIP. Conclusions: In our study, as inconsistent with the literature the most common pattern in RA patients was found to be NSIP.

Concordance between transbronchial lung cryobiopsy and surgical lung biopsy in patients with idiopathic multicentric Castleman disease: A report of four cases

BackgroundIdiopathic multicentric Castleman disease (iMCD) is a rare polyclonal lymphoproliferative disease often associated with pulmonary involvement. Recently, transbronchial lung cryobiopsy (TBLC) has been reported to be useful for the diagnosis of diffuse interstitial lung disease. However, there have been no reports of pathological assessment of TBLC for iMCD. MethodTo clarify the efficacy of TBLC in the diagnosis of iMCD, we retrospectively reviewed four iMCD patients who had undergone both TBLC and surgical lung biopsy (SLB). ResultsThe median age was 44 years; 2 males and 2 females. Two or three TBLC specimens were taken from each patient. All patients had no complications other than minimal bleeding. The size of the TBLC specimens was approximately 5–6 × 3–4 mm, and the alveolar region, and centrilobular and perilobular areas were adequately sampled. As with SLB, the extent of lung lesions and inflammatory cell infiltration could be sufficiently evaluated by TBLC. The presence of lymphoid follicles could also be assessed by TBLC; however, the germinal centers with lymphoid follicles were difficult to evaluate. The TBLC specimens could also be evaluated for immunostaining, especially IgG4 immunostaining, to rule out IgG4-related lung disease. Pulmonary pathological grading showed a high concordance rate between major pathological findings of TBLC and SLB. The pathologist's confidence level of TBLC for the diagnosis of iMCD was high in all cases. ConclusionsTBLC exhibits a high concordance rate with SLB in the pathological evaluation of iMCD, which may be useful for the diagnosis of iMCD.

Defining the pathway to timely diagnosis and treatment of interstitial lung disease: a US Delphi survey

IntroductionTimely diagnosis of interstitial lung disease (ILD) is limited by obstacles in the current patient pathway. Misdiagnosis and delays are common and may lead to a significant burden of diagnostic...

Symposium 4

Extra-articular manifestations are common in Rheumatoid-arthritis (RA), involving the skin, eyes, heart, and lung, causing significant comorbidities. In particular, clinically significant rheumatoid arthritis-associated interstitial lung disease (RA-ILD) has a prevalence of about 10%-19% among RA patients. However, the exact prevalence of ILD in RA patients is not well known and there is also a lack of local data in Hong Kong. Early screening and identification of ILD in RA patients is important for improving patient outcomes. Currently, there is no established algorithm for screening in asymptomatic patients with RA. High resolution computed tomography (HRCT) is a standard assessment in ILD diagnosis but is associated with high cost. Pulmonary function test and lung ultrasound might also be useful in identifying ILD. Besides, multiple clinical risk factors and novel biomarkers have been explored for early identification of RA-ILD. To evaluate the usefulness of these predictors and to evaluate the burden of interstitial lung disease (ILD) among patients with RA, a local study (RAISE, Rheumatoid Arthritis-associated ILD: Screening and Evaluation in high-risk patients) was designed in Hong Kong to assess the prevalence of RA-ILD among RA patients with high risk, and to identify potential clinical and biochemical markers associated with the condition. In this session, Dr. Shirley Chan will share the protocol of the RAISE study and the screening approach. The interim results will also be analyzed and presented.

Plasma LTBP2 as a potential biomarker in differential diagnosis of connective tissue disease-associated interstitial lung disease and idiopathic pulmonary fibrosis: a pilot study.

Few biomarkers distinguish connective tissue disease-associated interstitial lung disease (CTD-ILD) from idiopathic pulmonary fibrosis (IPF). Latent transforming growth factor-β binding protein-2 (LTBP2), a secreted extracellular matrix protein, is involved in pulmonary fibrosis. However, the role of LTBP2 in differentially diagnosing CTD-ILD and IPF is unclear. In this study, enzyme-linked immunosorbent assays quantified plasma LTBP2 concentrations in 200 individuals (35 healthy controls, 42 CTD patients without ILD, 89 CTD-ILD patients, and 34 IPF patients). CTD-ILD and IPF were further classified based on chest imaging pattern and pulmonary function test results. Plasma LTBP2 levels were significantly elevated in the IPF group compared with the CTD-ILD group. ROC analysis further suggested the possible value of LTBP2 in differentially diagnosing CTD-ILD and IPF. Additionally, CTD-ILD patients with progressive lung fibrosis had higher plasma LTBP2 concentrations than those who did not. Similarly, patients with IPF developing acute exacerbation showed higher plasma LTBP2 levels than those with stable IPF. This is the first study showing that LTBP2 was closely associated with the usual interstitial pneumonia (UIP) pattern in rheumatoid arthritis-associated ILD (RA-ILD). Moreover, the optimal cutoff values of LTBP2 for distinguishing IPF from CTD-UIP/RA-UIP were 33.75 and 38.33ng/mL with an AUC of 0.682 and 0.681, respectively. Our findings suggest that plasma LTBP2 levels may differentially diagnose CTD-ILD and IPF, and assess their fibrotic activity. Additionally, clinical LTBP2 evaluation may be a great aid to identifying the presence of the UIP pattern in RA-ILD and to discriminating IPF from CTD-UIP, particularly RA-UIP.

Characteristics and risk of interstitial lung disease in dermatomyositis and polymyositis: a retrospective cohort study in Japan

Dermatomyositis and polymyositis are rare, idiopathic inflammatory myopathies. Interstitial lung disease is one of the most common and potentially severe extra-muscular manifestations of dermatomyositis and polymyositis and is strongly linked to poor prognosis and early mortality. We aimed to characterise the demographic and clinical characteristics, incidence, and treatment of interstitial lung disease in patients with dermatomyositis or polymyositis. We conducted a retrospective cohort study using the Japan Medical Data Center healthcare claims database. Patients in the database with dermatomyositis (International Classification of Disease version 10 M33.0, M33.1, M33.9) or polymyositis (M33.2) from 01-Jan-2011 until 31-Dec-2019 were identified and followed-up for interstitial lung disease (J84.x) until death, dis-enrolment, or study end (31 December 2020). Cumulative risk curves compared interstitial lung disease risk in dermatomyositis versus polymyositis. Risk factors were evaluated by Cox proportional hazard models. There were 886 patients with dermatomyositis and 745 patients with polymyositis included in the cohort analysis. Mean (standard deviation) age at dermatomyositis/polymyositis diagnosis was 46.0 (16.0)/49.7 (13.3) years and 300 (34%)/104 (14%) developed interstitial lung disease during follow-up. The incidence rate of interstitial lung disease per 100 person-years was 18.42 (95% CI 16.42–20.59) for dermatomyositis and 5.39 (95% CI 4.43–6.50) for polymyositis. In the analysis adjusted for sex, age, and comorbidity score, the risk of interstitial lung disease was significantly higher in patients with dermatomyositis than with polymyositis (hazard ratio 2.72, 95% CI 2.18–3.41). The rate diverged markedly between the groups in the first year after diagnosis. Risk factors for interstitial lung disease were older age in dermatomyositis, female sex and rheumatoid arthritis in polymyositis. Glucocorticoids with/without tacrolimus were the most common newly prescribed drugs after the interstitial lung disease diagnosis. In conclusion, the risk of developing interstitial lung disease was significantly higher in patients with dermatomyositis than with polymyositis, and risk factors were different in the 2 patient groups.

THE FIRST EXPERIENCE OF USING NON-INTUBATION VIDEO-ASSISTED THORACOSCOPIC RESECTIONS FOR LUNG CANCER IN THE REPUBLIC OF KAZAKHSTAN

Relevance:Several studies have shown that non-intubation surgical interventions, especially, have proven themselves well in patients with low reserve of the cardiovascular and respiratory systems, as well as in the diagnosis of interstitial lung diseases.&#x0D; The study aimed to describe the experience of introducing into clinical practice surgical interventions without intubation on spontaneous breathing for malignant neoplasms of the lungs and mediastinal organs.&#x0D; Methods: two clinical cases are described of the main points of the anesthesia and technical features of surgical intervention.&#x0D; Results: A surgery in two patients with peripheral lung cancer was performed. In the first case, the operation included NI-VATS on the right and the resection of the upper (S2) and lower lobe (S6, S9); in the second case – NI-VATS on the right, lower lobectomy, and lymph node dissection. Based on the results of histological examination, both patients were diagnosed with NSCLC in the initial stages. Postoperative chemoradiotherapy is not indicated. The patients were registered at the dispensary in clinical group III.&#x0D; Conclusion: As the literature data and our preliminary results of NI-VATS show, surgical interventions in the surgical treatment of lung cancer have places to be and should be widely introduced into clinical practice in oncothoracic departments of the Republic of Kazakhstan.

Surgical Treatment of Secondary Pneumothorax-Complicated Interstitial Lung Disease.

To investigate the feasibility of early surgical treatment and perioperative steroid use in patients with interstitial lung disease (ILD) complicated by pneumothorax. We retrospectively examined data, including patient characteristics, laboratory findings, surgical treatment details, postoperative complications, and deaths, of nine patients with ILD complicated by secondary pneumothorax. The patients had been treated at our hospital during the past 10 years. All nine patients were male (median age, 69.0 years). A total of nine patients had a histopathologic diagnosis of ILD after surgery. Of these, five were clinically diagnosed with ILD before surgery. Collagen disease was diagnosed in one case, drug-induced in one case, and idiopathic ILD (IILD) in three cases. All nine patients were diagnosed with postoperative ILD, including one case of collagen disease, one case of drug-induced, three cases of idiopathic pulmonary fibrosis (IPF)/cryptogenic fibrosing alveolitis, one case of nonspecific interstitial pneumonia (NSIP), and three cases of cryptogenic organizing pneumonia (COP). Regarding preoperative clinical characteristics, the performance status (PS) was 0 or 1 in all patients. Overall, three patients received oxygen (0-3 L/min), whereas steroids were administered to five patients. The mean drainage period was 23.5 days, and this was consistent with the time taken from pneumothorax occurrence to surgery. Video-assisted thoracic surgery (VATS) and thoracoscopic-assisted surgery were performed in seven and two patients, respectively. No postoperative recurrence or surgery-related deaths occurred. Early surgery for secondary pneumothorax complicated by ILD may be a viable option for patients in good preoperative condition. For patients who are preoperatively treated with steroids, continued use of steroids should be carefully considered.

Prognostic value of serum Krebs von den Lungen-6 (KL-6) levels in COVID-19 pneumonia

Background and objectivesKrebs von den Lungen-6 (KL-6), expressed by damaged type II pneumocytes, is useful in the diagnosis and severity assessment of many diffuse interstitial lung diseases. The objective of our study was to determine the prognostic value of the initial KL-6 plasma level in COVID-19 pneumonia. MethodsAll patients hospitalized for a suspected COVID-19 pneumonia between March and May 2020 in our Chest department of a French university hospital were included. KL-6 serum concentrations were measured within 72 h of diagnostic suspicion by chemiluminescence enzyme immunoassay Survival analysis was performed using a Cox regression and modeled by a Kaplan-Meier curve. ResultsSixty-six COVID-19 patients (average age = 64 ± 14 years, 71.2 % males) with KL-6 serum measurement were included. Median KL-6 serum concentration was 409 ± 312 U/mL. KL-6 was significantly higher in men (p = 0.003), elders (p = 0.0001) and in patients with greater Charlson's score (p = 0.002). Higher KL-6 concentration was significantly associated with in-hospital mortality (HR: 8.66; 95 % CI:1.1–69.2, p = 0.014), radiological extension of lesions on chest CT scan (p = 0.004) and higher WHO severity score (p = 0.042), but not with admission in intensive care unit. In 9 (14 %) non-surviving COVID-19 patients, KL-6 serum concentration increased whereas it remained stable or decreased in survivors. At 3 months follow-up (n = 48), DLCO was negatively correlated with the initial KL-6 value (r = 0.47, p = 0.001), while FVC, FEV1 and MRC score were not. ConclusionInitial KL-6 serum concentration is significantly associated with in-hospital mortality, unfavorable outcome, and persistent impairment of DLCO at 3 months. Initial KL-6 plasma determination appears as a prognostic biomarker in COVID-19 pneumonia.

A NSCLC patient with novel ALK fusion responded to crizotinib therapy after alectinib-induced interstitial lung disease.

A 54-year-old, non-smoking woman was diagnosed as stage ⅣB adenocarcinoma with widespread bone metastasis (cT4N2M1c) in our hospital. Immunohistochemistry (Ventana D5F3 antibody) result showed the presence of ALK rearrangement; and next-generation sequencing assay (NGS) indicated EML4-ALK fusion (E6:A20) with concurrent CCDC148-ALK (C1:A20), PKDCC-ALK (Pintergenic:A20)and VIT-ALK (V15:A20) fusions. After 32 weeks of alectinib treatment, the patient complained cough and exertional chest distress but had no sign of infection. Computed tomography (CT) showed bilateral diffuse ground glass opacities, suggesting a diagnosis of alectinib-related interstitial lung disease (ILD). Following corticosteroid treatment and discontinuation of alectinib, clinical presentations and CT scan gradually improved, but the primary lung lesions enlarged in the regular follow-up. The administration of a first-generation ALK inhibitor crizotinib was then initiated and the disease was stable for 25 months without recurrence of ILD.

Clinical Application of Confocal Laser Endomircoscopy Combined with Cryobiopsy in the Diagnosis of Interstitial Lung Disease

Introduction: Confocal laser endomicroscopy (CLE) has the characteristics of high resolution, real-time imaging, and no radiation, which is helpful for the precise and effective implementation of transbronchial cryobiopsy (TBCB). The study aimed to compare the efficacy and safety of TBCB combined with CLE (CLE group) or fluoroscopy (fluoroscopy group) in the diagnosis of interstitial lung disease (ILD). Methods: From a prospective randomized controlled trial, 80 patients with undiagnosed ILD or ILD requiring biopsy between January 2022 and November 2022 were randomly assigned to CLE group and fluoroscopy group. The rate to reach an etiological diagnosis of ILD, maximum cross-sectional area of specimens, operation time, and complications were compared between the two groups. Results: The rate to reach an etiological diagnosis in the CLE group was significantly higher than that in the fluoroscopy group (95.0% vs. 80.0%, p < 0.05), but there was no difference in the maximum cross-sectional area of the specimens (42.1 ± 10.1 mm2 vs. 41.5 ± 10.3 mm2, p > 0.05). In terms of operation time, the CLE group was significantly shorter than the fluoroscopy group (37.6 ± 10.6 min vs. 54.8 ± 24.9 min, p < 0.05). The bleeding volume in the CLE group was significantly lower than that in the fluoroscopy group (4.9 ± 3.6 mL/case vs. 9.0 ± 9.2 mL/case, p < 0.05). Further analysis showed that the incidence of moderate bleeding was also lower in the CLE group (20.0% vs. 75.0%, p < 0.001). In addition, the incidence of pneumothorax in the CLE group was significantly lower than that in the fluoroscopy group (0 vs. 25.0%, p < 0.001). Conclusions: Compared with simple fluoroscopy, the combination of CLE significantly improves the rate of etiological diagnosis, shortens the operation time, and reduces complications such as bleeding and pneumothorax.

Effect of Nintedanib in Patients with Progressive Pulmonary Fibrosis in Subgroups with Differing Baseline Characteristics.

In the INBUILD trial in patients with progressive pulmonary fibrosis other than idiopathic pulmonary fibrosis (IPF), nintedanib slowed the rate of decline in forced vital capacity (FVC; mL/year) over 52weeks compared with placebo. We assessed the efficacy of nintedanib across subgroups in the INBUILD trial by baseline characteristics. We assessed the rate of decline in FVC over 52weeks and time to progression of interstitial lung disease (ILD) (absolute decline from baseline in FVC % predicted>10%) or death over the whole trial in subgroups based on sex, age, race, body mass index (BMI), time since diagnosis of ILD, FVC % predicted, diffusing capacity of the lungs for carbon monoxide (DLco) % predicted, composite physiologic index (CPI), GAP (gender, age, lung physiology) stage, use of anti-acid therapy and use of disease-modifying antirheumatic drugs (DMARDs) at baseline. The effect of nintedanib versus placebo on reducing the rate of decline in FVC over 52weeks was consistent across the subgroups by baseline characteristics analysed. Interaction p values did not indicate heterogeneity in the treatment effect between these subgroups (p>0.05). Over the whole trial (median follow-up time ∼19months), progression of ILD or death occurred in similar or lower proportions of patients treated with nintedanib than placebo across the subgroups analysed, with no heterogeneity detected between the subgroups. In the INBUILD trial, no heterogeneity was detected in the effect of nintedanib on reducing the rate of ILD progression across subgroups based on demographics, ILD severity or use of anti-acid therapy or DMARDs. These data support the use of nintedanib as a treatment for progressive pulmonary fibrosis. ClinicalTrials.gov Identifier: NCT02999178.

Progressive pulmonary fibrosis and its impact on survival in systemic sclerosis related interstitial lung disease.

To describe the frequency of progressive pulmonary fibrosis (PPF) in an incident cohort of systemic sclerosis (SSc) related interstitial lung disease (ILD) and its impact on survival. Incident ILD was defined as the new development of characteristic fibrotic changes on chest HRCT scan. PPF was defined as per the 2022 American Thoracic Society. Determinants of PPF were identified using generalised estimating equations. Impact on survival was analysed using accelerated failure time regression modelling. Of our incident SSc-ILD cases, 38.8% (n = 180) experienced PPF within a 12-month period after ILD diagnosis. Determinants of PPF included older age (OR 1.02, 95%CI 1.00-1.03, p= 0.011), dcSSc (OR 1.54, 95% CI 1.06-2.25, p= 0.024) and SSc specific antibodies (anticentomere antibody OR 0.51, 95%CI 0.29-0.91, p= 0.021 and anti-Scl-70 antibody OR 1.46, 95%CI 1.01-2.09, p= 0.043). Raised CRP was numerically associated with PPF but did not reach statistical significance (OR 1.29, 95%CI 0.99-1.68, p= 0.064) nor did GORD or dysphagia (OR 1.18, 95%CI 0.57-2.42, p= 0.658 and OR 1.17, 95%CI 0.57-2.40, p= 0.664 respectively). The presence of PPF significantly impacted survival in SSc-ILD (hazard ratio 2.66, 95%CI 1.59-4.41, p< 0.001). PPF occurred in a third of our incident SSc-ILD cohort; however, its occurrence was significantly associated with mortality indicating an at-risk group who may be suitable for earlier introduction of immunosuppressive and/or antifibrotic therapy.

Impact of chronic fibrosing interstitial lung disease on healthcare use: association between fvc decline and inpatient hospitalization

BackgroundMany types of interstitial lung diseases (ILDs) may transition to progressive chronic-fibrosing ILDs with rapid lung function decline and a negative survival prognosis. In real-world clinical settings, forced vital capacity (FVC) measures demonstrating progressive decline may be linked to negative outcomes, including increased risks of costly healthcare resource utilization (HRU). Thus, we assessed the relationship between rate of decline in lung function and an increase in HRU, specifically inpatient hospitalization, among patients with chronic fibrosing ILD.MethodsThis study utilized electronic health records from 01-Oct-2015 to 31-Oct-2019. Eligible patients (≥ 18 years old) had ≥ 2 fibrosing ILD diagnosis codes, clinical activity for ≥ 15 months, and ≥ 2 FVC tests occurring 6 months apart. Patients with missing demographic data, IPF, or use of nintedanib or pirfenidone were excluded. Two groups were defined by relative change in percent of predicted FVC (FVC% pred) from baseline to 6 months: significant decline (≥ 10%) vs. marginal decline/stable FVC (decrease < 10% or increase). The primary outcome was defined as the occurrence of an inpatient hospitalization 6 months after the first FVC value. Descriptive and multivariable analysis was conducted to examine the impact of FVC decline on occurrence of inpatient hospitalization.ResultsThe sample included 566 patients: 13% (n = 75) with significant decline and 87% (n = 491) with marginal decline/stable FVC; their mean age (SD) was 65 (13.7) years and 56% were female. Autoimmune diagnoses were observed among 40% of patients with significant decline, and 27% with marginal decline/stable FVC. The significant decline group had better lung function at baseline than the marginal/stable group. For patients with FVC% <80% at baseline, reduction of FVC% ≥10% was associated with significantly increased odds of an inpatient hospitalization (odds ratio [OR] 2.85; confidence interval [CI] 1.17, 6.94 [p = 0.021]).ConclusionDecline in FVC% ≥10% was associated with increased odds of inpatient hospitalization among patients with reduced lung function at baseline. These findings support the importance of preserving lung function among patients with fibrosing ILD.

Utility and Safety of Bronchoscopic Cryotechniques-A Comprehensive Review.

Cryosurgical techniques are employed for diagnostic and therapeutic bronchoscopy and serve as important tools for the management of pulmonary diseases. The diagnosis of interstitial lung disease requires multidisciplinary team discussions after a thorough assessment of history, physical exam, computed tomography, and lung-function testing. However, histological diagnosis is required in selected patients. Surgical lung biopsy has been the gold standard but this can be associated with increased morbidity and mortality. Transbronchial lung cryobiopsy is an emerging technique and multiple studies have shown that it has a high diagnostic yield with a good safety profile. There is wide procedural variability and the optimal technique for cryobiopsy is still under investigation. There is emerging data that demonstrate that cryobiopsy is safe and highly accurate in the diagnosis of thoracic malignancies. Furthermore, cryorecanalization procedures are a useful adjunct for the palliation of tumors in patients with central airway obstruction. One should keep in mind that these procedures are not free from complications and should be carried out in a specialized center by a trained and experienced bronchoscopy team. We present a review of the literature on the diagnostic and therapeutic utility of bronchoscopy-guided cryosurgical procedures and their safety profile.

Tomographic Fibrosis Score in the Patients with Systemic Sclerosis-Associated Interstitial Lung Disease.

Various visual semi-quantitative staging systems based on high-resolution computed tomography are used to evaluate inflammatory rheumatologic disease-associated interstitial lung disease. We aimed in this retrospective study to evaluate whether tomographic fibrosis score, a new visual semi-quantitative staging system, was a predictor of mortality and the relationship between tomographic fibrosis score and respiratory function tests in patients with systemic sclerosis-associ- ated interstitial lung disease. The patients who have been followed up at a single-center rheumatology clinic for the last 5 years and met the American College of Rheumatology / European League Against Rheumatism (ACR-EULAR) 2013 systemic sclerosis classification criteria were included in the study. Clinical data were obtained retrospectively from patient records, including patients' characteristics, pulmonary function test (forced vital capacity), diffusing capacity of the lung for carbon monoxide test, high-reso- lution computed tomography results, medication history, and serological test results. High-resolution computed tomography of the patients diagnosed with interstitial lung disease were assessed for the study. The radiologists scored the extent of parenchymal abnormalities (ground glass opacification, reticulation, honeycombing, and consolidation) and calculated tomographic fibrosis score and also traction bronchiectasis score for each patient. Fifty-two patients (46 female, median age 60 (Q1-Q3:47-66) years) were included in this study. The median disease duration, follow-up time, interstitial lung disease duration, and time from sys- temic sclerosis diagnosis to interstitial lung disease diagnosis were 80 (59-143) months, 78 (50-119) months, 63 (43-81) months, and 4 (0-58) months, respectively. The median tomographic fibrosis score and traction bronchiectasis score of the patients were 3.08% (1.33-8.06) and 0 (0-2), respectively. There was a moderate direct correlation between tomographic fibrosis score and traction bronchiectasis score (r = +0.472, P < .001). Additionally, there was a mod- erate inverse correlation between tomographic fibrosis score and diffusing capacity of the lung for carbon monoxide at diagnosis (r = -0.554, P = .011). During the follow-up period, 12 (23%) patients died. Kaplan-Meier Test (P = 0.009) and Cox regression analysis (B: 4.673, 95% confidence interval, 1.321-16.529, P = .017) revealed that tomographic fibrosis score ≥ 5% was associated with mortality. Multivariate analysis was not performed due to the small number of patients. An inverse relationship was found between tomographic fibrosis score and diffusing capacity of the lung for carbon monoxide at diagnosis. The odds ratio for mortality was 4.7 when tomographic fibrosis score was ≥5%. Tomographic fibrosis score may be useful for predicting mor- tality and respiratory function in patients with systemic sclerosis-associated interstitial lung disease.

Design of a phase III, double-blind, randomised, placebo-controlled trial of BI 1015550 in patients with progressive pulmonary fibrosis (FIBRONEER-ILD)

IntroductionProgressive pulmonary fibrosis (PPF) includes any diagnosis of progressive fibrotic interstitial lung disease (ILD) other than idiopathic pulmonary fibrosis (IPF). However, disease progression appears comparable between PPF and IPF, suggesting...

Transbronchial Cryobiopsy Is Superior to Forceps Biopsy for Diagnosing both Fibrotic and Non-Fibrotic Interstitial Lung Diseases

Background: Interstitial lung disease (ILD) evaluation often requires lung biopsy for definite diagnosis. In recent years, transbronchial cryobiopsy (TBCB) emerged as a procedure with higher diagnostic yield than transbronchial forceps biopsy (TBFB), especially for fibrotic ILDs. Nonetheless, studies comparing these modalities in non-fibrotic ILDs and for specific ILD diagnoses are scarce. Objectives: The aim of this study was to evaluate the diagnostic yield and safety of TBCB and TBFB in patients with fibrotic and non-fibrotic ILDs. Method: An observational retrospective multicenter study including patients with ILD diagnosis by multidisciplinary discussion that underwent TBCB or TBFB between 2017 and 2021. Chest CT scans were reviewed by a chest radiologist. Biopsy specimens were categorized as diagnostic (with specific histological pattern), nondiagnostic, or without lung parenchyma. Nondiagnostic samples were reassessed by a second lung pathologist. TBCB and TBFB diagnostic yields were analyzed by multivariate regression. Procedural complications were evaluated as well. Results: 276 patients were included, 116 (42%) underwent TBCB and 160 (58%) TBFB. Fibrotic ILDs were present in 148 patients (54%). TBCB diagnostic yield was 78% and TBFB 48% (adjusted odds ratio [AOR] 4.2, 95% CI: 2.4–7.6, p < 0.01). The diagnostic yield of TBCB was higher than TBFB among patients with fibrotic ILD (AOR 3.8, p < 0.01), non-fibrotic ILD (AOR 5.8, p < 0.01), and across most ILD diagnoses. TBCB was associated with higher risk for significant bleeding (10% vs. 3%, p < 0.01), but similar risk for pneumothorax. Conclusions: Diagnostic yield of TBCB was superior to that of TBFB for both fibrotic and non-fibrotic ILDs, and across most diagnoses.