Radiological and histopathological features and treatment response by subtypes of interstitial pneumonia with autoimmune features: A prospective, multicentre cohort study.

BACKGROUND: Patients with idiopathic interstitial pneumonia (IIP) have a favourable prognosis when they have interstitial pneumonia with autoimmune features (IPAF). However, precise IPAF-related findings from high-resolution computed tomography (HRCT) and lung histopathological specimens and the treatment response have not been fully determined. Therefore, this study was conducted to evaluate the relationship between findings on HRCT or lung histopathological specimens and the progression of interstitial pneumonia in patients with IPAF. METHODS: This multicentre cohort study prospectively enrolled consecutive patients with IIP. At the diagnosis of IIP, we systematically evaluated 74 features suggestive of connective tissue diseases and followed them up. HRCT, lung specimens, serum antibodies, and the clinical course were also evaluated. RESULTS: Among 222 patients with IIP, 26 (11.7%) fulfilled the IPAF criteria. During a median observation period of 36 months, patients with IPAF showed better survival than those without IPAF (p = 0.034). While histopathological findings were not related to IPAF, nonspecific interstitial pneumonia (NSIP) with organizing pneumonia (OP) overlap was the most prevalent HRCT pattern (p < 0.001) and the consolidation opacity was the most common radiological finding in IPAF (p = 0.017). Furthermore, in patients with IPAF, the diagnosis of COP or NSIP with OP overlap was associated with a higher increase in %FVC in 1 year than in those with idiopathic pulmonary fibrosis, NSIP, or unclassifiable IIP (p = 0.002). CONCLUSIONS: This study shows the presence of consolidation opacity on HRCT and the diagnosis of COP or NSIP with OP overlap are associated with IPAF and its favourable treatment response in patients with IPAF.

Idiopathic myointimal hyperplasia of mesenteric veins: radiological evaluation using CT angiography.

A 44-year-old man presented with a chief complaint of constipation. Initial contrast-enhanced CT showed extensive bowel wall thickening, mainly in the left colon, with a thin cord-like inferior mesenteric vein (IMV), in contrast to ectatic mesenteric venous branches, suggesting bowel ischaemia owing to venous stasis. One month later, at the time of symptom exacerbation, CT angiography showed a cord-like IMV and ectatic mesenteric venous branches with early enhancement, suggesting the presence of an arteriovenous fistula (AVF). Owing to the progression of bowel ischaemia and necrosis with peritonitis, emergency surgery was performed. Surgical specimens showed focal myointimal hyperplasia of the proximal mesenteric veins in both ischaemic and non-ischaemic lesions of the resected colon, thus leading to the diagnosis of idiopathic myointimal hyperplasia of mesenteric veins (IMHMV) when combined with the clinical and imaging findings. IMHMV is a bowel ischaemic disease caused by non-thrombotic venous obstruction that requires bowel resection and has been suggested to be associated with AVF. Cord-like IMV and AVF in the mesentery are important CT findings that characterize IMHMV. CT angiography is useful in diagnosing IMHMV.

Invasive candidiasis presenting bronchiectatic cavity as chest radiological findings: A case report.

Invasive candidiasis is rare but is associated with high mortality in immunocompromised or critically ill patients. Here, we present a case of a 55-year-old man with untreated diabetes who was diagnosed with coronavirus disease 2019 and subsequently developed invasive candidiasis. The patient presented with fever, tachycardia, and tachypnea. Chest computed tomography revealed multiple consolidations mainly distributed around the bronchovascular bundles with bronchiectatic cavity formation, which initially raised suspicion for invasive pulmonary aspergillosis. However, subsequent testing confirmed Candida albicans infection; hence, we changed the antifungal agents effective for invasive candidiasis. This improved the patient's respiratory status, and he was then successfully weaned from mechanical ventilation. This case report highlights the importance of considering invasive candidiasis in the differential diagnosis of patients with bronchiectatic cavities on chest computed tomography, particularly in immunocompromised or critically ill patients with risk factors for invasive candidiasis.

Longitudinal changes in radiographic features of pulmonary Mycobacterium avium complex diseases.

Background: The radiographic features of Mycobacterium avium complex pulmonary disease (MAC-PD), a major component of nontuberculous mycobacteria, consist of a variety of lesions; however, the responsiveness of each type of radiographic factor to treatment is unclear. Thus, we evaluated the longitudinal changes of each factor in serial computed tomography (CT) images using a mixed-effects model, and investigated the radiographic transition in patients with MAC-PD whose progress could be followed. Methods: In this retrospective study, eighty-four patients diagnosed with MAC-PD and with yearly CT records were recruited after a review of 328 medical records with culture-positive MAC in respiratory specimens. The study participants were divided into two groups: treatment (n = 43) and no-treatment (n = 41) groups. Radiographic images were scored using the nodule (N), infiltration (I), cavity (C), ectasis (E) scoring system. Longitudinal changes in each radiographic lesion factor were analyzed using a mixed-effects model in treated and untreated patients. Results: All factors tended to progress without treatment, and significant longitudinal changes were observed in the N, I, and E factors (N: p = 0.010, I: p = 0.004, E: p < 0.001). Although treatment tended to improve N and I in radiographic images (N: p = 0.006, I: p = 0.203), cavities and ectasis progressed, regardless of treatment (C: p = 0.057 and E: p = 0.033). Conclusion: Radiographic changes of MAC-PD can be categorized into reversible (nodules and infiltrations) and irreversible (cavities and ectasis) lesions. Early treatment may prevent the accumulation of irreversible factors.

Cystic fibrosis with multiple pulmonary arteriovenous malformations: A case report.

Cystic fibrosis is an autosomal recessive genetic disorder that damages the exocrine function of the body, resulting in alterations of multiple organs. In the respiratory system, it is known to cause bronchiectasis, recurrent bronchitis, and pneumonia; however, to the best of our knowledge, there are no reported cases of pulmonary arteriovenous malformations associated with this disease. Herein, we report a case of cystic fibrosis with multiple pulmonary arteriovenous malformations. A 16-year-old girl, who has been monitored since childhood for pancreatitis of unknown cause, experienced respiratory symptoms and hypoxemia (PaO2 = 57 mmHg). At 13 years of age, chest computed tomography revealed bronchiectasis, bronchial wall thickening, and tree-in-bud sign. Genetic testing was performed, and the patient was diagnosed with cystic fibrosis. However, the computed tomography scan also showed incidental nodular lesions in the left superior and both the inferior pulmonary lobes, suggesting multiple arteriovenous malformations. Dynamic computed tomography was performed which, confirmed the presence of 3 pulmonary arteriovenous malformations. Coil embolization was performed on all lesions, and the hypoxemia was corrected. Marked hypoxemia in a patient with cystic fibrosis may not be explained only by the presence of bronchiectasis and/or bronchial wall thickening; in such cases, it may be necessary to examine possible additional findings on computed tomography images, such as arteriovenous malformations.

Retrospective evaluation of clinical signs, clinical course, and prognosis between dogs with left atrial rupture secondary to myxomatous mitral valve disease and those with neoplastic cardiac tamponade (2015-2019): 70 cases.

OBJECTIVE: To describe the clinical signs, clinical course, and prognosis of suspected left atrial rupture (LAR) secondary to myxomatous mitral valve disease (MMVD) in dogs and to compare them with dogs with suspected neoplastic cardiac tamponade (NCT). DESIGN: Retrospective study from November 2015 to October 2019 SETTING: An out-of-hours Emergency Animal Hospital. ANIMALS: Twenty-three dogs with LAR secondary to MMVD (LAR group) and 47 dogs with NCT (NCT group). INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The following were the characteristics of the study population (LAR group vs NCT groups) with P < 0.05 as the significance threshold: male sex, 83% vs 66%; median age, 11.9 vs 12.5 years; and median weight, 3.8 vs 6.4 kg (P < 0.001). Chihuahuas and Miniature Dachshunds were overrepresented in the LAR and NCT groups, respectively. Statistically different clinical findings between the 2 groups were as follows (LAR vs NCT): pulmonary edema, 43% vs 0%; pericardial thrombus, 70% vs 6% (P < 0.001); ineffectiveness of pericardiocentesis (whether aspiration of pericardial fluid was successful or not), 58% vs 2% (P < 0.001); mortality rate within 48 hours of visiting hospital, 35% vs 9% (P < 0.01). No significant difference was observed in survival time after discharge between the 2 groups. CONCLUSIONS: The proportion of dogs with a diagnosis of LAR secondary to MMVD in dogs with cardiac tamponade was higher than the previously reported rate. Furthermore, the frequency of pulmonary edema, ineffectiveness of pericardiocentesis, and short-term mortality rate was higher in the LAR group than in the NCT group.

Effect of Ultra-High-Resolution CT on Pseudoenhancement in Renal Cysts: A Phantom Experiment and Clinical Study.

BACKGROUND. Ultra-high-resolution CT (UHRCT) allows acquisition using a small detector element size, in turn allowing very high spatial resolutions. The high resolution may reduce partial-volume averaging and thereby renal cyst pseudoenhancement. OBJECTIVE. The purpose of this article was to assess the impact of UHRCT on renal cyst pseudoenhancement. METHODS. A phantom was constructed that contained 7-, 15-, and 25-mm simulated cysts within compartments simulating unenhanced and nephrographic phase renal parenchyma. The phantom underwent two UHRCT acquisitions using 0.25- and 0.5-mm detector elements, with reconstruction at varying matrices and slice thicknesses. A retrospective study was performed of 36 patients (24 men, 12 women; mean age, 75.7 ± 9.4 [SD] years) with 118 renal cysts who underwent renal-mass protocol CT using UHRCT and the 0.25-mm detector element, with reconstruction at varying matrices and slice thicknesses; detector element size could not be retrospectively adjusted. ROIs were placed to measure cysts' attenuation increase from unenhanced to nephrographic phases (to reflect pseudoenhancement) and SD of unenhanced phase attenuation (to reflect image noise). RESULTS. In the phantom, attenuation increase was lower for the 0.25- than 0.5-mm detector element for the 15-mm cyst (4.6 ± 2.7 HU vs 6.8 ± 2.9 HU, p = .03) and 25-mm cyst (2.3 ± 1.4 HU vs 3.8 ± 1.2 HU, p = .02), but not the 7-mm cyst (p = .72). Attenuation increase was not different between 512 × 512 and 1024 × 1024 matrices for any cyst size in the phantom or patients (p > .05). Attenuation increase was not associated with slice thickness for any cyst size in the phantom or in patients for cysts that were between 5 mm and less than 10 mm and those that were 10 mm and larger (p > .05). For cysts smaller than 5 mm in patients, attenuation increase showed decreases with thinner slices, though there was no significant difference between 0.5-mm and 0.25-mm (3-mm slice: 23.7 ± 22.5 HU; 2-mm slice: 20.2 ± 22.7 HU; 0.5-mm slice: 11.6 ± 17.5 HU; 0.25-mm slice: 12.6 ± 19.7 HU; p < .001). Smaller detector element size, increased matrix size, and thinner slices all increased image noise for cysts of all sizes in the phantom and patients (p < .05). CONCLUSION. UHRCT may reduce renal cyst pseudoenhancement through a smaller detector element size and, for cysts smaller than 5 mm, very thin slices; however, these adjustments result in increased noise. CLINICAL IMPACT. Although requiring further clinical evaluation, UHRCT may facilitate characterization of small cystic renal lesions, thereby reducing equivocal interpretations and follow-up recommendations.

Treatment of massive hemoptysis after thoracic aortic aneurysm repair.

BACKGROUND: Massive hemoptysis after thoracic aortic aneurysm (TAA) repair is a rare but potentially lethal condition. Endovascular management is a challenging treatment option due to the complexity of culprit vessel access. CASE PRESENTATION: An 81-year-old woman was referred to our hospital with massive hemoptysis. She had a history of graft replacement and thoracic endovascular aortic repair (TEVAR) for dissecting TAA. Computed tomography (CT) showed massive atelectasis with hematoma in the left lower lung lobe adjacent to the descending aortic aneurysm treated with TEVAR. Contrast-enhanced CT revealed a pseudoaneurysm and proliferation of abnormal vessels at the peripheral side of the left pulmonary ligament artery (PLA) in the atelectasis. The PLA continued to the right subscapular artery via a complex collateral pathway. Diagnostic angiography of the right subcapsular artery revealed a pseudoaneurysm and abnormal vessels at the peripheral side of the left PLA with a systemic-pulmonary artery shunt. Transcatheter arterial embolization (TAE) for the left PLA via the collateral pathway with N-butyl cyanoacrylate achieved complete embolization. The patient's hemoptysis was controlled and she was discharged. CONCLUSIONS: Here we presented a case of massive hemoptysis due to PLA disruption that occurred after TAA repair. TAE via a complex collateral pathway is a feasible and effective treatment for hemoptysis, even in patients who have undergone surgical or endovascular TAA repair.

Kinetics of Anti-SARS-CoV-2 Antibody Response Following Two Doses of the BNT162b2 mRNA Vaccine: A Japanese Single-Center Primary Care Clinic Report Involving Volunteers and Patients with Autoimmune Disease.

Despite the promising effectiveness of the coronavirus disease 2019 vaccination using an mRNA vaccine, the short efficacy duration and some poor responses to the vaccination remain major concerns. We aimed to clarify the monthly kinetics of the anti-SARS-CoV-2 spike receptor-binding domain antibody response after two doses of the BNT162b2 vaccine in a Japanese population. A chemiluminescent enzyme immunoassay (CLIA) and an enzyme-linked immunosorbent assay were used to measure the antibody levels in 81 Japanese adults (age, <65 years). The antibody levels increased 10-fold at 2−3 weeks following the second dose of BNT162b2 and declined thereafter to approximately 50%, 20%, and 10% of the peak levels at 2, 3, and 6 months, respectively. To compare the antibody titers among different groups, older adults (age, >65 years; n = 38) and patients with systemic lupus erythematosus (SLE, n = 14) were also investigated. A decline in the mean relative antibody titers was observed in older men compared with younger men and in patients with SLE compared with individuals aged <65 years. Although the antibody levels increased drastically following two BNT162b2 doses, they then declined rapidly. Furthermore, poor responders to the vaccination were observed. Repeated vaccinations are required to maintain high antibody levels.

Development and external validation of a deep learning-based computed tomography classification system for COVID-19.

BACKGROUND: We aimed to develop and externally validate a novel machine learning model that can classify CT image findings as positive or negative for SARS-CoV-2 reverse transcription polymerase chain reaction (RT-PCR). METHODS: We used 2,928 images from a wide variety of case-control type data sources for the development and internal validation of the machine learning model. A total of 633 COVID-19 cases and 2,295 non-COVID-19 cases were included in the study. We randomly divided cases into training and tuning sets at a ratio of 8:2. For external validation, we used 893 images from 740 consecutive patients at 11 acute care hospitals suspected of having COVID-19 at the time of diagnosis. The dataset included 343 COVID-19 patients. The reference standard was RT-PCR. RESULTS: In external validation, the sensitivity and specificity of the model were 0.869 and 0.432, at the low-level cutoff, 0.724 and 0.721, at the high-level cutoff. Area under the receiver operating characteristic was 0.76. CONCLUSIONS: Our machine learning model exhibited a high sensitivity in external validation datasets and may assist physicians to rule out COVID-19 diagnosis in a timely manner at emergency departments. Further studies are warranted to improve model specificity.

Radiological pleuroparenchymal fibroelastosis-like lesion in idiopathic interstitial pneumonias.

BACKGROUND: Pleuroparenchymal fibroelastosis (PPFE) is characterised by predominant upper lobe pleural and subpleural lung parenchymal fibrosis. Radiological PPFE-like lesion has been associated with various types of interstitial lung diseases. However, the prevalence and clinical significance of radiological PPFE-like lesion in patients with idiopathic interstitial pneumonias (IIPs) are not fully understood. We aimed to determine the prevalence and clinical impact on survival of radiological PPFE-like lesion in patients with IIPs. METHODS: A post-hoc analysis was conducted using data from the Japanese nationwide cloud-based database of patients with IIPs. All the patients in the database were diagnosed as having IIPs by multidisciplinary discussion. Patients diagnosed with idiopathic PPFE were excluded. Clinical data and chest computed tomography (CT) image of 419 patients with IIPs were analysed. The presence of radiological PPFE-like lesion was independently evaluated by two chest radiologists blind to the clinical data. RESULTS: Of the 419 patients with IIPs, radiological PPFE-like lesions were detected in 101 (24.1%) patients, mainly in idiopathic pulmonary fibrosis (IPF) and unclassifiable IIPs, but less in idiopathic nonspecific interstitial pneumonia. Prognostic analyses revealed that radiological PPFE-like lesion was significantly associated with poor outcome in patients with IIPs, which was independent of age, IPF diagnosis and %FVC. In survival analyses, the patients with radiological PPFE-like lesions had poor survival compared with those without (log-rank, p < 0.0001). Subgroup analyses demonstrated that radiological PPFE-like lesion was significantly associated with poor survival both in patients with IPF and those with unclassifiable IIPs. CONCLUSION: Radiological PPFE-like lesion is a condition that could exist in IIPs, mainly in IPF and unclassifiable IIPs. Importantly, the radiological PPFE-like lesion is a non-invasive marker to predict poor outcome in patients with IIPs, which should be carefully considered in clinical practice.

Characteristics of chronic obstructive pulmonary disease patients with robust progression of emphysematous change.

Emphysema is a major pathological change in chronic obstructive pulmonary disease (COPD). However, the annual changes in the progression of emphysematous have not been investigated. We aimed to determine possible baseline predicting factors of the change in emphysematous progression in a subgroup of COPD patients who demonstrated rapid progression. In this observational study, we analyzed patients with COPD who were followed up by computed tomography (CT) at least two times over a 3-year period (n = 217). We divided the annual change in the low attenuation area percentage (LAA%) into quartiles and defined a rapid progression group (n = 54) and a non-progression group (n = 163). Predictors of future changes in emphysematous progression differed from predictors of high LAA% at baseline. On multivariate logistic regression analysis, low blood eosinophilic count (odds ratio [OR], 3.22; P = 0.04) and having osteoporosis (OR, 2.13; P = 0.03) were related to rapid changes in emphysematous progression. There was no difference in baseline nutritional parameters, but nutritional parameters deteriorated in parallel with changes in emphysematous progression. Herein, we clarified the predictors of changes in emphysematous progression and concomitant deterioration of nutritional status in COPD patients.

Diffuse Alveolar Hemorrhage Associated with Dilated Cardiomyopathy and Sleep Apnea Syndrome.

We herein report a case of diffuse alveolar hemorrhage (DAH) associated with dilated cardiomyopathy (DCM) and sleep apnea syndrome (SAS) in a 47-year-old man. The patient exhibited recurring dyspnea and bloody sputum. Chest radiography showed bilateral diffuse infiltrative opacities without pleural effusion. A bronchoscopic analysis of bronchoalveolar lavage fluid revealed hemosiderin-laden macrophages. Based on these findings, he was diagnosed with DAH. Laboratory and pathological findings ruled out the possibility of collagen diseases and vasculitis. Overnight polysomnography revealed concomitant severe obstructive SAS. Treatment with continuous positive-pressure ventilation and pharmacological therapy for DCM prevented recurrence of DAH.

Thymus variants on imaging in patients with rheumatoid arthritis-clinical and immunological significance.

OBJECTIVES: We sought to clarify the presence of radiographic thymus variants using a scoring system, and their association with clinical and immunological features in RA patients. METHODS: A total of 387 RA patients were randomly selected from all patients visiting our department who underwent chest CT scanning, with exclusion of patients with thymoma or thymic cyst, or age < 30 years. Thymus size and attenuation score in axial CT images were quantitatively interpreted and assessed. Associations between immunophenotype data and clinical and serological features were analysed in a subset of patients. RESULTS: Thymic enlargement was found in 76 (19.6%) patients, and a thymus attenuation score ≥ 2 was found in 50 (12.9%) patients. The score was significantly associated with antibodies to ACPA positivity. Thymic enlargement was significantly associated with the proportions of CD4+ effector memory T cells. CONCLUSION: Radiographic thymus variants were frequently observed in RA patients and may reflect an abnormal immune response involved in the pathogenesis of RA.

Clinical impact of the radiological indeterminate for usual interstitial pneumonia pattern on the diagnosis of idiopathic pulmonary fibrosis.

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a fatal lung disease associated with significant morbidity and mortality. The international clinical practice guidelines for the diagnosis of IPF have recently been revised. METHODS: In this single-center retrospective study conducted between June 2006 and March 2018, 27 patients with a newly classified indeterminate for usual interstitial pneumonia (UIP) pattern on high-resolution computed tomography (HRCT) who had undergone surgical lung biopsy were enrolled at the Japanese Red Cross Medical Center. Clinical and pathological characteristics and prognosis were retrospectively analyzed from patient records. RESULTS: On the basis of multidisciplinary discussion (MDD), IPF was diagnosed in six patients (22%), unclassifiable interstitial pneumonia in 5 (19%), chronic hypersensitivity pneumonitis in 10 (37%), collagen vascular disease-associated interstitial lung disease in 5 (19%), and lymphoproliferative disorder in 1 (4%) patient. Ground-glass opacity, peribronchovascular distribution, upper or middle lobe distribution, mosaic attenuation, consolidation patterns, and honeycombing were found on HRCT. Histological UIP or probable UIP was observed in seven patients. The median survival time from the initial visit was 2770 days (92.3 months). There was a significant difference in survival time in the GAP stage and honeycombing on HRCT according to the log-rank test. CONCLUSIONS: Patients with an indeterminate for UIP pattern on HRCT were more likely to have non-IPF than IPF through pathological diagnosis and MDD. GAP stage and honeycombing on HRCT may be significant risk factors for all-cause mortality.

Radiographic features and poor prognostic factors of interstitial lung disease with nivolumab for non-small cell lung cancer.

Nivolumab can cause interstitial lung disease (ILD), which may be fatal; however, mortality risk factors have not been identified. This postmarketing study evaluated the poor prognostic factors of ILD in nivolumab-treated patients with non-small cell lung cancer (NSCLC) in Japan. Clinical and chest imaging findings for each ILD case were assessed by an expert central review committee, and prognosis was evaluated by radiographic findings, including the presence/absence of peritumoral ground-glass opacity (peritumoral-GGO). Poor prognostic factors were identified by univariate and multivariate Cox regression analysis. Of the 238 patients with nivolumab-induced ILD, 37 died. The main radiographic patterns of ILD were cryptogenic organizing pneumonia/chronic eosinophilic pneumonia-like (53.4%), faint infiltration pattern/acute hypersensitivity pneumonia-like (20.2%), diffuse alveolar damage (DAD)-like (10.9%), and nonspecific interstitial pneumonia-like (6.3%). The main poor prognostic factors identified were DAD-like pattern (highest hazard ratio: 10.72), ≤60 days from the start of nivolumab treatment to the onset of ILD, pleural effusion before treatment, lesion distribution contralateral or bilateral to the tumor, and abnormal change in C-reactive protein (CRP) levels. Of the 37 deaths due to ILD, 17 had DAD-like radiographic pattern, three had peritumoral-GGO, and five had a change in radiographic pattern from non-DAD at the onset to DAD-like. Patients with NSCLC who develop ILD during nivolumab treatment should be managed carefully if they have poor prognostic factors such as DAD-like radiographic pattern, onset of ILD ≤60 days from nivolumab initiation, pleural effusion before nivolumab treatment, lesion distribution contralateral or bilateral to the tumor, and abnormal changes in CRP levels.

Treatment and relapse of interstitial lung disease in nivolumab-treated patients with non-small cell lung cancer.

Nivolumab, a human monoclonal antibody against programmed death-1, is approved for the treatment of non-small cell lung cancer (NSCLC). Although nivolumab is generally well tolerated, it can cause interstitial lung disease (ILD), a rare but potentially fatal immune-related adverse event. Currently, there are limited data available on the treatment of nivolumab-induced ILD and its outcome. This retrospective cohort study based on a post-marketing study described the treatment of nivolumab-induced ILD and its outcome in NSCLC patients in Japan through the assessment of clinical and chest imaging findings by an expert central review committee. Treatment details for patients who experienced a relapse of ILD were also analyzed. Of the 238 patients identified as having nivolumab-induced ILD, 37 patients died of ILD. Corticosteroids were used in 207 (87.0%) patients. Of those, 172 (83.1%) patients responded well and survived and 35 (16.9%) died (most died during corticosteroid treatment). A total of nine patients experienced a relapse; at the time of relapse, four patients were taking nivolumab. Of those who were receiving corticosteroids at the time of relapse, three of four patients were taking low doses or had nearly completed dose tapering. All patients (except one, whose treatment was unknown) received corticosteroids for the treatment of relapse, but one patient died. Patients with NSCLC who experience nivolumab-induced ILD are treated effectively with corticosteroids, and providing extra care when ceasing or reducing the corticosteroid dose may prevent relapse of ILD.

Pleuroparenchymal fibroelastosis-like lesions in patients with interstitial pneumonia diagnosed by multidisciplinary discussion with surgical lung biopsy.

PURPOSE: The present study aimed to evaluate the significance of Pleuroparenchymal fibroelastosis (PPFE)-like lesions in predicting prognosis in patients with chronic interstitial pneumonia (IP). METHOD: The present study enrolled 207 patients with IP in whom surgical lung biopsy was performed. Among the patients enrolled in the present study, 77 had idiopathic pulmonary fibrosis (IPF), 15 had nonspecific interstitial pneumonia (NSIP), 13 had chronic hypersensitivity pneumonitis (CHP), 41 had connective tissue disease (CTD), three had PPFE, and 58 had unclassifiable diagnosis. The incidence, characteristics, and thickness of PPFE-like lesions were evaluated in each patient with IP. Additionally, the influence of PPFE-like lesions on the prognosis was also determined. RESULTS: Of 207 patients, 160 (77.3 %) showed PPFE-like lesions. The frequency of PPFE-like lesions was similar in patients with IPF, NSIP, CHP, CTD, and unclassifiable diagnosis (79.5 %, 79.5 %, 73.2 %, 65.9 %, and 81 %, respectively); however, PPFE-like lesions were present in all patients with PPFE (p = 0.42). Consequently, there was no significant difference in the characteristics of PPFE-like lesions among patients with all forms of IP, except PPFE. PPFE-like lesions were not a significant predictor of prognosis (hazard ratio [HR], 1.16; 95 % confidence interval [CI], 0.64-2.10, p = 0.62); however, patients with PPFE-like lesions under the aortic arch had significantly poorer prognoses (HR, 2.70; 95 % CI, 1.66-4.39, p < 0.001). For craniocaudal extent comparison, patients with IPF with PPFE-like lesions below the level of the carina had significantly poorer prognoses than those without PPFE-like lesions (p = 0.001, overall survival 53.1 and 80.6, respectively). CONCLUSION: PPFE-like lesions are common in patients with IP, and their characteristics were not significantly different among all forms of IP, except idiopathic PPFE. The broad extent of PPFE-like lesions is an important predictor of prognosis in patients with IPF.