Safety and Effectiveness of Mycophenolate Mofetil in Interstitial Lung Diseases: Insights from a Machine Learning Radiographic Model

Respiration ◽  
2021 ◽  
pp. 1-10
Author(s):  
Theodoros Karampitsakos ◽  
Christina Kalogeropoulou ◽  
Vasilios Tzilas ◽  
Ourania Papaioannou ◽  
Alexandra Kazantzi ◽  
...  

<b><i>Introduction:</i></b> Treatment of interstitial lung diseases (ILDs) other than idiopathic pulmonary fibrosis (IPF) often includes systemic corticosteroids. Use of steroid-sparing agents is amenable to avoid potential side effects. <b><i>Methods:</i></b> Functional indices and high-resolution computed tomography (HRCT) patterns of patients with non-IPF ILDs receiving mycophenolate mofetil (MMF) with a minimum follow-up of 1 year were analyzed. Two independent radiologists and a machine learning software system (Imbio 1.4.2.) evaluated HRCT patterns. <b><i>Results:</i></b> Fifty-five (<i>n</i> = 55) patients were included in the analysis (male: 30 [55%], median age: 65.0 [95% CI: 59.7–70.0], mean forced vital capacity %predicted [FVC %pred.] ± standard deviation [SD]: 69.4 ± 18.3, mean diffusing capacity of lung for carbon monoxide %pred. ± SD: 40.8 ± 14.3, hypersensitivity pneumonitis: 26, connective tissue disease-ILDs [CTD-ILDs]: 22, other ILDs: 7). There was no significant difference in mean FVC %pred. post-6 months (1.59 ± 2.04) and 1 year (−0.39 ± 2.49) of treatment compared to baseline. Radiographic evaluation showed no significant difference between baseline and post-1 year %ground glass opacities (20.0 [95% CI: 14.4–30.0] vs. 20.0 [95% CI: 14.4–25.6]) and %reticulation (5.0 [95% CI: 2.0–15.6] vs. 7.5 [95% CI: 2.0–17.5]). A similar performance between expert radiologists and Imbio software analysis was observed in assessing ground glass opacities (intraclass correlation coefficient [ICC] = 0.73) and reticulation (ICC = 0.88). Fourteen patients (25.5%) reported at least one side effect and 8 patients (14.5%) switched to antifibrotics due to disease progression. <b><i>Conclusion:</i></b> Our data suggest that MMF is a safe and effective steroid-sparing agent leading to disease stabilization in a proportion of patients with non-IPF ILDs. Machine learning software systems may exhibit similar performance to specialist radiologists and represent fruitful diagnostic and prognostic tools.

Diagnostics ◽  
2021 ◽  
Vol 11 (3) ◽  
pp. 439
Author(s):  
Donato Lacedonia ◽  
Giulia Scioscia ◽  
Angelamaria Giardinelli ◽  
Carla Maria Irene Quarato ◽  
Ennio Vincenzo Sassani ◽  
...  

Transthoracic ultrasound (TUS) is a readily available imaging tool that can provide a quick real-time evaluation. The aim of this preliminary study was to establish a complementary role for this imaging method in the approach of interstitial lung diseases (ILDs). TUS examination was performed in 43 consecutive patients with pulmonary fibrosis and TUS findings were compared with the corresponding high-resolution computed tomography (HRCT) scans. All patients showed a thickened hyperechoic pleural line, despite no difference between dominant HRCT patterns (ground glass, honeycombing, mixed pattern) being recorded (p > 0.05). However, pleural lines’ thickening showed a significant difference between different HRCT degree of fibrosis (p < 0.001) and a negative correlation with functional parameters. The presence of >3 B-lines and subpleural nodules was also assessed in a large number of patients, although they did not demonstrate any particular association with a specific HRCT finding or fibrotic degree. Results allow us to suggest a complementary role for TUS in facilitating an early diagnosis of ILD or helping to detect a possible disease progression or eventual complications during routine clinical practice (with pleural line measurements and subpleural nodules), although HRCT remains the gold standard in the definition of ILD pattern, disease extent and follow-up.


Author(s):  
Aryeh Fischer ◽  
Mahalakshmi Krishnamoorthy ◽  
Amy L. Olson ◽  
Joshua J. Solomon ◽  
Evans R. Fernandez-Perez ◽  
...  

2019 ◽  
Vol 8 (5) ◽  
pp. 643 ◽  
Author(s):  
Ekaterina Krauss ◽  
Maike Froehler ◽  
Maria Degen ◽  
Poornima Mahavadi ◽  
Ruth C. Dartsch ◽  
...  

Background: New biomarkers are urgently needed to facilitate diagnosis in Interstitial Lung Diseases (ILD), thus reducing the need for invasive procedures, and to enable tailoring and monitoring of medical treatment. Methods: In this study we investigated if patients with idiopathic pulmonary fibrosis (IPF; n = 21), non-IPF ILDs (n = 57) and other lung diseases (chronic obstructive pulmonary disease (COPD) n = 24, lung cancer (LC) n = 16) as well as healthy subjects (n = 20) show relevant differences in exhaled NO (FeNO; Niox MINO), or in eicosanoid (PGE2, 8-isoprostane; enzyme-linked immunosorbent assay (ELISA)) levels as measured in exhaled breath condensates (EBC) and bronchoalveolar lavage fluids (BALF). Results: There was no significant difference in FeNO values between IPF, non-IPF ILDs and healthy subjects, although some individual patients showed highly elevated FeNO. On the basis of the FeNO signal, it was neither possible to differentiate between the kind of disease nor to detect exacerbations. In addition, there was no correlation between FeNO values and lung function. The investigation of the eicosanoids in EBCs was challenging (PGE2) or unreliable (8-isoprostane), but worked out well in BALF. A significant increase of free 8-isoprostane was observed in BALF, but not in EBCs, of patients with IPF, hypersensitivity pneumonitis (HP) and sarcoidosis, possibly indicating severity of oxidative stress. Conclusions: FeNO-measurements are not of diagnostic benefit in different ILDs including IPF. The same holds true for PGE2 and 8-isoprostane in EBC by ELISA.


2013 ◽  
Vol 66 (suppl. 1) ◽  
pp. 29-33
Author(s):  
Ruza Stevic ◽  
Vucinic Mihailovic ◽  
Dragana Jovanovic ◽  
Nada Vasic

Introduction. Interstitial lung diseases include the entities of idiopathic pulmonary fibrosis, nonspecific interstitial pneumonia, cryptogenic organizing pneumonia, acute interstitial pneumonia, respiratory bronchiolitis-associated interstitial lung disease, desquamative interstitial pneumonia, and lymphocytic interstitial pneumonia. Recent introduction of high-resolution computed tomography has made the diagnosis of interstitial lung disease much easier. Usual interstitial pneumonia A predominantly subpleural reticulation and honeycombing at the basal posterior part of the lung with a progression to anterior and superior parts are characteristic of usual interstitial pneumonia/ idiopathic pulmonary fibrosis. Nonspecific interstitial pneumonia Typical findings of nonspecific interstitial pneumonia are bilateral, relatively symmetrical subpleural ground glass opacifications and irregular linear opacities. Desquamative interstitial pneumonia is characterized by diffuse symmetrical ground glass opacifications. Respiratory bronchiolitisassociated interstitial lung disease Centrilobular nodules and irregular ground glass opacifications are present. Cryptogenic organizing pneumonia Subpleural and peribronchial consolidations are prominent findings that are not present in other idiopathic interstitial pneumonias. Acute interstitial pneumonia. Bilateral ground-glass opacifications are present and areas of peripheral consolidations may also be seen in acute interstitial pneumonia. Lymphocytic interstitial pneumonia. Diffuse or patchy areas of ground glass opacification with centrilobular nodules and occasionally well-defined cysts are seen. Conclusion. Imaging plays a crucial role in identifying interstitial lung diseases but precise diagnosis requires a dynamic interdisciplinary approach that correlates clinical, radiological and pathologic features.


2020 ◽  
Vol 113 (10) ◽  
pp. 630-641
Author(s):  
Charles Fauvel ◽  
Olivier Raitière ◽  
Nassima Si Belkacem ◽  
Stéphane Dominique ◽  
Elise Artaud-Macari ◽  
...  

2021 ◽  
Author(s):  
Yoichi Tagami ◽  
Yu Hara ◽  
Kota Murohashi ◽  
Ryo Nagasawa ◽  
Yurika Nishikawa ◽  
...  

Abstract Introduction: Serum Krebs von den Lungen-6 (KL-6) measurement is widely used to assess disease activity or prognosis in patients with interstitial lung diseases (ILDs). However, the clinical differences between high and low serum KL-6 levels at the time of acute exacerbation (AE) of ILD are not well known.Methods: Clinical parameters including age, sex, Charlson Comorbidity Index score (CCIS), blood biomarkers, high-resolution CT findings, and disease mortality were retrospectively compared between high and low KL-6 (cut-off value: 1000 U/mL) patients at the time of diagnosis of AE of ILDs.Results: 38 high serum KL-6 and 57 low serum KL-6 patients were included. There was no significant difference in 6-month mortality between them (P = 0.685). Whereas serum lactate dehydrogenase was a significant predictor of 6-month mortality in the high serum KL-6 patients (odds ratio (OR), 1.006; 95% confidence interval (CI), 1.003-1.009; P < 0.001), CCIS (OR, 1.502; 95% CI, 1.242-1.838; P < 0.001) and sex (OR, 5.751; 95% CI, 1.121-105.163; P = 0.033) were significant predictors in low serum KL-6 patients. In addition, the incidences of congestive heart failure, symptomatic chronic pulmonary disease, cerebrovascular disease, and second metastatic solid tumours were significantly higher in non-survivors with low serum KL-6 than in other groups (p < 0.05).Conclusions: The clinical features in patients with AEs of ILDs may differ depending on the serum KL-6 level, and clinicopathological examination according to this subtyping guided by the serum KL-6 level is essential.


2019 ◽  
Vol 20 (1) ◽  
pp. 35-39
Author(s):  
Nannapat Trisiripanit ◽  
Soraya Suntornsawat ◽  
Worapan Phonkaew

Diffuse interstitial lung diseases (ILDs) include more than hundreds of diseases which have different causes or underlying, target groups, signs and symptoms, clinical courses, radiographic appearances, treatments, and prognosis. Among them, idiopathic pulmonary fibrosis (IPF) is the most fatal, with prognosis worse than many cancers. After decades of no specific treatment, new medications that may help slow the progression of the fibrosis have been introduced and approved in some countries.  Similar to corticosteroid, anti-inflammatory and immunosuppressive drugs which are used to treat some ILDs; these antifibrotic medications could cause certain side effects. In contrast, the cost of treatment is much higher. To monitor ILDs in terms of incidence, demographic and geographic distributions, and life expectancy;  T.S.T. is developing a national ILD database. To ensure that this data base will provide the most accurate information, diagnosis should be as much precise as possible. However, the diagnoses of most ILDs are multidisciplinary. With the facts that surgical lung biopsies are available in patients fewer than 20% in most countries1, HRCT plays important role in showing disease characters and extension. Certain HRCT patterns are accepted to replace surgical lung biopsies (SLB) in some diseases. Unfortunately typical diagnostic HRCT patterns to replace SLB are not possible in all cases; for example, only about half of usual interstitial pneumonia2. Initially, diagnosis could not be made in some cases whose HRCT patterns are not specific and other clinical information is not sufficient. Longitudinal study by following up HRCTs and adding subsequently exhibited clinical data, or even surgical lung biopsy, could eventually establish the diagnosis.  These patients need a system that provide regular clinical and HRCT follow up, also the multidisciplinary team to evaluate those newly acquired clinical and radiographic information . As an important role in managing patients with ILDs, standard HRCT is required to ensure that the initial examination will provide sufficient radiographic information, both the initial and follow-up examinations could be compared,  the interpretation of all examinations is reproducible, and it could be performed in most institutes. To develop national standard HRCT protocol; current situation of interstitial lung diseases in Thailand,the purpose to develop the protocol, and a probable draft of the standard protocol (made by the committee from RCRT) were presented to a panel consisted of thoracic radiologist experts from all parts of Thailand in a meeting held on 11 January 2019 by Foundation of Orphan and Rare Lung Disease (FORD) and Imaging Academic Outreach Center (iAOC).  Knowledge sharing, benefits and disadvantages of the drafted protocol were discussed. Adjustment was done based on feasibility, coverage of all lung diseases, diagnostic accuracy, and radiation safety. The panel provided a standard protocol describing scan coverage, technique, collimation, rotation time, pitch, radiation dose, and reconstruction images. The standard protocol recommends a mandatory acquisition for the first HRCT and optional or additional ones for the follow-up or particular cases.


2021 ◽  
Vol 2021 ◽  
pp. 1-9
Author(s):  
Yoichi Tagami ◽  
Yu Hara ◽  
Kota Murohashi ◽  
Ryo Nagasawa ◽  
Yurika Nishikawa ◽  
...  

Background. Serum Krebs von den Lungen-6 (KL-6) measurement is widely used to assess disease activity or prognosis in patients with interstitial lung diseases (ILDs). However, the clinical differences between high and low serum KL-6 levels at the time of acute exacerbation (AE) of ILD are not well known. Methods. Clinical parameters including age, sex, Charlson Comorbidity Index score (CCIS), blood biomarkers, high-resolution CT findings, and disease mortality were retrospectively compared between high and low KL-6 (cutoff value: 1000 U/mL) patients at the time of diagnosis of AE of ILDs. Results. Thirty-eight high serum KL-6 and 57 low serum KL-6 patients were included. There was no significant difference in 6-month mortality between them ( P  = 0.685), whereas serum lactate dehydrogenase was a significant predictor of 6-month mortality in the high serum KL-6 patients (odds ratio (OR): 1.006; 95% confidence interval (CI): 1.003–1.009; P  < 0.001), and CCIS (OR: 1.502; 95% CI: 1.242–1.838; P  < 0.001) and sex (OR: 5.751; 95% CI: 1.121–105.163; P  = 0.033) were significant predictors in low serum KL-6 patients. In addition, the incidences of congestive heart failure, symptomatic chronic pulmonary disease, cerebrovascular disease, and second metastatic solid tumours were significantly higher in nonsurvivors with low serum KL-6 than in other groups ( P  < 0.05). Conclusions. The clinical features in patients with AEs of ILDs may differ depending on the serum KL-6 level, and clinicopathological examination according to this subtyping guided by the serum KL-6 level is essential.


Sign in / Sign up

Export Citation Format

Share Document