scholarly journals Interstitial Lung Disease in Childhood: Clinical and Genetic Aspects

2015 ◽  
Vol 9s1 ◽  
pp. CCRPM.S23282 ◽  
Author(s):  
Hiroshi Kitazawa ◽  
Shigeo Kure
Keyword(s):  
Genetic Testing ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Clinical Features ◽  
Clinical Studies ◽  
Treatment Strategies ◽  
Classification Criteria ◽  
Heterogeneous Group ◽  
New Classification ◽  
Respiratory Disorders

Interstitial lung disease (ILD) in childhood is a heterogeneous group of rare pulmonary conditions presenting chronic respiratory disorders. Many clinical features of ILD still remain unclear, making the treatment strategies mainly investigative. Guidelines may provide physicians with an overview on the diagnosis and therapeutic directions. However, the criteria used in different clinical studies for the classification and diagnosis of ILDs are not always the same, making the development of guidelines difficult. Advances in genetic testing have thrown light on some etiologies of ILD, which were formerly classified as ILDs of unknown origins. The need of genetic testing for unexplained ILD is growing, and new classification criteria based on the etiology should be adopted to better understand the disease. The purpose of this review is to give an overview of the clinical and genetic aspects of ILD in children.

scholarly journals AB0660 COVID-19 SHARES CLINICAL FEATURES WITH ANTI-MELANOMA DIFFERENTIATION ASSOCIATED PROTEIN 5 POSITIVE DERMATOMYOSITIS AND ADULT STILL’S DISEASE

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1362.1-1362
Author(s):  
Y. Kondo ◽  
Y. Kaneko ◽  
H. Takei ◽  
H. Tamai ◽  
T. Takeuchi
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Clinical Features ◽  
Serum Ferritin ◽  
Rheumatic Diseases ◽  
Macrophage Activation ◽  
Cytokine Storm ◽  
Still’S Disease ◽  
Still's Disease ◽  
Adult Still's Disease

Background:The coronavirus disease 2019 (COVID-19), caused by a novel corona virus named SARS-CoV-2, has emerged as a global pandemic. Severe inflammatory process is one of main pathogenesis of COVID-19 and this involves cytokine storm along with overactivation of macrophage. On another front, cytokine storm with macrophage activation is frequently observed in various connective tissue diseases including dermatomyositis with positive antimelanoma differentiation-associated protein 5 (anti-MDA5) autoantibodies and adult Still’s disease. Macrophage activation during inflammatory states is partially characterized by an increased serum ferritin levels and hyperferritinaemia and characteristics shared by the three diseases are a topic of interest to rheumatologists, however, no study has evaluated anti-MDA5-positive dermatomyositis and adult Still’s disease in comparison to COVID-19.Objectives:The aim of this study was to highlight the homology and heterogeneity of COVID-19, anti-MDA5 dermatomyositis, and adult Still’s disease by comparing clinical pictures of each disease in order to discuss their respective pathogeneses.Methods:We reviewed consecutive, newly diagnosed, untreated patients with COVID-19, anti-MDA5 dermatomyositis, or adult Still’s disease. We compared their clinical, laboratory, and radiological characteristics, including the prevalence of macrophage activation syndrome and lung involvement in each disease.Results:The numbers of patients with COVID-19, anti-MDA5 dermatomyositis, and adult-onset Still’s disease with hyperferritinaemia (serum ferritin ≥ 500ng/dL) who were included for main analysis were 22, 14, and 59, respectively. COVID-19 and adult Still’s disease both featured hyperinflammatory status, such as high fever and elevated serum C-reactive protein, whereas COVID-19 and anti-MDA5 dermatomyositis both presented with severe interstitial lung disease and hypoxaemia. While two-thirds of the patients in each group met the criteria for macrophage-activated syndrome that is used in systemic juvenile idiopathic arthritis, the HScore, an indicator of haemophagocytic lymphohistiocytosis, was low in anti-MDA5 dermatomyositis and COVID-19 even in severe or critical cases. The findings of chest computed tomography were similar between COVID-19 and anti-MDA5 dermatomyositis (Figure 1).Conclusion:COVID-19 shared clinical features with rheumatic diseases characterised by hyperferritinaemia, including anti-MDA5 dermatomyositis and adult Still’s disease. These findings should be investigated further in order to shed light on the pathogenesis of not only COVID-19 but also the aforementioned rheumatic diseases.References:[1]Mehta P, McAuley DF, Brown M, et al. COVID-19: consider cytokine storm syndromes and immunosuppression. Lancet. (2020) 395: 1033-4.[2]Gono T, Sato S, Kawaguchi Y, et al. Anti-MDA5 antibody, ferritin and IL-18 are useful for the evaluation of response to treatment in interstitial lung disease with anti-MDA5 antibody-positive dermatomyositis. Rheumatology (Oxford). 2012; 51(9):1563-70.Figure 1.Imaging characteristics of chest CT scans in patients with COVID-19, anti-MDA5 dermatomyositis, and adult Still’s disease A)Bilateral ground-glass and consolidative opacities with peripheral distribution in COVID-19. B)Bilateral ground-glass opacities with peripheral consolidations in anti-MDA5 dermatomyositis. C)Pleural effusion with pleural thickening on the left side in adult Still’s disease.Disclosure of Interests:Yasushi Kondo: None declared., Yuko Kaneko: None declared., Hisoshi Takei: None declared., Hiroya Tamai: None declared., Tsutomu Takeuchi Grant/research support from: received research grants outside the submitted work from Abbvie, Astra Zeneca, Bristol Myers Squibb, Chugai Pharmaceutical, Eisai Pharmaceutical, Janssen Pharmaceutical, Mitsubishi Tanabe Pharma Corporation, Novartis, Takeda Pharmaceutical, Abbott Japan Co., Ltd., Astellas Pharma, Ltd., Daiichi Sankyo, Pfizer, Sanofi–Aventis, Santen Pharmaceutical, Teijin Pharma Ltd., Asahikasei Pharma Corp., SymBio Pharmaceuticals Ltd., Celtrion, Nipponkayaku Co. Ltd., Eli Lilly Japan, and Taisho Toyama Pharmaceutical.

scholarly journals THU0305 PREVALENCE AND CLINICAL OUTCOME OF INTERSTITIAL LUNG DISEASE IN ANCA ASSOCIATED VASCULITIS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 381.2-381
Author(s):  
J. Fernandes Serodio ◽  
J. Hernández-Rodríguez ◽  
G. Espígol-Frigolé ◽  
M. Alba ◽  
J. Marco-Hernández ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Cox Regression ◽  
Clinical Course ◽  
Usual Interstitial Pneumonia ◽  
Classification Criteria ◽  
Lung Involvement ◽  
Anca Associated Vasculitis ◽  
Inflammatory Infiltrates

Background:Lung involvement is frequent in ANCA-associated vasculitis (AAV). Classical lung manifestations consist of capillaritis with lung haemorrhage, inflammatory infiltrates and nodules. Interstitial lung disease (ILD) is increasingly recognized among patients with AAV. However, little is known concerning risk factors and clinical course of these patients.Objectives:The aim of our study was to characterize the prevalence and clinical course of ILD in patients with AAV.Methods:We have performed a clinical retrospective single-centre observational analysis (1990-2019) of all patients with the diagnosis of microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA) diagnosed according to 2018 Draft Classification Criteria for GPA and MPA1. Demographic, clinical and immunologic data were reviewed. Radiologic pattern of ILD were assessed by high-resolution-CT. Main outcome evaluated was overall-all survival.Results:The study population consisted of 123 patients, 56% female, aged 59.3±18.2 years old at the time of diagnosis. Clinical diagnosis was of MPA in 54% of patients and GPA in 46%. While 108 (88%) ANCA positive patients had PR3 (n=25) or MPO (n=83), 15 (12%) patients had negative or atypical ANCA. Any lung involvement was present in 82 (71%) and ILD was identified in 24 (20%) of all patients. ILD pattern was of usual interstitial pneumonia (UIP) in 12 patients, non-specified interstitial pneumonia (NSIP) in 9 and chronic organizing pneumonia (OP) in 3. There was an association between the presence of ILD and ANCA specificity: MPO were present in 100% of patients with UIP and in 75% of patients with NSIP/OP (p=0.017). Bronchiectasis were more prevalent among patients with ILD (19/24; p<0.001). During the median follow-up time period of 68 (23-126) months, mortality was of 42% among patients with ILD-AAV compared with 11% in no ILD-AAV (log-rank p=0.0001). On the multivariate Cox regression model, ILD was an independent predictor of mortality HR 2.95 (95%CI 1.09-7.96; p=0.033).Conclusion:ILD is a frequent manifestation of MPA and GPA patients. The presence of ILD, particularly UIP, is associated with ANCA-MPO and is a predictor of mortality. Therefore, a better management of fibrotic lung involvement in AAV is warranted.References:[1]Robson JC, Grayson PC, Ponte C, et al. Draft classification criteria for the ANCA associated vasculitides. Ann Rheum Dis 2018;77 (suppl 2):60-1.Disclosure of Interests:João Fernandes Serodio: None declared, José Hernández-Rodríguez: None declared, Georgina Espígol-Frigolé: None declared, Marco Alba: None declared, Javier Marco-Hernández: None declared, Marcelo Sánchez: None declared, Fernanda Hernández-González: None declared, Jacobo Sellarés: None declared, Maria C. Cid Grant/research support from: Kiniksa Pharmaceuticals, Consultant of: Janssen, Abbvie, Roche, GSK, Speakers bureau: Vifor, Sergio Prieto-González: None declared

6050 Clinical features of interstitial lung disease induced by FOLFOX or FOLFIRI for colorectal cancer

2009 ◽  
Vol 7 (2) ◽  
pp. 336
Author(s):  
T. Shimura ◽  
N. Fuse ◽  
T. Yoshino ◽  
K. Minashi ◽  
M. Tahara ◽  
...  
Keyword(s):  
Colorectal Cancer ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Clinical Features

Relationship between quantitative radiographic assessments of interstitial lung disease and physiological and clinical features of systemic sclerosis

2014 ◽  
Vol 75 (2) ◽  
pp. 374-381 ◽  
Author(s):  
Donald P Tashkin ◽  
Elizabeth R Volkmann ◽  
Chi-Hong Tseng ◽  
Hyun J Kim ◽  
Jonathan Goldin ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Clinical Features

scholarly journals Clinical features of Anti-synthetase syndrome associated interstitial lung disease: a retrospective cohort in China

2020 ◽  
Author(s):  
Xi Zhan ◽  
YAN Wei ◽  
WANG Ying ◽  
LI Qing ◽  
SHI Xuhua ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Clinical Features ◽  
Retrospective Cohort ◽  
Small Sample Size ◽  
Inflammatory Myopathy ◽  
Small Sample ◽  
Positive Group ◽  
The Difference

Abstract Background Antisynthetase Syndrome (ASSD) is a chronic autoimmune condition characterized by antibodies directed against an aminoacycl transfer RNA synthetase (ARS) along with a group of clinical features including the classical clinical triad: inflammatory myopathy, arthritis, interstitial lung disease (ILD). ASSD is highly heterogenous due to the different organs involvement, and ILD is the main cause of mortality and function loss, which presents as different patterns when diagnosed. We designed this retrospective cohort to describe the clinical features and disease behavior of the ASSD) associated ILD. Methods Retrospectively collected the data of 108 cases of ASSD associated ILD in Beijing Chaoyang Hospital since 2017.12 to 2019.3. Data including age, gender, physical examination, laboratory test, pulmonary function and High Resolution Computer Tomography (HRCT), treatment, were obtained from the Electronic Medical Record (EMR) system. Patients were divided into 5 groups according to the distinct Aminoacyl tRNA synthetases (ARS) antibodies, and all patients had a multiple discussion team (MDT) to make a radiological and pathological diagnosis of the ILD pattern. Each patient had at least 1 follow up for no less than 6 months. Patients with missing data of MDT evaluation or follow up were excluded. Results: 108 consecutive patients were recruited in this retrospective cohort. 30 cases received bronchoscopy for a transbronchial lung biopsy. 3 had bronchoscopy for a transbronchial cryobiopsy. 33 were with JO-1 positive and 30 were with PL-7 positive. 23 with EJ positive, 13 with PL12 positive and 9 with OJ positive. JO-1 group had a significant higher rate of mechanic’s hand (57.6%) than other 4 groups, the skin involvement (Gottron Papules and/or Heliotrope Rash) was found in 38 (35%) and no difference was found among the 5 groups. Polymyositis/Dermatomyositis (PM/DM) was diagnosed in 25 (23.1%) patients and no difference was observed among the 5 groups. The anti-PL7 positive group had a higher frequency of UIP pattern (13.3%) than other 4 groups but the difference was not significant due to the small sample size, and EJ group had the most frequent OP pattern(78.2%), which was significantly higher than PL-7 group (P<0.001) and PL-12 group (P=0.025). The median follow-up time were 10.7 months. All received prednisone treatment, with or without immunosuppressants ,and at the 6-month-follow up, the JO-1 group and EJ group had the significantly higher improvement of forced vital capacity that the other 3 groups (P<0.05), and PL-7 group had the lowest FVC improvement (P<0.05). The anti-Jo1-positive group and anti-EJ-positive group had significantly higher anti-RO52-positive occurrence than other 3 groups (P<0.05).Conclusion Anti PL-7 antibody had the same frequency as anti-JO1 in ASSD-ILD, in which the ILD pattern was different with distinct anti-ARS antibodies. All ASSD-ILD responded to therapy of steroids, with or without immunosuppressants. PL-7 group had a highest occurrence of UIP pattern, and significantly lower respondence to therapy.

Sign in / Sign up

Export Citation Format

Share Document