Demystifying idiopathic interstitial pneumonia: time for more etiology-focused nomenclature in interstitial lung disease

2022 ◽  
Author(s):  
Nevins W. Todd ◽  
Sergei P. Atamas ◽  
Stella E. Hines ◽  
Irina G. Luzina ◽  
Nirav G. Shah ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Idiopathic Interstitial Pneumonia

scholarly journals The Association between MUC5B Rs35705950 and Risks of Idiopathic Interstitial Pneumonia, Systemic Sclerosis Interstitial Lung Disease, and Familial Interstitial Pneumonia: A Meta-Analysis

2020 ◽  
Author(s):  
Huai-Qing LOU ◽  
Chun-Xia HUANG ◽  
Guang-Yi LI ◽  
Ping LI ◽  
Shou-Ming ZHANG ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Lung Diseases ◽  
Meta Analysis ◽  
Biomedical Literature ◽  
Idiopathic Interstitial Pneumonia ◽  
Normal Lung ◽  
Cochrane Central Register

Background: Interstitial lung disease (ILD) is a category of chronic lung diseases with more than 200 subtypes. Idiopathic interstitial pneumonia (IIP), systemic sclerosis (SSc) ILD, and familial interstitial pneumonia (FIP) are three major groups of lung diseases with different causes or with unknown causes. Mucin5B (MUC5B) belongs to the mucin family, which contribute to the lubricating and viscoelastic properties of the whole saliva, normal lung mucus, and cervical mucus. The association between MUC5B rs35705950 and ILDs risks has been widely studied. However, the results were inconclusive and inconsistent. Methods: In the present meta-analysis, the database PubMed, Embase, Cochrane Central Register of Controlled Trials, CNKI and Chinese Biomedical Literature Database were searched till Aug 20th, 2018. Overall 16 publications with 28 studies, 76345 cases and 18402 controls were included. Results: The results indicated a significant increase of overall IIP risk for TT genotype and T allele of the rs35705950 in all genetic models (TT vs GG, OR=9.11; TT vs GT+TT, OR=5.80; GT+TT vs GG, OR=4.34; T vs G, OR=4.03. P<0.0001). Subgroup analysis by subtypes of IIP revealed higher risks of TT genotype and T allele for IPF and iNSIP (P<0.05). A significant increase of FIP risk was also found for the TT genotype and T allele of the rs35705950 (TT vs GG, OR=17.08; GT+TT vs GG, OR=6.02; T vs G, OR=1.64.P<0.05). Conclusion: No significant relations existed between the rs35705950 and SSc-ILD risks. MUC5B rs35705950 might be a predictor for the susceptibility of IIP and FIP.

scholarly journals Successful treatment of interstitial pneumonia with autoimmune features using rituximab as salvage therapy

2021 ◽  
Author(s):  
Abeline Kapuczinski ◽  
Michel Cotils ◽  
Yannick Gombeir
Keyword(s):  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Connective Tissue Disease ◽  
Successful Treatment ◽  
Salvage Therapy ◽  
Rescue Therapy ◽  
Specific Treatment ◽  
Idiopathic Interstitial Pneumonia

Interstitial pneumonia with autoimmune features is a concept to identify patients with idiopathic interstitial pneumonia and features suggestive of a connective tissue disease. There is no specific treatment which consists mainly of corticosteroids and immunossupressive agents. Rituximab is an effective rescue therapy for refractory interstitial lung disease.

scholarly journals Limitations to the 6-Minute Walk Test in Dermatomyositis with Interstitial Lung Disease in comparison with Idiopathic Interstitial pneumonia

2013 ◽  
Vol 7 ◽  
pp. CCRPM.S10764 ◽  
Author(s):  
Fujiko Someya ◽  
Naoki Mugii
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Exercise Capacity ◽  
Interstitial Pneumonia ◽  
Idiopathic Interstitial Pneumonia ◽  
Walk Test ◽  
Exertional Dyspnea ◽  
Lung Dysfunction ◽  
6 Minute Walk Test ◽  
Minute Walk

Although the relationship between muscle strength and exercise capacity has been demonstrated in dermatomyositis without lung dysfunction, little is known about the association between exercise capacity and interstitial lung disease in dermatomyositis. Eleven patients with dermatomyositis with interstitial lung disease without the manifestation of muscle weakness and 12 patients with idiopathic interstitial pneumonia underwent the 6-minute walk test (6MWT). PaO2, creatine kinase, percentage predicted 6MWT distance (6MWD%), and SpO2 at rest were similar between patients. Percentage predicted vital capacity, carbon monoxide diffusing capacity (DLCO%), and SpO2 after 6MWT were higher and exertional dyspnea was lower in patients with dermatomyositis than in patients with idiopathic interstitial pneumonia. SpO2 after 6MWT was positively correlated with 6MWD% in patients with dermatomyositis, while DLCO% and PaO2 were positively correlated with 6MWD% in patients with idiopathic interstitial pneumonia. Lung dysfunction in dermatomyositis might not be a major limitation factor in exercise capacity.

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