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Last update 08 May 2025

Interstitial lung disease due to connective tissue disease

Last update 08 May 2025

Basic Info

Synonyms

Interstitial lung disease due to connective tissue disease, Interstitial lung disease due to connective tissue disease (disorder), interstitial lung disease due to connective tissue disease

+ [1]

Introduction-

Drugs associated with Interstitial lung disease due to connective tissue disease

Belimumab

Target

BAFF

Mechanism

BAFF inhibitors

Active Org.

GlaxoSmithKline (Ireland) Ltd. [+3]

Originator Org.

GSK Plc

Active Indication

Lupus Nephritis [+20]

Inactive Indication

Allograft Rejection [+10]

Drug Highest PhaseApproved

First Approval Ctry. / Loc.

United States

First Approval Date09 Mar 2011

Genakumab

Target

IL-1β

Mechanism

IL-1β inhibitors

Active Org.

Changchun Genescience Pharmaceuticals Co., Ltd.

Originator Org.

Changchun Genescience Pharmaceuticals Co., Ltd.

Active Indication

Arthritis, Gouty [+8]

Inactive Indication-

Drug Highest PhaseNDA/BLA

First Approval Ctry. / Loc.-

First Approval Date20 Jan 1800

Human cord derived MSC cell(Shanghai Aisar Biotechnology)

Target-

Mechanism

Stem cell replacements

Active Org.

IxCell Biotechnology Co., Ltd.

Originator Org.

IxCell Biotechnology Co., Ltd.

Active Indication

Interstitial lung disease due to connective tissue disease [+1]

Inactive Indication

Osteoarthritis, Knee

Drug Highest PhasePhase 1

First Approval Ctry. / Loc.-

First Approval Date20 Jan 1800

Clinical Trials associated with Interstitial lung disease due to connective tissue disease

NCT06823063

/ Not yet recruitingPhase 1

A Phase I Clinical Trial to Evaluate the Safety and Tolerability of a Single Dose of Human Umbilical Cord Mesenchymal Stem Cell Injection (IxCell hUC-MSC-P) in Patients With Connective Tissue Disease-associated Interstitial Lung Disease

To evaluate the safety and tolerability of IxCell hUC-MSC-P in the treatment of patients with connective tissue disease-related interstitial lung disease.
To evaluate the efficacy, pharmacokinetics and immunogenicity of IxCell hUC-MSC-P in the treatment of connective tissue disease-associated interstitial lung disease (CTD-ILD).

Start Date01 Apr 2025

Sponsor / Collaborator

IxCell Biotechnology Co., Ltd.

ChiCTR2500098990

/ RecruitingNot ApplicableIIT

Study on the Efficacy and Safety of Pirfenidone and Nintedanib in Connective Tissue Disease-Interstitial Lung Disease (CTD-ILD)

Start Date17 Mar 2025

Sponsor / Collaborator

Tongji Medical College of Huazhong University of Sci & Tech

ChiCTR2500096430

/ SuspendedNot ApplicableIIT

Analysis of the Incidence and Types of Interstitial Lung Disease (ILD) In Patients with Connective Tissue Disease-Associated Interstitial Lung Disease (CTD-ILD), Risk Factors and Prognosis Analysis Model: A Real-World Study Based on the National Rheumatic Disease Data Center-CTD-ILD Database

Start Date18 Feb 2025

Sponsor / Collaborator

Peking Union Medical College Hospital

100 Clinical Results associated with Interstitial lung disease due to connective tissue disease

100 Translational Medicine associated with Interstitial lung disease due to connective tissue disease

0 Patents (Medical) associated with Interstitial lung disease due to connective tissue disease

482

Literatures (Medical) associated with Interstitial lung disease due to connective tissue disease

31 Dec 2025·Pulmonology

Early detection of interstitial lung disease in rheumatic diseases: A joint statement from the Portuguese Pulmonology Society, the Portuguese Rheumatology Society, and the Portuguese Radiology and Nuclear Medicine Society

Review

Author: Pereira, J.M. ; Freitas, S. ; Fernandes, M.O. ; Branco, J.C. ; Duarte, A.C. ; Marques, I.D. ; Cordeiro, A. ; Morais, A. ; Khmelinskii, N. ; Bernardes, M. ; Pinto, P. ; Borba, A. ; Pinto-Basto, R. ; Alfaro, T. ; Ruano, C. ; Calha, J. ; Campainha, S. ; Salvador, M.J.

INTRODUCTIONInterstitial lung disease (ILD) contributes significantly to morbidity and mortality in connective tissue disease (CTD). Early detection and accurate diagnosis are essential for informing treatment decisions and prognosis in this setting. Clear guidance on CTD-ILD screening, however, is lacking.OBJECTIVETo establish recommendations for CTD-ILD screening based on the current evidence.METHODFollowing an extensive literature research and evaluation of articles selected for their recency and relevance to the characterization, screening, and management of CTD-ILD, an expert panel formed by six pulmonologists from the Portuguese Society of Pulmonology, six rheumatologists from the Portuguese Society of Rheumatology, and six radiologists from the Portuguese Society of Radiology and Nuclear Medicine participated in a multidisciplinary discussion to produce a joint statement on screening recommendations for ILD in CTD.RESULTSThe expert panel achieved consensus on when and how to screen for ILD in patients with systemic sclerosis, rheumatoid arthritis, mixed connective tissue disease, Sjögren syndrome, idiopathic inflammatory myopathies and systemic lupus erythematous.CONCLUSIONSDespite the lack of data on screening for CTD-ILD, an expert panel of pulmonologists, rheumatologists and radiologists agreed on a series of screening recommendations to support decision-making and enable early diagnosis of ILD to ultimately improve outcomes and prognosis in patients with CTD.

01 Jun 2025·Computers in Biology and Medicine

A computed tomography-based deep learning radiomics model for predicting the gender-age-physiology stage of patients with connective tissue disease-associated interstitial lung disease

Article

Author: Wang, Ronghua ; Zhuang, Ziyi ; Yin, Anyu ; Long, Bingqing ; Li, Rui ; Jing, Yang ; E, Linning

OBJECTIVESTo explore the feasibility of using a diagnostic model constructed with deep learning-radiomics (DLR) features extracted from chest computed tomography (CT) images to predict the gender-age-physiology (GAP) stage of patients with connective tissue disease-associated interstitial lung disease (CTD-ILD).MATERIALS AND METHODSThe data of 264 CTD-ILD patients were retrospectively collected. GAP Stage I, II, III patients are 195, 56, 13 cases respectively. The latter two stages were combined into one group. The patients were randomized into a training set and a validation set. Single-input models were separately constructed using the selected radiomics and DL features, while DLR model was constructed from both sets of features. For all models, the support vector machine (SVM) and logistic regression (LR) algorithms were used for construction. The nomogram models were generated by integrating age, gender, and DLR features.RESULTSThe DLR model outperformed the radiomics and DL models in both the training set and the validation set. The predictive performance of the DLR model based on the LR algorithm was the best among all the feature-based models (AUC = 0.923). The comprehensive models had even greater performance in predicting the GAP stage of CTD-ILD patients. The comprehensive model using the SVM algorithm had the best performance of the two models (AUC = 0.951).CONCLUSIONThe DLR model extracted from CT images can assist in the clinical prediction of the GAP stage of CTD-ILD patients. A nomogram showed even greater performance in predicting the GAP stage of CTD-ILD patients.

12 Apr 2025·Zhonghua jie he he hu xi za zhi = Zhonghua jiehe he huxi zazhi = Chinese journal of tuberculosis and respiratory diseases

[Clinicopathological characteristics,prognosis and related factors of lung cancer arising in the native lung following single lung transplantation].

Article

Author: Mao, M L ; Zhao, R S ; Cheng, N ; Wang, B ; Guo, L J ; Zhong, D R ; Guo, J ; Guo, R C ; Liu, H Y

Objective: To explore the clinicopathological characteristics and prognosis of lung cancer arising in the native lung after single lung transplantation for end-stage lung disease. Methods: We conducted a respective analysis of the clinical, pathological, and follow-up data of 12 recipients who developed lung cancer in the native lung after single-lung transplantation at China-Japan Friendship Hospital from September 2017 to June 2021, among a total of 247 single-lung transplantations performed during this period. Eleven were male and 1 was female, with ages ranging from 46-67 (59.25±5.75) years. Results: Of the 12 recipients, 11 had a smoking history before transplantation. The underlying diagnosis of lung diseases before transplantation included 8 cases of idiopathic pulmonary fibrosis, 3 cases of connective tissue disease-associated interstitial lung disease, and 1 case of chronic obstructive pulmonary disease. The time interval from transplantation to the development of lung cancer in the native lung was 3 to 53 months, with an average of (30.0±16.2) months. Eleven patients had elevated levels of serum tumor markers at the time of lung cancer diagnosis. CT/PET-CT showed new nodules or FDG avidity in the native lung. The histological types of lung cancer in the 12 cases included 1 case of small cell carcinoma and 11 cases of non-small cell lung cancer (7 cases of squamous cell carcinoma, 3 cases of adenocarcinoma, and 1 case of SMARCA4-deficient undifferentiated carcinoma). There were 8 cases in clinical stage Ⅳ, 1 case in stage Ⅲ, and 3 cases in stageⅠ. Three patients in stage Ⅰ and one patient in stage Ⅲ underwent surgical treatment, while patients in stage Ⅳ were treated with radiotherapy, chemotherapy, and palliative care. At the end of this study, 10 patients had died, 1 patient survived, and 1 patient was lost to follow-up. The median survival time was 7 months (ranging from 2 to 47 months), and the 1-year cumulative survival rate was 9.2%. Conclusions: The risk of developing lung cancer in the native lung after single lung transplantation is increased. The prognosis is very poor. Most of the histological types are squamous cell carcinoma. Close monitoring of high-risk populations after transplantation for early tumor detection may prolong survival time.

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Interstitial lung disease due to connective tissue disease

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