Diagnosis and Management of Interstitial Lung Disease in Patients with Connective Tissue Diseases

Koslow, Matthew; Maleki-Fischbach, Mehrnaz; Keith, Rebecca C.

doi:10.1155/2021/6677353

Cited by 4 publications

(5 citation statements)

References 21 publications

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“…Ideally, a review of imaging findings by an expert radiologist should be part of an MDT discussion of all information available on a patient with an autoimmune diseaserelated ILD. [60][61][62][63][64][65] A surgical lung biopsy is rarely required in patients with autoimmune disease-related ILDs found to have ILD on HRCT. MDT discussion of imaging findings is not only important for informing an initial diagnosis.…”

Section: Importance Of Multidisciplinary Discussion Of Imaging Findingsmentioning

confidence: 99%

Imaging Features of Autoimmune Disease-Related Interstitial Lung Diseases

Walsh,

Lafyatis,

Cottin

2023

Journal of Thoracic Imaging

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Interstitial lung diseases (ILDs) associated with autoimmune diseases show characteristic signs of imaging. Radiologic signs are also used in the identification of ILDs with features suggestive of autoimmune disease that do not meet the criteria for a specific autoimmune disease. Radiologists play a key role in identifying these signs and assessing their relevance as part of multidisciplinary team discussions. A radiologist may be the first health care professional to pick up signs of autoimmune disease in a patient referred for assessment of ILD or with suspicion for ILD. Multidisciplinary team discussion of imaging findings observed during follow-up may inform a change in diagnosis or identify progression, with implications for a patient’s treatment regimen. This article describes the imaging features of autoimmune disease-related ILDs and the role of radiologists in assessing their relevance.

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Section: Importance Of Multidisciplinary Discussion Of Imaging Findingsmentioning

confidence: 99%

Imaging Features of Autoimmune Disease-Related Interstitial Lung Diseases

Walsh,

Lafyatis,

Cottin

2023

Journal of Thoracic Imaging

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“…From 2018 to 2020, patients initially diagnosed with CTD-ILD in our hospital were included. The diagnostic criteria were (I) interstitial lung disease diagnosed according to ILD diagnostic criteria issued by the European Respiratory Society (10) and (II) connective tissue disease diagnosed according to the 2012 SICCA guidelines (11). The patients were divided into the study group (pirfenidone treatment) and control group (without pirfenidone treatment) based on whether they received pirfenidone treatment.…”

Section: Ctd-ild Patient Inclusionmentioning

confidence: 99%

Pirfenidone inhibits cell fibrosis in connective tissue disease-associated interstitial lung disease by targeting the TNF-α/STAT3/KL6 pathway

Zuo¹,

Liu²,

Xu³

et al. 2022

J Thorac Dis

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Background: To explore the effect and mechanism of pirfenidone in inhibiting pulmonary fibrosis in connective tissue disease-associated interstitial lung disease (CTD-ILD). Methods: From 2018 to 2020, 50 CTD-ILD patients were enrolled in the clinical study. Based on whether pirfenidone was used during treatment, patients were enrolled into the pirfenidone group and the control group. Pulmonary function tests were compared before and after treatment. Enzyme-linked immunosorbent assay (ELISA) was performed to detect the expression of tumor necrosis factor-α (TNF-α), signal transducer and activator of transcription 3 (STAT3), and Krebs Von den Lungen-6 (KL-6) in venous blood before and after treatment. Rat type II (RLE-6TN) lung epithelial cells were cultivated for in vitro experiments, and they were sorted into the control group, bleomycin group, pirfenidone group, TNF-α group, Stattic group, and TNF-α/Stattic combined treatment group. For the in vitro experiments, 3-(4,5-dimethylthiazol-2-yl)-2,5diphenyltetrazolium bromide (MTT) tests were used to evaluate cell proliferation, Reverse Transcription-Polymerase Chain Reaction(RT-PCR) was performed to detect STAT3 and KL-6 mRNA expression levels, ELISA was utilized to detect TNF-α and E-cadherin expression levels, and Western blotting (WB) was performed to determine α-smooth muscle actin (α-SMA), vimentin, TNF-α, STAT3, phosphorylated signal transducer and activator of transcription 3 (PSTAT3) and KL-6 expression.Results: In the clinical study, the pulmonary function indices including forced expiratory volume in one second (FEV1), forced vital capacity (FVC), FEV1/FVC, peak expiratory flow (PEF) and partial pressure (PaO 2 ) of the patients in the study group were superior to those in the control group (P<0.05). The serum TNF-α, STAT3 and KL-6 levels in the study group were significantly lower than those in the control group (P<0.05). In the in vitro experiments, the α-SMA, vimentin, STAT3 and KL-6 levels in the treatment group were significantly lower than those in the bleomycin group (P<0.05). Compared with those in the pirfenidone group, the α-SMA, vimentin, STAT3 and KL-6 levels in the TNF-α-treated group were significantly upregulated (P<0.05). Meanwhile, cell viability was further upregulated (P<0.05), and the expression of STAT3 and KL-6 was further decreased in the Stattic-treated group (P<0.05). In the group treated with infliximab combined with Stattic, TNF-α expression was significantly increased (P<0.05), cell activity was significantly restored (P<0.05), and the STAT3, KL-6 and E-cadherin expression levels were inhibited (P<0.05).Conclusions: Pirfenidone improved pulmonary function 1 and decreased TNF-α, STAT3, and KL-6 expression in CTD-ILD patients. Moreover, pirfenidone inhibits cell fibrosis through the TNF-α/STAT3/ Mucin 1(MUC1) pathway.

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“…Interstitial lung disease (ILD) is a chronic lung disease characterized by inflammation and/or fibrosis of the interstitium and alveolar parenchyma ( 1 ). Patients experience variable clinical courses dependent on both the etiology and phenotype of the ILD, as well as other individual patient characteristics ( 2 – 4 ). A person may develop ILD as a result of an environmental or occupational exposure, a systemic connective tissue disease, or an unidentifiable cause, as in the case of idiopathic pulmonary fibrosis (IPF) ( 4 – 8 ).…”

Section: Introductionmentioning

confidence: 99%

“…Patients experience variable clinical courses dependent on both the etiology and phenotype of the ILD, as well as other individual patient characteristics ( 2 – 4 ). A person may develop ILD as a result of an environmental or occupational exposure, a systemic connective tissue disease, or an unidentifiable cause, as in the case of idiopathic pulmonary fibrosis (IPF) ( 4 – 8 ). Patients who live with ILD experience varying degrees of symptomatology, including chronic dyspnea, cough, and fatigue, which have the potential to interfere with daily activities and quality of life ( 2 , 6 , 9 – 11 ).…”

Section: Introductionmentioning

confidence: 99%

Current Landscape and Future Directions of Patient Education in Adults with Interstitial Lung Disease

Savvaides,

Demetres,

Aronson

2024

ATS Scholar

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Background Understandable, comprehensive, and accessible educational materials for patients with interstitial lung disease (ILD) are lacking. Patients consistently ask for improved access to evidence-based information about ILD. Nonetheless, few research studies focus directly on developing and evaluating interventions to improve patient knowledge. Objective We describe the current landscape of patient education in ILD, identify gaps in current approaches to information delivery, and provide frameworks to address these challenges through novel educational tools. Methods A literature review was conducted in collaboration with a medical librarian (M.R.D.) in April 2022 using Ovid MEDLINE (1946–), Embase (1947–), Cochrane Central (1993–), and CINAHL (1961–). Search terms included “interstitial lung disease,” “pulmonary fibrosis,” “patient education,” and “information seeking behavior” ( see the data supplement for full search terms). Reference lists from selected articles were used to identify additional studies. Results Currently, patient education is commonly combined with exercise regimens in pulmonary rehabilitation programs in which benefits of the educational component alone are unclear. Few studies investigate improving knowledge access and acquisition for patients with ILD and their caregivers regarding self-management, oxygen use, and palliative care plans. Online distribution of health information through social media runs the risk of being unregulated and outdated, although it is an avenue of increasing accessibility. Conclusion By expanding access to novel ILD-specific education programs and accounting for social determinants of health that impact healthcare access, patient education has the potential to become more attainable, improving patient-centered outcomes. Further research into optimal development, delivery, and efficacy testing of patient education modalities in ILD is warranted.

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Diagnosis and Management of Interstitial Lung Disease in Patients with Connective Tissue Diseases

Cited by 4 publications

References 21 publications

Imaging Features of Autoimmune Disease-Related Interstitial Lung Diseases

Imaging Features of Autoimmune Disease-Related Interstitial Lung Diseases

Pirfenidone inhibits cell fibrosis in connective tissue disease-associated interstitial lung disease by targeting the TNF-α/STAT3/KL6 pathway

Current Landscape and Future Directions of Patient Education in Adults with Interstitial Lung Disease

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