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2.
Int J Mol Sci ; 25(5)2024 Feb 26.
Artigo em Inglês | MEDLINE | ID: mdl-38473928

RESUMO

Rheumatoid arthritis (RA) is a systemic autoimmune disease characterized by swelling in at least one joint. Owing to an overactive immune response, extra-articular manifestations are observed in certain cases, with interstitial lung disease (ILD) being the most common. Rheumatoid arthritis-associated interstitial lung disease (RA-ILD) is characterized by chronic inflammation of the interstitial space, which causes fibrosis and the scarring of lung tissue. Controlling inflammation and pulmonary fibrosis in RA-ILD is important because they are associated with high morbidity and mortality. Pirfenidone and nintedanib are specific drugs against idiopathic pulmonary fibrosis and showed efficacy against RA-ILD in several clinical trials. Immunosuppressants and disease-modifying antirheumatic drugs (DMARDs) with anti-fibrotic effects have also been used to treat RA-ILD. Immunosuppressants moderate the overexpression of cytokines and immune cells to reduce pulmonary damage and slow the progression of fibrosis. DMARDs with mild anti-fibrotic effects target specific fibrotic pathways to regulate fibrogenic cellular activity, extracellular matrix homeostasis, and oxidative stress levels. Therefore, specific medications are required to effectively treat RA-ILD. In this review, the commonly used RA-ILD treatments are discussed based on their molecular mechanisms and clinical trial results. In addition, a computational approach is proposed to develop specific drugs for RA-ILD.


Assuntos
Antirreumáticos , Artrite Reumatoide , Fibrose Pulmonar Idiopática , Doenças Pulmonares Intersticiais , Humanos , Doenças Pulmonares Intersticiais/etiologia , Artrite Reumatoide/tratamento farmacológico , Fibrose Pulmonar Idiopática/tratamento farmacológico , Inflamação/tratamento farmacológico , Antirreumáticos/uso terapêutico , Imunossupressores/uso terapêutico
3.
BMJ Open Respir Res ; 11(1)2024 Mar 13.
Artigo em Inglês | MEDLINE | ID: mdl-38479818

RESUMO

OBJECTIVES: Vasoactive drugs have exhibited clinical efficacy in addressing pulmonary arterial hypertension, manifesting a significant reduction in morbidity and mortality. Pulmonary hypertension may complicate advanced interstitial lung disease (PH-ILD) and is associated with high rates of disability, hospitalisation due to cardiac and respiratory illnesses, and mortality. Prior management hinged on treating the underlying lung disease and comorbidities. However, the INCREASE trial of inhaled treprostinil in PH-ILD has demonstrated that PH-ILD can be effectively treated with vasoactive drugs. METHODS: This comprehensive systematic review examines the evidence for vasoactive drugs in the management of PH-ILD. RESULTS: A total of 1442 pubblications were screened, 11 RCTs were considered for quantitative synthesis. Unfortunately, the salient studies are limited by population heterogeneity, short-term follow-up and the selection of outcomes with uncertain clinical significance. CONCLUSIONS: This systematic review underscores the necessity of establishing a precision medicine-oriented strategy, directed at uncovering and addressing the intricate cellular and molecular mechanisms that underlie the pathophysiology of PH-ILD. PROSPERO REGISTRATION NUMBER: CRD42023457482.


Assuntos
Hipertensão Pulmonar , Doenças Pulmonares Intersticiais , Humanos , Hipertensão Pulmonar/tratamento farmacológico , Hipertensão Pulmonar/etiologia , Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/tratamento farmacológico , Doenças Pulmonares Intersticiais/epidemiologia , Comorbidade
4.
BMJ Open Respir Res ; 11(1)2024 Mar 13.
Artigo em Inglês | MEDLINE | ID: mdl-38479819

RESUMO

BACKGROUND: Fibrotic interstitial lung disease (ILD) is frequently associated with abnormal oxygenation; however, little is known about the accuracy of oxygen saturation by pulse oximetry (SpO2) compared with arterial blood gas (ABG) saturation (SaO2), the factors that influence the partial pressure of carbon dioxide (PaCO2) and the impact of PaCO2 on outcomes in patients with fibrotic ILD. STUDY DESIGN AND METHODS: Patients with fibrotic ILD enrolled in a large prospective registry with a room air ABG were included. Prespecified analyses included testing the correlation between SaO2 and SpO2, the difference between SaO2 and SpO2, the association of baseline characteristics with both the difference between SaO2 and SpO2 and the PaCO2, the association of baseline characteristics with acid-base category, and the association of PaCO2 and acid-base category with time to death or transplant. RESULTS: A total of 532 patients with fibrotic ILD were included. Mean resting SaO2 was 92±4% and SpO2 was 95±3%. Mean PaCO2 was 38±6 mmHg, with 135 patients having PaCO2 <35 mmHg and 62 having PaCO2 >45 mmHg. Correlation between SaO2 and SpO2 was mild to moderate (r=0.39), with SpO2 on average 3.0% higher than SaO2. No baseline characteristics were associated with the difference in SaO2 and SpO2. Variables associated with either elevated or abnormal (elevated or low) PaCO2 included higher smoking pack-years and lower baseline forced vital capacity (FVC). Lower baseline lung function was associated with an increased risk of chronic respiratory acidosis. PaCO2 and acid-base status were not associated with time to death or transplant. INTERPRETATION: SaO2 and SpO2 are weakly-to-moderately correlated in fibrotic ILD, with limited ability to accurately predict this difference. Abnormal PaCO2 was associated with baseline FVC but was not associated with outcomes.


Assuntos
Doenças Pulmonares Intersticiais , Oxigênio , Humanos , Oximetria , Gasometria , Doenças Pulmonares Intersticiais/diagnóstico
5.
BMJ Case Rep ; 17(3)2024 Mar 13.
Artigo em Inglês | MEDLINE | ID: mdl-38479828

RESUMO

Overlap syndromes are diseases that meet the criteria of two or more rheumatic diseases. In this case report, a woman in her 20s presented with a constellation of symptoms, including skin thickening, Raynaud's phenomenon, hypertension, absent pulse in both lower limbs with bilateral renal artery bruit. The antinuclear antibody profile revealed Scl-70 positivity. CT thorax identified early interstitial lung disease, and nailfold capillaroscopy showed severe capillary loss. CT angiogram features were suggestive of Takayasu arteritis. Notably, there have been only four documented cases of systemic sclerosis coexisting with Takayasu arteritis, highlighting the rarity of this overlap syndrome. The diagnosis of overlap syndrome was made after a thorough history recording and clinical examination. In the presence of bilateral renal artery stenosis, managing the scleroderma renal crisis may be challenging . This patient received treatment with mycophenolate mofetil and oral corticosteroids, aiming to address both systemic sclerosis and Takayasu arteritis effectively.


Assuntos
Doenças Autoimunes , Doenças do Tecido Conjuntivo , Doenças Pulmonares Intersticiais , Esclerodermia Localizada , Escleroderma Sistêmico , Arterite de Takayasu , Feminino , Humanos , Arterite de Takayasu/diagnóstico , Arterite de Takayasu/diagnóstico por imagem , Escleroderma Sistêmico/complicações , Escleroderma Sistêmico/diagnóstico , Doenças do Tecido Conjuntivo/complicações , Esclerodermia Localizada/complicações , Doenças Pulmonares Intersticiais/complicações , Doenças Autoimunes/complicações
7.
BMC Pulm Med ; 24(1): 108, 2024 Mar 04.
Artigo em Inglês | MEDLINE | ID: mdl-38438922

RESUMO

BACKGROUND: Safely implementing transbronchial lung cryobiopsy (TBLC) in patients with interstitial lung disease (ILD) requires accurate navigation. Traditional fluoroscopy falls short in reducing the risk of post-procedure pneumothorax. The potential of electromagnetic navigation bronchoscopy (ENB) as a more precise navigation method warrants further exploration. METHODS: A prospective cohort study was conducted on ILD patients undergoing TBLC. Patients were assigned either fluoroscopy or ENB for cryoprobe positioning. Navigation accuracy was evaluated using cone beam computed tomography (CBCT) images as the standard. Safety and diagnostic yield were also observed. RESULTS: Seventeen patients underwent TBLC, with 10 guided by fluoroscopy and seven by ENB. Fluoroscopy-guided cryoprobe navigation required more adjustments [9/15 (60%) v.s. 1/9 (11%), p = 0.018] for subsequent TBLC compared to ENB, as confirmed by CBCT images. Clinical characteristics, post-procedure complications, and biopsy specimen size showed no significant differences between the groups. Fourteen patients obtained a pathological diagnosis, and 15 received a multidisciplinary discussion (MDD) diagnosis. In the fluoroscopy group, three patients failed to obtain a pathological diagnosis, and two failed to obtain an MDD diagnosis. CONCLUSIONS: ENB demonstrates significantly superior accuracy in TBLC navigation compared to traditional fluoroscopy when CBCT images are used as a reference. Further studies are necessary to determine the value of ENB in TBLC navigation for ILD patients.


Assuntos
Broncoscopia , Doenças Pulmonares Intersticiais , Humanos , Estudos Prospectivos , Doenças Pulmonares Intersticiais/diagnóstico por imagem , Fluoroscopia , Pulmão/diagnóstico por imagem , Fenômenos Eletromagnéticos
9.
Int J Tuberc Lung Dis ; 28(3): 130-135, 2024 Mar 01.
Artigo em Inglês | MEDLINE | ID: mdl-38454180

RESUMO

INTRODUCTIONTo determine the frequency of TB among patients with interstitial lung diseases (ILDs).METHODSWe performed a comprehensive search in the PubMed/Medline, EMBASE and Scopus databases up to 1 August 2023 of studies reporting on the prevalence of TB among patients with ILDs.RESULTSTwelve studies comprising 3,817 patients with ILD were found: the pooled prevalence of TB among ILD patients was 11.0% (95% CI 5.4-21.0). In the subgroup analysis, the TB rate among patients with silicosis and idiopathic pulmonary fibrosis (IPF) was respectively 35.6% (95% CI 32.6-38.8) and 4.4% (95% CI 3.6-5.3) (P = 0.00). The frequency of TB among ILD patients was higher in high TB burden countries than in low/intermediate-burden countries: 26.3%, 95% CI 17.7-37.3 vs. 4.9%, 95% CI 3.3-7.2; P = 0.00.CONCLUSIONSThis study shows the frequency of TB among ILD patients. The meta-analysis reveals a significantly increased prevalence of TB among ILD patients with silicosis compared to IPF, and among individuals in high TB burden countries than in those with low/intermediate burden. The study results can help physicians and policymakers make efficient decisions for prompt screening and anti-TB treatment initiation in ILD patients..


Assuntos
Fibrose Pulmonar Idiopática , Doenças Pulmonares Intersticiais , Silicose , Tuberculose , Humanos , Tuberculose/epidemiologia , Prevalência , Doenças Pulmonares Intersticiais/epidemiologia , Silicose/epidemiologia , Fibrose Pulmonar Idiopática/epidemiologia
10.
BMC Pulm Med ; 24(1): 136, 2024 Mar 15.
Artigo em Inglês | MEDLINE | ID: mdl-38491506

RESUMO

BACKGROUND: Lung cancer (LC) is an important comorbidity of interstitial lung disease (ILD) and has a poor prognosis. The clinical characteristics and outcome of each ILD subtype in LC patients have not been sufficiently investigated. Therefore, this study aimed to evaluate the difference between idiopathic pulmonary fibrosis (IPF) and non-IPF ILD as well as prognostic factors in patients with ILD-LC. METHODS: The medical records of 163 patients diagnosed with ILD-LC at Asan Medical Center from January 2018 to May 2023 were retrospectively reviewed. Baseline characteristics and clinical outcomes were compared between the IPF-LC and non-IPF ILD-LC groups, and prognostic factors were analyzed using the Cox proportional-hazard model. RESULTS: The median follow-up period was 11 months after the cancer diagnosis. No statistically significant differences were observed in clinical characteristics and mortality rates (median survival: 26 vs. 20 months, p = 0.530) between the groups. The independent prognostic factors in patients with ILD-LC were higher level of Krebs von den Lungen-6 (≥ 1000 U/mL, hazard ratio [HR] 1.970, 95% confidence interval [CI] 1.026-3.783, p = 0.025) and advanced clinical stage of LC (compared with stage I, HR 3.876 for stage II, p = 0.025, HR 5.092 for stage III, p = 0.002, and HR 5.626 for stage IV, p = 0.002). In terms of treatment, surgery was the significant factor for survival (HR 0.235; 95% CI 0.106-0.520; p < 0.001). CONCLUSIONS: No survival difference was observed between IPF-LC and non-IPF ILD-LC patients. A higher level of Krebs von den Lungen-6 may act as a prognostic marker in ILD-LC patients.


Assuntos
Fibrose Pulmonar Idiopática , Doenças Pulmonares Intersticiais , Neoplasias Pulmonares , Humanos , Estudos Retrospectivos , Neoplasias Pulmonares/complicações , Doenças Pulmonares Intersticiais/diagnóstico , Fibrose Pulmonar Idiopática/complicações , Fibrose Pulmonar Idiopática/epidemiologia , Fibrose Pulmonar Idiopática/diagnóstico , Modelos de Riscos Proporcionais , Prognóstico
11.
Clin Exp Rheumatol ; 42(2): 288-294, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38488091

RESUMO

OBJECTIVES: To characterise clinical amyopathic dermatomyositis (CADM) from a clinical, histological, and prognostic perspective. METHODS: We retrospectively recorded data from our DM cohort. Patients were categorised into three groups: classic DM, hypomyopathic DM (HDM), characterised by normal muscle strength and evidence of muscle involvement in laboratory tests and/or instrumental examinations and CADM, featured by normal muscle strength and unremarkable findings in both laboratory tests and instrumental examinations. Available muscle biopsies from each group were also compared. RESULTS: Our cohort included 63 DM (69.2%), 12 HDM (13.2%) and 16 CADM (17.6%) patients. Compared to DM, CADM patients were younger at onset and diagnosis (45.5±17 vs. 57±18, and 46±17 vs. 58±18 years, respectively; p<0.05). They were more likely to test positive for anti-MDA5 (37.5% vs. 4.8%) and anti- TIF1-γ (31.3% vs. 6.3%), had a higher incidence of arthritis (37.5% vs. 12.6%) and interstitial lung disease (ILD) (43.8% vs. 15.9%) (all comparisons with p<0.05). Muscle biopsies were available for 44 DM, 7 CADM, and 11 HDM patients, revealing similar sarcolemma MHC-I expression rates. Five-year survival rates were comparable across groups (DM: 74.6%, CADM: 75%, HDM: 83.3%). Cox analysis indicated the main mortality predictors in overall cohort were ILD (HR: 3.57, CI: 1.11-11.5) and cancer (HR: 3.67, CI: 1.17-11.5), not CADM (HR: 1.46, CI: 0.33-6.68). CONCLUSIONS: CADM patients differ in disease onset, autoantibody profiles, joint and lung involvement. While laboratory and instrumental tests have not shown muscle involvement in CADM, many muscle biopsies have shown MHC-I overexpression.


Assuntos
Dermatomiosite , Doenças Pulmonares Intersticiais , Humanos , Prognóstico , Estudos Retrospectivos , Doenças Pulmonares Intersticiais/etiologia , Autoanticorpos , Helicase IFIH1 Induzida por Interferon
12.
BMC Complement Med Ther ; 24(1): 121, 2024 Mar 14.
Artigo em Inglês | MEDLINE | ID: mdl-38486172

RESUMO

BACKGROUND: Drug-induced interstitial lung disease (DIILD) is a severe adverse event leading to morbidity and mortality. This study evaluated the adverse event indicators of DIILD and time-to-onset profiles following the daily intake of herbal drugs (Scutellariae radix ["ogon" in Japanese], Bupleuri radix ["saiko" in Japanese], and Pinelliae tuber ["hange" in Japanese]) using the Japanese Adverse Drug Event Report database. DIILD was defined in accordance with the Medical Dictionary for Regulatory Activities. METHODS: The Japanese Adverse Drug Event Report database contained 830,079 reports published between April 2004 and April 2023. The association between herbal medicines and DILLD was evaluated using the pharmacovigilance index as the reporting odds ratio (ROR), logistic regression models, propensity score-matching techniques, and Weibull shape parameters. RESULTS: The adjusted RORs using multivariate logistic regression models for Scutellariae radix (daily intake), Pinelliae tuber (daily intake), sex (male), age (≥ 60 years), Scutellariae radix (daily intake)*age (≥ 60 years), and Scutellariae radix (daily intake)* Pinelliae tuber (daily intake) were 1.47 (1.36 - 1.59), 1.05 (1.01 - 1.10), 1.45 (1.34 - 1.57), 1.92 (1.74 - 2.11), 3.35 (3.12 - 3.60), and 1.49 (1.46 - 1.53), respectively. DIILD onset profiles were evaluated using the Weibull shape parameter. A logistic plot of daily intake and onset of DIILD was drawn. ROR signals were detected in 32 of 54 herbal medicines, including Scutellariae radix, Bupleuri radix, and Pinelliae tuber. The median duration (days) (interquartile range) to DIILD onset was 36.0 (27.0-63.0) for Saikokaryukotsuboreito, 35.0 (21.0-55.0) for Saireito, and 31.0 (13.5-67.5) for Shosaikoto. The Weibull shape parameter beta (95% confidence interval) values for Saikokaryukotsuboreito, Saireito, and Shosaikoto were 1.36 (1.08-1.67), 1.36 (1.20-1.52), and 1.31 (0.98-1.68), respectively. CONCLUSIONS: DIILD demonstrated a dose-dependent to crude drugs. Clinicians should strive for the early detection of DIILD and avoid the inadvertent administration of herbal medicines.


Assuntos
Efeitos Colaterais e Reações Adversas Relacionados a Medicamentos , Doenças Pulmonares Intersticiais , Plantas Medicinais , Preparações Farmacêuticas , Japão/epidemiologia , Doenças Pulmonares Intersticiais/induzido quimicamente , Doenças Pulmonares Intersticiais/epidemiologia
13.
RMD Open ; 10(1)2024 Mar 22.
Artigo em Inglês | MEDLINE | ID: mdl-38519110

RESUMO

BACKGROUND: This study aims to establish a reliable prediction model of progressive fibrosing interstitial lung disease (PF-ILD) in patients with systemic sclerosis (SSc)-ILD, to achieve early risk stratification and to help better in preventing disease progression. METHODS: 304 SSc-ILD patients with no less than three pulmonary function tests within 6-24 months were included. We collected data at baseline and compared differences between SSc patients with and without PF-ILD. Least absolute shrinkage and selection operator regularisation regression and multivariable Cox regression were used to construct the prediction model, which were presented as nomogram and forest plot. RESULTS: Among the 304 patients with SSc-ILD included, 92.1% were women, with a baseline average age of 46.7 years. Based on the 28 variables preselected by comparison between SSc patients without PF-ILD group (n=150) and patients with SSc PF-ILD group (n=154), a 9-variable prediction model was constructed, including age≥50 years (HR 1.8221, p=0.001), hyperlipidemia (HR 4.0516, p<0.001), smoking history (HR 3.8130, p<0.001), diffused cutaneous SSc subtype (HR 1.9753, p<0.001), arthritis (HR 2.0008, p<0.001), shortness of breath (HR 2.0487, p=0.012), decreased serum immunoglobulin A level (HR 2.3900, p=0.002), positive anti-Scl-70 antibody (HR 1.9573, p=0.016) and usage of cyclophosphamide/mycophenolate mofetil (HR 0.4267, p<0.001). The concordance index after enhanced bootstrap resampling adjustment was 0.874, while the optimism-corrected Brier Score was 0.144 in internal validation. CONCLUSION: This study developed the first prediction model for PF-ILD in patients with SSc-ILD, and internal validation showed favourable accuracy and stability of the model.


Assuntos
Doenças Pulmonares Intersticiais , Escleroderma Sistêmico , Humanos , Feminino , Pessoa de Meia-Idade , Masculino , Estudos de Coortes , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/epidemiologia , Doenças Pulmonares Intersticiais/etiologia , Ciclofosfamida , Progressão da Doença , Escleroderma Sistêmico/complicações
14.
BMJ Open Respir Res ; 11(1)2024 Mar 22.
Artigo em Inglês | MEDLINE | ID: mdl-38519114

RESUMO

BACKGROUND: Inhaled treprostinil (iTre) is the only treatment approved for pulmonary hypertension due to interstitial lung disease (PH-ILD) to improve exercise capacity. This post hoc analysis evaluated clinical worsening and PH-ILD exacerbations from the 16-week INCREASE study and change in 6-minute walking distance (6MWD) in the INCREASE open-label extension (OLE) in patients with less severe haemodynamics. METHODS: Patients were stratified by baseline pulmonary vascular resistance (PVR) of <4 Wood units (WU) versus ≥4 WU and <5 WU versus ≥5 WU. Exacerbations of underlying lung disease, clinical worsening and change in N-terminal prohormone of brain natriuretic peptide (NT-proBNP) in INCREASE were evaluated. For the OLE, patients previously assigned to placebo were considered to have a 16-week treatment delay. 6MWD and clinical events in the OLE were evaluated by PVR subgroup. RESULTS: Of the 326 patients enrolled in INCREASE, patients with less severe haemodynamics receiving iTre had fewer exacerbations of underlying lung disease and clinical worsening events. This was supported by the Bayesian analysis of the risk of disease progression (HR<1), and significant decreases in NT-proBNP levels. In the OLE, patients without a treatment delay had improved exercise capacity after 1-year compared with those with a 16-week treatment delay (22.1 m vs -10.3 m). Patients with a PVR of ≤5 WU without a treatment delay had a change of 5.5 m compared with -8.2 m for those with a treatment delay. Patients without a treatment delay had a prolonged time to hospitalisation, lung disease exacerbation and death. CONCLUSION: Treatment with iTre led to consistent benefits in clinical outcomes in patients with PH-ILD and less severe haemodynamics. Earlier treatment in less severe PH-ILD may lead to better exercise capacity long-term, however, the subgroup analyses in this post hoc study were underpowered and confirmation of these findings is needed.


Assuntos
Epoprostenol/análogos & derivados , Hipertensão Pulmonar , Doenças Pulmonares Intersticiais , Humanos , Hipertensão Pulmonar/tratamento farmacológico , Hipertensão Pulmonar/etiologia , Teorema de Bayes , Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/tratamento farmacológico , Hemodinâmica
15.
Respir Res ; 25(1): 138, 2024 Mar 23.
Artigo em Inglês | MEDLINE | ID: mdl-38521926

RESUMO

BACKGROUND: The prognostic and theragnostic role of histopathological subsets in systemic sclerosis interstitial lung disease (SSc-ILD) have been largely neglected due to the paucity of treatment options and the risks associated with surgical lung biopsy. The novel drugs for the treatment of ILDs and the availability of transbronchial cryobiopsy provide a new clinical scenario making lung biopsy more feasible and a pivotal guide for treatment. The aim of our study was to investigate the usefulness of lung biopsy in SSc ILD with a systematic literature review (SLR). METHODS: PubMed, Embase and Cochrane databases were searched up to June 30, 2023. Search terms included both database-specific controlled vocabulary terms and free-text terms relating to lung biopsy and SSc-ILD diagnostic and prognosis. The SLR was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-analysis (PRISMA). Studies were selected according to the PEO (population, exposure, and outcomes) framework and Quality assessment of diagnostic accuracy studies (QUADAS) were reported. RESULTS: We selected 14 articles (comprising 364 SSc-ILD patients). The paucity and heterogeneity of the studies prevented a systematic analysis. Diffuse cutaneous SSc was present in 30-100% of cases. Female predominance was observed in all studies (ranging from 64 to 100%). Mean age ranged from 42 to 64 years. Mean FVC was 73.98 (+/-17.3), mean DLCO was 59.49 (+/-16.1). Anti-Scl70 antibodies positivity was detected in 33% of cases (range: 0-69.6). All patients underwent surgical lung biopsies, and multiple lobes were biopsied in a minority of studies (4/14). Poor HRCT-pathologic correlation was reported with HRCT-NSIP showing histopathologic UIP in up to 1/3 of cases. Limited data suggest that SSc-UIP patients may have a worse prognosis and response to immunosuppressive treatment compared to other histopathologic patterns. CONCLUSIONS: The data from this SLR clearly show the paucity and heterogeneity of the studies reporting lung biopsy in SSc ILD. Moreover, they highlight the need for further research to address whether the lung biopsy can be helpful to refine prognostic prediction and guide therapeutic choices.


Assuntos
Doenças Pulmonares Intersticiais , Escleroderma Sistêmico , Humanos , Feminino , Adulto , Pessoa de Meia-Idade , Masculino , Doenças Pulmonares Intersticiais/patologia , Pulmão/patologia , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/complicações , Biópsia , Prognóstico
16.
Radiol Cardiothorac Imaging ; 6(2): e230287, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38483245

RESUMO

Purpose To investigate quantitative CT (QCT) measurement variability in interstitial lung disease (ILD) on the basis of two same-day CT scans. Materials and Methods Participants with ILD were enrolled in this multicenter prospective study between March and October 2022. Participants underwent two same-day CT scans at an interval of a few minutes. Deep learning-based texture analysis software was used to segment ILD features. Fibrosis extent was defined as the sum of reticular opacity and honeycombing cysts. Measurement variability between scans was assessed with Bland-Altman analyses for absolute and relative differences with 95% limits of agreement (LOA). The contribution of fibrosis extent to variability was analyzed using a multivariable linear mixed-effects model while adjusting for lung volume. Eight readers assessed ILD fibrosis stability with and without QCT information for 30 randomly selected samples. Results Sixty-five participants were enrolled in this study (mean age, 68.7 years ± 10 [SD]; 47 [72%] men, 18 [28%] women). Between two same-day CT scans, the 95% LOA for the mean absolute and relative differences of quantitative fibrosis extent were -0.9% to 1.0% and -14.8% to 16.1%, respectively. However, these variabilities increased to 95% LOA of -11.3% to 3.9% and -123.1% to 18.4% between CT scans with different reconstruction parameters. Multivariable analysis showed that absolute differences were not associated with the baseline extent of fibrosis (P = .09), but the relative differences were negatively associated (ß = -0.252, P < .001). The QCT results increased readers' specificity in interpreting ILD fibrosis stability (91.7% vs 94.6%, P = .02). Conclusion The absolute QCT measurement variability of fibrosis extent in ILD was 1% in same-day CT scans. Keywords: CT, CT-Quantitative, Thorax, Lung, Lung Diseases, Interstitial, Pulmonary Fibrosis, Diagnosis, Computer Assisted, Diagnostic Imaging Supplemental material is available for this article. © RSNA, 2024.


Assuntos
Doenças Pulmonares Intersticiais , Fibrose Pulmonar , Idoso , Feminino , Humanos , Masculino , Modelos Lineares , Doenças Pulmonares Intersticiais/diagnóstico , Estudos Prospectivos , Tomografia Computadorizada por Raios X , Pessoa de Meia-Idade
17.
Immunotherapy ; 16(7): 465-480, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38511241

RESUMO

Aim: This study aims to clarify the efficacy and adverse effects of immune checkpoint inhibitors (ICIs) in the lung cancer patients with a history of interstitial lung disease (ILD). Methods: From the inception of the database to 4 April 2023, we systematically searched the four databases. Results: The objective remission rate, disease control rate, incidence of immune-associated pneumonitis (ICIP) in the combined ILD group were significantly higher than those in the non-combined ILD group. There were no significant differences between the two groups in progression-free survival, overall survival, renal insufficiency, thyroid dysfunction and gastrointestinal toxicity. Conclusion: Generally, a pre-existing ILD history can increase the efficacy and incidence of ICIs' adverse reactions. Therefore, ICIs should be administered with caution.


Immune checkpoint inhibitors are a type of immunotherapy used to treat lung cancer. Some experts disagree over whether it is safe and effective to use this type of immunotherapy in people with lung cancer who also have lung disease. In this paper, the researchers analyzed the results of lots of different studies relating to the use of immune checkpoint inhibitors to treat lung cancer in patients with and without lung disease. They wanted to find out whether immune checkpoint inhibitors differed in their effectiveness between the two groups of patients. They also looked at whether patients with lung disease experienced more negative side effects from the immunotherapy treatment. The researchers found that patients with lung disease had a bigger response to immune checkpoint inhibitor treatment than patients without lung disease. This means that this type of immunotherapy is likely to be effective at treating lung cancer in patients with lung disease. However, the researchers also found that this patient group was more likely to experience negative side effects from the immunotherapy treatment. In particular, there were many more cases of pneumonia in this group than in the patients without lung disease. Therefore, doctors should be cautious when using immunotherapy to treat lung cancer patients with lung disease, ensuring they take measures to prevent pneumonia and be better prepared in case negative side effects occur.


Assuntos
Carcinoma Pulmonar de Células não Pequenas , Doenças Pulmonares Intersticiais , Neoplasias Pulmonares , Pneumonia , Humanos , Inibidores de Checkpoint Imunológico/efeitos adversos , Carcinoma Pulmonar de Células não Pequenas/tratamento farmacológico , Doenças Pulmonares Intersticiais/tratamento farmacológico , Doenças Pulmonares Intersticiais/etiologia , Estudos Retrospectivos
18.
BMJ Case Rep ; 17(3)2024 Mar 21.
Artigo em Inglês | MEDLINE | ID: mdl-38514155

RESUMO

Antimelanoma differentiation-associated protein 5 positive dermatomyositis (MDA5 DM) is a rare subtype of idiopathic inflammatory myopathy. There are limited data available regarding the cutaneous manifestations of MDA5 DM in the African American population. We presented the case of a male patient in his early 20s who presented with debilitating cutaneous ulceration and myopathy. Workup revealed interstitial lung disease (ILD) and positive MDA5 serology consistent with MDA5 DM. He made a remarkable recovery in terms of myopathy and cutaneous ulcerations with a multipronged regimen of prednisone, intravenous immunoglobulin and mycophenolate mofetil. However, there was a progression of ILD on this regimen which warranted use of rituximab.


Assuntos
Dermatomiosite , Doenças Pulmonares Intersticiais , Úlcera Cutânea , Humanos , Masculino , Dermatomiosite/complicações , Dermatomiosite/tratamento farmacológico , Úlcera , Helicase IFIH1 Induzida por Interferon , Autoanticorpos , Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/tratamento farmacológico , Úlcera Cutânea/tratamento farmacológico , Úlcera Cutânea/etiologia , Estudos Retrospectivos
19.
Int J Rheum Dis ; 27(3): e15069, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38514918

RESUMO

AIM: To clarify the prognosis and prognostic factors for lung cancer in patients with rheumatoid arthritis (RA). METHODS: In this retrospective longitudinal study, we investigated the medical records of patients with RA among 1422 patients diagnosed with lung cancer and registered in a hospital-based cancer registry between January 2013 and May 2022. The Kaplan-Meier method and Cox proportional hazards model were used to analyze survival and identify predictive factors. RESULTS: Of 26 patients with RA complicated with lung cancer (median age, 69 years), the 2-year overall survival rates for stages I-II were 90%-100%, and those for stages III-IV were 20%, respectively. Positivity of anti-citrullinated protein/peptide antibody, smoking history, interstitial lung disease, poorly controlled RA, stage III and IV lung cancer, histological types other than adenocarcinoma and squamous cell carcinoma, and RF ≧ 50 IU/mL were associated with increased mortality. After the surgical resection of stage I and II lung cancer, 5 of the 16 patients experienced cancer recurrence after resumption of RA treatment, and the histology of the recurrent cancers was mostly squamous cell carcinoma. CONCLUSIONS: Early detection of lung cancer is needed, especially in patients with RA who have a history of smoking, seropositivity, or interstitial lung disease. Even after surgical resection, it should be noted that squamous cell carcinoma is prone to recurrence.


Assuntos
Artrite Reumatoide , Carcinoma de Células Escamosas , Doenças Pulmonares Intersticiais , Neoplasias Pulmonares , Humanos , Idoso , Prognóstico , Neoplasias Pulmonares/diagnóstico , Neoplasias Pulmonares/epidemiologia , Neoplasias Pulmonares/complicações , Estudos Retrospectivos , Estudos Longitudinais , Recidiva Local de Neoplasia/complicações , Artrite Reumatoide/complicações , Artrite Reumatoide/diagnóstico , Artrite Reumatoide/epidemiologia , Doenças Pulmonares Intersticiais/diagnóstico , Doenças Pulmonares Intersticiais/epidemiologia , Doenças Pulmonares Intersticiais/etiologia , Anticorpos Antiproteína Citrulinada , Carcinoma de Células Escamosas/diagnóstico , Carcinoma de Células Escamosas/epidemiologia , Carcinoma de Células Escamosas/terapia
20.
Am J Case Rep ; 25: e942736, 2024 Mar 19.
Artigo em Inglês | MEDLINE | ID: mdl-38500257

RESUMO

BACKGROUND Patients with obesity with interstitial lung diseases (ILD) are encouraged to lose weight, as it improves lung function and lung transplant eligibility. As exercise tolerance in these patients is low and weight gain is a common adverse effect of corticosteroids, bariatric surgery can be an effective method for the management of obesity in this patient group. However, perioperative complications in such high-risk patients remain a concern. Therefore, we aimed to demonstrate successful anesthetic management for obese patients with ILD, which may be practically utilized to reduce perioperative pulmonary complications and improve outcomes. CASE REPORT Our case report presents a 42-year-old man with ILD who underwent laparoscopic sleeve gastrectomy (LSG). Preoperative studies revealed severe restrictive disease, right ventricular overload with assessed intermediate risk of pulmonary hypertension, and heart failure, with preserved left ventricle fraction but with poor exercise tolerance. Patient had opioid-free anesthesia (OFA) and postoperative multimodal analgesia. Following a 24-h stay in the Post-Anesthesia Care Unit, the patient was transferred to the ward and ultimately discharged home 2 days thereafter. At the 1-year follow-up, the patient reduced his weight by 40 kg and reported a significant improvement in physical capacity. CONCLUSIONS Our record demonstrates that OFA can be successfully used in high-risk patients with ILD undergoing LSG. In a period of a year, the patient improved so much that he no longer required lung transplantation, which may encourage clinicians to provide bariatric surgery using the OFA technique in the population of patients with obesity and severe respiratory illness.


Assuntos
Anestésicos , Laparoscopia , Doenças Pulmonares Intersticiais , Transplante de Pulmão , Obesidade Mórbida , Masculino , Humanos , Adulto , Obesidade Mórbida/complicações , Obesidade Mórbida/cirurgia , Laparoscopia/métodos , Estudos Retrospectivos , Obesidade/complicações , Obesidade/cirurgia , Gastrectomia/efeitos adversos , Doenças Pulmonares Intersticiais/complicações , Doenças Pulmonares Intersticiais/cirurgia , Resultado do Tratamento , Índice de Massa Corporal
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