Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG最新文献

{"title":"Can mycophenolate mofetil be an effective option in the treatment of vascular Behçet's Syndrome.","authors":"Emine Uslu, Nilgün Göveç Giynaş, Zehra Karaman Ongun, Serdar Sezer, Müçteba Enes Yayla, Tahsin Murat Turgay, Aşkın Ateş","doi":"10.36141/svdld.v42i4.17166","DOIUrl":"10.36141/svdld.v42i4.17166","url":null,"abstract":"<p><p>Background/Aim Behcet's syndrome (BS) is a type of vasculitis that primarily affects veins and tend to involve pulmonary arteries more often than peripheral arteries or the aorta. Inflammation is a central role in the development of thrombotic events in BS.  Given its critical role in etiopathogenesis, the use of immunosuppressive therapy is fundamental to the treatment strategy. The aim of our study is to report our center's experience with mycophenolate mofetil (MMF) treatment in vascular BS (VBS) patients. Materials and Methods The clinical, laboratory and imaging findings of the patients receiving MMF treatment for vascular BS were retrospectively evaluated.  It was noted whether MMF treatment was induction or maintenance treatment. Treatment related side effects were noted. On the 12th month, patients were evaluated for the development of new events and being in remission. Results Five patients underwent MMF treatment for remission induction, while another five patients received MMF for maintenance. Relapses occurred in 2 out of 5 patients who were administered MMF as induction therapy, resulting in the development of acute deep vein thrombosis. Among the five patients receiving MMF as maintenance treatment, only one exhibited an active vascular event. Additionally, one patient was classified as vascularly active based on a CRP level of 10.3 mg/l. Conclusion MMF therapy may be an effective and safe treatment agent that can be preferred as azathioprine and cyclosporine in VBS patients, especially in maintenance therapy. This study is the first investigation into MMF treatment in patients with vascular BS.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"42 4","pages":"17166"},"PeriodicalIF":0.0,"publicationDate":"2025-12-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12815285/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145759155","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Does combined pulmonary fibrosis and emphysema syndrome affect response to antifibrotic therapy and survival? A single-center retrospective cohort study.","authors":"Nazli Huma Teke, Meltem Agca, Ayla Turkar, Tülin Sevim, Eylem Tuncay, Sinem Gungor, Elif Yıldırım, Fatma Ozbaki, Baran Gundogus, Sibel Arınc, Huriye Berk Takır, Ipek Ozmen","doi":"10.36141/svdld.v42i4.17187","DOIUrl":"10.36141/svdld.v42i4.17187","url":null,"abstract":"<p><strong>Objective: </strong>Combined pulmonary fibrosis and emphysema (CPFE) is a clinicoradiological syndrome characterized by upper lobe emphysema and lower lobe fibrosis, most commonly associated with idiopathic pulmonary fibrosis (IPF). This study compared the clinical characteristics, functional parameters, and mortality of patients with CPFE and IPF who received antifibrotic therapy.</p><p><strong>Methods: </strong>Between October 2015 and August 2022, patients with IPF treated with antifibrotics for at least 6 months were retrospectively evaluated and divided into two groups: CPFE (emphysema present) and IPF (emphysema absent). Demographic data, antifibrotic therapy, functional parameters before and after treatment (FEV1%, FVC%, DLco %), clinical outcomes (hospital admissions, mortality) were compared.</p><p><strong>Results: </strong>Of the 204 patients with IPF, 90 (44%) had CPFE. CPFE patients were more often male, had greater smoking history, higher pack-years, and more lung cancer than IPF patients (p < 0.001 for all). Post-treatment FEV1% and FVC% did not significantly differ between the groups, whereas DLco% declined significantly in both (p < 0.001 and p = 0.002). DLco% decreased more in IPF than CPFE, but the difference was not statistically significant [-3 (-11-3) vs. -0.43 (-1.1-0.2), p = 0.36]. The hospital admission rates were similar. Independent risk factors for mortality included CPFE diagnosis (HR: 1.73, 95% CI: 1.06-2.83, p = 0.029), low FVC% (HR: 0.970, 95% CI: 0.96-0.98, p < 0.001), and device use (long-term oxygen therapy [LTOT]  or home non-invasive mechanical ventilation [NIMV]) (HR: 2.48, 95% CI: 1.50-4.09, p < 0.001). Mean survival was shorter in patients with emphysema than in those without emphysema (5.08 vs. 5.68 years, p = 0.08).</p><p><strong>Conclusions: </strong>Despite a decline in DLco%, changes remained below the futility threshold. Clinical outcomes and mortality were comparable. CPFE diagnosis, low FVC%, and LTOT/NIMV use independently predicted higher mortality.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"42 4","pages":"17187"},"PeriodicalIF":0.0,"publicationDate":"2025-12-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12815298/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145759150","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The association between sarcoidosis and lymphoma: a retrospective and multicentre study of 46 patients.","authors":"Romé Charriere, Thomas El Jammal, Arthur Bert, Emilie Chalayer, Martin Kilian, Mathieu Gerfaud-Valentin, Yvan Jamilloux, Hervé Ghesquieres, Pascal Seve","doi":"10.36141/svdld.v42i4.16866","DOIUrl":"10.36141/svdld.v42i4.16866","url":null,"abstract":"<p><strong>Background: </strong>Studies on sarcoidosis-lymphoma syndrome are scarce, and the association between these diseases is poorly understood.   Aim: To analyse the features of three types of sarcoidosis-lymphoma association (SLA) according to the time of diagnosis of both diseases and to compare the features of patients who presented with sarcoidosis before lymphoma with those of patients with sarcoidosis alone (SA) in order to identify predictive factors of lymphoma development.   Design: A multicentre retrospective comparative study was conducted in two hospitals in Lyon and one hospital in Saint-Etienne, France, between 1999 and 2022.   Methods: In the SLA group, 46 patients were included and compared to 92 patients of the SA group. Demographic characteristics, medical history, clinical presentation, laboratory and imaging findings, treatments, and outcomes were collected and analysed from medical records.   Results: The age at lymphoma diagnosis was significantly higher in the group in which sarcoidosis preceded lymphoma [65.9 years when sarcoidosis preceded lymphoma vs 48.3 years when both diseases occurred simultaneously vs 44.5 years when sarcoidosis followed lymphoma, p<0.001]. Hodgkin's lymphoma was significantly more frequent in patients from group \"simultaneous diseases\" (p<0.001). There were significantly more autoimmune diseases (15.2% vs 2.17% p=0.007) in the SLA group, and patients with SA presented more frequently with night sweats (45.7% vs 11.6% p<0.001).   Conclusions: No robust predictive factor of lymphoma development in patients with sarcoidosis was identified. However, it highlighted distinct clinical scenarios according to when lymphoma is diagnosed that must be taken into account by clinicians for disease management.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"42 4","pages":"16866"},"PeriodicalIF":0.0,"publicationDate":"2025-12-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12815286/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145759125","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The prognostic value of peripheral hematological biomarkers in patients with hypersensitivity pneumonitis: A single-center retrospective study.","authors":"Ayshan Mammadova, Zeynep Yalçınkaya, Nilgün Yılmaz Demirci, Haluk Türktaş","doi":"10.36141/svdld.v42i4.17291","DOIUrl":"10.36141/svdld.v42i4.17291","url":null,"abstract":"<p><strong>Background/aim: </strong> Investigating inflammation-related biomarkers in hypersensitivity pneumonitis (HP) patients may provide valuable insights, particularly in determining their clinical course and prognosis. This study aimed to explore the potential role of peripheral hematological biomarkers in predicting the clinical course and prognosis of HP patients.</p><p><strong>Materials and methods: </strong> This retrospective study included 75 patients diagnosed with HP. Demographic data, symptoms, exposure history, pulmonary function tests, systemic immune-inflammation index (SII), and aggregate index of systemic inflammation (AISI) were analyzed.</p><p><strong>Results: </strong> Elevated SII (1353.6±915.15) and AISI (1001.1±695.38) values were significantly higher in patients with fibrotic HP compared to non-fibrotic HP (SII: 607.84±334.53, AISI: 375.01±235.18) (p<0.001). ROC analysis showed that SII (cut-off: 644.9, AUC: 0.788) and AISI (cut-off: 438.79, AUC: 0.767) had good predictive value for fibrotic HP. For mortality, SII ≥690.12 (AUC: 0.736) and AISI ≥490.35 (AUC: 0.753) were significant predictors. These elevated values were also associated with higher mortality (46.3% vs. 8.8%, p=0.001 for SII, and 42.5% vs. 14.3%, p=0.004 for AISI). In univariate analysis, older age, digital clubbing, low FVC%, and high SII/AISI were associated with fibrotic HP, while multivariate analysis identified only increased SII (OR: 1.45, p=0.021) and reduced FVC% (OR: 0.999, p=0.043) as independent predictors.</p><p><strong>Conclusion: </strong> SII and AISI appear as valuable prognostic tools in determining the clinical course and prognosis of HP patients. These biomarkers could be beneficial in the early diagnosis and management of HP, offering high sensitivity and specificity.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"42 4","pages":"17291"},"PeriodicalIF":0.0,"publicationDate":"2025-12-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12815288/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145759146","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Unveiling CCL2 and CXCL1: Key biomarkers and therapeutic targets in idiopathic pulmonary fibrosis.","authors":"Jing Pan, Guanhua Li, Yuanyuan Pu, Peilin Li, Yan Zhou","doi":"10.36141/svdld.v42i4.17182","DOIUrl":"10.36141/svdld.v42i4.17182","url":null,"abstract":"<p><p>Idiopathic Pulmonary Fibrosis (IPF) is a chronic, progressive lung disease characterized by fibrosis and impaired lung function. Understanding the molecular mechanisms underlying IPF is crucial for developing effective therapeutic strategies. This study aims to identify differentially expressed genes (DEGs) in alveolar macrophages of IPF patients and validate these findings in an independent cohort. We analyzed gene expression data from the GSE49072 dataset, which includes 23 IPF and 61 normal alveolar macrophage samples. Differential expression analysis was performed using the LIMMA package, and Gene Set Enrichment Analysis (GSEA) identified significant biological pathways. Validation of key DEGs (CCL2, CCL7, and CXCL1) was conducted using ELISA in serum samples from an independent cohort of 50 IPF patients and 50 normal controls. Correlations with clinical parameters such as forced vital capacity (FVC) and diffusing capacity for carbon monoxide (DLCO) were also evaluated. The analysis identified several DEGs, with CCL2 and CXCL1 showing significant upregulation in IPF samples. GSEA highlighted pathways related to inflammatory responses and extracellular matrix remodeling. ELISA validation confirmed elevated serum levels of CCL2 and CXCL1 in IPF patients, correlating with reduced FVC and DLCO. CCL7 did not show significant differences between groups. Targeting these chemokines may offer new therapeutic avenues for IPF treatment. Further research is needed to explore the therapeutic potential and validate findings in larger cohorts. This study enhances our understanding of IPF pathogenesis and identifies promising targets for clinical intervention.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"42 4","pages":"17182"},"PeriodicalIF":0.0,"publicationDate":"2025-12-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12815301/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145759098","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Utility of microRNAs in Exhaled Breath Condensate as Diagnostic Biomarkers to Differentiate Pulmonary Sarcoidosis from Pulmonary Tuberculosis.","authors":"Divyanjali Rai, Adwika Pp, Jaya Tak, Sunil Bangaru, Bijay Pattnaik, Sunita Yadav, Seetu Kumari, Jyoti Kumari, Umashankar Verma, Geetika Yadav, R S Dhaliwal, Naveen Bhatraju, Saurabh Mittal, Pawan Tiwari, Vijay Hadda, Karan Madan, Anurag Agrawal, Randeep Guleria, Rohit Vadala, Sachin Kumar, Anant Mohan","doi":"10.36141/svdld.v42i4.16920","DOIUrl":"10.36141/svdld.v42i4.16920","url":null,"abstract":"<p><strong>Background and aim: </strong>Pulmonary sarcoidosis and pulmonary tuberculosis (PTB) are two granulomatous diseases with similar clinical presentations. This study explored the potential of microRNAs (miRNAs) in exhaled breath condensate (EBC) as non-invasive diagnostic biomarkers to distinguish between pulmonary sarcoidosis and PTB.  Methods: EBC samples were collected from 46 participants (20 with PTB, 26 with pulmonary sarcoidosis) and 20 healthy controls and miRNA profiling was done. Differentially expressed miRNAs were further validated using qRT-PCR in independent cohorts in EBC (17 PTB, 18 sarcoidosis, and 25 controls) and blood (10 PTB, 10 sarcoidosis, and 10 controls).  Results: Initial profiling identified 19 miRNAs differentially expressed between pulmonary sarcoidosis and PTB, one between sarcoidosis and controls, and 22 between PTB and controls. The top five upregulated miRNAs (miR-132, miR-362-5p, miR-181c, miR-181a, and miR-512-3p) and two downregulated miRNAs (miR-454 and miR-139-5p) in sarcoidosis compared to PTB were selected for validation. Validation showed significant downregulation (p value <0.05) of only miR-454 in EBC of sarcoidosis patients compared to PTB patients with an optimal AUC of 0.663. miR-150* discriminated sarcoidosis patients from healthy controls with an AUC of 0.982. When PTB were compared to healthy controls, miR-454 and miR-139-5p were discriminating both groups with AUCs of 0.991 and 0.994 respectively in EBC.</p><p><strong>Conclusions: </strong>These findings suggest that while miR-454 differentiated pulmonary sarcoidosis from pulmonary tuberculosis, their clinical utility in EBC is limited. Further investigation is needed to explore their diagnostic potential, and to improve diagnostic accuracy in EBC.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"42 4","pages":"16920"},"PeriodicalIF":0.0,"publicationDate":"2025-12-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12815296/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145759119","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"The prevalence of interstitial lung disease and bronchiectasis in rheumatoid arthritis: A systematic review and meta-analysis.","authors":"Jonas Aggerholm Baekdal, Laurits Kramer Janns, Erik Sören Halvard Hansen, Howraman Meteran, Elisabeth Bendstrup, Charlotte Suppli Ulrik","doi":"10.36141/svdld.v42i4.17112","DOIUrl":"10.36141/svdld.v42i4.17112","url":null,"abstract":"<p><strong>Background and aim: </strong>Rheumatoid arthritis (RA) is associated with an increased risk of interstitial lung disease (ILD) and bronchiectasis. Understanding the prevalence of these diseases is essential for timely diagnosis and management. Our aim was to estimate the prevalence of ILD and bronchiectasis in RA patients.</p><p><strong>Study design and methods: </strong>A systematic literature search was performed on PubMed, Embase, and Google Scholar to identify all studies performing HRCT in consecutive RA patients. Articles were screened by three independent authors in accordance with PRISMA guidelines. Random-effects meta-regression was performed to estimate the prevalence according to RA duration and C-reactive protein.</p><p><strong>Results: </strong>Twenty-four studies comprising 2,532 RA patients were included. The estimated prevalence of ILD at the time of RA diagnosis was 8.5% (95% CI: 4.4-12.5%), increasing by 3.0 percentage points (95% CI: 2.1-3.9%) per year after RA diagnosis (P<0.0001, R²=95%). The estimated prevalence of bronchiectasis at RA diagnosis was 8.2% (95% CI: 1.6-14.7%), increasing 1.1 percentage points (95% CI: 0.3-1.9) per year after RA diagnosis (P<0.01, R²=34%). Bronchiectasis prevalence was strongly associated with C-reactive protein and increased by 3.1 percentage points (95% CI: 0.9-5.4) per unit increase in CRP (mg/dL) (p<0.01, R2=77%) Conclusion: Bronchiectasis and ILD are common pulmonary manifestations of RA, particularly in patients with long-term disease. Possibly due to the cumulative effect of chronic inflammation. Therefore, we suggest HRCT for all patients with respiratory symptoms or RA duration exceeding 5 years. This approach may facilitate earlier detection of preclinical ILD and timely intervention.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"42 4","pages":"17112"},"PeriodicalIF":0.0,"publicationDate":"2025-12-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12815293/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145759121","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Pulmonary rehabilitation improves distance-saturation product in IPF: Greater benefits in patients with exercise-induced desaturation.","authors":"Hülya Şahin, Ilknur Naz Gursan, Fatma Demirci Ucsular, Gulistan Karadeniz","doi":"10.36141/svdld.v42i4.17284","DOIUrl":"10.36141/svdld.v42i4.17284","url":null,"abstract":"<p><strong>Background and aim: </strong>Exercise-induced dyspnea and desaturation are hallmark features of idiopathic pulmonary fibrosis (IPF) and are linked to disease severity and poor outcomes. The distance-saturation product (DSP), calculated by multiplying the walking distance by oxygen saturation, has been proposed as a mortality predictor. This study aimed to investigate the effects of a pulmonary rehabilitation (PR) program on clinical outcomes and DSP in two IPF patient groups: those with and without desaturation during the six-minute walk test (6MWT).</p><p><strong>Methods: </strong>IPF patients who completed a standardized PR program were included. Assessments before and after PR included spirometry, arterial blood gas analysis, Medical Research Council (MRC) dyspnea scale, 6MWT, St. George's Respiratory Questionnaire (SGRQ), 36-Item Short Form Survey (SF-36), and Hospital Anxiety and Depression Scale (HADS). DSP was calculated at the end of the 6MWT. Patients with oxygen saturation ≤88% during baseline 6MWT were assigned to the desaturated group; others were assigned to the non-desaturated group.</p><p><strong>Results: </strong>Fifty patients were enrolled (20 desaturated, 30 non-desaturated). Following PR, only the desaturated group showed significant improvements in forced vital capacity (FVC), oxygen saturation, and SF-36 domains. In both groups, PaO₂, 6MWT distance, and DSP increased significantly, while MRC, SGRQ, and HADS scores decreased. However, the gains in 6MWT distance and DSP were significantly greater in the desaturated group.</p><p><strong>Conclusion: </strong>PR improved functional, psychological, and quality-of-life outcomes in both groups, with more pronounced benefits on sa in desaturated patients. Referral to PR is especially recommended for this subgroup.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"42 4","pages":"17284"},"PeriodicalIF":0.0,"publicationDate":"2025-12-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12815300/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145759087","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Janus kinase inhibitors in pulmonary and extra-pulmonary sarcoidosis: A case series and a systematic review of the literature.","authors":"Maxime Quaggetto, Thouraya Ben Salem, Julien Haroche, Julien Campagne, Quentin Moyon, Raphael Lhote, Zahir Amoura, Fleur Cohen Aubart","doi":"10.36141/svdld.v42i4.17246","DOIUrl":"10.36141/svdld.v42i4.17246","url":null,"abstract":"<p><strong>Background and aim: </strong>Sarcoidosis is an inflammatory granulomatous disease of unknown cause affecting the lungs and frequently extra-thoracic organs. Several immunosuppressive agents have been evaluated in patients refractory to steroids or as steroids-sparing treatments with low level of evidence and conflicting results. JAK inhibitors (JAKi) are novel immunosuppressive agents and recent reports suggested their potential to induce disease response. We report four new sarcoidosis cases treated with JAKi and present a systematic review of the literature.</p><p><strong>Methods: </strong>We describe four new cases and conducted a literature review using Pubmed, Medline, Embase and Cochrane Library. All English-language reports of sarcoidosis patients treated with JAKi published between January 2004 and 2024 were included.</p><p><strong>Results: </strong>We included 4 cases, as well as 45 cases from a systematic review of the literature, reported in 21 articles, leading to a cohort of 49 patients (female 53%, median [IQR 25-75] age 55 [43-60] years). Most cases had pulmonary and cutaneous sarcoidosis, while cardiac and neurological sarcoidosis were less frequent. Indication for JAKi were: steroid sparing agent (29%), refractory disease (22%), non-sarcoidosis indication (10%) and not specified (39%). Most patients received Tofacitinib 5 mg twice daily. Median corticosteroids dosage at treatment initiation was 15 [10-25] mg/day. After a median follow-up of 8 [6-12] months, complete response was achieved in 45% of cases and partial response in 49%, with few adverse events reported. Corticosteroids were discontinued in 11/23 (48%) and reduced in 6/23 (26%).</p><p><strong>Conclusions: </strong>JAK inhibitors appear to be a promising therapeutic option in sarcoidosis patients who are corticosteroid-dependent or refractory to conventional immunosuppressants. Prospective studies are now needed to confirm their efficacy and long-term safety.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"42 4","pages":"17246"},"PeriodicalIF":0.0,"publicationDate":"2025-12-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12815289/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145759089","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Unveiling the role of circulating periostin in patients with idiopathic pulmonary fibrosis: A prognostic meta-analysis.","authors":"Wasfi Alghwyeen, Shrouk F Mohamed, Ahmed Shamil Hashim, Abdelrahman M Tawfik, Ahmed Kertam","doi":"10.36141/svdld.v42i4.16980","DOIUrl":"10.36141/svdld.v42i4.16980","url":null,"abstract":"<p><strong>Background: </strong> Periostin measurement has been suggested as a noninvasive biomarker for assessing the likelihood of IPF progression and predicting patient outcomes, but these results aren't entirely consistent. Therefore, we conducted a meta-analysis to study the relation between serum periostin and IPF.</p><p><strong>Methods: </strong> We conducted a comprehensive search in major electronic biomedical databases of (PubMed, Scopus, Cochrane Library, EMBASE and Web of Science) spanning from inception to September 2024.  Meta-analysis of included studies was done, and quantitative data were pooled as standardized mean difference (SMD), odd rations (ORs) and coefficient (r) values , with corresponding 95% confidence intervals (CIs).</p><p><strong>Results: </strong>Eleven articles were included in final meta-analysis. Our results showed that there was no significant difference in periostin levels between IPF patients and healthy controls (SMD: 2.59, 95% CI: -0.59 to 5.77, p= 0.11). Moreover, it was shown that IPF patients with a progressive disease have higher periostin levels compared to those who remain stable (SMD: 0.52, 95% CI: 0.29 to 0.75, p<0.0001). In addition to, higher serum periostin levels were significantly associated with shortened overall survival among IPF patients (RR: 3.70, 95% CI: 1.84 to 7.43, p<0.0001). Finally, there was a significant negative correlation between periostin levels and  relative decline in DLCO and VC over the follow-up period (COR: -0.36, 95% CI, -0.58 to -0.10),  (COR: -0.49, 95% CI, -0.63 to -0.34)  respectively.</p><p><strong>Conclusions: </strong> This study concludes that periostin may be a valuable biomarker for predicting prognosis in IPF patients.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"42 4","pages":"16980"},"PeriodicalIF":0.0,"publicationDate":"2025-12-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12815292/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145759139","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}