Jonas A Baekdal, Laura Bjerken, Erik Sören Halvard Hansen, Laurits K Janns, Howraman Meteran, Charlotte Suppli Ulrik, Elisabeth Bendstrup
{"title":"Interstitial lung disease in psoriasis: A systematic review and meta-analysis.","authors":"Jonas A Baekdal, Laura Bjerken, Erik Sören Halvard Hansen, Laurits K Janns, Howraman Meteran, Charlotte Suppli Ulrik, Elisabeth Bendstrup","doi":"10.36141/svdld.2026.17851","DOIUrl":"10.36141/svdld.2026.17851","url":null,"abstract":"<p><strong>Background and aim: </strong>Psoriatic disease is associated with several extra-cutaneous complications, including cardiovascular disease and type 2 diabetes. Whether psoriatic disease is associated with interstitial lung disease (ILD) remains uncertain. The aim of this study was toquantify the prevalence of ILD overall and across specific subtypes of psoriatic disease.</p><p><strong>Methods: </strong>We systematically searched PubMed, Embase, and Scopus for original studies reporting the prevalence of ILD in adults with psoriatic disease. Pooled prevalence estimates were calculated using proportional random-effects meta-analysis, with subgroup analyses stratified by factors including psoriatic disease type, sex, and smoking history. Random-effects meta-regression was conducted to examine the influence of age and disease duration.</p><p><strong>Results: </strong>Of 863 records screened, 11 studies were included. The pooled ILD prevalence in any psoriatic disease was 3.6% (95% CI: 2.1-5.0%). Meta-regression indicated that ILD prevalence increased by 0.19 percentage points per year (95% CI: 0.16-0.22) from approximately 38 years of age. Subgroup analysis showed ILD prevalences of 2.4% (95% CI: 1.1-3.6%) in psoriasis vulgaris, 5.7% (95% CI: 2.5-8.9%) in psoriatic arthritis, and 8.4% (95% CI: 6.4-10.4%) in generalized pustular psoriasis (GPP). ILD prevalence was 1.2% (95% CI: 0.1-2.2%) in never-smokers versus 6.7% (95% CI: 2.3-11.1%) in ever-smokers.</p><p><strong>Conclusions: </strong>ILD may represent a clinically relevant comorbidity in patients with psoriatic disease, particularly psoriatic arthritis and GPP. This increased prevalence may reflect chronic systemic inflammation and exposures such as medications and cigarette smoke. Further research is needed to elucidate the underlying mechanisms and to assess whether early detection and targeted interventions can improve outcomes.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"43 1","pages":"17851"},"PeriodicalIF":0.0,"publicationDate":"2026-03-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13101020/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147535463","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":"Combination antifibrotic and immunosuppressive therapy in progressive fibrosing ILD.","authors":"Ryan Fischer, Khawaja Abdul Rehman","doi":"10.36141/svdld.2026.18462","DOIUrl":"10.36141/svdld.2026.18462","url":null,"abstract":"<p><strong>Background: </strong>Progressive fibrosing interstitial lung disease is characterized by continuous functional decline and early mortality despite conventional immunosuppressive therapy. The dual pathophysiology involves both inflam-mation and fibrosis and provides rationale for combination antifibrotic and immunosuppressive therapy.</p><p><strong>Objectives: </strong>To review and synthesize current clinical evidence on the efficacy, safety, and clinical positioning of combination antifibrotic and immunosuppressive therapy in progressive fibrosing interstitial lung disease.</p><p><strong>Methods: </strong>We conducted a comprehensive literature review of randomized controlled trials, observational studies, and real-world cohorts published between 2015 and 2025 that evaluated combined antifibrotic (nintedanib or pirfe-nidone) and immunosuppressive therapy in adults with non-IPF progressive fibrosing ILD. We synthesized evidence from randomized controlled trials, observational studies, and real-world data regarding their methodolo-gies, patient population, therapies and outcomes.</p><p><strong>Results: </strong>Forty-one studies were identified, including 14 RCTs and 12 observational cohorts. The available evidence suggests that combination therapy demonstrates promising results across various PF-ILDs. Clinical trials and real-world data have shown improved lung function and forced vital capacity with combination therapy in PF-ILD. Evi-dence supports combination therapy to be well-tolerated, with manageable safety and tolerability profile consistent with individual agents rather than additive effects. Limitations of the available evidence include heterogeneity of PF-ILD populations, variable background immunosuppression, and limited long-term outcome data.</p><p><strong>Conclusions: </strong>Combination antifibrotic and immunosuppressive therapy shows meaningful efficacy in select PF-ILD patients. Evidence supports individualized combination approaches, particularly for progressive systemic sclerosis-ILD and connective tissue disease-ILD with nonspecific interstitial pneumonia pattern. Critical evidence gaps remain regarding optimal sequencing, patient selection, and long-term outcomes, requiring future investigation.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"43 1","pages":"18462"},"PeriodicalIF":0.0,"publicationDate":"2026-03-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13101010/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147535417","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":"Methylprednisolone in the management of post-COVID-19 interstitial lung disease: A randomized trial (STERCOV-ILD).","authors":"Aycan Yüksel, Dilek Karadoğan, Nur Hürsoy, Tahsin Gökhan Telatar, Neslihan Köse Kabil, Feride Marım, İlknur Kaya, Aslıhan Banu Er, Merve Erçelik, Demet Polat Yuluğ, Merve Yumrukuz Şenel, Ceren İlgar, Ökkeş Gültekin, Selin Çakmakcı Karakaya, Bilge Yılmaz Kara, Neslihan Özçelik, İnci Selimoğlu, Kübra Uyar Er, Abdurrahman Kotan, Hasan Veysel Keskin, Metin Akgün","doi":"10.36141/svdld.2026.17022","DOIUrl":"10.36141/svdld.2026.17022","url":null,"abstract":"<p><strong>Background and aim: </strong>Post-COVID-19 Interstitial Lung Disease (ILD) is controversial and need for treatment is unclear.The aim of this study was to investigate the efficacy of methylprednisolone in the management of post-COVID-19 ILD in comparison to standard of care.</p><p><strong>Methods: </strong>In this multicentre, randomized controlled clinical trial, patients with post-COVID ILD were assigned to two groups: the steroid group received oral methylprednisolone at a dose of 0.5 mg/kg/day, while the control group received supportive therapy. The primary outcome was proportion of patients with functional improvement (defined as the absence of hypoxemia/desaturation during 6MWT) at twelve-weeks.</p><p><strong>Results: </strong>A total of 229 patients with post-COVID ILD patients (124 in the steroid group and 104 in the control group) completed the study. At 12-weeks, functional improvement rate was higher in the steroid group compared to the control group (74.2% vs. 55.2%, OR:2.33 [95% CI:1.34-4.06], p=0.0041). Radiological improvement was observed in 61.3% of the steroid group compared to 46.7% of the controls (OR:1.81 [95% CI:1.07-3.06], p=0.037). The mean increase in FVC (7.2% vs 3.7%, p=0.03), 6MW distance (91 vs 41 meters, p<0.001), and SpO2 (2.33 vs 1.21, p=0.002) was significantly higher in the steroid group. Multivariate regression analysis revealed that the following variables were associated with poorer outcomes: smoking (OR:0.932 [95% CI:0.875-0.992], p=0.028), older age (OR:0.951 [95% CI:0.912-0.99], p=0.035), and severe COVID-19 (OR:0.233 [95% CI:0.068-0.799], p=0.029.</p><p><strong>Conclusions: </strong>Methylprednisolone improved oxygen saturation, FVC, exercise capacity, and radiological resolution in patients with post-COVID-19 ILD compared to the natural course of the disease.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"43 1","pages":"17022"},"PeriodicalIF":0.0,"publicationDate":"2026-03-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13101009/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147535378","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}
Linling Jin, Yiting He, Chenyang Liu, Songtao Liu, Shuying Ma, Nan Li, Hui Kong, Weiping Xie, Mengyu He
{"title":"Intrapulmonary unicentric Castleman's Disease: A clinical analysis of 8 patients.","authors":"Linling Jin, Yiting He, Chenyang Liu, Songtao Liu, Shuying Ma, Nan Li, Hui Kong, Weiping Xie, Mengyu He","doi":"10.36141/svdld.2026.18073","DOIUrl":"10.36141/svdld.2026.18073","url":null,"abstract":"<p><strong>Background and aims: </strong>Castleman's disease (CD) is a rare, chronic, lymphoproliferative disorder, increasingly recognized as a clonal neoplasm of lymph node stromal cells that can affect multiple organs and tissues. Intrapulmonary unicentric Castleman's disease (UCD) represents an exceptionally uncommon subtype, primarily involving the lung parenchyma and hilum, with only a few pathologically confirmed cases reported to date. This study aimed to characterize the clinical, radiological, and pathological features of intrapulmonary UCD and to summarize diagnostic and therapeutic experiences.</p><p><strong>Methods: </strong>We retrospectively analyzed eight patients diagnosed with intrapulmonary UCD. Clinical manifestations, laboratory data, radiologic and bronchoscopic findings, histopathological characteristics, treatment approaches, and follow-up outcomes were systematically reviewed.</p><p><strong>Results: </strong>No patient exhibited specific clinical symptoms or distinctive laboratory abnormalities. Chest computed tomography (CT) served as the optimal imaging modality, consistently revealing solitary, well-circumscribed, soft-tissue masses with smooth lobulated contours. Definitive diagnosis relied on histopathological examination. All patients underwent complete surgical resection, followed by regular postoperative surveillance with chest CT. During follow-up, no local recurrence or disease progression was detected.</p><p><strong>Conclusions: </strong>Intrapulmonary UCD is an exceedingly rare form of Castleman's disease that can be easily misdiagnosed as primary lung tumors, lymphoma, granulomatous disease, or tuberculosis due to its nonspecific clinical and radiological features. Complete surgical excision remains the mainstay of treatment and is associated with an excellent prognosis following complete resection.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"43 1","pages":"18073"},"PeriodicalIF":0.0,"publicationDate":"2026-03-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13101018/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147535458","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}
Elias Giallafos, Evangelos Oikonomou, Ourania Katsarou, Lykourgos Kolilekas, Evangelos Markozanes, Kostas Zisimos, Gesthimani Seitaridi, Maria Gazouli, Efrosyni Manali, Spyros Papiris, Gerasimos Siasios, Ioannis Ilias
{"title":"Prevalence of thyroid autoantibodies in a large series of sarcoidosis patients: Implications for autoimmune thyroid disease screening.","authors":"Elias Giallafos, Evangelos Oikonomou, Ourania Katsarou, Lykourgos Kolilekas, Evangelos Markozanes, Kostas Zisimos, Gesthimani Seitaridi, Maria Gazouli, Efrosyni Manali, Spyros Papiris, Gerasimos Siasios, Ioannis Ilias","doi":"10.36141/svdld.2026.18306","DOIUrl":"10.36141/svdld.2026.18306","url":null,"abstract":"<p><strong>Background and aim: </strong>Sarcoidosis is a potentially multisystem granulomatous disease frequently associated with autoimmunity. Previous cohort studies have reported an elevated risk of developing autoimmune thyroid disease (AITD) in sarcoidosis patients. Aim of the study was to assess the prevalence of thyroid autoimmunity in a large, biopsy-proven sarcoidosis cohort and compare it to established general population rates, thereby highlighting the clinical significance of screening.</p><p><strong>Methods: </strong>To explore the prevalence of AITD, we examined a cross-sectional cohort of 668 patients with biopsy-proven sarcoidosis. Serum Thyroperoxidase Antibodies (TPOAb) and Thyroglobulin Antibodies (TgAb) were quantified using standard electrochemiluminescence immunoassays (ECLIA). The prevalence of positive thyroid autoantibodies was compared to previously published regional healthy control rates (11.4%, n=1,108) using a two-sample Z-test for comparing two proportions.</p><p><strong>Results: </strong>The overall prevalence of positive thyroid autoantibodies (TPOAb and/or TgAb) in the sarcoidosis cohort (n=668) was found to be 27.4% (95% CI: 21.7%-28.3%). This rate was significantly higher compared to the pooled historical control group prevalence of 11.4% (p < 0.001.</p><p><strong>Conclusion: </strong>The prevalence of positive thyroid autoantibodies in patients with sarcoidosis is significantly elevated compared to the healthy population. This finding underscores the strong autoimmune linkage between sarcoidosis and AITD and supports the recommendation for systematic screening for thyroid autoimmunity in patients diagnosed with sarcoidosis.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"43 1","pages":"18306"},"PeriodicalIF":0.0,"publicationDate":"2026-03-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13101019/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147535426","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":"Renal tubular lysozyme in tubulointerstitial diseases and renal sarcoidosis.","authors":"Satoru Sanada, Mitsuhiro Sato","doi":"10.36141/svdld.v42i4.16937","DOIUrl":"10.36141/svdld.v42i4.16937","url":null,"abstract":"","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"42 4","pages":"16937"},"PeriodicalIF":0.0,"publicationDate":"2025-12-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12815299/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145759153","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":"Bioinformatics analysis identifies candidate biomarkers associated with sarcoidosis.","authors":"Linling Jin, Chunfeng Pan, Yujie Sun, Jiayi Zhang, Weiping Xie, Mengyu He","doi":"10.36141/svdld.v42i4.17074","DOIUrl":"10.36141/svdld.v42i4.17074","url":null,"abstract":"<p><strong>Background and aim: </strong>Sarcoidosis is a complex inflammatory disorder characterized by the formation of non-caseating granulomas, which can affect multiple systems, with a predominant impact on the lungs and thoracic lymph nodes. The aim of the study is to identify potential biomarkers and explore the infiltration of immune cells associated with sarcoidosis. Methods: The datasets of GSE19314, GSE83456, and GSE37912 were obtained from the Gene Expression Omnibus (GEO) database. Differentially expressed genes (DEGs) were screened by comparing sarcoidosis samples to healthy controls in GSE19314 and GSE83456. Functional enrichment analyses and protein-protein interactions (PPIs) network construction were performed to elucidate the functional roles of the DEGs. Hub genes were identified through PPI network analysis. The GSE37912 dataset was used as a validation set. Immune cell infiltration in sarcoidosis patients was investigated. Furthermore, the trehalose 6,6'-dimycolate-granuloma (TDM)-induced granuloma experimental model was employed to resemble human sarcoid granulomas, and the expression of hub genes in lung tissues was detected by qRT-PCR.</p><p><strong>Results: </strong>A total of 71 common DEGs, including 53 upregulated DEGs and 18 downregulated DEGs, were identified between sarcoidosis patients and controls. The signal transducer and activator of transcription 1 (STAT1), C-X-C motif chemokine ligand 10 (CXCL10) and basic leucine zipper ATF-like transcription factor 2 (BATF2) were considered as hub genes, showing promising diagnostic potential for sarcoidosis. Immune infiltration analysis indicated that compared with the controls, sarcoidosis samples exhibited increased infiltration of activated NK cells, monocytes, macrophage, activated dendritic cells and resting mast cells. In the TDM-induced lung granuloma model, STAT1, CXCL10 and BATF2 expression was upregulated in sarcoidosis lung tissue.</p><p><strong>Conclusions: </strong>Bioinformatics analysis indicated that STAT1, CXCL10 and BATF2 may as candidate biomarkers associated with sarcoidosis.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"42 4","pages":"17074"},"PeriodicalIF":0.0,"publicationDate":"2025-12-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12815294/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145759079","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}
Inci Uslu Biner, Seval Aslan, Ilknur Ak Sivrikoz, Nazli Sena Seker
{"title":"A unique case of extrapulmonary sarcoidosis on F18 FDG PET/CT: \"Tiger man\".","authors":"Inci Uslu Biner, Seval Aslan, Ilknur Ak Sivrikoz, Nazli Sena Seker","doi":"10.36141/svdld.v42i4.17033","DOIUrl":"10.36141/svdld.v42i4.17033","url":null,"abstract":"<p><p>Sarcoidosis is a systemic granulomatous disease in which muscle involvement is a rare extrapulmonary manifestation. We present the case of a 34-year-old man with shortness of breath lasting for three months. Computerized tomography showed intrathoracic lymph nodes and perihilar consolidations in both lungs. She received treatment for pneumonia, but because of insufficient regression, she was referred to our hospital's chest diseases department for further examination. Laboratory results showed an elevated calcium. We conducted an F-18 FDG PET CT scan because the clinicians suspected cancer. PET/CT scan demonstrated hypermetabolism in bilateral lung parenchyma, multiple lymph nodes, the spleen, muscles, and cutaneous/subcutaneous lesions. With the guidance of PET/CT with a suspicion of lymphoproliferative diseases, an axillary lymph node biopsy revealed granulomas; sarcoidosis was assumed, and corticosteroids were started. This case shows rarely seen muscle involvement of sarcoidosis by emphasizing the ability of PET/CT in detecting extrapulmonary involvement of the disease.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"42 4","pages":"17033"},"PeriodicalIF":0.0,"publicationDate":"2025-12-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12815291/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145759091","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}
Joel Francesqui, Mariana Benegas, Elena Laserna, Leticia Valera, Sandra Cuerpo, Fernanda Hernández-González, Mario Serrano, Marcelo Sánchez, Jacobo Sellares
{"title":"Online multidisciplinary discussion on interstitial lung disease: Using new technologies to connect general hospitals to expert units.","authors":"Joel Francesqui, Mariana Benegas, Elena Laserna, Leticia Valera, Sandra Cuerpo, Fernanda Hernández-González, Mario Serrano, Marcelo Sánchez, Jacobo Sellares","doi":"10.36141/svdld.v42i4.17105","DOIUrl":"10.36141/svdld.v42i4.17105","url":null,"abstract":"<p><p>.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"42 4","pages":"17105"},"PeriodicalIF":0.0,"publicationDate":"2025-12-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12815284/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145759143","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":"What's the connection? Sarcoidosis and combined variable immunodeficiency: A Single center retrospective case series.","authors":"Sarah MacDowell, Arindam Singha, Elliott Crouser","doi":"10.36141/svdld.v42i4.17211","DOIUrl":"10.36141/svdld.v42i4.17211","url":null,"abstract":"<p><strong>Background and aim: </strong>Sarcoidosis is a disorder hallmarked by non-caseating granulomas and can involve multi-organ systems but most frequently affects the lungs, eyes, central nervous system, and skin.1 Combined Variable Immunodeficiency (CVID) is a disorder featuring hypogammaglobulinemia, an inadequate response to vaccination, and a history of recurrent bacterial and viral infections, most commonly pneumonia, sinusitis, and ear infections. 2 CVID can also present with non-caseating granulomas, including in Granulomatous Lymphocytic Interstitial Lung Disease (GLILD), making distinguishing between these conditions challenging. There continues to be a debate about how these two conditions are related and what the guidelines should be related to checking quantitative immunoglobulins among those suspected of having sarcoidosis.</p><p><strong>Methods: </strong>This single-center retrospective review was performed at a large midwestern academic center with a large sarcoidosis referral base using available data from the past seventeen years. ICD-9 and ICD-10 codes for CVID and Sarcoidosis were used to identify individuals in billing for outpatient encounters Results: A total of 1920 cases of sarcoidosis and 231 cases of CVID were identified, with eight individuals identified as having both CVID and sarcoidosis diagnoses coded in the same outpatient clinical encounter, which is just 0.042% of the total number of sarcoidosis cases.</p><p><strong>Conclusions: </strong>In place of universal quantitative immunoglobulin screening among those with sarcoidosis, which has additional costs and leads to further challenges with other causes of hypogammaglobulinemia other than CVID, this review points out essential patient features where testing should instead be focused. Identified patient features based on this review that should lead to quantitative immunoglobulin testing include a personal history of recurrent sinopulmonary infections, individual or family history of autoimmune conditions, computed tomography (CT) chest with non-traction-related bronchiectasis, and middle to lower lobe pattern of lung involvement on CT.</p>","PeriodicalId":502142,"journal":{"name":"Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG","volume":"42 4","pages":"17211"},"PeriodicalIF":0.0,"publicationDate":"2025-12-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12815295/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145759137","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}