Clinical RheumatologyPub Date : 2025-06-01Epub Date: 2025-04-16DOI: 10.1007/s10067-025-07440-w
Esra Mehmetoglu, Anvitha Mummadisetty, Andreas Chatzittofis, Konstantinos Parperis, Nora Sandorfi, Chris T Derk
{"title":"A systematic review of prevalence and predictors of depression in systemic sclerosis based on the CES-D, BDI, and PHQ- 9 self-assessment questionnaires.","authors":"Esra Mehmetoglu, Anvitha Mummadisetty, Andreas Chatzittofis, Konstantinos Parperis, Nora Sandorfi, Chris T Derk","doi":"10.1007/s10067-025-07440-w","DOIUrl":"10.1007/s10067-025-07440-w","url":null,"abstract":"<p><strong>Objectives: </strong>This study aimed to investigate the prevalence of depression in Systemic Sclerosis (SSc) patients using depression self-assessment tools such as the Beck Depression Inventory (BDI), the Centre for Epidemiologic Studies Depression Scale (CES-D), and the Patient Health Questionnaire (PHQ-9) and to explore the clinical characteristics of SSc patients with depression and identify potential risk factors for depression.</p><p><strong>Methods: </strong>Based on PRISMA guidelines, an electronic search was conducted in PubMed, Embase, PsycINFO, and Google Scholar to collect studies assessing systemic sclerosis and depression up to April 2024. Original research studies in SSc patients and depression using BDI > 10, CES-D > 16, and PHQ-9 > 10 that met our inclusion criteria were included and appraised using the Joanna Briggs Institute (JBI) instrument, then data extraction was performed.</p><p><strong>Results: </strong>We identified 497 articles, of which 22 were included in this systematic review. Among the 4,165 patients who completed the self-assessment questionnaires, 1486 (35.6%) met the criteria for depression where 564 (31.9%) of patients were identified based on the CES-D, 410 (55.1%) from the BDI, and 512 (30.8%) from the PHQ-9 group. Gastrointestinal involvement was the most identified predictor for depression, while pain, disease activity, and pulmonary and joint symptoms of SSc were also commonly associated with depression. Non-SSc-related predictors of depression included unemployment, low educational level, and unmarried status.</p><p><strong>Conclusion: </strong>Higher prevalence of depression is seen among patients with SSc. Although the prevalence varies according to the assessment tool used, we found correlation of depression estimates in SSc-patients between the CES-D and PHQ-9 scores. Moreover, this review identifies the significant predictors of depression in SSc patients and highlights the need of mental healthcare professionals to engage in the care of SSc patients.</p><p><strong>Conclusion: </strong>HB level was significantly related to disease activity and structural damage in RA patients. Key Points • There is significant variability in estimates of depression among different self-assessment questionnaires in patients with SSc. • We found correlation of depression estimates in SSc-patients between the CES-D and PHQ9 scores. • Among the studies reviewed strong predictors of depression in SSc-patients were identified. These were significant GI and arthritic manifestations, increased pain and disease severity, and a lower educational level. • Importance in engaging mental healthcare professionals in the care of SSc- patients as per our study up to a third of patients may benefit from this.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":"2327-2341"},"PeriodicalIF":2.9,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12141151/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143975875","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Infrapatellar fat pad stiffness is associated with knee symptoms in patients with knee osteoarthritis.","authors":"Sayaka Okada, Masashi Taniguchi, Masahide Yagi, Shogo Okada, Kaede Nakazato, Yoshiki Motomura, Yoshihiro Fukumoto, Masashi Kobayashi, Kyoseki Kanemitsu, Noriaki Ichihashi","doi":"10.1007/s10067-025-07442-8","DOIUrl":"10.1007/s10067-025-07442-8","url":null,"abstract":"<p><strong>Objective: </strong>To clarify the association between infrapatellar fat pad (IFP) stiffness and size with knee osteoarthritis (OA) severity and knee symptoms.</p><p><strong>Method: </strong>This study included 93 participants: 66 with symptomatic medial knee OA (Kellgren-Lawrence grade [KL] ≥ 2) and 27 healthy controls (KL grade 0 or 1) with asymptomatic knees. Shear modulus (stiffness) and thickness (size) of the IFP were measured using ultrasonography. The Knee Society Score (KSS) was used to assess knee symptoms. Maximum knee extension strength and external knee adduction moment during walking, both related to the symptoms of knee OA, were measured. The shear modulus and thickness of the IFP were compared among three groups: control, mild OA (KL grade 2), and severe OA (KL grades 3 and 4). Multiple regression analysis was performed to determine the association of IFP shear modulus and thickness with knee symptoms.</p><p><strong>Results: </strong>The IFP shear modulus was higher in the severe OA group than that in the control group, with no significant differences among the other groups. There were no significant differences in thickness between the groups. The shear modulus was significantly associated with the KSS symptom scores, indicating that a stiffer IFP correlated with more severe knee OA symptoms. Conversely, the IFP thickness was not associated with the KSS symptom scores.</p><p><strong>Conclusions: </strong>Infrapatellar fat pad stiffness was higher in severe knee OA, and greater stiffness was associated with more severe symptoms, suggesting that IFP stiffness may serve as both an assessment marker and therapeutic target in knee OA management.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":"2455-2462"},"PeriodicalIF":2.9,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143980554","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Interaction of nutritional and inflammatory levels on all-cause mortality among individuals with rheumatoid arthritis: a prospective cohort study.","authors":"Zhuang Ma, Shixin Wu, Lin-En Xiong, Juncheng Zhong, Baiwen Lin, Liangkai Chen, Ting Xiong, Yuanjue Wu","doi":"10.1007/s10067-025-07458-0","DOIUrl":"10.1007/s10067-025-07458-0","url":null,"abstract":"<p><strong>Background: </strong>Malnutrition and systemic inflammation are frequently prevalent in individuals with rheumatoid arthritis (RA). However, limited studies have explored the combined impacts of nutritional and inflammatory levels on all-cause mortality among RA. This study is aimed at investigating these potential associations.</p><p><strong>Methods: </strong>We involved 2213 RA patients from the National Health and Nutrition Examination Survey (2001-2018). Nutritional risk index (NRI) and systemic inflammatory response index (SIRI) were used to evaluate the nutritional and inflammatory status of participants, respectively. Weight-based Kaplan-Meier survival curves and COX proportional hazard models were employed to ascertain the independent and joint association.</p><p><strong>Results: </strong>Over a median follow-up of 7.98 years, 544 deaths occurred. Following the adjustment for confounding factors, we found that individuals with moderate/severe malnutrition (HR, 2.13; 95%CI, 1.50-3.03) or high SIRI (HR, 1.49; 95%CI, 1.07-2.07) were independently associated with an increased risk of all-cause mortality. Furthermore, the simultaneous moderate/severe malnutrition and high SIRI further elevated the mortality risk (HR, 2.34; 95%CI, 1.53-3.56). An additive interaction was observed between malnutrition and high SIRI, yielding an excess risk of 0.34 (95%CI 0.06-0.62, P = 0.014), and the attributable proportion for the interaction was 20.9% (95%CI 7.6-34.2%).</p><p><strong>Conclusions: </strong>This research indicated that malnutrition and high systemic inflammation levels were independent risk factors for the prognosis of RA patients, and co-occurrence can further deteriorate the prognosis. Our findings highlight the importance of maintaining nutrition and anti-inflammation in patients with RA, providing new insights into the prognosis of RA patients.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":"2131-2141"},"PeriodicalIF":2.9,"publicationDate":"2025-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143977527","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Letter to the editor: CD3 +CD4 +T cells counts refect the severity and prognosis of invasive pulmonary aspergillosis in patients with connective tissue disease-associated interstitial lung disease.","authors":"Ling Xu, Dong Shi, Diao Yu","doi":"10.1007/s10067-025-07513-w","DOIUrl":"https://doi.org/10.1007/s10067-025-07513-w","url":null,"abstract":"","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2025-05-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144191573","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Anne-Mari Mustonen, Juha Savinainen, Marko Lehtonen, Petri Lehenkari, Tommi Kääriäinen, Antti Joukainen, Heikki Kröger, Petteri Nieminen
{"title":"Synovial fluid o-tyrosine is a potential biomarker for autoimmune-driven rheumatoid arthritis.","authors":"Anne-Mari Mustonen, Juha Savinainen, Marko Lehtonen, Petri Lehenkari, Tommi Kääriäinen, Antti Joukainen, Heikki Kröger, Petteri Nieminen","doi":"10.1007/s10067-025-07491-z","DOIUrl":"https://doi.org/10.1007/s10067-025-07491-z","url":null,"abstract":"<p><strong>Introduction/objectives: </strong>The aim of the present study was to identify key amino acid (AA) pathways in osteoarthritis (OA) and autoimmune-driven rheumatoid arthritis (RA), as AAs have emerged as potential biomarkers for the detection of degenerative joint diseases. It was hypothesized that we would detect distinct metabolic pathways activated in OA and RA due to different degrees of inflammation.</p><p><strong>Method: </strong>Samples of synovial fluid (SF) and infrapatellar Hoffa's fat pad (IFP) were collected from end-stage knee OA (n = 10) and RA patients (n = 10), and from non-inflammatory controls (n = 5). Metabolites were analyzed utilizing a liquid chromatography high-resolution mass spectrometry approach, followed by univariate and multivariate statistical testing and pathway analysis by MetaboAnalyst. Receiver operating characteristic analysis was used to examine diagnostic values.</p><p><strong>Results: </strong>SF results identified o-tyrosine as a promising biomarker for distinguishing RA patients from OA patients and controls, and cystine, cysteine, and methionine separating OA patients from controls. Regarding IFPs, β-alanine could have diagnostic value to discriminate RA and OA. The present data indicate alterations in metabolic pathways, such as cysteine and methionine metabolism in RA and OA SFs compared to control SF, selenocompound metabolism in RA vs. OA SFs, and pyrimidine metabolism in RA vs. OA IFPs.</p><p><strong>Conclusions: </strong>The identified nitrogen compounds, particularly o-tyrosine, and AA metabolism pathways have potential as novel diagnostic and therapeutic targets for degenerative joint diseases. Key Points • Synovial fluid o-tyrosine can distinguish rheumatoid arthritis from osteoarthritis and control. • Synovial fluid cystine, cysteine, and methionine separate osteoarthritis from control. • β-Alanine in intra-articular fat has diagnostic value between rheumatoid arthritis and osteoarthritis. • A routine measurement of o-tyrosine would be useful in the future as an indicator of rheumatoid arthritis.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2025-05-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144191574","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Multidimensional assessment of Juvenile Sjögren disease.","authors":"Şeyma Türkmen, Taner Coşkuner, Betül Sözeri","doi":"10.1007/s10067-025-07486-w","DOIUrl":"https://doi.org/10.1007/s10067-025-07486-w","url":null,"abstract":"<p><strong>Objectives: </strong>This study aims to provide a multidimensional assessment of juvenile Sjögren's disease (jSjD), focusing on diagnostic and management processes through various instrumental tests.</p><p><strong>Methods: </strong>A cross-sectional and retrospective study was conducted on 21 jSjD patients diagnosed between June 2016 and September 2023. In addition, 37 healthy children were included only for comparison of nailfold videocapillaroscopy (NVC) findings. Clinical data, parotid gland ultrasound (US), NVC, salivary gland biopsy, and Schirmer test results were analyzed.</p><p><strong>Results: </strong>Positive correlations were found between parotitis scores in parotid gland US and bizarre capillary, neoangiogenesis, and microhemorrhage scores in NVC (r = 0.35, 0.32, 0.52; p < 0.05). Diagnostic delay was associated with higher parotitis frequency and scores in US (p = 0.025) and increased dilated capillaries in NVC (p < 0.05). Clinically evident parotitis correlated with higher parotitis frequency in US (p = 0.009) and higher parotitis scores (r = 0.480, p = 0.051). Higher ESSDAI scores at diagnosis correlated with increased parotitis frequency and scores in US (p = 0.001), with higher scores in patients with high disease activity (p < 0.05). ENA-positive patients had higher parotitis scores in US (p = 0.022) and increased cross capillaries in NVC (p = 0.024). Capillary density was lower in the jSjD group compared to healthy controls (p = 0.021), with healthy children having higher median capillary density (p = 0.005). Dilated, bushy, bizarre capillaries, and microhemorrhages were significantly higher in the jSjD group (p < 0.001).</p><p><strong>Conclusion: </strong>Early and accurate diagnosis is crucial for managing jSjD. The integration of US and NVC provides a comprehensive framework for detecting glandular and microvascular abnormalities, emphasizing the need for multidimensional approach. Key Points • This study provides a multidimensional and comprehensive assessment of Juvenile Sjögren's disease (jSjD) by combining various instrumental tests used in the diagnosis and monitoring of Sjögren's disease. • It is the first study in the literature to demonstrate the connection between glandular and microvascular abnormalities in jSjD patients. • The comparison of nailfold videocapillaroscopy findings in healthy children highlights the pronounced microvascular abnormalities present in jSjD patients. • The results emphasize the importance of early diagnosis and intervention, offering critical insights into the systemic effects and clinical progression of the disease.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2025-05-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144186661","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Dionicio Ángel Galarza-Delgado, Jorge Eduardo Juárez-Silva, Jesús Alonso Baca-Soto, Andrea Citlalli Flores-Álvarez, José Luis Treviño-González
{"title":"Subclinical sensorineural hearing loss in systemic lupus erythematosus: a comparative cross-sectional study.","authors":"Dionicio Ángel Galarza-Delgado, Jorge Eduardo Juárez-Silva, Jesús Alonso Baca-Soto, Andrea Citlalli Flores-Álvarez, José Luis Treviño-González","doi":"10.1007/s10067-025-07488-8","DOIUrl":"https://doi.org/10.1007/s10067-025-07488-8","url":null,"abstract":"<p><strong>Introduction: </strong>Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease that damages multiple target organs, including the ear. This study aims to determine the prevalence of hearing loss in patients with a confirmed diagnosis of SLE compared to controls and to assess frequency-specific hearing thresholds using extended audiometry (500 to 16,000 Hz).</p><p><strong>Methods: </strong>This comparative cross-sectional study included 75 patients diagnosed with SLE and 150 matched control subjects. All subjects underwent otolaryngological evaluation, conventional and extended audiometry (500 to 8,000 and 10,000 to 16,000 Hz, respectively), and tympanometry.</p><p><strong>Results: </strong>Patients with SLE showed a higher prevalence of sensorineural hearing loss (SNHL) at all frequencies and elevated thresholds at very high frequencies compared to the controls (p < 0.05). The most affected rage was 10-16 kHz, with an average threshold of 24.7 dB (IQR 13.1 - 44.4) and a SNHL prevalence of 50.7% in patients with SLE vs. 9.3% and 18.7% in both control groups (p < 0.001). Multivariate analysis showed that being a patient with SLE (OR = 10.84; p < 0.001) and older age (OR = 1.13/year; p < 0.001) were independent predictors of SNHL. In the SLE subgroup, age remained the only significant predictor (OR = 1.16; p < 0.001).</p><p><strong>Conclusion: </strong>Patients with SLE have higher odds of developing SNHL compared to healthy controls and demonstrate a significantly increased prevalence of SNHL, particularly at very-high frequencies, which can only be detected through extended audiometry. These findings underscore the utility of extended audiometry in early detection of subclinical SNHL. Key Points • Patients with SLE shows a higher prevalence of SNHL, especially in very-high frequencies. • Patients with SLE demonstrated increased hearing thresholds when compared to control groups, particularly in very-high frequencies. • No significance was identified when compared chloroquine use and SNHL prevalence. • SLE and age were significant predictors of SNHL, whereas comorbidities, disease activity, and treatment were not.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2025-05-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144186662","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Gabriel Ştefan, Andreea Niculescu, Simona Cinca, Corina Chiriac, Adrian Zugravu, Pavel Cristina, Razvan Adrian Ionescu, Valer Mihai Pompilian, Nicoleta Petre, Cristina Căpusa, Simona Stancu
{"title":"Kidney involvement in VEXAS syndrome: insights from a rare case of secondary amyloidosis and systematic review of renal biopsy-confirmed reports.","authors":"Gabriel Ştefan, Andreea Niculescu, Simona Cinca, Corina Chiriac, Adrian Zugravu, Pavel Cristina, Razvan Adrian Ionescu, Valer Mihai Pompilian, Nicoleta Petre, Cristina Căpusa, Simona Stancu","doi":"10.1007/s10067-025-07506-9","DOIUrl":"https://doi.org/10.1007/s10067-025-07506-9","url":null,"abstract":"<p><p>VEXAS syndrome is a recently identified autoinflammatory disorder caused by somatic mutations in the UBA1 gene, leading to systemic inflammation and hematologic abnormalities. While its renal involvement remains poorly understood, reported cases suggest a diverse spectrum of kidney pathology. We present a 69-year-old male with a history of systemic inflammation who developed nephrotic syndrome and worsening kidney function. His disease course included recurrent fevers, pulmonary infiltrates, cutaneous vasculitis, and hematologic abnormalities. A kidney biopsy revealed secondary amyloidosis, indicating chronic inflammation. Genetic testing confirmed a UBA1 mutation (c.121A > G, p.Met41Val), establishing the diagnosis of VEXAS syndrome. Despite treatment with corticosteroids, cyclosporine, and interleukin-1 blockade, the patient deteriorated and ultimately succumbed to septic shock. A systematic review of biopsy-confirmed renal involvement in VEXAS syndrome identified 23 cases, with interstitial nephritis as the most frequent histopathologic finding, followed by vasculitis, IgA nephropathy, minimal change disease, and amyloidosis. Treatment responses varied, with limited efficacy of immunosuppressive therapies. This case highlights the under-recognized renal manifestations of VEXAS syndrome, demonstrating its diagnostic challenges and the need for heightened clinical suspicion. Further research is required to define optimal management strategies and improve patient outcomes.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2025-05-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144186659","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Letter to the Editor: Assessing early therapeutic drug monitoring of adalimumab as a predictor of treatment efficacy and immunogenicity in rheumatic diseases: \"early therapeutic drug monitoring of adalimumab\".","authors":"Syeda Hani Ammad, Tayyaba Malik, Waleed Ahmad","doi":"10.1007/s10067-025-07516-7","DOIUrl":"https://doi.org/10.1007/s10067-025-07516-7","url":null,"abstract":"","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2025-05-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144186660","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Exploring the clinical characteristics and long-term outcome of serologically active clinically quiescent IgG4-related disease: knowledge from a prospective cohort.","authors":"Jingna Li, Yuxue Nie, Fei Teng, Linyi Peng, Jie Meng, Qinhuan Luo, Nianyi Zhang, Jialei Zhang, Xinli Yang, Yifei Wang, Yunyun Fei, Jiaxin Zhou, Wen Zhang","doi":"10.1007/s10067-025-07507-8","DOIUrl":"https://doi.org/10.1007/s10067-025-07507-8","url":null,"abstract":"<p><strong>Objective: </strong>In the long-term management of IgG4-related disease (IgG4-RD), it is common to observe clinical remission accompanied by elevated IgG4 levels, namely, serologically active clinically quiescent (SACQ). We aimed to determine and characterize the SACQ status in IgG4-RD and evaluate its prognosis.</p><p><strong>Methods: </strong>We performed this single-center study based on a prospective IgG4-RD cohort. SACQ and serologically quiescent clinically quiescent (SQCQ) were defined as clinical remission with elevated but steady (for SACQ)/normal (for SQCQ) serum IgG4 for at least two years. Characteristics and prognoses between the SACQ and SQCQ groups were compared. Univariate and multivariate Cox regressions were used to investigate risk factors for relapse in SACQ patients.</p><p><strong>Results: </strong>The study included 268 SACQ and 108 SQCQ patients with IgG4-RD. Compared to the SQCQ group, the SACQ group had higher baseline levels of IgG4 and a greater prevalence of proliferative subtype (both P < 0.001). Plasmablasts were increased, while unswitched memory B cells and regulatory B cells were decreased in SACQ patients compared with SQCQ patients. Both groups experienced glucocorticoid (GC) tapering and had similar relapse rates (P = 0.659) during an average follow-up of 55 months, although the SACQ group was at a higher GC maintenance dose. In the SACQ group, serum IgG4 level re-elevation to ≥ 1.269 times the start of SACQ status was associated with relapse (HR 1.40, 95% CI: 1.16-1.42, P < 0.001; AUC = 0.791).</p><p><strong>Conclusion: </strong>SACQ patients achieved outcomes similar to SQCQ with cautious GC tapering. Monitoring serum IgG4 re-elevation might be useful for predicting relapses in the long-term management of SACQ status in IgG4-RD. Key Points • During clinical remission in IgG4-RD, a considerable proportion of patients still have elevated serum IgG4, which we first define as serologically active and clinically quiescent (SACQ). • SACQ patients achieved relapse rates similar to SQCQ patients with cautious glucocorticoid tapering. • Monitoring serum IgG4 re-elevation might help predict relapses in the long-term management of IgG4-RD SACQ patients.</p>","PeriodicalId":10482,"journal":{"name":"Clinical Rheumatology","volume":" ","pages":""},"PeriodicalIF":2.9,"publicationDate":"2025-05-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144186657","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}