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Anti-synthetase and myelodysplastic syndromes with deep morphea: an example of shared immunopathogenesis? A case-based review. 抗合成酶和骨髓增生异常综合征伴深部斑秃:共同免疫发病机制的范例?基于病例的综述。
IF 3.2 3区 医学 Q2 RHEUMATOLOGY Pub Date : 2024-11-01 Epub Date: 2024-09-09 DOI: 10.1007/s00296-024-05717-y
Agustín Hernández-López, Yatzil Reyna-Juárez, María José Ostos-Prado, Beatriz Alcalá-Carmona, Jiram Torres-Ruiz, Silvia Méndez-Flores, Salvador Escobar-Ceballos, Braulio Martínez-Benitez, Diana Gómez-Martín

Anti-synthetase syndrome (AS) is a subset of idiopathic inflammatory myopathy (IIM) characterized by the presence of anti-aminoacyl-transfer RNA synthetase accompanied by myositis, interstitial lung disease and other clinical features. According to a recent multicentric study, 31% of AS patients present skin lesions compatible with dermatomyositis, but sclerodermiform features are rare. Therefore, we aimed to report the case of a patient with simultaneous diagnosis of AS, deep morphea, vasculitic neuropathy, and myelodysplastic syndrome and review the current literature regarding these uncommon associations. A 57 year old man with axial and symmetrical proximal muscle weakness, skin thickening and B symptoms, later diagnosed with PL7 + AS, deep morphea, myelodysplastic syndrome (MDS) and vasculitic neuropathy documented by histopathologic studies and immunologic assessments. Since both AS and deep morphea share the vasculopathic changes and type II interferon-induced inflammation, we hypothesize that they may share pathogenic mechanisms. The muscle biopsy of the patient was consistent with AS and showed focal neutrophil infiltration. The patient received intensive immunosuppressive therapy for AS and vasculitic neuropathy, with high dose steroids, intravenous immunoglobulin (IVIg) and rituximab. Nonetheless, he suffered an unfavorable evolution with a fatal outcome due to septic shock. Albeit sclerodermiform features are rare in patients with AS, we propose a pathogenic link among AS, deep morphea and the autoimmune/autoinflammatory signs of MDS. The vasculopathic changes along with the activation of the innate and adaptive immune system leading to the production of proinflammatory cytokines may have been one of the contributing factors for the coexisting diagnosis of the patient.

抗合成酶综合征(AS)是特发性炎症性肌病(IIM)的一个分支,其特点是存在抗氨基酸酰转移核糖核酸合成酶,并伴有肌炎、间质性肺病和其他临床特征。根据最近的一项多中心研究,31%的强直性脊柱炎患者出现了与皮肌炎相符的皮肤病变,但硬皮样特征却很少见。因此,我们旨在报告一例同时被诊断为强直性脊柱炎、深部病变、血管神经病和骨髓增生异常综合征的患者,并回顾目前有关这些不常见关联的文献。一名 57 岁的男子患有轴性和对称性近端肌无力、皮肤增厚和 B 症状,后经组织病理学研究和免疫学评估确诊为 PL7 + AS、深部病变、骨髓增生异常综合征(MDS)和血管炎性神经病。由于强直性脊柱炎和深度病变都具有血管病理变化和II型干扰素诱导的炎症,我们推测它们可能具有相同的致病机制。患者的肌肉活检结果与强直性脊柱炎一致,并显示局灶性中性粒细胞浸润。患者因强直性脊柱炎和血管性神经病接受了强化免疫抑制治疗,包括大剂量类固醇、静脉注射免疫球蛋白(IVIg)和利妥昔单抗。然而,他的病情发展并不乐观,最终因脓毒性休克而死亡。尽管硬皮样特征在强直性脊柱炎患者中很少见,但我们认为强直性脊柱炎、深部病变和MDS的自身免疫/自体炎症症状之间存在致病联系。血管病理变化以及先天性和适应性免疫系统的激活导致了促炎细胞因子的产生,这可能是导致患者同时被诊断为强直性脊柱炎的原因之一。
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引用次数: 0
Correction: Paradoxical psoriasis induced by IL-17 inhibitors: a case series of patients with axial spondyloarthritis and a systematic literature review. 更正:IL-17抑制剂诱发的矛盾性银屑病:轴性脊柱关节炎患者病例系列及系统性文献综述。
IF 3.2 3区 医学 Q2 RHEUMATOLOGY Pub Date : 2024-11-01 DOI: 10.1007/s00296-024-05687-1
Nikolaos Chaitidis, Zoi Papadopoulou, Stavritsa Taxiarchoula Varvara, Michail Panagiotidis, Ioanna Katsigianni, Grigorios T Sakellariou
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引用次数: 0
The impact of histopathological criteria for definite vasculitis in giant cell arteritis: retrospective analysis of temporal artery biopsies. 组织病理学标准对巨细胞动脉炎明确血管炎的影响:颞动脉活检的回顾性分析。
IF 3.2 3区 医学 Q2 RHEUMATOLOGY Pub Date : 2024-11-01 Epub Date: 2024-09-08 DOI: 10.1007/s00296-024-05708-z
Güllü Sandal Uzun, Özay Gököz, Betül Oğüt, Aylin Heper, Servet Güreşçi, Rıza Can Kardaş, Mehmet Akif Öztürk, Emine Uslu, Aşkın Ateş, Berkan Armağan, Ahmet Omma, Levent Kılıc, Omer Karadag

Histopathological findings associated with definite vasculitis in temporal artery biopsy (TAB) defined in 2022 ACR/EULAR classification criteria for Giant Cell Arteritis (GCA) was published in 2022. We aimed to evaluate the TAB of our GCA patients for histopathological findings associated with definite vasculitis. Patients who were diagnosed with GCA by clinicians and underwent TAB between January 2012 and May 2022 were included. Hospital electronic records and patients' files were reviewed retrospectively. A total of 90 patients' pathology reports were evaluated by a pathologist and a rheumatologist. In cases where microscopic findings were not specified in the pathology reports, histopathologic specimens were re-evaluated (n = 36). A standard checklist was used for histopathological findings of definite vasculitis. Patients were divided into two groups; (i) definite vasculitis-GCA and (ii) non-definite-GCA group, and the clinical and demographic characteristics for all patients were compared. The mean age of patients was 69.8 (± 8.5) years and 52.2% were female. In the first evaluation, 66 (73.3%) patients had a diagnosis of vasculitis according to pathology reports. In the re-evaluation of biopsy specimens, at least one definite finding of vasculitis was observed in TAB of 10/24 (41.6%) patients whose microscopic findings were not specified in the pathology reports. The ROC analysis showed that biopsy length had diagnostic value in predicting the diagnosis of definite vasculitis (AUC: 0.778, 95% CI: 0.65-0.89, p < 0.001). In those with a biopsy length of ≥ 1 cm, sensitivity was 76.5%, specificity was 64.3%, and PPV value was 92. In multivariate analysis, the most significant factor associated with definite vasculitis was biopsy length (OR: 1.18 (1.06-1.31), p = 0.002). Microscopic findings were reported in over 70% of patients. Reinterpretation of results according to a standard check-list improved the impact of TAB in the diagnosis of GCA. A biopsy length ≥ 1 cm was found to contribute towards a definitive histopathological vasculitis diagnosis.

2022 年发布的 ACR/EULAR 巨细胞动脉炎(GCA)分类标准中定义了颞动脉活检(TAB)中与明确的血管炎相关的组织病理学结果。我们的目的是评估 GCA 患者的颞动脉活检组织病理学结果是否与明确的血管炎相关。我们纳入了在2012年1月至2022年5月期间被临床医生诊断为GCA并接受TAB检查的患者。对医院电子病历和患者档案进行了回顾性审查。病理学家和风湿病学家共评估了90名患者的病理报告。如果病理报告中没有明确说明显微镜检查结果,则对组织病理学标本进行重新评估(36 例)。组织病理学检查结果明确为血管炎的,采用标准检查表。患者被分为两组:(i) 明确的血管炎-GCA 组和 (ii) 非明确的血管炎-GCA 组,并对所有患者的临床和人口统计学特征进行了比较。患者的平均年龄为 69.8(± 8.5)岁,52.2% 为女性。在首次评估中,66 例(73.3%)患者根据病理报告确诊为血管炎。在对活检标本进行再次评估时,10/24(41.6%)名患者的活检标本中至少有一项明确的血管炎发现,而这些患者的病理报告中并未明确说明显微镜下的发现。ROC 分析显示,活检样本长度在预测确诊脉管炎方面具有诊断价值(AUC:0.778,95% CI:0.65-0.89,p
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引用次数: 0
Secukinumab is not associated with cancer recurrence or progression in patients with spondyloarthritis and history of neoplastic disease. 对于患有脊柱关节炎并有肿瘤病史的患者来说,塞库单抗与癌症复发或进展无关。
IF 3.2 3区 医学 Q2 RHEUMATOLOGY Pub Date : 2024-11-01 Epub Date: 2024-03-20 DOI: 10.1007/s00296-024-05571-y
Nicola Farina, Alessandro Tomelleri, Nicola Boffini, Adriana Cariddi, Stefania Calvisi, Elena Baldissera, Marco Matucci-Cerinic, Lorenzo Dagna

Secukinumab is a monoclonal antibody directed against interleukin-17 approved for the treatment of psoriasis and spondyloarthritis. The favorable oncological profile of secukinumab in patients with a history of malignancy has been shown in patients with psoriasis. However, systematic data to this regard have not been published yet for patients with spondyloarthritis. The objective of the present study was to evaluate the oncological safety of secukinumab in patients affected by this group of diseases. We performed a retrospective study in which we identified from our cohort patients with spondyloarthritis treated with secukinumab and with a history of malignancy. These patients' baseline demographic, treatment, rheumatological, and oncological data were collected. The neoplastic outcome (i.e., cancer recurrence or progression) after secukinumab start was then analyzed. Our study included 22 patients with spondyloarthritis. The most frequently reported oncological diagnosis was breast cancer (9 [41%] patients). Secukinumab was started after a median of 24 months following cancer diagnosis. At this time point, all but three patients were in oncological remission. No case of cancer relapse or progression was recorded over a median follow-up of 30 months. In the largest cohort reported to date to this regard, secukinumab was not associated with oncological recurrence or progression in patients with spondyloarthritis with a history of malignancy. Secukinumab may, therefore, represent a safe option in this clinical scenario.

secukinumab 是一种针对白细胞介素-17 的单克隆抗体,已被批准用于治疗银屑病和脊柱关节炎。secukinumab 对有恶性肿瘤病史的银屑病患者具有良好的抗肿瘤作用。然而,脊柱关节炎患者在这方面的系统数据尚未公布。本研究旨在评估 secukinumab 在这类疾病患者中的肿瘤安全性。我们进行了一项回顾性研究,从队列中找出了接受过赛库单抗治疗且有恶性肿瘤病史的脊柱关节炎患者。我们收集了这些患者的人口统计学、治疗、风湿病学和肿瘤学基线数据。然后对开始使用secukinumab后的肿瘤结果(即癌症复发或进展)进行了分析。我们的研究包括 22 名脊柱关节炎患者。最常见的肿瘤诊断是乳腺癌(9 例 [41%])。塞库单抗在确诊癌症后的中位 24 个月后才开始使用。在这个时间点上,除 3 名患者外,其他患者的肿瘤均得到缓解。在 30 个月的中位随访期间,没有记录到癌症复发或进展的病例。在迄今为止报告的最大规模队列中,赛库单抗与有恶性肿瘤病史的脊柱关节炎患者的肿瘤复发或恶化无关。因此,在这种临床情况下,赛库单抗可能是一种安全的选择。
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引用次数: 0
Posture, balance and gait in axial spondyloarthritis: a case-control study. 轴性脊柱关节炎的姿势、平衡和步态:一项病例对照研究。
IF 3.2 3区 医学 Q2 RHEUMATOLOGY Pub Date : 2024-11-01 Epub Date: 2024-09-04 DOI: 10.1007/s00296-024-05710-5
Erdem Türk, Fatma Gül Yurdakul, Tuba Güler, Hatice Bodur

Axial spondyloarthritis (axSpA) is a chronic inflammatory disease that primarily involves the axial skeleton but may also present with peripheral joint involvement and extra-articular involvement. The present study aims to quantitatively analyze posture, balance, and gait parameters in patients with axSpA and and assess associated factors. This cross-sectional case-control study included 51 axSpA patients (30 males, 21 females; mean age 40.94 ± 10.48 years) and 51 age- and sex-matched healthy controls. In patients with axSpA, the Ankylosing Spondylitis Disease Activity Score CRP, the Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), the Bath Ankylosing Spondylitis Functional Index (BASFI), the Bath Ankylosing Spondylitis Metrology Index (BASMI), the Maastrich Ankylosing Spondylitis Enthesitis Score (MASES), and the Ankylosing Spondylitis Quality of Life (ASQoL) scale were used. For postural analysis, DIERS formetric (Diers GmbH, Schlangenbad, Germany) videoraster- stereography device was utilized. HUR SmartBalance BTG4 (HUR-labs Oy, Kokkola, Finland) balance platform was used for postural balance and limit of stability (LOS) measurement. Participants were evaluated using Berg Balance Scale (BBS), Functional Reach Test (FRT) and Timed Up and Go Test (TUG). The Zebris FDM type 3 (Zebris Medical GmbH, Germany) walking platform was used to measure the spatiotemporal parameters of the participants. Comparison of postural parameters showed that sagittal imbalance and cervical depth distance were increased in the axSpA group than in the healthy participants (p < 0.004). Comparison of functional balance parameters showed that BBS and FRT scores were significantly lower (p < 0.001) in the axSpA group than in the control group, while TUG scores were significantly higher (p < 0.001). The LOS values, which evaluate dynamic balance were significantly lower, indicating impairment, in the axSpA group. In the measurement of postural sway, which indicates static balance, all 23 subparameters were found to be similar. When analyzing the spatiotemporal gait parameters, in the axSpA group compared with those in the control group; Foot angles (p= 0.028) and stride width (p = 0.004) were increased, whereas step lengths (p = 0.004) and stride lengths (P = 0.004) were decreased. In the axSpA group the gait speed was decreased (p = 0.004). When axSpA was analyzed separately as radiographic and nonradiographic axSpA, similar findings were observed in posture, balance, and gait parameters. No significant difference was observed. We found that the clinical assessments most closely associated with posture, balance, and gait analyses were BBS, FRT, TUG, and BASFI.

轴性脊柱关节炎(axSpA)是一种慢性炎症性疾病,主要累及轴性骨骼,但也可能出现外周关节受累和关节外受累。本研究旨在定量分析 axSpA 患者的姿势、平衡和步态参数,并评估相关因素。这项横断面病例对照研究包括 51 名 axSpA 患者(30 名男性,21 名女性;平均年龄为 40.94 ± 10.48 岁)和 51 名年龄和性别匹配的健康对照者。在 axSpA 患者中,使用了强直性脊柱炎疾病活动度评分 CRP、巴斯强直性脊柱炎疾病活动度指数 (BASDAI)、巴斯强直性脊柱炎功能指数 (BASFI)、巴斯强直性脊柱炎计量指数 (BASMI)、马斯特里赫强直性脊柱炎关节炎评分 (MASES) 和强直性脊柱炎生活质量 (ASQoL) 量表。体位分析使用的是 DIERS formetric(Diers GmbH,德国施朗根巴德)录像机-立体成像设备。HUR SmartBalance BTG4(HUR-labs Oy,芬兰科科拉)平衡平台用于姿势平衡和稳定性极限(LOS)测量。参与者使用伯格平衡量表(BBS)、功能性前伸测试(FRT)和定时上下测试(TUG)进行评估。Zebris FDM 3 型(Zebris Medical GmbH,德国)行走平台用于测量参与者的时空参数。姿势参数比较显示,axSpA 组患者的矢状不平衡和颈椎深度距离比健康组患者增加(P < 0.004)。功能平衡参数比较显示,axSpA 组的 BBS 和 FRT 评分明显低于对照组(P < 0.001),而 TUG 评分则明显高于对照组(P < 0.001)。评估动态平衡的 LOS 值在 axSpA 组明显较低,表明存在障碍。在测量表示静态平衡的姿势摇摆时,发现所有 23 个子参数都相似。在分析时空步态参数时,axSpA 组与对照组相比,足角(P= 0.028)和步幅(P= 0.004)增加,而步长(P= 0.004)和步幅(P= 0.004)减少。在 axSpA 组中,步速降低(P = 0.004)。如果将轴SpA分为放射学轴SpA和非放射学轴SpA进行单独分析,则会在姿势、平衡和步态参数方面观察到类似的结果。没有观察到明显差异。我们发现,与姿势、平衡和步态分析最密切相关的临床评估是 BBS、FRT、TUG 和 BASFI。
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引用次数: 0
Paradoxical psoriasis induced by IL-17 inhibitors: a case series of patients with axial spondyloarthritis and a systematic literature review. IL-17抑制剂诱发的矛盾性银屑病:轴性脊柱关节炎患者病例系列及系统性文献综述。
IF 3.2 3区 医学 Q2 RHEUMATOLOGY Pub Date : 2024-11-01 Epub Date: 2024-06-25 DOI: 10.1007/s00296-024-05647-9
Nikolaos Chaitidis, Zoi Papadopoulou, Stavritsa Taxiarchoula Varvara, Michail Panagiotidis, Ioanna Katsigianni, Grigorios T Sakellariou

Following the market authorization of interleukin (IL)-17 inhibitors, a growing number of cases of IL-17 inhibitor-induced paradoxical psoriasis (PsO) have been reported. Our objectives were to present two cases of IL-17 inhibitor-induced paradoxical PsO and to systematically review the literature for similar cases, summarizing and presenting the relevant data. A systematic literature review of previously presented cases of paradoxical PsO induced by IL-17 inhibitors was conducted. We presented two patients with axial spondyloarthritis (axSpA) and paradoxical PsO induced by secukinumab (SEC). One patient's psoriatic lesions responded well to adjuvant topical treatment, while the other patient required a combination of topical treatment and cyclosporine Α for successful treatment. SEC was continued in both cases. We also identified 35 patients with IL-17 inhibitor-induced paradoxical PsO in the literature review. The most frequent types of paradoxical PsO were palmoplantar pustular and plaque PsO, while the median latency period was 11 weeks. Approximately one-third of patients continued IL-17 inhibitor treatment with adjunctive therapy, primarily topical, which produced satisfactory results in most patients. Almost two-thirds of the patients discontinued the IL-17 inhibitor, with the majority of patients switching to another biological agent with a different mechanism of action or initiating other systemic antipsoriatic treatments, resulting in mainly satisfactory outcomes. Therefore, paradoxical PsO induced by IL-17 inhibitors appears to respond well in both patients who continue IL-17 inhibitors with adjunctive treatment and those who discontinue IL-17 inhibitors while switching to a different class of biological agent or initiating other systemic antipsoriatic treatments.

白细胞介素(IL)-17抑制剂上市后,越来越多的IL-17抑制剂诱发的矛盾性银屑病(PsO)病例被报道。我们的目的是介绍两例IL-17抑制剂诱发的矛盾性银屑病病例,并系统回顾类似病例的文献,总结和介绍相关数据。我们对以前发表的由IL-17抑制剂诱发的矛盾性PsO病例进行了系统的文献回顾。我们介绍了两名患有轴性脊柱关节炎(axSpA)并由secukinumab(SEC)诱发矛盾性PsO的患者。其中一名患者的银屑病皮损对局部辅助治疗反应良好,而另一名患者则需要结合局部治疗和环孢素Α才能成功治疗。这两名患者都继续接受了 SEC 治疗。我们还在文献综述中发现了 35 例由 IL-17 抑制剂诱发的矛盾性 PsO 患者。最常见的矛盾性 PsO 类型是掌跖脓疱型和斑块型 PsO,中位潜伏期为 11 周。约有三分之一的患者在继续接受 IL-17 抑制剂治疗的同时还接受了辅助治疗,主要是局部治疗,大多数患者的治疗效果令人满意。近三分之二的患者停用了 IL-17 抑制剂,大多数患者转用了另一种作用机制不同的生物制剂,或开始接受其他系统的抗银屑病治疗,结果主要令人满意。因此,IL-17 抑制剂诱发的矛盾性 PsO 似乎对继续使用 IL-17 抑制剂并进行辅助治疗的患者和停用 IL-17 抑制剂同时改用另一类生物制剂或开始其他全身性抗银屑病治疗的患者都有很好的疗效。
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引用次数: 0
Elevated uric acid is associated with a low bone mineral density in pre- but not post-menopausal women with rheumatoid arthritis: a pilot study. 尿酸升高与类风湿性关节炎绝经前而非绝经后妇女的低骨矿物质密度有关:一项试点研究。
IF 3.2 3区 医学 Q2 RHEUMATOLOGY Pub Date : 2024-11-01 Epub Date: 2024-07-29 DOI: 10.1007/s00296-024-05655-9
Sandra Hermann, Andriko Palmowski, Kay Geert A Hermann, Burkhard Muche, Nadége Léprêtre, Tobias Alexander, Zhivana Boyadzhieva, Gerhard Krönke, Paula Hoff, Edgar Wiebe, Frank Buttgereit

Introduction: The role of uric acid (UA) on bone metabolism is controversially discussed. Higher UA levels have been associated with higher T-scores and a reduced incidence of fractures in postmenopausal women. However, in the context of rheumatoid arthritis (RA), the role of UA remains unclear. This pilot study aimed to investigate the association of UA levels with bone mineral density in RA female and male patients.

Methods: This pilot study analyzed patients with RA to explore preliminary associations. We utilized data from the Rh-GIOP cohort, a prospective monocentric observational study focusing on bone health in chronic rheumatic diseases. To assess the association between UA levels and the lowest T-scores measured at the lumbar spine, hip, or femur, we used linear regression with adjustment for various confounders. An interaction term was included to evaluate differential associations in pre- and postmenopausal women.

Results: Data on dual X-ray absorptiometry (DXA) measurements and serum UA levels were analyzed in a total of 206 patients. Among the 167 women 16 were premenopausal (age 40 ± 8 years) and 149 postmenopausal (age 65 ± 10 years). As expected, postmenopausal had lower T-scores than premenopausal patients (-1.53 ± 1.01 versus - 0.41 ± 1.29, respectively). No association of UA levels with T-scores was found when analyzing the whole cohort (Slope β: -0.04; p = 0.45). However, a significant negative correlation of UA with T-scores in premenopausal (Slope β: -0.98; p = 0.014), but not postmenopausal (Slope β: -0.04; p > 0.05) women was found.

Conclusion: Uric acid appears to be negatively associated with bone mineral density in premenopausal but not in postmenopausal women with RA. Thus, the impact of UA on bone health seems to depend on the hormonal status of women. Further investigations are required to validate these results in a larger cohort of patients and to investigate the underlying mechanisms.

导言:尿酸(UA)对骨代谢的作用一直备受争议。尿酸水平越高,绝经后妇女的 T 值越高,骨折发生率越低。然而,在类风湿性关节炎(RA)中,尿酸的作用仍不明确。这项试验性研究旨在调查类风湿性关节炎女性和男性患者体内尿酸水平与骨矿物质密度的关系:这项试验性研究分析了 RA 患者,以探索初步的关联。我们利用了Rh-GIOP队列的数据,该队列是一项前瞻性单中心观察研究,重点关注慢性风湿性疾病患者的骨健康状况。为了评估 UA 水平与腰椎、髋部或股骨测量的最低 T 值之间的关联,我们使用了线性回归,并对各种混杂因素进行了调整。为了评估绝经前和绝经后妇女的不同关联,我们加入了一个交互项:共对 206 名患者的双 X 射线吸收测量(DXA)测量数据和血清 UA 水平进行了分析。在这 167 名妇女中,16 人绝经前(40 ± 8 岁),149 人绝经后(65 ± 10 岁)。不出所料,绝经后患者的 T 值低于绝经前患者(分别为-1.53 ± 1.01 和 - 0.41 ± 1.29)。在分析整个队列时,未发现 UA 水平与 T 值有任何关联(斜率 β:-0.04;p = 0.45)。然而,在绝经前(斜率 β:-0.98;p = 0.014)而非绝经后(斜率 β:-0.04;p > 0.05)的妇女中,发现尿酸与 T 值呈明显的负相关:结论:尿酸似乎与绝经前 RA 妇女的骨矿物质密度呈负相关,但与绝经后 RA 妇女的骨矿物质密度无关。因此,尿酸对骨骼健康的影响似乎取决于女性的荷尔蒙状况。要在更大的患者群体中验证这些结果并研究其潜在机制,还需要进一步的研究。
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引用次数: 0
Respiratory failure in systemic sclerosis. 系统性硬化症的呼吸衰竭。
IF 3.2 3区 医学 Q2 RHEUMATOLOGY Pub Date : 2024-11-01 Epub Date: 2023-10-14 DOI: 10.1007/s00296-023-05482-4
Joaquim Ivo Vasques Dantas Landim, Andre Silva Franco, Percival Degrava Sampaio-Barros, Renata Miossi, Ana Cristina Medeiros-Ribeiro, Rosa Maria R Pereira, Ana Paula Luppino Assad

Systemic sclerosis (SSc) can lead to dyspnea and respiratory failure through multiple mechanisms, making a precise diagnosis particularly challenging, especially amid the current COVID-19 pandemic. In this report, we present a case involving a 26-year-old female who had previously undiagnosed SSc. She experienced acute respiratory failure necessitating orotracheal intubation. Following an extensive evaluation, the patient exhibited skin thickening, kidney failure, thrombocytopenia, microangiopathic anemia, and an antinuclear antibody with a nuclear fine speckled pattern at a titer of 1:320. A diagnosis of SSc complicated by scleroderma renal crisis (SRC) was established. The patient's condition improved after undergoing hemodialysis, receiving an angiotensin-converting enzyme inhibitor, and undergoing cyclophosphamide treatment. Subsequently, she demonstrated sustained improvement during a follow-up period of 20 months.

系统性硬化症(SSc)可通过多种机制导致呼吸困难和呼吸衰竭,这使得精确诊断尤其具有挑战性,尤其是在当前新冠肺炎大流行的情况下。在本报告中,我们介绍了一例涉及一名26岁女性的病例,她之前患有未确诊的SSc。她经历了急性呼吸衰竭,需要经口气管插管。经过广泛的评估,患者表现出皮肤增厚、肾衰竭、血小板减少、微血管病性贫血和具有核细斑点图案的抗核抗体,滴度为1:320。对合并硬皮病肾危象(SRC)的SSc进行了诊断。患者在接受血液透析、接受血管紧张素转化酶抑制剂和环磷酰胺治疗后病情有所改善。随后,她在20个月的随访期间表现出持续改善。
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引用次数: 0
Tuberculosis is the predominant infection in systemic sclerosis: thirty-year retrospective study of serious infections from a single centre. 结核病是系统性硬化症的主要感染病症:对一个中心三十年来严重感染病症的回顾性研究。
IF 3.2 3区 医学 Q2 RHEUMATOLOGY Pub Date : 2024-11-01 Epub Date: 2024-08-24 DOI: 10.1007/s00296-024-05688-0
Abhishek Gollarahalli Patel, Sakir Ahmed, Jyoti Ranjan Parida, Sarit Sekhar Pattanaik, Latika Gupta, Amita Aggarwal, Able Lawrence, Durga Prasanna Misra, Alok Nath, Zia Hashim, Ajmal Khan, Richa Mishra, Akshatha Ravindra, Namita Mohindra, Neeraj Jain, Vikas Agarwal

To look for the spectrum of infections and the factors predisposing to infection in patients with systemic sclerosis (SSc). In this retrospective study, demographic, clinical features, details of infections, immunosuppressive therapy, and outcomes of patients with SSc attending clinics at department of Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India from 1990 to 2022 were captured. Multivariable-adjusted logistic regression was applied to identify independent predictors of infection. Data of 880 patients, mean age 35.5 ± 12 years, and female: male ratio 7.7:1, were analyzed. One hundred and fifty-three patients had at least 1 infection with a total of 233 infectious episodes. Infections were most common in lung followed by skin and soft tissue. Tuberculosis was diagnosed in 45 patients (29.4%). Klebsiella was the commonest non-tubercular organism in lung and Escherichia coli in urinary tract infections. In comparison to matched control group, patients with infection had a greater number of admissions due to active disease, odds ratio (OR) 6.27 (CI 3.23-12.18), were receiving immunosuppressive medication OR, 5.05 (CI 2.55-10.00), and had more digital ulcers OR, 2.53 (CI 1.17-5.45). Patients who had infection had more likelihood for death OR, 13.63 (CI 4.75 -39.18). Tuberculosis is the commonest infection and lung remains the major site of infection in patients with SSc. Number of hospital admissions, digital ulcers and immunosuppressive therapy are predictors of serious infection in patients with SSc. Patients with infections had more likelihood of death.

研究系统性硬化症(SSc)患者的感染谱和易感因素。在这项回顾性研究中,研究人员收集了 1990 年至 2022 年期间在印度勒克瑙桑贾伊-甘地研究生医学院临床免疫学和风湿病学系就诊的系统性硬化症患者的人口统计学特征、临床特征、感染详情、免疫抑制疗法和治疗结果。采用多变量调整逻辑回归来确定感染的独立预测因素。分析了 880 名患者的数据,这些患者的平均年龄为 35.5 ± 12 岁,男女比例为 7.7:1。153 名患者至少感染过一次,共发生了 233 次感染。肺部感染最为常见,其次是皮肤和软组织。45名患者(29.4%)被确诊为肺结核。肺部感染中最常见的非结核菌是克雷伯菌,泌尿道感染中最常见的是大肠埃希菌。与匹配对照组相比,感染患者因活动性疾病入院的次数更多,几率比(OR)为 6.27(CI 3.23-12.18),接受免疫抑制药物治疗的几率比(OR)为 5.05(CI 2.55-10.00),出现数字溃疡的几率比(OR)为 2.53(CI 1.17-5.45)。感染患者死亡的可能性更大,OR 为 13.63(CI 为 4.75 -39.18)。肺结核是最常见的感染,肺部仍然是 SSc 患者的主要感染部位。住院次数、数字溃疡和免疫抑制治疗是预测 SSc 患者严重感染的因素。感染患者死亡的可能性更大。
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引用次数: 0
Psoriatic arthritis with psychological comorbidities: an overview of systematic reviews on incidence, prevalence, and geographic disparities. 伴有心理并发症的银屑病关节炎:关于发病率、流行率和地域差异的系统综述。
IF 3.2 3区 医学 Q2 RHEUMATOLOGY Pub Date : 2024-11-01 Epub Date: 2024-05-26 DOI: 10.1007/s00296-024-05617-1
Juan-Carlos Hernández-Rodríguez, Marta Infante-Cano, Cristina García-Muñoz, Javier Matias-Soto, Javier Martinez-Calderon

Systematic reviews and meta-analysis evaluating the prevalence, incidence, and psychological comorbidities of psoriatic arthritis (PsA) are increasing, so it's time to perform an overview of systematic reviews. To summarize the pooled prevalence, incidence, and psychological comorbidities rates of PsA, and to explore possible continent disparities. In this overview of systematic reviews the CINAHL, EMBASE, PsycINFO, and PubMed were searched to October 25, 2023. This overview included systematic reviews with meta-analysis of people with PsA, providing the pooled prevalence or incidence rates of PsA in general, or clinical populations and/or psychological comorbidities. The Preferred Reporting Items for Overviews of Reviews (PRIOR) statement was followed. AMSTAR-2 assessed the quality of reviews. The degree of overlap was calculated using the corrected covered area (CCA). Maps were developed using the location of where primary studies were conducted using DataWrapper App. The protocol was prospectively registered with Open Science Framework registry. Pooled prevalence and incidence rates of PsA or its associated psychological comorbidities in general or specific populations. We also collected locations from the primary studies of the included meta-analyses. Only the assessment of prevalence rates of PsA in people with psoriasis showed slight overlap (CCA = 3.3%). Items 2, 3, 4, 7, 8, 10, 12, and 13 were poorly reported in AMSTAR-2. The pooled prevalence of PsA ranged from 0.13 to 0.15% in the general population, and 15.5% to 19.7% in people with psoriasis. The pooled incidence of PsA ranged from 8.26 to 9.27 cases per 100,000 inhabitants to 0.87 cases in individuals with hidradenitis suppurativa. The pooled prevalence of psychological comorbidities was 11.9-20% for depression, 19-33% anxiety, 38% alexithymia, and 72.9% in poor sleep quality. Only the pooled incidence of depression was assessed with 21.3 per 1000-person year. PsA seems to be prevalent and incident not only in people with psoriasis, but also in general population. Depression and anxiety symptoms may be present in some patients with PsA. Finally, continent disparities exist, and methodological and clinical issues were found, which could be helpful in the future agenda of the epidemiology of PsA.

评估银屑病关节炎(PsA)患病率、发病率和心理并发症的系统综述和荟萃分析越来越多,因此是时候对系统综述进行概述了。总结PsA的患病率、发病率和心理并发症的汇总情况,并探讨可能存在的大陆差异。在本系统综述中,检索了截至 2023 年 10 月 25 日的 CINAHL、EMBASE、PsycINFO 和 PubMed。本综述包括对PsA患者进行荟萃分析的系统综述,提供了一般PsA或临床人群和/或心理并发症的总体流行率或发病率。研究遵循了 "综述首选报告项目"(PRIOR)声明。AMSTAR-2 评估了综述的质量。使用校正覆盖面积(CCA)计算重叠程度。使用 DataWrapper 应用程序根据主要研究的开展地点绘制地图。研究方案在开放科学框架注册中心进行了前瞻性注册。汇总一般或特定人群中 PsA 或其相关心理并发症的流行率和发病率。我们还收集了纳入荟萃分析的主要研究的地点。只有对银屑病患者 PsA 患病率的评估略有重叠(CCA = 3.3%)。AMSTAR-2的第2、3、4、7、8、10、12和13项报告较少。在普通人群中,PsA 的合计患病率为 0.13% 至 0.15%,在银屑病患者中为 15.5% 至 19.7%。在每 10 万居民中,PsA 的总发病率为 8.26 至 9.27 例,在化脓性扁平苔藓患者中为 0.87 例。心理合并症的汇总发病率为:抑郁 11.9%-20%、焦虑 19%-33%、失眠 38%、睡眠质量差 72.9%。仅评估了抑郁症的总发病率,为每千人年 21.3 例。PsA 似乎不仅在银屑病患者中流行,在普通人群中也有发生。一些 PsA 患者可能会出现抑郁和焦虑症状。最后,研究还发现了大陆差异、方法学和临床问题,这些都有助于今后开展 PsA 流行病学研究。
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Rheumatology International
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