sacroiliitis

骶髂关节炎
  • DOI:
    文章类型: Journal Article
    背景:慢性骶髂关节炎具有不同的病因和多种疗效的治疗方法。近年来,一种利用骨基质的新型后路已经开发出来,尽管迄今为止,关于这种方法的疗效和安全性的文献数据有限.与横向入路相比,所描述的益处包括减少的不良后果和更快的恢复。
    目的:本研究集中于后路骶髂关节融合术和包括残疾在内的转归,疼痛,术后使用镇痛药。
    方法:本回顾性研究,进行了单中心研究,评估了骶髂融合同种异体移植植入物(来自PainTEQ的LinQ植入系统;来自OmniaMedical的PsiF系统)的安全性和有效性.
    方法:共72例后入路骶髂关节融合术。在2020年8月至2024年6月期间,有53人在LSUHealthShreveport注册并随访为唯一的研究地点。选定的参与者年龄在28至79岁之间,平均年龄53.4岁.LinQ植入系统是选择用于植入的主要手术硬件(83.0%),在其余情况下选择PsiF系统。
    方法:VAS评分,残疾变化,不良结果,经后路骶髂关节融合术后,比较镇痛的使用情况。
    结果:到指定终点时,SIJ疼痛强度的平均VAS评分从9.5cm的基线评分显着降低了3.6cm(6月1日,2024).在这方面,65.4%的患者经历了20%或更多的疼痛改善,38.5%的患者经历了50%或更多的疼痛改善,26.9%的患者疼痛改善了70%或更多.在整个研究期间,零(0)个与手术相关的不良事件,也没有发生术中或术后并发症。
    结论:没有对照组的回顾性研究。54%(72个中的39个)完成了至少一年的随访。Further,退出率为26%。
    结论:本研究的结果表明,在迄今为止涉及后入路骶髂关节融合术的最大单中心研究中,在三年期间内,有效的结果具有最小的不良反应和残疾改善。
    BACKGROUND: Chronic sacroiliitis has variable etiologies with numerous treatments of varying efficacy. In recent years, a novel posterior approach utilizing bone matrix has been developed although to date, there is limited data in the literature regarding efficacy and safety through this approach. Benefits described include reduced adverse outcomes and quicker recovery when compared to the lateral approach.
    OBJECTIVE: The present investigation focused on sacroiliac joint fusion through the posterior approach and outcomes including disability, pain, and use of analgesics post-surgery.
    METHODS: This retrospective, single-center study was conducted evaluating safety and efficacy of sacroiliac fusion allograft implants (LinQ Implant System from PainTEQ; PsiF System from Omnia Medical).
    METHODS: A total of 72 posterior approach sacroiliac joint fusions were performed. Fifty-three individuals were enrolled and followed at LSU Health Shreveport as the sole investigational site between August 2020 and June 2024. Selected participant age ranged between 28 and 79 years, with a mean age of 53.4 years. The LinQ Implant System was the primary surgical hardware selected for implantation (83.0%), with the PsiF System chosen in the remaining cases.
    METHODS: VAS Scores, disability changes, adverse outcomes, and analgesic use were compared after sacroiliac joint fusion via the posterior approach.
    RESULTS: Mean VAS Scores for SIJ Pain Intensity significantly decreased by 3.6 cm from a baseline score of 9.5 cm by the Specified End (June 1st, 2024). In this regard, 65.4% of patients experienced a 20% or greater improvement in pain, 38.5% of patients experienced a 50% or greater improvement in pain, and 26.9% of patients experienced a 70% or greater improvement in pain.  Zero (0) procedure-related adverse events nor intra- or post-operative complications occurred throughout the duration of the investigation.
    CONCLUSIONS: Retrospective nature of the study without a control group. Fifty-four percent (39 of 72) completed minimum one year follow up. Further, the withdrawal rate was 26%.
    CONCLUSIONS: The results of the present investigation demonstrated effective outcomes with minimal adverse effects and improvements in disability over a three-year period in the largest single center study to date involving posterior approach sacroiliac joint fusion.
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  • 文章类型: Journal Article
    比较断层合成表现与X线摄影术的区别,以区分可疑轴性脊柱关节炎(SpA)患者的骶髂关节正常或退行性变化。
    射线照相术,对疑似SpA的连续患者在同一天进行了骶髂关节的断层合成和CT检查(29例患者)。检查是回顾性独立阅读的,被两名放射科医生(一名初级和一名高级,和初级学生两次)。使用kappa系数评估观察者之间和观察者之间的一致性。每种成像灵敏度的有效剂量,评估了特异性和准确性,并与作为金标准的CT进行了比较.
    CT检测到15/58个关节伴有骶髂关节炎。成像灵敏度,特异性和准确性为60%,84%和44%,分别,射线照相和87%,断层合成分别为91%和77%。断层合成的平均有效剂量显着低于CT(少5倍),而显着高于放射线照相术(多8倍)。
    对于疑似SpA的患者,断层合成优于X线摄影,辐射暴露比CT少5倍。
    UNASSIGNED: To compare tomosynthesis performance to radiography for the differentiation of sacroiliitis versus normal or degenerative changes in sacroiliac joints in patients with suspected axial spondyloarthritis (SpA).
    UNASSIGNED: Radiography, tomosynthesis and CT of sacroiliac joints (29 patients) were performed on the same day in consecutive patients with suspected SpA. The examinations were retrospectively read independently, blinded by two radiologists (one junior and one senior, and twice by one junior). Interobserver and intraobserver agreement was evaluated using the kappa coefficient. Effective doses for each imaging sensitivity, specificity and accuracy were assessed and compared with CT as gold standard.
    UNASSIGNED: CT detected 15/58 joints with sacroiliitis. The imaging sensitivity, specificity and accuracy were 60%, 84% and 44%, respectively, for radiography and 87%, 91% and 77% for tomosynthesis. The mean effective dose for tomosynthesis was significantly lower than that of CT (5-fold less) and significantly higher than that of radiography (8-fold more).
    UNASSIGNED: Tomosynthesis is superior to radiography for sacroiliitis detection in patients with suspected SpA, with 5-fold less radiation exposure than CT.
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  • 文章类型: Journal Article
    目的:家族性地中海热(FMF)是一种单基因自身炎症性疾病,以反复发作的发热和多发性浆膜炎为特征。骶髂关节受累在FMF患者中很少见。这项研究的目的是评估人口统计学,临床,FMF患者发生骶髂关节炎的实验室和影像学检查结果。
    方法:对0-18岁的儿科患者的档案进行回顾性分析,随访诊断为FMF。在磁共振成像(MRI)上有骶髂关节炎证据的FMF患者被纳入研究。
    结果:在1062名FMF患者中,其中22名(12名男性;中位年龄8.5)(2.1%)被发现患有骶髂关节炎。FMF在9例(40.9%)患者和13例(59.1%)患者的骶髂关节炎之前被诊断出。骶髂关节炎患者最常见的症状是下腰痛(n=21,95.5%)。在MEFV基因分析中,M694V在16例(72.7%)患者中发现,是最常见的突变。MRI显示所有患者均有骶髂关节炎。所有患者均使用秋水仙碱。FMF相关骶髂关节炎患者,12例(54.5%)使用非甾体抗炎药实现缓解,6例(27.3%)常规抗风湿药和4例(31.8%)肿瘤坏死因子抑制剂治疗。当秋水仙碱不相容时,四名(31.8%)患者出现骶髂关节炎,四名(31.8%)患者在使用生物制剂治疗秋水仙碱抗性FMF时出现骶髂关节炎。
    结论:FMF相关骶髂关节炎尤其是M694V突变患者,如果他们有腰背痛等症状。对秋水仙碱耐药的FMF患者应在每次就诊时评估骶髂关节炎症状。
    OBJECTIVE: Familial Mediterranean fever (FMF) is a monogenic autoinflammatory disease characterised by recurrent episodes of fever and polyserositis. Sacroiliac joint involvement is rare in FMF patients. The purpose of this study was to evaluate the demographic, clinical, laboratory and imaging findings of patients with FMF who developed sacroiliitis.
    METHODS: The files of paediatric patients aged 0-18 years who were followed up with a diagnosis of FMF were retrospectively reviewed. FMF patients with evidence of sacroiliitis on magnetic resonance imaging (MRI) were included in the study.
    RESULTS: Among 1062 FMF patients, 22 (12 males; median age 8.5) (2.1%) of them were found to have sacroiliitis. FMF was diagnosed before sacroiliitis in nine (40.9%) patients and after in 13 (59.1%) patients. The most common symptom in patients with sacroiliitis was low back pain (n = 21, 95.5%). In MEFV gene analysis, M694V was found in 16 (72.7%) patients and was the most common mutation. MRI showed evidence of sacroiliitis in all patients. All patients were using colchicine. Patients with FMF-associated sacroiliitis, remission was achieved with non-steroidal anti-inflammatory drugs in 12 (54.5%), conventional disease-modifying antirheumatic drugs in six (27.3%) and tumour necrosis factor inhibitor treatment in four (31.8%). Four (31.8%) patients experienced sacroiliitis when colchicine incompatible and four (31.8%) patients experienced sacroiliitis while using biologic agents for colchicine-resistant FMF.
    CONCLUSIONS: FMF-associated sacroiliitis should be considered especially in patients with M694V mutation if they have symptoms such as low back pain. Colchicine-resistant FMF patients should be evaluated for sacroiliitis symptoms at each visit.
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  • 文章类型: Journal Article
    目的:确定T2*软骨标测在诊断和评估早期轴向脊柱关节炎(axSpA)疾病活动中的性能,并探讨软骨损伤与临床特征的相互作用,骶髂关节炎MRI评分,和扩散指标。
    方法:这项前瞻性研究包括83名axSpA患者和37名非axSpA患者。临床特征,在MRI上评估国际社会定义的活动性骶髂关节炎,并记录T2*SIJs值。在axSpA中,使用强直性脊柱炎疾病活动评分-C反应蛋白评估疾病活动;使用加拿大关节炎研究协会评估活动性骶髂关节炎,体素内不连贯运动,和弥散峰度成像;使用复合结构损伤评分(CSDS)和结构评分脂肪评估慢性骶髂关节炎。Mann-WhitneyU-test,具有错误发现率(FDR)的Kruskal-Wallis测试,ROC曲线,采用线性回归进行统计分析。
    结果:AxSpA患者的T2*SIJs值明显高于非axSpA患者。(22.86±2.42msvs20.36±1.30ms,p<0.001)。MRI上T2*SIJs值和活动性骶髂关节炎的组合具有用于鉴定axSpA的最高AUC。T2*SIJs值在非活跃和非常高之间有显著差异,中等和非常高,又高又高,以及不活跃和高疾病活动度组(所有pFDR<0.05)。Dk(β=0.48)和CSDS(β=0.48)与T2*SIJs值独立相关。
    结论:T2*值可能是诊断和区分axSpA早期疾病活动的有希望的生物标志物。急性和慢性骶髂关节炎都会影响软骨特性。
    结论:骶髂关节软骨异常可以通过T2*弛豫时间来量化,并可以更好地表征早期axSpA。
    结论:T2*作图可能对评估axSpA有价值。MRI上T2*值和活动性骶髂关节炎的组合增强了axSpA的诊断性能。用T2*值测量的异常与疾病活动相关,急性骶髂关节炎,和结构损伤程度。
    OBJECTIVE: To determine the performance of T2* cartilage mapping in diagnosing and assessing disease activity in early axial spondyloarthritis (axSpA), and to investigate the interaction of cartilage damage with clinical characteristics, sacroiliitis MRI scorings, and diffusion metrics.
    METHODS: This prospective study included 83 axSpA patients and 37 no-axSpA patients. Clinical characteristics, the Assessment of SpondyloArthritis International Society-defined active sacroiliitis on MRI, and T2* SIJs values were recorded. In axSpA, disease activity was evaluated using the ankylosing spondylitis disease activity score-C-reactive protein; active sacroiliitis was evaluated using Spondyloarthritis Research Consortium of Canada, intravoxel incoherent motion, and diffusion kurtosis imaging; chronic sacroiliitis was assessed using composite structural damage score (CSDS) and structural score fat. Mann-Whitney U-test, Kruskal-Wallis test with false discovery rate (FDR), ROC curve, and linear regression were used for statistical analysis.
    RESULTS: AxSpA patients had significantly higher T2*SIJs values than no-axSpA patients. (22.86 ± 2.42 ms vs 20.36 ± 1.30 ms, p < 0.001). The combination of T2*SIJs values and active sacroiliitis on MRI had the highest AUC for identifying axSpA. T2*SIJs values were significantly different between the inactive and very high, moderate and very high, high and very high, as well as inactive and high disease activity groups (all pFDR < 0.05). Dk (β = 0.48) and CSDS (β = 0.48) were independently associated with T2*SIJs values.
    CONCLUSIONS: T2* values may be a promising biomarker for diagnosing and differentiating disease activity in early axSpA. Both acute and chronic sacroiliitis influence cartilage properties.
    CONCLUSIONS: Sacroiliac joint cartilage abnormalities can be quantified with T2* relaxation time and allow better characterization of early axSpA.
    CONCLUSIONS: T2* mapping may have value in evaluating axSpA. The combination of T2* values and active sacroiliitis on MRI enhances diagnostic performance for axSpA. Abnormalities measured with T2* values correlate with disease activity, acute sacroiliitis, and degree of structural damage.
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  • 文章类型: Journal Article
    背景:骶髂关节炎的单侧表现是一个诊断难题,尤其是感染和与脊柱关节炎相关的炎性骶髂关节炎,需要早期和准确的诊断。
    目的:评估磁共振成像(MRI)在区分单侧骶髂关节炎的感染性和炎性病因中的应用。
    方法:回顾90例单侧骶髂关节炎患者的MRI,有一个确定的最终诊断。使用预定义标准评估MR图像的各种骨骼和软组织变化,并使用单变量和多变量回归分析进行分析。
    结果:在90例患者中,感染性病因诊断为66例(73.3%),炎症病因诊断为24例(26.7%)。大侵蚀,髂和骶骨侧水肿,关节间隙累及积液或滑膜炎,软组织水肿,ESR/CRP升高,无包膜炎和附着点炎与感染相关(p<.001)。在多变量分析中,有利于感染的独立区分变量是髂侧和骶侧水肿(OR4.79,95%CI:0.96-23.81,p=0.05),大的侵蚀(OR17.96,95%CI:2.66-121.02,p=0.003),和关节间隙受累(OR9.9,95%CI:1.36-72.06,p=.02)。感染的唯一特征是骨髓炎,sequestra,脓肿,窦道,大的侵蚀,和多焦点。所有感染病例均有软组织水肿,联合空间参与,ESR升高,也没有囊炎.
    结论:MRI评估骨和关节间隙受累的存在和模式,软组织受累,仔细注意某些独特的特征将有助于区分感染性骶髂关节炎和炎性骶髂关节炎。
    BACKGROUND: Unilateral presentation of sacroiliitis is a diagnostic dilemma, especially between infection and inflammatory sacroiliitis associated with spondyloarthritis, requiring an early and accurate diagnosis.
    OBJECTIVE: To assess the utility of magnetic resonance imaging (MRI) in differentiating infective versus inflammatory etiology in unilateral sacroiliitis.
    METHODS: Retrospective review of the MRI of 90 patients with unilateral sacroiliitis, having an established final diagnosis. MR images were evaluated for various bone and soft tissue changes using predefined criteria and analyzed using univariate and multivariate regression analysis.
    RESULTS: Among the 90 patients, infective etiology was diagnosed in 66 (73.3%) and inflammatory etiology in 24 (26.7%). Large erosions, both iliac and sacral-sided edema, joint space involvement with effusion or synovitis, soft tissue edema, elevated ESR/CRP, and absence of capsulitis and enthesitis were associated with infection (p < .001). The independently differentiating variables favoring infection on multivariate analysis were-both iliac and sacral-sided edema (OR 4.79, 95% CI: 0.96-23.81, p = .05), large erosions (OR 17.96, 95% CI: 2.66-121.02, p = .003), and joint space involvement (OR 9.9, 95% CI: 1.36-72.06, p = .02). Exclusive features of infection were osteomyelitis, sequestra, abscesses, sinus tracts, large erosions, and multifocality. All infective cases had soft tissue edema, joint space involvement, elevated ESR, and no capsulitis.
    CONCLUSIONS: MRI evaluation for the presence and pattern of bone and joint space involvement, soft tissue involvement, and careful attention to certain exclusive features will aid in differentiating infectious sacroiliitis from inflammatory sacroiliitis.
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  • 文章类型: Case Reports
    髂腰肌脓肿是一种罕见的疾病,其特征是在髂腰肌隔室中积聚脓液。近年来,随着各种合并症和危险因素的出现,该病的患病率一直在增加。更新的成像方式的可用性也改善了对新病例的检测。沙门氏菌病是髂腰肌脓肿和骶髂关节炎的罕见病因。文献报道的大多数病例与金黄色葡萄球菌有关,链球菌种类,和大肠杆菌。糖尿病,血液恶性肿瘤,艾滋病毒,和其他免疫受损状态是髂腰肌脓肿的重要合并症/危险因素。我们报告了一例18岁男性,有发烧和右髋部疼痛10天的病史。放射显像显示右侧骶髂关节炎和髂腰肌脓肿。血培养显示泛敏感伤寒沙门氏菌。抗生素疗程延长后(静脉注射头孢曲松,然后口服左氧氟沙星),患者病情好转,症状无进一步复发。在排除金黄色葡萄球菌和结核分枝杆菌等常见病因后,伤寒沙门氏菌应该是流行地区髂腰肌脓肿的重要区别。
    Iliopsoas abscess is an infrequent condition characterized by the collection of pus in the iliopsoas compartment. The prevalence of the disease has been increasing in recent years with the emergence of various comorbidities and risk factors. The availability of newer imaging modalities has also improved the detection of new cases. Salmonellosis is an uncommon etiology in iliopsoas abscess and sacroiliitis. Most cases reported in the literature are associated with Staphylococcus aureus, Streptococci species, and Escherichia coli. Diabetes, hematological malignancies, HIV, and other immunocompromised states are important comorbidities/risk factors for iliopsoas abscess. We report a case of an 18-year-old male who presented with a history of fever and right hip pain for 10 days. Radioimaging revealed right sacroiliitis and iliopsoas abscess. Blood culture revealed pan-sensitive Salmonella typhi. After the prolonged course of antibiotics (intravenous ceftriaxone followed by oral levofloxacin), the patient improved with no further relapse in symptoms. Salmonella typhi should be an important differential of iliopsoas abscess in endemic regions after ruling out the common etiology such as S. aureus and Mycobacterium tuberculosis.
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  • 文章类型: Case Reports
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  • 文章类型: Journal Article
    该研究的目的是分析轴性银屑病关节炎(axPsA)患者是否符合轴性脊柱关节炎(axSpA)和强直性脊柱炎(AS)的分类标准。根据CASPAR标准,总共检查了104例PsA患者(66例男性和38例女性),所有患者都有背痛。通过ASAS标准评估患者是否存在炎性背痛(IBP)。不符合ASAS标准的背痛被认为是慢性背痛(chrBP)。患者接受了手,脚和骨盆,颈椎和腰椎X光片。侵蚀,骨质溶解,并评估了关节间的新骨形成。明确的放射照相骶髂关节炎(d-rSI)定义为双侧≥2级或单侧≥3级。19例无d-rSI的患者接受了骶髂关节MRI检查。93例患者接受了HLAB27检查。确定符合axSpA(ASAS)标准和改良纽约(mNY)AS标准的患者人数。在67例(64.4%)患者中发现了IBP;chrBP,37例(35.6%)患者中;31例(29.8%)患者在IBP/chrBP发病时年龄较大(40岁以上);57例(58.8%)患者有d-rSI;6例(31.6%)患者有MRI-SI;57例(58.8%)患者中检测到了复合体。在40例无d-rSI的患者中,19例(47.5%)有合生体。在38/97(39.2%)的患者中,d-rSI与合成植物一起被检测到,而19/97(19.6%)患者有孤立性d-rSI,无脊柱炎,和19/97(19.6%)患者有分离的无d-rSI的合成植物。28例(30.1%)患者存在HLA-B27。51例(55.4%)患者符合axSpA标准。41例(44.6%)患者不符合axSpA标准;然而,其中27例(65.9%)有人工林。48例(48.5%)PsA患者符合AS的mNY标准。在这些患者中,揭示了一组具体特征:18人(37.5%)没有IBP,18(37.5%)在IBP/chrBP发作时年龄较大(40岁以上),34人(70.8%)患有指炎,38例(79.2%)患有糜烂性多关节炎,23人(48.8%)有关节间新骨形成,14例(30.2%)骨溶解,23人(48.9%)有“厚实”的非边缘性复合体,40例(82.6%)患有指甲牛皮癣;28例(66.6%)患者HLA-B27阴性。45%的axPsA患者不符合axSpA标准。已经确定了将axPsA与AS区分开的特征。
    The objective of the study was to analyze whether axial psoriatic arthritis (axPsA) patients meet classification criteria for axial spondyloarthritis (axSpA) and ankylosing spondylitis (AS). A total of 104 patients (66 men and 38 women) with PsA according to CASPAR criteria were examined, all patients had back pain. Patients were evaluated for presence of inflammatory back pain (IBP) by ASAS criteria. Back pain not meeting the ASAS criteria was taken to be chronic back pain (chrBP). Patients underwent hands, feet and pelvis, cervical spine and lumbar spine X-rays. Erosions, osteolysis, and juxta-articular new bone formation were evaluated. Definite radiographic sacroiliitis (d-rSI) was defined as bilateral grade ≥ 2 or unilateral grade ≥ 3. Nineteen patients without d-rSI underwent sacroiliac joints MRI. Ninety-three patients underwent HLA B27 examination. The number of patients who met the criteria for axSpA (ASAS) and the modified New York (mNY) criteria for AS was determined. IBP was identified in 67 (64.4%) patients; chrBP, in 37 (35.6%) patients; 31 (29.8%) patient were of older age (over 40) at the onset of IBP/chrBP; 57 (58.8%) patients had d-rSI; 6 (31.6%) patients had MRI-SI; syndesmophytes were detected in 57 (58.8%) cases. Among 40 patients without d-rSI, 19 (47.5%) had syndesmophytes. In 38/97 (39.2%) patients d-rSI was detected along with syndesmophytes, while 19/97 (19.6%) patients had isolated d-rSI without spondylitis, and 19/97 (19.6%) patients had isolated syndesmophytes without d-rSI. HLA B27 was present in 28 (30.1%) cases. 51 (55.4%) patients met criteria for axSpA. Forty-one (44.6%) patients did not meet criteria for axSpA; however, 27 (65.9%) of them had syndesmophytes. Forty-eight (48.5%) PsA patients met mNY criteria for AS. Among these patients, a set of specific features was revealed: 18 (37.5%) had no IBP, 18 (37.5%) were of older age (over 40) at the onset of IBP/chrBP, 34 (70.8%) had dactylitis, 38 (79.2%) had erosive polyarthritis, 23 (48.8%) had juxta-articular new bone formation, 14 (30.2%) had osteolysis, 23 (48.9%) had \"chunky\" non-marginal syndesmophytes, and 40 (82.6%) had nail psoriasis; 28 (66.6%) patients were HLA-B27 negative. Forty-five percent of axPsA patients do not meet criteria for axSpA. Characteristic features have been identified to differentiate axPsA from AS.
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  • 文章类型: Case Reports
    VEXAS综合征是一种最近描述的自身炎症综合征,由髓样前体中UBA1突变的体细胞获得引起,并且通常与血液恶性肿瘤有关。主要是骨髓增生异常综合征。疾病表现可以模拟几种风湿病,延迟诊断。我们描述了一个非典型表现,类似迟发性轴向脊椎关节炎的病例,后来进展为软骨炎的全身性炎症综合征,皮肤血管炎,输血依赖性贫血,需要高剂量的类固醇.Ruxolitinib被用作第一个类固醇保留策略,没有反应。然而,阿扎胞苷显示出控制炎症和突变克隆的活性。该病例提出了阿扎胞苷的抗炎作用是否依赖于或独立于克隆控制的问题。我们讨论了分子缓解在VEXAS综合征中的潜在相关性,并强调了多学科团队对此类复杂患者护理的重要性。
    VEXAS syndrome is a recently described autoinflammatory syndrome caused by the somatic acquisition of UBA1 mutations in myeloid precursors and is frequently associated with hematologic malignancies, chiefly myelodysplastic syndromes. Disease presentation can mimic several rheumatologic disorders, delaying the diagnosis. We describe a case of atypical presentation resembling late-onset axial spondylarthritis, later progressing to a systemic inflammatory syndrome with chondritis, cutaneous vasculitis, and transfusion-dependent anemia, requiring high doses of steroids. Ruxolitinib was used as the first steroid-sparing strategy without response. However, azacitidine showed activity in controlling both inflammation and the mutant clone. This case raises the question of whether azacitidine\'s anti-inflammatory effects are dependent on or independent of clonal control. We discuss the potential relevance of molecular remission in VEXAS syndrome and highlight the importance of a multidisciplinary team for the care of such complex patients.
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  • 文章类型: Journal Article
    Familial Mediterranean fever (FMF) is an autosomal recessive disease distributed among populations of Mediterranean origin - Armenians, Sephardi Jews, Arabs, Turks. There are numerous clinical observations regarding combination of FMF, as a classical representative of autoinflammatory diseases, with systemic diseases of connective tissue. Seronegative spondyloarthritis (SpA) are the most interesting disorders from this point of view, as far as sacroiliitis - an essential feature of SpA, may also present as a part of joint syndrome in FMF. The main objective of this clinical study was the investigation of the peculiarities of courses of FMF and SpA in case of their coexistence. We studied 126 patients with FMF, SpA and coexistence of both. According to results, patients with the overlap of FMF with SpA had relatively milder course of disease in comparison with each disease separately. Comparative clinical and instrumental characteristics of FMF-associated disorders had shown that in FMF-SpA overlap the symptoms of both diseases are less severe.
    Периодическая болезнь (ПБ), или семейная средиземноморская лихорадка, – аутосомно-рецессивное заболевание, распространенное в популяциях средиземноморского происхождения, в частности среди армян, евреев-сефардов, арабов, турков. Существует множество клинических наблюдений о сочетании ПБ как типичного представителя аутовоспалительных заболеваний и системных заболеваний соединительной ткани как классических аутоиммунных заболеваний. В этом плане большой интерес представляют серонегативные спондилоартриты (СНСА), так как сакроилеит, являющийся патогномоничным признаком данного заболевания, довольно часто проявляется в качестве составной части суставного синдрома при ПБ. Целью данного исследования явилось изучение особенностей течения ПБ и СНСА при их сочетании. Изучены 126 больных с ПБ и СНСА, а также их сочетанием. Согласно полученным результатам сочетание ПБ-СНСА имеет относительно мягкое течение по сравнению и с СНСА, и с ПБ в отдельности. Сравнительная клинико-инструментальная характеристика ПБ-ассоциированных заболеваний выявила, что при сочетании ПБ-СНСА наблюдается менее тяжелое течение как в отношении симптомов ПБ, так и симптомов СНСА.
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