Pigmented Villonodular Tenosynovitis of the Temporomandibular Joint—A Case Report

Background: Pigmented villonodular synovitis is a rare condition typically affecting the major joints and the involvement of the temporomandibular joint is rare. Although, it is histologically benign, it can be locally aggressive, capable of extensive bone destruction. The disease has aggressive behavior and a high incidence of recurrence (40% - 60%). Case report: A 35 years old male presented with history of unilateral hearing loss and otalgia of six month duration. On examination, there was a soft bulge in the superior wall of external auditory canal. Contrast enhanced CT scan showed soft tissue density occupying the right external auditory canal and bulging into the middle ear cavity, displacing the intact ossicular chain medially with evidence of destruction of the posterior wall of the external auditory canal, glenoid fossa, mastoid bone and the scutum. The squamous part of the right temporal bone showed evidence of lateral bowing with discontinuity. Surgical resection was done through post aural approach. Histopathological examination revealed pigmented villonodular tenosynovitis which stained positive for CD 68. Post operatively patient was evaluated clinically and with radiological modalities which showed healthy mastoid cavity and no increase in post operative granulations on magnetic resonance imaging. Conclusion: Pigmented villonodular tenosynovitis of temporomandibular joint is a rare entity. Due to the aggressive behavior of the lesion, radical or aggressive surgical resection should be done. Long term follow up with radiological modality should be done due to the high recurrence rate.

FABP4 secreted by M1-polarized macrophages promotes synovitis and angiogenesis to exacerbate rheumatoid arthritis

Increasing evidence shows that adipokines play a vital role in the development of rheumatoid arthritis(RA).Fatty acid-binding protein 4(FABP4),a novel adipokine that regulates inflammation and angiogenesis,has been extensively studied in a variety of organs and diseases.However,the effect of FABP4 on RA remains unclear.Here,we found that FABP4 expression was upregulated in synovial M1-polarized macrophages in RA.The increase in FABP4 promoted synovitis,angiogenesis,and cartilage degradation to exacerbate RA progression in vivo and in vitro,whereas BMS309403(a FABP4 inhibitor)and anagliptin(dipeptidyl peptidase 4 inhibitor)inhibited FABP4 expression in serum and synovial M1-polarized macrophages in mice to alleviate RA progression.Further studies showed that constitutive activation of mammalian target of rapamycin complex 1(mTORC1)by TSC1 deletion specifically in the myeloid lineage regulated FABP4 expression in macrophages to exacerbate RA progression in mice.In contrast,inhibition of mTORC1 by ras homolog enriched in brain(Rheb1)disruption specifically in the myeloid lineage reduced FABP4 expression in macrophages to attenuate RA development in mice.Our findings established an essential role of FABP4 that is secreted by M1-polarized macrophages in synovitis,angiogenesis,and cartilage degradation in RA.BMS309403 and anagliptin inhibited FABP4 expression in synovial M1-polarized macrophages to alleviate RA development.Hence,FABP4 may represent a potential target for RA therapy.

Serum matrix metalloproteinase 3 in detecting remitting seronegative symmetrical synovitis with pitting edema syndrome: A case report

We report a case of remitting seronegative symmetrical synovitis with pitting edema(RS3 PE) syndrome in a 71-year-old woman. She referred to our hospital with finger stiffness, edema of both hands and feet, pain of bilateral shoulder, wrist, metacarpophalangeal, proximal interphalangeal, and ankle joints. Rheumatoid factor was negative, human leukocyte antigen-B7 antigen was positive. Moreover, matrix metalloproteinase 3(MMP-3) was high. She was diagnosed with RS3 PE syndrome, and treatment with prednisolone(15 mg/d) was started. One week after prednisolone treatment initiation, CRP decreased to negative, and joint pain was almost completely resolved. However, hand stiffness persisted, and MMP-3 level was still high. Thus, prednisolone dose was increased to 20 mg/d, and the stiffness resolved. Twenty days after treatment initiation, MMP-3 was normalized. MMP-3 was more indicative of RS3 PE syndrome symptoms than CRP. Thus, MMP-3 seems to be more sensitive to RS3 PE syndrome symptoms.

Rapid remission of refractory synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome in response to the Janus kinase inhibitor tofacitinib: A case report

BACKGROUND Synovitis,acne,pustulosis,hyperostosis,and osteitis(SAPHO)syndrome is a rare autoinflammatory disease for which clinical treatment has not been standardized.Janus kinase(JAK)inhibitors represent a novel therapeutic option for rheumatoid arthritis,psoriatic arthritis,and some other autoinflammatory diseases.However,the clinical utility of JAK inhibitors in treating SAPHO syndrome has not been thoroughly investigated.In this study,we describe a patient with SAPHO syndrome who failed to respond to conventional treatment but demonstrated a remarkable and rapid response to the JAK inhibitor tofacitinib.CASE SUMMARY A 62-year-old female patient presented with swelling and pain at the sternoclavicular joints,back pain that limited her activities,arthralgia in the right knee,and cutaneous lesions.Her symptoms were unresponsive to nonsteroidal anti-inflammatory drugs,disease-modifying antirheumatic drugs,Tripterygium wilfordii hook f,and bisphosphonates.SAPHO syndrome was diagnosed in accordance with dermatological and osteoarticular manifestations and abnormal inflammatory factors.Multiple image studies have illustrated bone lesions and pathological fractures of vertebral bodies.Oral treatment with tofacitinib at 5 mg twice daily with methotrexate and bisphosphonates was initiated.The patient reported that her pain symptoms were relieved after 3 d and her cutaneous lesions were reduced after 4 wk of treatment.Vertebral lesions were improved after 6 mo on tofacitinib.No serious adverse effects were noted.CONCLUSION JAK inhibitor therapy may be a promising strategy to treat SAPHO syndrome.

Subtalar joint pigmented villonodular synovitis misdiagnosed at the first visit:A case report

BACKGROUND Pigmented villonodular synovitis(PVNS)is an uncommon disease that usually occurs in large joints,and involvement of the subtalar joint is rare.The lack of comprehensive knowledge of subtalar joint PVNS could lead to misdiagnosis.CASE SUMMARY We present a 64-year-old woman who,at her first visit,complained of discomfort in the right ankle when she walked.Based on the physical signs and X-ray report,the physician failed to make the suspected diagnosis of PVNS.Eighteen months later,the patient returned with a complaint of a mass in her right lateral malleolus with intermittent blunt pain.The X-ray presented an osteophyte formation and soft tissue calcification at the margin of the subtalar joint.The laboratory tests were normal,whereas magnetic resonance imaging(MRI)showed a low-intensity area on both T1-and T2-weighted images.A suspected diagnosis of PVNS was made and later confirmed by postoperative pathology.Subsequently,the patient received radiotherapy with 32 Gy in 16 fractions.At 6 mo postoperatively,the patient only complained of discomfort after walking three blocks.The American Orthopedic Foot and Ankle Society Ankle-Hindfoot score was 97.CONCLUSION MRI is necessary for the diagnosis of PVNS.Early soft tissue calcification and painless joint swelling should be of concern.

New scoring system in assessment of Hoffa's fat pad synovitis: A comparative study with established scoring systems

AIM To investigate the reliability of the established and new scoring methods for Hoffa's fat pad synovitis using magnetic resonance imaging(MRI).METHODS A total of 139 knees of 115 patients who underwent MRI of the knee with and without gadolinium contrast were enrolled in this study. Proton density(PD)-weighted, PD-weighted fat-suppressed(PD-FS), and postcontrast T1-weighted fat-suppressed(T1CE) images were used for evaluation. Using contrast and noncontrast images, our grading method for synovitis was performed to measure synovial thickness and signal intensity changes of the fat pad [Synovial membrane(SM) score], which was compared with the established methods,including MRI Osteoarthritis Knee Score(MOAKS), parapatellar synovitis score,Whole-Organ Magnetic Resonance Imaging Score(WORMS), and suprapatellar effusion diameter. Intraclass correlation coefficients(ICC) for intra and interobserver reproducibility and Spearman correlation coefficients(r) were calculated for the parapatellar synovitis score and each scoring method.RESULTS All of the scores presented substantial to almost perfect intrareliability. Among three readers, effusion diameter had substantial to almost perfect interreliability(ICC = 0.68-0.81) and WORMS had substantial interreliability(ICC = 0.61-0.70).For two out of three readers, there was substantial interreliability for the thickness score in T1CE(ICC = 0.55-0.69), SM scores in T1CE(ICC = 0.56-0.78)and PD-FS(ICC = 0.51-0.79), and parapatellar synovitis score in T1CE(ICC =0.53-0.72). The parapatellar synovitis score was significantly correlated with the thickness score in T1CE(r = 0.70) and the SM score in T1CE(r = 0.81) and PD-FS(r = 0.65).CONCLUSION The newly proposed quantitative thickness score on T1CE and the semiquantitative SM score on T1CE and PD-FS can be useful for Hoffa's fat pad synovitis.

Effect of synoviectomy combined with postoperative radiotherapy on pigmented villonodular synovitis in knee:A meta-analysis

Objective:To evaluate the efficacy of synovectomy combined with postoperative radiotherapy in the treatment of pigmented villonodular synovitis compared with only synovectomy in knee. Methods:The related references were searched from SinoMed,CNKI,Wanfang,VIP,PubMed,Embase,Cochrane Library and Web of Science from date of databases establishment to October 2020,and the studies of synovectomy combined with postoperative radiotherapy were screened. According to the type of research,the corresponding evaluation methods were selected for quality evaluation of the literature. RevMan 5.3 software was used for meta-analysis. The primary outcome was recurrence,while the secondary outcomes included complications,excellent and good rate of function of knee,range of motion of knee,Lysholm score,and IKDC score of knee etcetera. Results:A total of 11 articles were included in meta-analysis,with 510 cases,280 in the combined group and 230 in the control group. The results of meta-analysis showed that:(1)The recurrence rate in the combination group was significantly lower than that in the control group,[OR=0.33,95%CI(0.20,0.55),P<0.000 1];(2)The complications in the combined group were higher than that in the control group[OR=2.70,95%CI(1.16,6.31),P=0.02];(3)The excellent and good rate of the combined group was significantly better than that of the control group[OR=2.86,95%CI(1.23,6.64),P=0.01],and it also may be the result of different evaluation criteria of each study;(4)There was no significant difference range of motion[MD=2.96,95%CI(-0.54,6.47),P=0.10],Lysholm score[MD=3.22,95%CI(-0.51,6.96),P=0.09],or IKDC score[MD=2.88,95%CI(-1.07,6.82),P=0.15]in knee between the two groups. Conclusion:The meta-analysis shows that synovectomy combined with radiotherapy can reduce the recurrence rate of pigmented villonodular synovitis in knee,but compared with only operation,there are higher complications. However,the above conclusions need to be verified by long-term follow-up of more high-quality clinical studies.

Potential mechanism of Simiao powder in treatment of synovitis based on network pharmacology

Objective:Based on the technology and method of network pharmacology,to explore the active components and specific mechanism of Simiao powder in the treatment of synovitis.Methods:by using tcmsp database,the potential active components and related target proteins of Simiao powder were screened.Genecards and digenet database were used to predict the target of synovitis.UniProt database was used to query the corresponding gene names.The compound synovitis target network and protein of Simiao powder were constructed by using Cytoscape and string software In R language,bio conductor data package was used to analyze the enrichment of go biological function and KEGG pathway,and the effective components and targets of Simiao powder in the treatment of synovitis were obtained.Results:64 key active components of Simiao powder were screened out from tcmsp database,71 of them were involved in the interaction of main chemical components of Simiao powder-chemical components and synovitis treatment target-synovitis.95 PPI core genes were obtained,and 90 go biological enrichment processes were obtained by go enrichment analysis.The results of network pharmacology indicate that Simiaosan can regulate 32 signaling pathways in the treatment of synovitis,including IL-17 signaling pathway,TNF signaling pathway,T cell receptor signaling pathway and toll like receptor signaling pathway.Conclusion:the treatment of synovitis with Simiao powder is a complex process of multi-component,multi-target and multi-channel,which is related to the coordinated regulation of multiple targets and signal pathways.

Therapeutic Effect Analysis of Sodium Aescinate Tablets on Knee Osteoarthritis Combined with Synovitis

Objective:To observe therapeutic effect of sodium aescinate Tablets on knee osteoarthritis complicated with synovitis.Methods:86 patients with knee osteoarthritis combined with synovitis treated in the Honghui Hospital Affiliated to Xi’an Jiaotong University from January 2018 to December 2019 were randomly divided into control group and intervention group,with 43 cases in each group.The control group was given routine treatment,and the intervention group was given sodium aescinate Tablets orally on the basis of routine treatment,twice a day,2 tablets each time.The curative effect was evaluated after 2 weeks of treatment.The knee pain and joint function of the two groups were evaluated by Visual Analogue Scale(VAS)and Western Ontario and McMaster Universities Arthritis Index(WOMAC),and the clinical effects of the two groups were compared.Results:Before treatment,there was no significant difference in general information,VAS and WOMAC scores between the two groups(P>0.05).After treatment,the VAS and WOMAC scores of the two groups were both lower than those before treatment,and the difference was statistically significant(P<0.05).The vas and WOMAC scores of the intervention group were lower than those of the control group(VAS,1.87±0.79 vs 3.38±0.81).In this study,sodium aescinate tablets were applied to the treatment of KOA combined with synovitis.The results showed that the intervention group was better than the control group in alleviating knee pain,improving joint function and improving clinical efficacy,and the difference was statistically significant.The total effective rate of the intervention group was higher than that of the control group(100%vs 86.05%),and the difference was statistically significant(P<0.05).Conclusion:Sodium aescinate tablet in the treatment of knee osteoarthritis combined with synovitis can effectively alleviate knee pain,improve joint function and improve clinical efficacy.

Combination of manual lymphatic drainage and Kinesio taping for treating pigmented villonodular synovitis:A case report

BACKGROUND Pigmented villonodular synovitis(PVNS)is a benign proliferative disorder that affects the synovial joints,bursae,and tendon sheaths.To date,few studies have reported on the treatment of postoperative pain and edema in patients with PVNS.Herein,we present the case of a woman who developed pain and edema in the left lower limb 1 wk after synovectomy and arthroscopic partial meniscectomy and was unable to walk due to limited flexion and extension of the left knee.CASE SUMMARY A 32-year-old woman underwent synovectomy and arthroscopic partial meniscectomy successively and was treated with a combination of manual lymphatic drainage(MLD)and kinesio taping(KT)in our hospital to alleviate postoperative pain and edema.The following parameters were assessed at 2 wk post-treatment and 1 wk post-discharge follow up:suprapatellar circumference,infrapatellar circumference,visual analog scale score,knee range of motion,pittsburgh sleep quality index score,hamilton anxiety rating scale(HAMA)score,and hamilton depression rating scale(HAMD)score.After treatment,the postoperative pain and edema in the patient’s left knee were effectively relieved,resulting in improved sleep quality and remarkably attenuated HAMA and HAMD scores.CONCLUSION Combined MLD and KT may be an effective approach for relieving postoperative pain and edema in patients with PVNS.

Cerebral activation during unilateral clenching in patients with temporomandibular joint synovitis and biting pain： an functional magnetic resonance imaging study

Background Functional magnetic resonance is a non-invasive method that can examine brain activity and has been widely used in various fields including jaw movement and pain processing. Temporomandibular disorder （TMD） is one of the most frequent facial pain problems. The objective of this study was to investigate the brain activities using functional magnetic resonance imaging （fMRI） during unilateral maximal voluntary clenching tasks in the TMD synovitis patients with biting pain.Methods Fourteen TMD synovitis patients with unilateral biting pain and 14 controls were included in the study.Contralateral biting pain was defined as right molar clenching causing left temporomandibular joint （TMJ） pain. Ipsilateral biting pain was defined as right molar clenching causing right TMJ pain. Symptom Check List-90 （SCL-90） was administered to the patients and controls. Independent sample t-test was used to compare the SCL-90 subscales between the two groups. Unilateral clenching tasks were performed by the patients and controls. Imaging data were analyzed using SPM99.Results Patients were divided into contralateral TMD biting pain group （n=8） and ipsilateral TMD biting pain group （n=6）. The SCL-90 subscales were significantly different between the two groups for somatization, depression, anxiety,phobic anxiety, and paranoid ideation. Group analysis of the controls demonstrated brain activations in the inferior frontal gyrus, precentral gyrus, middle frontal gyrus, superior temporal gyrus, and insular. The areas of activation were different between right and left clenching task. In TMJ synovitis patients with contralateral or ipsilateral biting pain, the group analysis showed activations in the inferior frontal gyrus, superior temporal gyrus, medium frontal gyrus, precentral gyrus,and anterior cingulate cortex.Conclusions The inferior frontal gyrus and precentral gyrus play essential roles during the unilateral clenching task.Activation of anterior cingulate cortex in the synovitis patients with biting pain was associated with higher levels of psychological distress.

Diagnostic value of diffusion-weighted MRI for imaging synovitis in pediatric patients with inflammatory conditions of the knee joint

Background Diffusion-weighted imaging(DWI)of synovitis has been suggested as a possible non-invasive alternative to contrast-enhanced T1w imaging(ce-T1w).We aimed to study DWI for diagnosing synovitis in the knee joint of pediatric patients,to quantify inter-observer agreement on DWI and ce-T1w and to calculate quantitative measures of synovial diffusivity and conspicuity.Methods Forty consecutive patients with known or suspected arthritis of the knee(25 girls,median age 12 years)underwent routine 1.5T MRI with ce-T1w and transverse DWI with b values 50 and 800 s/mm2.Mean apparent diffusion coefficient(ADC)values and signal intensity of inflamed synovium,joint effusion and muscle were measured with regions of interest retrospectively.Post-contrast T1 w images(diagnostic standard)and diffusion-weighted images at b=800 s/mm2 with ADC map were separately rated by three independent and blinded readers with different levels of expertise for the presence and degree of synovitis along with the level of diagnostic confidence.Results Thirty-one(78%)patients showed at least some synovial contrast enhancement,17(43%)children were diagnosed with synovitis on ce-T1w.Ratings by the 1st reader on ce-T1w and on DWI for synovitis showed very good agreement(kappa=0.90).Inter-observer agreement on DWI ranged from moderate to substantial with kappa values between 0.68 and 0.79(all P<0.001).Agreement and diagnostic confidence were generally lower in patients with mild and without synovial enhancement,compared to patients with synovitis.DWI yielded higher signal of inflamed synovium vs.muscle tissue,but lower signal vs.joint effusion,compared to ce-T1 w(all P<0.001).Conclusions Diffusion-weighted imaging is a promising,though reader-dependent alternative to contrast-enhanced imaging in patients with arthritis of the knee,based on our preliminary findings.It holds potential for increasing patient safety and comfort.

Successful Treatment of Synovitis, Acne, Pustulosis, Hyperostosis and Osteitis Syndrome with Thalidomide: A Case Report

IntroductionSynovitis,Acne,Pustulosis,Hyperostosis and Osteitis (SAPHO) syndrome is a rare clinical entity characterized by a wide range of dermatological and musculoskeletal manifestations.This condition has a heterogeneous presentation,at times chronic and relapsing,which results in diagnostic difficulties and delays as well as therapeutic challenges.

SAPHO综合征一例报告并文献复习

SAPHO综合征（synovitis-acne-pustulosis-hyperostosisos teitis syndrome）即滑膜炎-痤疮-脓疱病-骨肥厚-骨炎综合征,是一种主要累及骨、关节及皮肤的慢性疾病。传统观念认为,本病为少见病,发病率可能不超过1/10 000[1]。随着对疾病的研究深入和重视度提高,近年来国内外报道逐渐增多。故本病有可能被误诊而不是少见病。但SAPHO综合征的病因和发病机制尚不清楚,本文报道1例,并对其研究进展予以SAPHO综合征,旨在临床早期诊断和治疗SAPHO综合征,探寻新的有效的治疗方法。

免疫抑制剂联合生物制剂治疗1例SAPHO综合征患者的护理

SAPHO综合征即滑膜炎（synovitis,S）、痤疮（acne,A）、脓疱病（pustulosis,P）、骨肥厚（hyperostosis,H）和骨髓炎（osteomyelitis,O）综合征,是一种骨关节和皮肤等多器官受累的慢性无菌性炎性疾病。1987年Charmot等首次提出SAPHO综合征的概念[1]。

超声与MRI在儿童暂时性髋关节滑膜炎诊断中的对比研究

暂时性髋关节滑膜炎（transient synovitis of the hip,TSH）常见于儿童,是一种发生于关节、腱鞘及黏液滑囊附属纤维组织的滑膜增殖性疾病,严重影响患者生活质量。本组回顾性分析我院收治的87例TSH患儿的临床资料,旨在探讨MRI与高频超声在TSH中的诊断价值。

RS3PE综合征与迟发型类风湿关节炎临床特点分析

缓和的血清阴性对称性滑膜炎伴凹陷性水肿综合症(syndrome of remitting seronegative symmetric synovitis with pitting edema,RS3PE)是一种特殊类型的以关节炎为主要表现的风湿性疾病,该病1985年由Mc Cary等[1]首先报道,而迟发型类风湿关节炎(Elderly-onset rheumatoid arthritis,ERA)是指60岁以后发病的类风湿关节炎[2],为探讨二者之间的异同,现对其进行回顾性分析如下:

SAPHO综合征病例分享及文献复习

1987年,Chamot等[1]命名了SAPHO综合征,SAPHO是滑膜炎（synovitis）、痤疮（acne）、脓疱病（pustulosis）、骨肥厚（hyperostosis）和骨炎（osteitis）的英文首字母缩写。主要的症状为皮肤的损害和无菌性的骨关节炎。据估计SAPHO综合征的发病率低于1/10 000[2],但由于临床医师的不重视及误诊,

膝关节色素沉着绒毛结节性滑膜炎的超声表现与病理变化关系

目的:探讨膝关节色素沉着绒毛结节性滑膜炎(PVNS)的超声表现与病理变化的关系,评价超声诊断PVNS的临床价值。材料和方法:回顾性分析19例膝关节PVNS的超声表现,并与手术病理结果对照。结果:19例PVNS均为弥漫型,病理特征为滑膜增厚呈结节状或绒毛状,有丰富的血管和大量含铁血黄素沉着;超声显示病变关节内有不同程度积液;滑膜增厚并有结节状隆起或互相形成较大肿块,其回声为低、中或高回声;CDFI示滑膜内均可见较丰富血流信号;部分可见骨质及周围组织侵蚀性改变。结论:超声诊断PVNS有一定的应用价值,可作为首选筛检方法。

Bone marrow mononuclear cells for joint therapy: The role of macrophages in inflammation resolution and tissue repair

Osteoarthritis(OA)is the most prevalent joint disease causing major disability and medical expenditures.Synovitis is a central feature of OA and is primarily driven by macrophages.Synovial macrophages not only drive inflammation but also its resolution,through a coordinated,simultaneous expression of pro-and anti-inflammatory mechanisms that are essential to counteract damage and recover homeostasis.Current OA therapies are largely based on anti-inflammatory principles and therefore block pro-inflammatory mechanisms such as prostaglandin E2 and Nuclear factor-kappa B signaling pathways.However,such mechanisms are also innately required for mounting a pro-resolving response,and their blockage often results in chronic low-grade inflammation.Following minor injury,macrophages shield the damaged area and drive tissue repair.If the damage is more extensive,macrophages incite inflammation recruiting more macrophages from the bone marrow to maximize tissue repair and ultimately resolve inflammation.However,sustained damage and inflammation often overwhelms pro-resolving mechanisms of synovial macrophages leading to the chronic inflammation and related tissue degeneration observed in OA.Recently,experimental and clinical studies have shown that joint injection with autologous bone marrow mononuclear cells replenishes inflamed joints with macrophage and hematopoietic progenitors,enhancing mechanisms of inflammation resolution,providing remarkable and long-lasting effects.Besides creating an ideal environment for resolution with high concentrations of interleukin-10 and anabolic growth factors,macrophage progenitors also have a direct role in tissue repair.Macrophages constitute a large part of the early granulation tissue,and further transdifferentiate from myeloid into a mesenchymal phenotype.These cells,characterized as fibrocytes,are essential for repairing osteochondral defects.Ongoing“omics”studies focused on identifying key drivers of macrophagemediated resolution of joint inflammation and those required for efficient osteochondral repair,have the potential to uncover ways for developing engineered macrophages or off-the-shelf pro-resolving therapies that can benefit patients suffering from many types of arthropaties,not only OA.