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Mandibular involvement in SAPHO syndrome: a retrospective study.

ABSTRACT: BACKGROUND:Mandible osteomyelitis can occur in synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome, a rare chronic inflammatory disease; however, few studies have explored its characteristics and management. METHODS:We reviewed the medical records of consecutive SAPHO patients with mandible involvement diagnosed in Peking Union Medical College Hospital from September 2014 to July 2019. Demographic, clinical, laboratory, and imaging data were collected at baseline. Prescription data and follow-up magnetic resonance imaging (MRI) and cone beam computed tomography (CBCT) images were collected from the hospital information system. An electronic questionnaire was distributed to all patients to obtain their latest symptoms. RESULTS:A total of 26 SAPHO patients with mandibular involvement were involved, all of whom responded to the questionnaire (38.5% male; median age, 28 years; median follow-up duration, 2.1 years). Ten patients (38.5%) had undergone an oral procedure 1 month before the onset of mandibular symptoms. All 14 of the patients who underwent a surgical intervention relapsed within a median duration of 2 months (range 0.25-4.0 months), and 24 patients (92.3%) achieved improvement with conservative treatment. Following bisphosphonate treatment, remission of bone marrow oedema and osteolysis was observed on MRI and CBCT, and 5 patients receiving bisphosphonates with follow-up CBCT after remission did not relapse in 5.4 months (mean 6.0, range 3.2-9.9 months). CONCLUSION:Mandibular involvement of SAPHO syndrome predominantly occurs in young women. Dental procedures are a possible risk factor. Conservative treatment, especially intravenous bisphosphonates, can lead to oral improvement.

SUBMITTER: Wang M

PROVIDER: S-EPMC7643311 | biostudies-literature | 2020 Nov

REPOSITORIES: biostudies-literature

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Mandibular involvement in SAPHO syndrome: a retrospective study.

Wang Mu M Li Yueting Y Cao Yihan Y Lu Xinyu X Liu Yuchen Y Zhao Jizhi J Zhang Wen W Li Chen C

Orphanet journal of rare diseases 20201105 1

<h4>Background</h4>Mandible osteomyelitis can occur in synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome, a rare chronic inflammatory disease; however, few studies have explored its characteristics and management.<h4>Methods</h4>We reviewed the medical records of consecutive SAPHO patients with mandible involvement diagnosed in Peking Union Medical College Hospital from September 2014 to July 2019. Demographic, clinical, laboratory, and imaging data were collected at basel ...[more]

PMID: 33153463

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Project description:BackgroundThe anterior chest wall (ACW) involvement is characteristic of synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome, yet little research has focused on its magnetic resonance imaging (MRI) findings.PurposeTo characterize the MRI features of the ACW in patients with SAPHO syndrome.MethodsSeventy-one patients with SAPHO syndrome and ACW involvement evidenced by bone scintigraphy were recruited in this cross-sectional study. The ACW region was scanned using sagittal, axial, and oblique coronal Dixon T2-weighted sequences and axial Dixon T1-weighted sequences. The characteristics of both active inflammatory and chronic structural lesions were evaluated.ResultsThe ACW lesions exhibited an asymmetrical distribution and a predilection for the sternocostoclavicular region (93.0%). Notably, 91.5% of the patients had lesions in the area of the anterior first ribs. Bone marrow edema (BME) was observed in 63 (88.7%) patients, which mainly affected the sternocostal joints (87.3%) and the manubrium sterni (84.5%). All of the BMEs were distributed under the articular surface or the bone cortex, consistent with the distribution of the ligaments and joint capsules. Synovitis was detected in 64 (90.1%) patients, with a predilection for the sternoclavicular joints (76.1%). A soft tissue mass or infiltration was found in all the patients who had bone marrow edema. Thirteen (18.3%) patients showed venous stenosis. Structural changes included bone bridge formation (80.3%), hyperostosis (43.7%), and fat infiltration (39.4%). Four common patterns of involvement were observed: the first rib area, the sternoclavicular area, the sternal angle area, and the areas of the second to sixth sternocostal joints.ConclusionThe ACW lesions of SAPHO syndrome demonstrated a triad of enthesitis, synovitis, and osteitis, suggesting complex interactions among the ligaments, synovium, and bones in the region. The inflammatory changes in the first rib area were highlighted in SAPHO syndrome.

Project description:Background: Most children and adolescents infected with the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) develop a mild coronavirus disease 2019 (COVID-19) that usually does not require medical intervention. However, a small proportion of pediatric patients develop a severe clinical condition, Multisystem Inflammatory Syndrome in Children (MIS-C). The involvement of epigenetics in the control of the immune response and viral activity prompted us to carry out an epigenomic study to uncover target loci regulated by DNA methylation that could be altered upon the appearance of MIS-C. Methods: Peripheral blood samples were recruited from 43 confirmed MIS-C patients with a median age of 7.7 years, most of whom were of West-Eurasian origin. Four or more organ systems were involved in the majority of patients. 69 non-COVID-19 pediatric samples and 15 COVID-19 pediatric samples without MIS-C were used as controls. The cases in the two groups were mixed and divided into discovery (MIS-C = 29 and non-MIS-C = 56) and validation (MIS-C = 14 and non-MIS-C = 28) cohorts, and balanced for age, gender and ethnic background. We interrogated 850,000 CpG sites of the human genome for DNA methylation variants. Findings: The DNA methylation content of 33 CpG loci was linked with the presence of MIS-C. Of these sites, 18 (54.5%) were located in described genes. The top candidate gene was the immune T-cell mediator ZEB2; and others highly ranked candidates included the regulator of natural killer cell functional competence SH2D1B; VWA8, which contains a domain of the Von Willebrand factor A involved in the pediatric hemostasis disease; and human leukocyte antigen complex member HLA-DRB1; in addition to pro-inflammatory genes such as CUL2 and AIM2. The identified loci were used to construct a DNA methylation profile (EPIMISC) that was associated with MIS-C in both cohorts. The EPIMISC signature was also overrepresented in Kawasaki disease patients, a childhood pathology with also a possible viral trigger, that shares many of the clinical features of MIS-C. Interpretation: We have characterized DNA methylation loci that are associated with the onset of MIS-C. The identified genes are likely contributors to the characteristic exaggerated host inflammatory response observed in these patients. The described epigenetic signature could also provide new targets for more specific therapies for the disorder.

Project description:As the mandible is susceptible to fracture, the aim of this study was to use multivariate logistic regression analysis to identify and distinguish various internal factors that may influence the location of mandibular fractures. The study included 1131 patients with maxillofacial fractures during the period from January 2000 to December 2009 to evaluate the association of mandibular fracture location (unilateral symphysis, body, angle, condylar, or bilateral condylar fractures) with various internal factors. Among the 1131 patients, 869 had mandibular fractures. Data on age, sex, soft tissue injuries, dental trauma, and maxillofacial fracture type were collected and analyzed using multivariate logistic regression. In total, 387, 210, 139, 319, and 172 patients were diagnosed with unilateral symphysis, body, angle, unilateral, or bilateral condylar fractures, respectively. The dental trauma in patients with bilateral condylar fractures differed from that in patients with unilateral condylar fractures. Patients with mandibular fracture (unilateral symphysis, body, unilateral or bilateral condylar) possessed an approximately equal risk of soft tissue injuries in the mandible. Patients with either unilateral or bilateral condylar fractures were associated with a low risk of mandibular angle fracture (OR < 1). Similarly, patients with mandibular angle fracture were associated with a low risk of unilateral or bilateral condylar fractures (OR < 1). Moreover, patients with symphysis fracture were associated with a low risk of bilateral condylar fractures (90 of 387 [23.3%], OR 0.899). By contrast, patients with bilateral condylar fractures were associated with a high risk of symphysis fracture (90 of 172 [52.3%], OR 17.38). Patients with condylar fractures, particularly those with bilateral condylar fractures, were infrequently associated with secondary mandibular fractures. Mandibular fractures tended to have less of an association with midfacial fractures. The occurrence of mandibular fractures is strongly correlated with age, sex, soft tissue injuries, dental trauma, and the pattern and position of the maxillofacial fractures in patients.

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Mandibular involvement in SAPHO syndrome: a retrospective study.

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Mandibular involvement in SAPHO syndrome: a retrospective study.

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