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Wen Zhang - One of the best experts on this subject based on the ideXlab platform.
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axial skeletal lesions and disease duration in SAPHO Syndrome a retrospective review of computed tomography findings in 81 patients
International Journal of Rheumatic Diseases, 2020Co-Authors: Yihan Cao, Wen Zhang, Shuyang Zhang, Weihong ZhangAbstract:Aims To explore the relevance of disease duration with axial skeletal lesions in synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) Syndrome based on computed tomography (CT). Methods Patients who had undergone whole-spine CT were included from a previously reported single-center cohort of SAPHO Syndrome. Patients with previous treatments of bisphosphates, biological agents or surgeries were excluded. Sites of axial skeletal involvement were reviewed including the spine, sacroiliac joints, and anterior chest wall. Duration of osteoarticular involvement was defined as the interval between the onset of osteoarticular symptoms and the CT evaluation. Results Eighty-one patients (54 female and 27 male) were included in the study. The mean disease duration was 26.9 ± 34.9 months. Fifty (63.0%) patients showed spinal lesions on CT. The average numbers of involved vertebral segments for corner and endplate lesions were 3.16 ± 3.51 and 0.73 ± 1.85 respectively. Sixty-eight (84.0%) patients exhibited abnormalities in the anterior chest wall. The number of involved vertebral segments showed poor correlation with disease duration for both corner lesions (r2 = 0.051, P = 0.043) and endplate lesions (r2 = 0.137, P = 0.001). Patients with costoclavicular involvement showed remarkably longer disease duration than those without (49.3 ± 47.7 months vs 18.0 ± 23.5 months, P = 0.006), while no significant difference was detected regarding other structures in the anterior chest wall. Conclusion Costoclavicular involvement is a hallmark of long disease duration in SAPHO Syndrome. Corner and endplate lesions in the vertebrae and sacroiliac involvement may not relate to disease course.
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spinal and sacroiliac involvement in SAPHO Syndrome a single center study of a cohort of 354 patients
Seminars in Arthritis and Rheumatism, 2019Co-Authors: Yihan Cao, Weixin Hao, Xiaochuan Sun, Weihong Zhang, Hongli Jing, Siyi Yuan, Yuzhi Zuo, Jinhe Liu, Zhenhua Dong, Wen ZhangAbstract:Abstract Objective SAPHO Syndrome is a highly heterogeneous disease with distinct treatment response. We report the largest cohort of SAPHO Syndrome and explore its clinical classification with special interest in spinal and sacroiliac involvement. Methods A total of 354 patients with SAPHO Syndrome were recruited in Peking Union Medical College Hospital. The demographic, clinical and imaging data were collected at baseline. Spinal and sacroiliac involvement was determined by the co-existence of related symptoms and imaging evidence of lesions in the spine or sacroiliac joints on either bone scintigraphy, CT or MRI. Results A total of 197 (55.6%) patients were identified to have spinal or sacroiliac involvement. Compared to those without spinal or sacroiliac lesions, these patients were significantly older at onset (38 ± 12 vs 35 ± 10 years old, p = 0.019) but had comparable duration of disease. Therapeutically, patients with spinal or sacroiliac involvement had been treated more aggressively with more frequently prescribed NSAIDs, glucocorticoids, DMARDs, TNF-α inhibitors, and bisphosphonates (all p ≤ 0.001). Nonetheless, greater disease activity was observed for these patients at baseline, supported by both inflammatory markers (ESR and hs-CRP) and visual analog scale (VAS) for pain (all p Conclusions SAPHO patients with spinal or sacroiliac involvement are older at onset and have greater disease activity despite of more aggressive treatments compared to those without. Stratified management is in urgent need for this rare disease.
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case report successful treatment of refractory SAPHO Syndrome with the jak inhibitor tofacitinib
Medicine, 2018Co-Authors: Qiao Yang, Yumo Zhao, Yaping Luo, Weixin Hao, Wen ZhangAbstract:Introduction Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) Syndrome is an autoinflammatory disorder without standardized treatment. Janus kinase (JAK) inhibitors can block a range of cytokines and might possess significant anti-inflammatory activity. Here, we report the first case of efficacious treatment of refractory SAPHO Syndrome with the JAK inhibitor tofacitinib. Case presentation A 44-year-old woman presented with arthralgia in the right wrist and complained of having difficulty in doing housework. Symptoms were unresponsiveness to nonsteroidal anti-inflammatory drugs, disease-modifying antirheumatic drugs, and tumor necrosis factor inhibitors. A diagnosis of SAPHO Syndrome was made based on previous dermatological and osteoarticular manifestations and bone scintigraphy findings. Oral treatment with tofacitinib at 5 mg twice daily in combination with the basic methotrexate treatment was initiated. After 4 weeks of using tofacitinib, the patient reported marked improvement of symptoms and also reported being competent in completing housework. Conclusions The efficacy of JAK inhibitors in treating refractory SAPHO Syndrome should be noted.
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clinical heterogeneity of SAPHO Syndrome challenge of diagnosis
Modern Rheumatology, 2018Co-Authors: Yihan Cao, Wen ZhangAbstract:Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) Syndrome is spectrum of highly heterogeneous diseases firstly proposed by Chamot et al. [1]. Characterized by the coexisting of derma...
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Demographic features of the SAPHO Syndrome patients and healthy controls.
2018Co-Authors: Shuang Liu, Xin Sui, Jian Wang, Wei Song, Xinlun Tian, Wen ZhangAbstract:Demographic features of the SAPHO Syndrome patients and healthy controls.
Yihan Cao - One of the best experts on this subject based on the ideXlab platform.
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axial skeletal lesions and disease duration in SAPHO Syndrome a retrospective review of computed tomography findings in 81 patients
International Journal of Rheumatic Diseases, 2020Co-Authors: Yihan Cao, Wen Zhang, Shuyang Zhang, Weihong ZhangAbstract:Aims To explore the relevance of disease duration with axial skeletal lesions in synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) Syndrome based on computed tomography (CT). Methods Patients who had undergone whole-spine CT were included from a previously reported single-center cohort of SAPHO Syndrome. Patients with previous treatments of bisphosphates, biological agents or surgeries were excluded. Sites of axial skeletal involvement were reviewed including the spine, sacroiliac joints, and anterior chest wall. Duration of osteoarticular involvement was defined as the interval between the onset of osteoarticular symptoms and the CT evaluation. Results Eighty-one patients (54 female and 27 male) were included in the study. The mean disease duration was 26.9 ± 34.9 months. Fifty (63.0%) patients showed spinal lesions on CT. The average numbers of involved vertebral segments for corner and endplate lesions were 3.16 ± 3.51 and 0.73 ± 1.85 respectively. Sixty-eight (84.0%) patients exhibited abnormalities in the anterior chest wall. The number of involved vertebral segments showed poor correlation with disease duration for both corner lesions (r2 = 0.051, P = 0.043) and endplate lesions (r2 = 0.137, P = 0.001). Patients with costoclavicular involvement showed remarkably longer disease duration than those without (49.3 ± 47.7 months vs 18.0 ± 23.5 months, P = 0.006), while no significant difference was detected regarding other structures in the anterior chest wall. Conclusion Costoclavicular involvement is a hallmark of long disease duration in SAPHO Syndrome. Corner and endplate lesions in the vertebrae and sacroiliac involvement may not relate to disease course.
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three patterns of osteoarticular involvement in SAPHO Syndrome a cluster analysis based on whole body bone scintigraphy of 157 patients
Rheumatology, 2019Co-Authors: Yihan Cao, Qiao Yang, Xiaochuan Sun, Ximin Shi, Weihong Zhang, Qingqing Pan, Yanxue Zhao, Siyi Yuan, Yuzhi Zuo, Weixin HaoAbstract:OBJECTIVES To explore the patterns of osteoarticular involvement in SAPHO Syndrome. METHODS Baseline clinical characteristics and imaging data of 99mTc-MDP whole-body bone scintigraphy (WBBS) were collected from 157 out of 164 patients diagnosed with SAPHO Syndrome. The twelve most frequently involved osteoarticular sites were analysed by hierarchical cluster analysis with the Ward minimum-variance method. RESULTS Three distinctive patterns of osteoarticular involvement were identified: the spinal type (70 patients, 44.6%), with predominantly thoracic, lumbar or sacral vertebral lesions; the costal type (52 patients, 33.1%), with lesions of anterior ribs, particularly the first ribs; and the sternoclavicular type (35 patients, 22.3%), with predominantly sternal and bilateral sternoclavicular lesions, characterized by the typical bullhead sign. Notably, a total of 77 (49%) patients exhibited lesions of ribs on WBBS, of which 61.3% involved the first ribs. Interestingly, patients of spinal type were older at onset of cutaneous manifestations than those of sternoclavicular type (P = 0.036) and costal type (P = 0.035). The disease course was remarkably longer in sternoclavicular type than costal type (P = 0.001) and spinal type (P < 0.001). CONCLUSION The osteoarticular involvement in SAPHO Syndrome can be categorized as three distinct patterns with different corresponding clinical features. The costal involvement in SAPHO Syndrome, which was under-recognized previously, may define a distinct sub-type of the disease.
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spinal and sacroiliac involvement in SAPHO Syndrome a single center study of a cohort of 354 patients
Seminars in Arthritis and Rheumatism, 2019Co-Authors: Yihan Cao, Weixin Hao, Xiaochuan Sun, Weihong Zhang, Hongli Jing, Siyi Yuan, Yuzhi Zuo, Jinhe Liu, Zhenhua Dong, Wen ZhangAbstract:Abstract Objective SAPHO Syndrome is a highly heterogeneous disease with distinct treatment response. We report the largest cohort of SAPHO Syndrome and explore its clinical classification with special interest in spinal and sacroiliac involvement. Methods A total of 354 patients with SAPHO Syndrome were recruited in Peking Union Medical College Hospital. The demographic, clinical and imaging data were collected at baseline. Spinal and sacroiliac involvement was determined by the co-existence of related symptoms and imaging evidence of lesions in the spine or sacroiliac joints on either bone scintigraphy, CT or MRI. Results A total of 197 (55.6%) patients were identified to have spinal or sacroiliac involvement. Compared to those without spinal or sacroiliac lesions, these patients were significantly older at onset (38 ± 12 vs 35 ± 10 years old, p = 0.019) but had comparable duration of disease. Therapeutically, patients with spinal or sacroiliac involvement had been treated more aggressively with more frequently prescribed NSAIDs, glucocorticoids, DMARDs, TNF-α inhibitors, and bisphosphonates (all p ≤ 0.001). Nonetheless, greater disease activity was observed for these patients at baseline, supported by both inflammatory markers (ESR and hs-CRP) and visual analog scale (VAS) for pain (all p Conclusions SAPHO patients with spinal or sacroiliac involvement are older at onset and have greater disease activity despite of more aggressive treatments compared to those without. Stratified management is in urgent need for this rare disease.
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clinical heterogeneity of SAPHO Syndrome challenge of diagnosis
Modern Rheumatology, 2018Co-Authors: Yihan Cao, Wen ZhangAbstract:Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) Syndrome is spectrum of highly heterogeneous diseases firstly proposed by Chamot et al. [1]. Characterized by the coexisting of derma...
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F-18 FDG PET/CT in 26 patients with SAPHO Syndrome: a new vision of clinical and bone scintigraphy correlation
BMC, 2018Co-Authors: Xiaochuan Sun, Yihan Cao, Ximin Shi, Weihong Zhang, Hongli Jing, Wen ZhangAbstract:Abstract Backgrounds Whole-body bone scintigraphy (WBBS) and MRI are widely used in assessment of patients with synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) Syndrome. However, the value of F-18 fluorodeoxyglucose-positron emission tomography/computed tomography (18F-FDG PET/CT) in SAPHO Syndrome was unclear. The aim of this study was to characterize the manifestation of SAPHO Syndrome on 18F-FDG PET/CT and explore its relationship with clinical symptoms and WBBS. Methods Twenty-six patients who suffered from SAPHO Syndrome and had undergone whole-body 18F-FDG PET/CT were recruited in Peking Union Medical College Hospital from 2004 to 2016. Clinical manifestations and laboratory findings were recorded for all patients. Imaging data on 18F-FDG PET/CT and WBBS were collected and analyzed retrospectively. Results All the 26 patients (20 females and 6 males) exhibited skeletal abnormalities on 18F-FDG PET/CT. Multiple skeletal lesions affecting the anterior chest wall or spine with low to moderate 18F-FDG uptake and coexistence of osteolysis and osteosclerosis presented as the typical features of SAPHO Syndrome. Sixteen (61.5%) patients had abnormal 18F-FDG uptake outside the osteoarticular system. PET scan had moderate to substantial agreement with CT and WBBS in revealing lesions in the anterior chest wall and axial skeleton. Nonetheless, the correlation between increased 18F-FDG uptake and clinical symptoms was weak. Conclusions SAPHO Syndrome exhibits characteristic features on 18F-FDG PET/CT. It showed comparable capacity in revealing skeletal lesions with bone scintigraphy
Keiji Tanimoto - One of the best experts on this subject based on the ideXlab platform.
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Diagnostic points and possible origin of osteomyelitis in synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) Syndrome: a radiographic study of 77 mandibular osteomyelitis cases
Rheumatology, 2003Co-Authors: Yoshikazu Suei, Akira Taguchi, Keiji TanimotoAbstract:Objectives. To find diagnostic points and to identify the origin of osteomyelitis in synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) Syndrome. Methods. Fifty-two patients with mandibular suppurative osteomyelitis and 25 patients with mandibular osteomyelitis in SAPHO Syndrome were included in the study. Radiographic patterns of the lesion, types of periosteal reaction and the presence of external bone resorption and bone enlargement were investigated in each case and compared between the two entities. Results. Suppurative osteomyelitis demonstrated an osteolytic pattern and a lamellated type of periosteal reaction, whereas SAPHO Syndrome revealed a mixed-pattern, solid-type periosteal reaction, external bone resorption and bone enlargement. Conclusions. Radiographic examination is suggested to be convenient and a useful diagnostic method of differentiating osteomyelitis in SAPHO Syndrome from suppurative osteomyelitis. The periosteum is suspected to be the original site of osteomyelitic lesions in SAPHO Syndrome.
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diffuse sclerosing osteomyelitis of the mandible its characteristics and possible relationship to synovitis acne pustulosis hyperostosis osteitis SAPHO Syndrome
Journal of Oral and Maxillofacial Surgery, 1996Co-Authors: Yoshikazu Suei, Akira Taguchi, Keiji TanimotoAbstract:Abstract Purpose : This article reports on the possible relationship of diffuse sclerosing osteomyelitis (DSO) of the mandible to synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) Syndrome. Patients and Methods : The pathologic features in 12 new DSO patients and those in previously reported cases were reviewed and compared with those of SAPHO Syndrome. Results : Many similarities were noted between the two entities in terms of the clinical, radiographic, and histologic features. Furthermore, multiple bone lesions and skin lesions (palmoplantar pustulosis and psoriasis) were observed not only in SAPHO Syndrome but also in DSO patients. Conclusion : DSO is concluded to be one manifestation of SAPHO Syndrome.
Pingning Hsu - One of the best experts on this subject based on the ideXlab platform.
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increased neutrophil infiltration il 1 production and a SAPHO Syndrome like phenotype in pstpip2 deficient mice
Rheumatology, 2015Co-Authors: Hsiujung Liao, Itsu Chyuan, Shuwha Lin, Kunhung Chen, Hweifang Tsai, Pingning HsuAbstract:OBJECTIVE Proline-serine-threonine-phosphatase-interacting protein 2 (PSTPIP2) is involved in macrophage activation, neutrophil motility and osteoclast differentiation. However, the role of PSTPIP2 in inflammation and autoinflammatory diseases is still not clear. In this study, we generated PSTPIP2 knockout (Pstpip2(-/-)) mice to investigate its phenotype and role in autoinflammatory diseases. METHODS We constructed a Pstpip2-targeting vector and generated Pstpip2(-/-) mice. The phenotype and immunopathology of Pstpip2(-/-) mice were analysed. RESULTS All Pstpip2(-/-) mice developed paw swelling, synovitis, hyperostosis and osteitis, resembling SAPHO Syndrome, an inflammatory disorder of the bone, skin and joints. Multifocal osteomyelitis was found in inflamed paws, with increased macrophage and marked neutrophil infiltrations in the bone, joint and skin. Profound osteolytic lesions with markedly decreased bone volume density developed in paws and limbs. Neutrophil-attracting chemokines and IL-1β were markedly elevated in inflamed tissues. CONCLUSION Our study suggests that PSTPIP2 could play a role in innate immunity and development of autoinflammatory bone disorders, and may be associated with the pathogenesis of human SAPHO Syndrome.
B A Michel - One of the best experts on this subject based on the ideXlab platform.
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primary chronic osteomyelitis associated with synovitis acne pustulosis hyperostosis and osteitis SAPHO Syndrome
Journal of Oral Pathology & Medicine, 2007Co-Authors: Gerold Eyrich, C Harder, H F Sailer, T Langenegger, Elisabeth Bruder, B A MichelAbstract:Diffuse sclerosing osteomyelitis may indicate the mandibular localisation of the SAPHO Syndrome. Twelve patients with diffuse sclerosis of the mandible were examined for symptoms of the SAPHO Syndrome. Nine patients were found to have primary chronic osteomyelitis and eight of these represented a SAPHO Syndrome. Results in this series support the hypothesis of an association between primary chronic osteomyelitis and the SAPHO Syndrome.
