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Pleural effusion is a rare manifestation in synovitis-acne-pustulosis-hyperostosis-osteitis (SAPHO) syndrome, which is characterized by the presence of osteoarticular lesions and dermatological involvement. We herein report a 71-year-old man with pleural effusion resulting from SAPHO syndrome. He was successfully treated using corticosteroids and has experienced no recurrence for one year. We should consider SAPHO syndrome when encountering cases of anterior chest pain and pleural fluid.

We report a case of isolated lesions of the thoracic spine attributed to synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome. A 55-year-old woman who suffered from 6 months of back pain had vertebral osteomyelitis on magnetic resonance imaging (MRI). There were no laboratory findings suggestive of infection, malignancy, or autoimmune disease. Radiography, computed tomography (CT), and MRI of the thoracic spine showed mixed lesions of sclerosis and erosion, whereas bone scintigraphy did not show accumulation at any site except the thoracic spine. No lesions in the anterior chest wall or sacroiliac joints were apparent from CT and MRI. No lesions other than at the thoracic spine were observed. As the isolated lesions of the thoracic spine were considered not to have resulted from infection, malignancy, or autoimmune disease, the patient was referred to our department for differential diagnosis. Given that isolated sterile hyperostosis/osteitis among adults is included in the modified diagnostic criteria for SAPHO syndrome, we suspected that the mixed lesions of sclerosis and erosion of the thoracic spine in this case may reflect SAPHO syndrome with chronic non-bacterial osteitis (CNO) of the thoracic spine. Treatment with non-steroidal anti-inflammatory drugs (NSAIDs) was initiated and led to alleviation of her back pain, although the thoracic spine lesions remained on the 6-month MRI. Based on the CNO of the thoracic spine and the rapid response to NSAIDs, the final diagnosis was SAPHO syndrome with isolated lesions of the thoracic spine.

Synovitis-Acne-Pustulosis-Hyperostosis-Osteitis (SAPHO) syndrome is a rare inflammatory osteoarticular disorder, which encompassed many diseases, including pustulotic arthro-osteitis (PAO). Musculoskeletal manifestations, including osteitis, synovitis, and hyperostosis, are the hallmarks of the SAPHO syndrome and affect a variety of regions of the body. Recent survey indicated that more than 80% of cases of SAPHO syndrome in Japan were PAO, originally proposed by Sonozaki et al. in 1981, whereas severe acne was the most commonly reported skin ailment amongst participants with SAPHO syndrome in Israel. Prevalence of SAPHO syndrome remains unavailable, whereas the prevalence of palmoplantar pustulosis (PPP) was reported to be 0.12% in Japan, and 10–30% of patients with PPP had PAO. SAPHO syndrome and PAO are predominantly found in patients in the third through fifth decades of life, and a female predominance is seen in both groups. The diagnosis is typically made by a rheumatologist or dermatologist. Identification of a variety of the clinical, radiological, and laboratory features outlined, as well as diagnostic criteria, are used to make the diagnosis. Goals of treatment seek to maximize health-related quality of life, preventing structural changes and destruction, and normalizing physical function and social participation. Finally, we review the non-pharmacological and pharmacological managements.

Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome is a rare chronic inflammatory disease with osteoarticular and cutaneous involvements as its main manifestations. Acne conglobata as a severe acne form may affect adolescent patients. Tumor necrosis factor inhibitors are usually used as a second-line therapy for refractory SAPHO syndrome and has been reported to treat acne conglobata successfully. We herein report three cases of adolescent patients with SAPHO syndrome associated with acne conglobata who were successfully treated with TNFi therapy.

Objective A working group was established to develop a core domain set (CDS) for Chronic Nonbacterial Osteomyelitis (CNO) and Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis (SAPHO) following the OMERACT filter 2.1. Methods A scoping review to identify disease-related manifestations was performed, followed by a special interest group (SIG) session at OMERACT2020 to begin the CNO/SAPHO CDS framework. Results Candidate items were identified from the scoping review and most fell under Life Impact and Pathophysiology Manifestation core areas. A SIG agreed on the need to develop a CDS for CNO and SAPHO (100%) and for children and adults (91%). Conclusion Based on candidate items identified, qualitative research and Delphi surveys will be performed as next steps.

SAPHO syndrome is a rare clinical entity characterized by a wide range of dermatological and musculoskeletal manifestations. Treatment strategies are not standardized. Palmoplantar pustulosis (PPP) is the most common rash in patients with SAPHO syndrome.A 24-year-old Chinese woman with no relevant medical or familial history had a 1-year history of cutaneous lesions with PPP and pain in the sternoclavicular joint.Based on the diagnostic criteria for SAPHO syndrome proposed by Nguyen et al in 2012, we diagnosed SAPHO syndrome with severe PPP as the predominant manifestation.Due to the limited therapeutic efficacy of methotrexate and cyclosporin, we started therapy with subcutaneous secukinumab 150 mg weekly for the first month, then 150 mg monthly thereafter.After 4 weeks of secukinumab administration, the patient showed significant remission of pustular skin lesions, with almost no joint pain and no adverse reaction. Complete remission of skin symptoms was achieved after 3 months. Joint pain and adverse events have not reoccurred in follow-up thus far.In patients with SAPHO syndrome, we recommend personalized treatment, which may have excellent therapeutic efficacy in those with PPP or severe skin symptoms. Although data related to the use of IL-17 blockers for SAPHO syndrome are very limited, secukinumab provides a novel therapeutic option, especially for patients with PPP and severe skin lesions. Further prospective studies are needed to support our findings.

Synovitis,acne,pustulosis,hyperostosis,and osteitis (SAPHO) syndrome is a rare disease. The previous literature demonstrated that about 10% of the patients with SAPHO syndrome were complicated with inflammatory bowel disease.So far,few cases of SAPHO syndrome complicated with inflammatory bowel disease have been reported in China.Herein,we reported a SAPHO syndrome case complicated with ulcerative colitis. The patient suffered from recurrent attacks of colitis following treatment of SAPHO syndrome with etanercept.

Aim To present the prevalence of tonsillitis in synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) patients, to compare the clinical characteristics and disease activities between SAPHO patients with and without tonsillitis and to preliminarily explore the efficacy of tonsillectomy in SAPHO syndrome. Method A total of 58 SAPHO patients were included. Clinical data were collected, including demographic characteristics and acute phase reactants (erythrocyte sedimentation rate, high-sensitivity C-reactive protein). The visual analog scale (VAS), Palmoplantar Pustule Psoriasis Area Severity Index (PPPASI) and Nail Psoriasis Severity Index (NAPSI) were used to measure the severity of bone pain, skin lesions and nail lesions, respectively. Patients were referred to the otolaryngology department for tonsil examinations, including tonsil hypertrophy (grade ≥ 2), chronic congestion, inflammatory secretion and tonsil stones. The patients who underwent tonsillectomy were followed up after the surgery. Results A total of 67.2% of patients had tonsillitis. Patients with tonsillitis had markedly higher PPPASI (1.2 [0, 7.4] vs. 7.6 [1.75, 15.5], P = .018) and NAPSI (0 [0, 21] vs. 8 [3, 28], P = .032) scores. After tonsillectomy, the patients experienced significantly improved bone pain (VAS, 5 [4, 7] vs. 3 [1, 4], P = .034) and skin lesions (PPPASI, 16.2 [7.05, 18.35] vs 1.8 [0.7, 3.7], P = .028). Conclusion Approximately 2/3 of SAPHO patients had tonsillitis. Patients with tonsillitis had more severe skin and nail lesions. Tonsillectomy might be associated with improved bone and skin symptoms in SAPHO patients. Future prospective controlled studies are warranted.

The purpose of this review is to present the up-to-date evidence on the epidemiology, pathogenesis, musculoskeletal manifestations, and imaging of the synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome and to discuss its relationship with spondyloarthritis (SpA).SAPHO is a rare inflammatory disorder of bone, joints, and skin, with a worldwide distribution that predominantly affects the middle-age adults. The hallmark of the syndrome is a constellation of sterile inflammatory osteitis, hyperostosis, and synovitis involving the anterior chest wall, associated with acneiform and neutrophilic dermatoses, such as palmoplantar pustulosis and severe acne. The axial skeleton, sacroiliac, and peripheral joints can be involved in a similar fashion to SpA. The pathogenesis of the syndrome is multifactorial. The diagnosis is mainly based on the clinical and typical radiological features. The treatment approach is based on the off-label use of antibiotics, bisphosphonates, disease-modifying antirheumatic drugs, and anticytokine biologics.The SAPHO syndrome shares common features with SpA-related diseases, yet also shows some unique pathogenetic and clinical features. The nosology of SAPHO remains a subject of controversy, awaiting further research into the pathogenetic and clinical aspects of this syndrome. A better understanding of these aspects will improve the diagnostics and clinical care of patients with SAPHO.

A 66-year-old woman presented with anterior chest wall and knee joints pain and multiple skin lesions. 99mTc-MDP bone scintigraphy showed increased bone density in the sternal angle with abnormal bone metabolism. The patient was enrolled in a 68Ga-DOTA-FAPI-04 PET/CT tumor clinical trial, and it showed increased tracer uptake in the sternum and right knee joint. Subsequently, ultrasound of the right knee joint confirmed the existence of synovitis, and no malignant components were found in sternal biopsy. The patient's final diagnosis was synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome. The case confirmed that 68Ga-DOTA-FAPI-04 imaging is helpful for evaluating the involved sites of SAPHO syndrome.

Case report. To describe the first reported use of corrective surgery combined with C7 vertebral column resection (VCR) to treat an extremely rare case of severe, rigid cervical kyphotic deformity associated with synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome. Spinal lesions reportedly occur in 32–45% in SAPHO syndrome. However, bone and joint lesions are usually repaired such that severe joint destruction is rare. Therefore, there have been few reported cases of surgical treatment for spinal lesions. A 22-year-old woman had been diagnosed with cervical kyphotic deformity associated with SAPHO syndrome. She had difficulty looking upward. On radiography, the C4–C7 vertebral bodies were fused and exhibited severe rigid kyphotic deformity. Right convex scoliosis with a Cobb angle of 22° was apparent at C5–T2, and the C2–C7 angle of kyphosis was 75°. Corrective three-stage surgery was carried out from the anterior, posterior, and anterior, with C7 VCR. The C2–T1 angle improved to 21° and the patient was capable of looking up. At present, 2 years postoperatively, complete bony fusion has been achieved. Her cervical spine function and quality of life were markedly improved. Only a very limited number of patients have a deformity sufficiently severe that VCR of cervical vertebra is required, but as this is the technique that provides the greatest multiplanar alignment correction, its choice in the present case was appropriate. IV.

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.

The aim was to comparatively assess the clinical and imaging features in patients with SAPHO syndrome.The clinical data, laboratory results, imaging data of forty-six SAPHO patients were reviewed and the SAPHO patients were divided into spinal involvement group and non-spinal involvement group. Fifty patients with ankylosing spondylitis were recruited as control group. The clinical and radiological features of them were analyzed and compared.Thirty-four of 46 (73.9%) of all the SAPHO patients had spinal involvement. The lesions exhibited as abnormal hyper-intensity signal in vertebral bodies, vertebral body erosion or collapse, bone marrow edema, endplate inflammation, spondyldiscitis, paravertebral ossification, and facet joint involvement. Compared with patients in non-spinal involvement group, the age at disease onset was older (P = 0.033), the disease duration was longer (P = 0.048), and CRP level was elevated (P = 0.047) in patients in spinal involvement group. Compared with patients with ankylosing spondylitis, SAPHO patients were more likely to have cervical vertebra involvement (P = 0.024), endplate inflammation (P = 0.019), and spondyldiscitis (P = 0.001), but less multiple vertebral body and facet joint involvement (P = 0.002). Patients regularly received DMARDS or biologics treatment had symptoms relieved and lesions turned into chronic stage or better than before.A total of 73.9% of the SAPHO patients had spinal involvement and the involvement could affect any part of the spine. Cervical vertebral involvement, endplate inflammation, and sponlypodiscitis were more common in SAPHO than in patients with ankylosing spondylitis. In SAPHO patients with spinal involvement, the disease duration was longer and the inflammatory reaction was more intensive. DMARDs and biologics may help to prevent the disease progress.• To the best of our knowledge, this paper is the first one to comparatively study the clinical and radiological features of SAPHO syndrome, especially the characteristics of spinal involvement.

Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome and chronic recurrent multifocal osteomyelitis (CRMO) have been described as disorders of chronic osteoarthritic inflammation frequently associated with skin manifestations, and SAPHO and CRMO (SAPHO/CRMO) are rare autoinflammatory disorders of unknown etiology. SAPHO tends to occur in adults and CRMO predominantly occurs in children and adolescents. SAPHO/CRMO can affect any skeletal region (e.g., anterior chest wall, spine, or long bones). As SAPHO/CRMO are diagnoses of exclusion, the diagnoses might be difficult if skin manifestations are not clearly evident. However, knowledge of the imaging findings of skeletal disorders is helpful for correcting the diagnosis and avoiding unnecessary invasive procedures, as well as in facilitating early diagnosis and adequate treatment. This pictorial review describes the appearance of increased skeletal uptake for SAPHO/CRMO on bone scintigraphy along with findings from radiography, computed tomography, and magnetic resonance imaging.

Pulmonary vasculitis is an uncommon type of vasculitis that may cause pulmonary artery stenosis with ensuing ischemic damage to lung tissue. 99mTc-macroaggregated albumin scintigraphy and iodine lung perfusion mapping are useful imaging modalities for assessment of lung perfusion but are not useful for assessing disease activity. On the other hand, 18F-FDG PET/CT is widely used for assessment of vasculitis activity but cannot provide perfusion information. We reported the clinical utility of dual-phase 18F-FDG PET/CT for pulmonary vasculitis in patients with SAPHO (synovitis-acne-pustulosis-hyperostosis-osteitis) syndrome.

In the late 1960s, palmoplantar pustulosis (PPP) with sternocostoclavicular arthropathy was first described in Japan, predominantly affecting women in the perimenopausal age. In the 1970s, the chronic non-bacterial osteomyelitis and chronic recurrent multifocal osteomyelitis were initially observed in paediatric patients with approximately 70% girls. Acne fulminans accompanied by polyarthralgia have been observed since early 1970s, which almost exclusively occurs in adolescent boys. Report on spondyloarthropathy associated with hidradenitis suppurativa can be traced back to 1982. The SAPHO syndrome was coined in 1987 to lump together synovitis, acne, pustulosis, hyperostosis and osteitis to conceptualize a group of inflammatory osteocutaneous diseases of unclear etiopathogenesis and ill-defined associations spanning disparate age and gender groups. From historical view, Sasaki syndrome is proposed to replace SAPHO syndrome to represent PPP with sternocostoclavicular arthropathy in the absence of other skin manifestations. Hidradenitis suppurativa is folliculitis in pathogenesis and no longer classified as acne. PPP accompanied by psoriasis vulgaris is more likely psoriasis pustulosa palmoplantaris in dermatological aspect, and the associated arthritis is part of psoriatic arthropathy. Pathophysiology of these disorders is incompletely understood. To echo the advancement of high-throughput sequencing, splitting but not lumping of clinical findings would be a better strategy to decipher these multigenic complex inflammatory disorders.

We report the use of ixekizumab in treating synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome, the first such report to our knowledge. The patient presented with palmoplantar pustulosis and sternoclavicular joint pain, which was markedly improved with ixekizumab treatment.

Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome is an umbrella term covering a constellation of bone lesions and skin manifestations, but has rarely been clarified in the clinic. We report a 28-year-old woman who had initial onset of SAPHO syndrome with involvement of the femur, and she experienced a tortuous diagnostic course. We also performed a literature review of SAPHO syndrome cases involving the femur and summarize several empirical conclusions by integrating previous findings with our case. Furthermore, we propose our perspective that ailment of the skin caused by infection of pathogens might be the first hit for triggering or perpetuating the activation of the immune system. As a result, musculoskeletal manifestations are probably the second hit by crosstalk of an autoimmune reaction. The skin manifestations preceding bone lesions can be well explained. Current interventions for SAPHO syndrome remain controversial, but drugs aiming at symptom relief could serve as the first preference for treatment. An accurate diagnosis and appropriate treatment can cure patients in a timely manner. Although the pathogenesis of SAPHO syndrome remains to be determined, physicians and surgeons still need to heighten awareness of this entity to avoid invasive procedures, such as frequent biopsies or nonessential ostectomy.

Background Our understanding of work productivity impairment among patients with synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome is limited. The purpose of this study was to provide an overview of work productivity loss in SAPHO syndrome patients through the use of the work productivity and activity impairment (WPAI) questionnaire, as well as to investigate the relationship between the WPAI and other disease-related indicators. Methods Patients for this cross-sectional study were recruited from Peking Union Medical College Hospital (Beijing, China). The questionnaires incorporating the WPAI were administered, along with the inclusion of demographic data, disease-specific measures, and general health variables. The construct validity of the WPAI was evaluated via the correlations between WPAI outcomes and other measures. Wilcoxon rank-sum tests and nonparametric Kruskal‒Wallis tests were used for the comparison of the WPAI outcomes between known groups. Results A total of 376 patients were included, and 201 patients (53.5%) were employed. The medians (interquartile range [IQR]) of absenteeism, presenteeism, work productivity loss, and activity impairment were 0% (0–13%), 20% (0–40%), 20% (0–52%), and 30% (0–50%), respectively. All of the WPAI outcomes showed moderate to strong correlations with other generic and disease-specific measures (|r| = 0.43–0.75), except for absenteeism. Increasing disease activity and worse health status were significantly associated with increased impairments of work productivity and activity. Conclusion This study highlights the negative effects of SAPHO syndrome on the work productivity and activity of patients, thus indicating good construct validity and discriminative ability of the WPAI. To reduce the economic burden, it is important to improve the work productivity and daily activity of patients by ameliorating clinical care.

本文报道1例发生于中年男性的滑膜炎-痤疮-脓疱病-骨质增生-骨炎（synovitis-acne-pustulosis-hyperostosis-osteitis，SAPHO）综合征。患者男，40岁，因“反复咽痛30年，双手足皮疹伴关节疼痛4年”就诊。药物治疗效果不佳，全身麻醉下行扁桃体切除手术。术后1个月随访，疗效较好。.