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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.

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。.

Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome is a chronic inflammatory disease that severely affects patients’ quality of life. Therapies including tumour necrosis factor inhibitors (TNFi) and bisphosphonates (BPs) yield variable efficacy.1 Tofacitinib, a Janus kinase inhibitor, may suppress osteoclast-mediated joint damage by inhibiting the RANKL pathway.2 A previous study proved the safety and efficacy of tofacitinib in psoriatic arthritis resistant to TNFi over 3 months.3 Additionally, we reported a case where tofacitinib in combination with methotrexate and Tripterygium wilfordii Hook was effective in SAPHO syndrome.4 To further explore tofacitinib efficacy in SAPHO syndrome, we retrospectively reviewed the medical records of patients enrolled from January 2019 to December 2019 in our dynamic cohort of SAPHO syndrome.5 Patients were included if they: (1) received tofacitinib without concomitant TNFi, BPs, or other disease-modifying antirheumatic drugs and (2) had complete clinical data and MRI of SAPHO-related lesions with pain before (within 1 week) and during tofacitinib treatment. The pain visual analogue scale (VAS) score and …

SAPHO (synovitis-acne-pustulosis-hyperostosis-osteitis) syndrome is a chronic inflammatory disease involving multiple organs such as skin and bones. At present, its etiology and pathogenesis are still unclear. Due to the variety of clinical manifestations and the small number of SAPHO syndrome involving the mandible, accurate diagnosis is difficult for oral and maxillofacial surgeons. Here, the authors report that a male patient with SAPHO syndrome involving the maxillofacial skin and mandible, followed for 3 years. We used Tc-MDP (technetium-99 conjugated with methylene disphosphonate) (commercially known as Yunke) to treat this disease and achieved significant clinical treatment. This suggests that Tc-MDP can be used as a bisphosphonate to treat SAPHO syndrome.

Objective SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis, and osteitis) is an autoimmune disease of unknown etiology that seriously affects patients' daily lives. Family-based investigations support genetic contributions toward disease susceptibility. The present study evaluated whether the previously reported autoimmune disease-associated single nucleotide polymorphisms (SNPs) have any genetic overlap with SAPHO syndrome. Method Genomic DNA was obtained from 71 SAPHO patients and 104 healthy controls. The SNP genotypes of each patient were determined with polymerase chain reaction and matrix-assisted laser desorption/ionization time-of-flight mass spectrometry (MALDI-TOF MS). Genotype, allele, and haplotype frequencies were analyzed with SPSS software. Results Three SNP sites (rs10889677 and rs2201841 of interleukin [IL]-23R, and rs2243248 of IL-4) showed significant correlation with the occurrence of SAPHO syndrome in additive and dominant genetic models, while rs7517847 of IL-23R showed substantial correlation with SAPHO in the dominant genetic model. The G allele of rs2243248 (IL-4) was a high risk factor for SAPHO (P = 2.41e-5, odds ratio [OR] =7.79, 95% CI: 2.59-23.3). The haplotype (A-G-C-G-T), comprising 5 SNPs of the IL-23R gene, had a significantly higher frequency in the SAPHO cohort than in the controls (P = .011, OR = 2.05, 95% CI: 1.12-3.60). Conclusion Variants rs10889677, rs2201841, and rs7517847 of IL-23R, and variant rs2243248 of IL-4, showed strong associations with SAPHO syndrome. Patients carrying the A-G-C-G-T haplotype of IL-23 are significantly more likely to develop SAPHO syndrome.

SAPHO (synovitis, acne, pustulosis, hyperostosis and osteitis) syndrome is a heterogeneous condition combining osteoarticular and cutaneous manifestations. Conventional treatments are mostly ineffective. We hereby report two patients, the first with an aggressive form of disease and the second with an incomplete response to two different anti-TNF-α agents. Both were successfully treated with tocilizumab and ustekinumab, respectively, over a long period of time. A narrative review of a biological therapy in SAPHO syndrome yielded very little information on the specific use of these agents. We highlight the advantages of personalising therapy and describe emerging promising treatments for this disease. info:eu-repo/semantics/publishedVersion

Background The SAPHO syndrome is a relatively rare clinical entity characterized by a wide range of dermatological and musculoskeletal manifestations. Biologics have been used in cases refractory to conventional treatment. Methods We present herein a patient with refractory to treatment SAPHO syndrome who exhibited a dramatic and fast response to IL-17 blockade. Additionally, we performed a systematic review of all cases of patients with SAPHO syndrome treated with biologics to date. Results We identified 66 cases treated with biologics (45 with TNF blockers, 7 with IL-1 blockers, 13 with biologics targeting the IL-23/IL-17 axis, and 1 with tocilizumab). Data support a positive effect of anti-TNF treatment in SAPHO with a response rate in bone and joint manifestations of 93.3%. Skin disease also improved in 21/29 cases (72.4%). Data related to IL-1 inhibition in SAPHO are encouraging with most patients exhibiting a significant response in musculoskeletal manifestations (6/7, 85.7%). However, IL-1 inhibition is not effective in skin manifestations. Ustekinumab seems to have some efficacy with 2/4 patients responding in skin and 3/5 in bone/joint manifestations. Data related to IL-17 blockade indicate efficacy in skin disease with 4/7 patients responding (57.1%). Joint/bone manifestations improved in 3/8 patients (37.5%). Conclusions In SAPHO patients not responding to conventional treatment, TNF blockers appear to be the first choice. In patients failing TNF blockers, IL-1 inhibitors and biologics targeting the IL-17/IL-23 axis could be used.

Pustular psoriasis (PsO) is an uncommon variant of PsO that may present in a generalized or localized fashion with or without musculoskeletal or systemic inflammatory involvement. Generalized pustular PsO (GPP) presents as a widespread acute or subacute pustular eruption that may be familial and is often associated with severe flares and systemic inflammation. The palmoplantar pustulosis variant is localized to palms and soles, whereas acrodermatitis continua of Hallopeau is localized to the nail apparatus. Patients with pustular PsO may have overlapping plaque PsO and may develop psoriatic arthritis (PsA). Pustulosis is also a feature of both synovitis, acne, pustulosis, hyperostosis, osteomyelitis (SAPHO) syndrome and chronic nonbacterial osteomyelitis. At the 2020 Group for Research and Assessment of Psoriasis and Psoriatic Arthritis (GRAPPA) annual meeting, members were given an overview of the cutaneous features of pustular PsO, SAPHO, and recent insights into the genetics of GPP, leading to new targeted drug therapies and the development of validated endpoints.

Background SAPHO syndrome is a group of symptoms consisting of synovitis, acne, pustulosis, hyperostosis and osteosis. There is no specific laboratory index assist in the diagnosis of SAPHO because of its highly heterogeneous clinical manifestations. Pathogenic microorganisms had been identified in biopsies of some SAPHO cases and particular gene mutations were also linked to the occurrence of SAPHO. It is largely unknown whether intestinal microbiome plays a role in pathogenesis of SAPHO. To explore the intestinal microbiome structure of SAPHO syndrome, fecal samples from 17 SAPHO patients and 14 healthy controls (HC) were collected for 16S rDNA sequencing. Results Our results showed that there was no significant difference in alpha indexes and beta diversity between SAPHO and HC samples, while there were 14 operational taxonomic units (OTUs) in the Wilcoxon rank-sum test and 42 OTUs in the MetagenomeSeq analysis showed significant difference in distribution between the SAPHO and HC groups, 3 of which in Firmicutes were also observed in the random forest analysis and used to construct a receiver operating characteristic curve to evaluate the diagnostic value, the area under the curve was 0.86. Conclusion Fecal-associated microbiome in the SAPHO samples was characterized by the alteration in abundance of some nondominant species, and the 3 selected OTUs in Firmicutes could serve as candidate biomarkers for SAPHO syndrome diagnosis.

Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome is a rare chronic disease with marked clinical and radiological heterogeneity. It is characterized by a combination of dermatological and osteoarticular manifestations. The treatment of SAPHO syndrome is not yet codified. It includes several therapeutic options such as anti-inflammatory drugs, bisphosphonates, antibiotics, conventional disease-modifying antirheumatic drugs, and biological treatment.This article aims to provide an updated review of the different pharmacological options for SAPHO syndrome. We also propose a therapeutic algorithm for the management of this disease.

SAPHO syndrome is a rare disease that typically begins in the third-fifth decade of life, and is characterized by osteoarticular and dermatologic features [1]. The acronym summarizes the key clinic...

Synovitis, acne, palmoplantar pustulosis, hyperostosis and osteitis (SAPHO) syndrome is a rare entity that involves the skin, bones and joints. The estimated prevalence of SAPHO syndrome is lower than 1/10 000.1 The real prevalence may be underestimated because of lack of typical symptoms.2 Bone is one of the critical affected organs for SAPHO. The most common site is the anterior chest wall (65%–90%), followed by the thoracic spine.1 However, the effects of SAPHO syndrome on bone loss or osteoporosis have not been clarified. We performed a case–control study to show bone loss in patients with SAPHO syndrome in a Chinese population. From June 2014 to August 2019, a total of 27 new-onset SAPHO patients were included in the study after excluding patients who had been diagnosed for more than 6 months and who were younger than 25 years old. The diagnosis of SAPHO syndrome was based on clinical symptoms and radiological examinations.2 The diagnosis is based on the presence of at least one of four features: (1) osteoarticular manifestations with severe acne; (2) osteoarticular manifestations with palmoplantar pustulosis; (3) hyperostosis with or without skin lesions; (4) recurrent multifocal chronic osteomyelitis involving the axial or peripheral skeleton, with or without skin lesions. Two or three age-matched and gender-matched control subjects who underwent chest CT scan for physical examinations …

Acne fulminans (AF) is a rare and severe form of inflammatory acne presenting clinically with an abrupt outburst of painful, hemorrhagic pustules and ulceration, that may or may not be associated with systemic symptoms, such as fever, polyarthritis, and laboratory abnormalities. It typically affects male teenagers with a pre-existing acne. Although the pathogenetic mechanism has not been established yet, a role of genetic, abnormal immunologic response, drugs intake, hormonal imbalance and viral infection, as causal factors, has been identified. AF may occur as a single disease or may be associated with other disorders. Traditionally, AF has been classified, on the basis of the presence of systemic involvement, in "acne fulminans" and acne fulminans "sine fulminans," when no systemic involvement is present. Recently, four clinical variants have been proposed: acne fulminans with systemic symptoms (AF-SS), acne fulminans without systemic symptoms (AF-WOSS), isotretinoin-induced acne fulminans with systemic symptoms (IIAF-SS), isotretinoin-induced acne fulminans without systemic symptoms (IIAF-WOSS). The diagnosis of AF is usually based on clinical history and physical examination. No specific laboratory abnormalities are generally found. In selected cases, biopsy and/or radiologic imaging are helpful for a correct diagnosis. The treatment significantly differs from severe acne according to severity of clinical presentation and possible systemic involvement. Currently, systemic corticosteroids (prednisolone) and retinoids (isotretinoin) represent the first choice of treatment. Dapsone, cyclosporine A, methotrexate, azathioprine, levamisole, and biological agents such as anakinra, infliximab, adalimumab may be considered as alternative therapies in selected cases. Adjunctive topical and physical therapies may also be considered.

is missing (Short Communication)

SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome shows a wide variability in musculoskeletal and cutaneous manifestations, and it is therefore underrecognized and misdiagnosed in the clinic due to a lack of specific markers. In this study, we aimed to identify specific biomarkers by screening serum autoantibodies in SAPHO patients with a 17K human whole-proteome microarray. The serum anti-Sp17 autoantibody was identified and verified to be a specific biomarker in patients with SAPHO syndrome. Indeed, the level of the anti-Sp17 autoantibody was significantly increased in patients with active SAPHO compared to patients with an inactive disease and healthy controls (P

Background The 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. Purpose To characterize the MRI features of the ACW in patients with SAPHO syndrome. Methods Seventy-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. Results The 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. Conclusion The 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.

Synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome has been proposed to describe a clinicoradiological entity manifesting in the joints, skin and bones. We describe a patient with SAPHO syndrome for whom bilateral tonsillectomy was effective for the treatment of arthralgia, which was quantified using multiple pain scales.

Purpose of ReviewTo describe in detail the clinical synopsis and pathophysiology of chronic non-bacterial osteomyelitis and SAPHO syndrome.Recent FindingsChronic non-bacterial osteomyelitis (CNO) has been identified as a disease entity for almost 50 years. This inflammatory bone disorder is characterized by osteolytic as well as hyperostotic/osteosclerotic lesions. It is chronic in nature, but it can present with episodic flairs and phases of remission, which have led to the denomination “chronic recurrent osteomyelitis”, with its severe multifocal form “chronic recurrent multifocal osteomyelitis” (CRMO). For almost three decades, an infectious aetiology had been considered, since especiallyPropionibacterium acneshad been isolated from bone lesions of individual patients. However, this concept has been challenged since long-term antibiotic therapy did not alter the course of disease and modern microbiological techniques (including PCR) failed to confirm bone infection as an underlying cause. Over recent years, a profound dysregulation of cytokine expression profiles has been demonstrated in innate immune cells of CNO patients. A hallmark of monocytes from CNO patients is the failure to produce immune regulatory cytokines interleukin-10 (IL-10) and IL-19, which have been linked with genetic and epigenetic alterations. Subsequently, a significant upregulation of pro-inflammatory, NLRP3 inflammasome-dependent cytokines (IL-1β and TNF-α), has been demonstrated.SummaryThe current knowledge on CNO, the underlying molecular pathophysiology, and modern imaging strategies are summarized; differential diagnoses, treatment options, outcome measures, as well as quality of life studies are discussed.

A 28-year-old African American male with a history of synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome, tobacco use, and sickle cell trait was referred to a physiatrist at a multidisciplinary spine center with a one-year history of non-traumatic thoracic back pain that had significantly worsened over the prior four weeks. In the context of recurrent infections requiring hospital admissions and the patient's immunosuppressed status, magnetic resonance imaging (MRI) of his thoracic spine was obtained, showing an acute or subacute anterior compression deformity of the T7 vertebral body. He was subsequently provided with a hyperextension brace, physical therapy (PT) referral, and a trial of intranasal calcitonin. The patient reported significant improvement in pain at his three-month follow-up appointment and continued to show pain and functional improvement in PT sessions up to six months later.

Paraneoplastic arthritides are a group of immune-mediated inflammatory arthropathies associated with occult or manifest malignancy. Musculoskeletal spread of an underlying malignancy may also mimic many rheumatologic conditions. Distinguishing primary rheumatologic condition from paraneoplastic arthritides versus direct musculoskeletal spread of malignancy can be challenging especially in individuals with prior history of cancer and new musculoskeletal complaints. SAPHO (synovitis, acne, pustulosis, hyperostosis and osteitis) syndrome is an uncommon, although under recognized autoimmune disorder. Two musculoskeletal manifestations, namely inflammatory osteitis and hyperostosis of anterior chest wall with or without dermatologic manifestations, constitute a unifying feature of SAPHO syndrome. However, diagnosis of SAPHO syndrome is one of exclusion, and a wide variety of disorders including infections, malignancy (chondrosarcoma/osteosarcoma/metastasis), metabolic bone disorders (Paget's disease), osteoarthritis, seronegative spondyloarthropathy (spA) and osteonecrosis form part of a broad differential diagnosis. We present the case of a man, aged 72 years, with signs and symptoms of SAPHO syndrome and skin findings. Detailed history, radiological imaging, dermatology appearance, and role of immunohistochemical markers, especially staining for NKX3.1 protein with a novel antibody, led to a diagnosis of metastatic prostate adenocarcinoma. To our knowledge, this is the first case of metastatic adenocarcinoma of the prostate manifesting as SAPHO syndrome and cutaneous metastasis.