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Key Points Diffuse idiopathic skeletal hyperostosis (DISH) is characterized by entheseal ossification and/or calcification involving mainly the thoracic spine Peripheral joints and adjacent entheses can also be involved The pathogenesis of DISH is not clear, but several factors may promote the differentiation of mesenchymal cells into bone-forming cells DISH is often associated with a variety of metabolic derangements, which may increase cardiovascular morbidity Patients with DISH also have an increased risk of complicated spinal fractures, with associated morbidities Diffuse idiopathic skeletal hyperostosis (DISH) is a poorly understood condition characterized by ossification of ligaments and entheses. This comprehensive Review explains the epidemiology and clinical manifestations of DISH, as well as highlighting the latest insights into pathogenic mechanisms. The authors also argue for the development of new classification criteria that can identify early disease. Diffuse idiopathic skeletal hyperostosis (DISH) is a systemic condition characterized by the ossification and calcification of ligaments and entheses. DISH is observed on all continents and in all races, but most commonly in men over 50 years of age. Although DISH is asymptomatic in most individuals, the condition is often an indicator of underlying metabolic disease, and the presence of spinal or extraspinal ossifications can sometimes lead to symptoms including pain, stiffness, a reduced range of articular motion, and dysphagia, as well as increasing the risk of unstable spinal fractures. The aetiology of DISH is poorly understood, and the roles of the many factors that might be involved in the development of excess bone are not well delineated. The study of pathophysiological aspects of DISH is made difficult by the formal diagnosis requiring the presence of multiple contiguous fully formed bridging ossifications, which probably represent advanced stages of DISH. In this Review, the reader is provided with an up-to-date discussion of the epidemiological, aetiological and clinical aspects of DISH. Existing classification criteria (which, in the absence of diagnostic criteria, are used to establish a diagnosis of DISH) are also considered, together with the need for modified criteria that enable timely identification of early phases in the development of DISH.

ObjectiveThis study aimed to investigate the efficacy and safety of Tripterygium wilfordii Hook F. (TwHF) in the treatment of osteoarticular lesions in synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome.MethodsEligible SAPHO patients were recruited to this single-center trial to receive 12-week TwHF treatment. Two dose groups (1.0-mg/kg/day group and 1.5-mg/kg/day group) were designed and patients were allocated (1:1) to these two groups. The primary endpoint was the change from baseline in Ankylosing Spondylitis Disease Activity Score on the basis of C-reactive protein level (ASDAS) at week 12.ResultsAll the 30 included patients completed the trial. At week 12, both dose groups showed significant change from baseline in ASDAS (1.0-mg/kg/day group: − 1.34 (1.10), p = 0.000; 1.5-mg/kg/day group: − 1.53 (1.19), p = 0.000). Similar improvement was also found in the Visual Analogue Scale in global osteoarticular pain, Bath Ankylosing Spondylitis Disease Activity Index, and other efficacy measures. The results showed a fast-acting characteristic of TwHF that the maximum efficacy was achieved within the first 2–4 weeks and maintained at a stable level for the rest of the study. No significant differences were observed between the two dose groups under the current sample size. TwHF was well tolerated that no severe adverse events or irregular menstruation were recorded, except for one patient who developed severe alanine aminotransferase elevation at the last follow-up and has stopped the TwHF treatment after the 12-week follow-up.ConclusionsTwHF should be considered for the treatment of osteoarticular lesions in SAPHO syndrome in clinical practice because of significant efficacy, reliable safety, and high socioeconomic value.Trial registrationChiCTR1900025912Key points• This is the first clinical trial to evaluate Tripterygium wilfordii Hook F. (TwHF) in the treatment of synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome.• Twelve-week TwHF treatment in both dose groups designed (1.0-mg/kg/day group and 1.5-mg/kg/day group) was well tolerated and could lead to significant disease remission of SAPHO syndrome.• No significant differences were observed between the two dose groups under the current sample size.• TwHF should be considered for the treatment of osteoarticular lesions in SAPHO syndrome in clinical practice because of significant efficacy, reliable safety, and high socioeconomic value.

Diffuse idiopathic skeletal hyperostosis (DISH) is a common disease in older adults that causes extensive spinal ankylosis. However, its impact on quality of life (QOL) and locomotive syndrome (LS) remains unknown. Thus, we aimed to evaluate the DISH effect on QOL and LS in community-dwelling middle-aged and older adults. Data of community volunteers who attended a health checkup in 2018–2019 were assessed. A total of 455 subjects were included. Whole spine lateral radiographs were obtained to detect DISH according to the Resnik criteria. QOL was evaluated using SF-36 and EQ5D HUSV. LS was evaluated using the stand-up and two-step tests and GLFS-25. Multivariate regression analysis was performed, adjusting for sex, age, body mass index, and knee osteoarthritis. Additionally, a sex-stratified analysis was performed. DISH was detected in 83 (18.2%) participants. Multivariate analysis demonstrated that DISH was significantly associated with low EQ5D HSUV. Multivariate analysis in men demonstrated that DISH was associated with low PCS, low EQ5D HSUV, high LS stage. Multivariate analysis in women demonstrated that DISH was not associated with any QOL or LS. DISH may be associated with a poor QOL. Additionally, DISH is more common and produces a greater clinical impact in men than in women.

Background and objectivesSynovitis acne pustulosis hyperostosis osteitis (SAPHO) is a rare heterogeneous disease of unknown aetiopathology. Externally validated and internationally agreed diagnostic criteria or outcomes and, as a result, prospective randomised controlled trials in SAPHO are absent. Consequently, there is no agreed treatment standard. This study aimed to systematically collate and discuss treatment options in SAPHO.MethodsFollowing ‘Preferred Reporting Items for Systematic Reviews and Meta-Analyses’ guidance, a systematic literature search was conducted using PubMed, Scopus and Web of Science databases. Prospective clinical studies and retrospective case collections discussing management and outcomes in SAPHO involving five or more participants were included. Articles not published in English, studies not reporting defined outcomes, and studies solely relying on patient-reported outcomes were excluded.ResultsA total of 28 studies (20 observational, 8 open-label clinical studies) reporting 796 patients of predominantly European ethnicity were included. Reported therapies varied greatly, with many centres using multiple treatments in parallel. Most patients (37.1%) received non-steroidal anti-inflammatory drugs alone or in combination. Bisphosphonates (22.1%), conventional (21.7%) and biological (11.3%) disease-modifying antirheumatic drugs were the next most frequently reported treatments. Reported outcomes varied and delivered mixed results, which complicates comparisons. Bisphosphonates demonstrated the most consistent improvement of osteoarticular symptoms and were associated with transient influenza-like symptoms. Paradoxical skin reactions were reported in patients treated with TNF inhibitors, but no serious adverse events were recorded. Most treatments had limited or mixed effects on cutaneous involvement. A recent study investigating the Janus kinase inhibitor tofacitinib delivered promising results in relation to skin and nail involvement.ConclusionsNo single currently available treatment option sufficiently addresses all SAPHO-associated symptoms. Variable, sometimes descriptive outcomes and the use of treatment combinations complicate conclusions and treatment recommendations. Randomised clinical trials are necessary to generate reliable evidence.

This report highlights an unusual case of a woman in her 70s who presented with diffuse idiopathic skeletal hyperostosis and an initial symptom of spinal cord compression and associated spinal degeneration. She presented with progressive thoracolumbar pain, bilateral lower limb weakness, and sensory deficits. Imaging showed continuous osteophytes in the anterior and lateral spine, multiple levels of intervertebral space narrowing, marked ligament ossification at T10/11, and severe spinal stenosis. Diffuse idiopathic skeletal hyperostosis was diagnosed and spinal cord compression was significantly reduced after laminectomy. Although diffuse idiopathic skeletal hyperostosis is relatively common in elderly patients, cases of spinal cord compression are still rare, and the combination of intervertebral space stenosis, and ossification of the ligamentum flavum may be misdiagnosed as degenerative spondylopathy. This case suggests the possibility of intervertebral stenosis and ossification of the thoracic ligamentum flavum coexisting with diffuse idiopathic skeletal hyperostosis, highlighting the importance of diagnostic imaging in the early stage of patient management.

This review provides an overview of SAPHO (Synovitis, Acne, Pustulosis, Hyperostosis, and Osteitis), a rare autoinflammatory disease that primarily affects bones, skin, and joints. We conducted a search on Medline/PubMed using keywords such as SAPHO syndrome, chronic recurrent multifocal osteitis/osteomyelitis, and related terms. SAPHO syndrome is rare, with a reported frequency of 1 in 10,000 in the Caucasian population. However, the actual incidence of SAPHO syndrome is unknown, and the incidence of the disease is likely higher. The pathogenesis of SAPHO syndrome remains incompletely understood. Current evidence suggests that SAPHO results from a complex interplay between immune dysregulation, genetic susceptibility, and environmental factors. It's not clear if SAPHO syndrome is an autoimmune disease or an autoinflammatory disease, but current evidence suggests that it's more likely an autoinflammatory disease because of things like neutrophil hyperactivity, fewer natural killer (NK) cells, high levels of interleukin (IL)-1, and a good response to treatments that block IL-1. Osteo-articular (OA) involvement is a key clinical feature of SAPHO. It affects the anterior chest wall, axial skeleton, peripheral joints, mandible, long bones of the extremities, and pelvis. Dermatological involvement is a common target in SAPHO, with lesions observed in 60–90% of cases. Common skin lesions include psoriasis and acne, with hidradenitis suppurativa and neutrophilic dermatoses being less commonly seen. Other clinical findings include constitutional symptoms caused by systemic inflammation, such as fever, weight loss, and fatigue. There is no specific laboratory finding for SAPHO syndrome. However, during active disease, there may be an increase in positive acute phase markers, such as erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), complement levels, mild leukocytosis, and thrombocytosis. Diagnosis is crucial for SAPHO syndrome, which lacks a specific diagnostic finding and is often underrecognized. A comprehensive evaluation of a patient's medical history and physical examination is crucial. Treatment options include non-steroidal anti-inflammatory drugs (NSAIDs), corticosteroids, conventional and synthetic disease-modifying agents (cDMARDs and sDMARDs), biological therapies, bisphosphonates, and antibiotics. Biological treatments have emerged as a viable alternative for SAPHO patients who do not respond to conventional treatments.

PurposePatients with diffuse idiopathic skeletal hyperostosis (DISH) that extends to the lumbar segments (L-DISH) have a high risk of further surgery after lumbar decompression. However, few studies have focused on the ankylosis status of the residual caudal segments, including the sacroiliac joint (SIJ). We hypothesized that patients with more ankylosed segments beside the operated level, including the SIJ, would be at a higher risk of further surgery.MethodsA total of 79 patients with L-DISH who underwent decompression surgery for lumbar stenosis at a single academic institution between 2007 and 2021 were enrolled. The baseline demographics and radiological findings by CT imaging focusing on the ankylosing condition of the residual lumbar segments and SIJ were collected. Cox proportional hazard analysis was conducted to investigate the risk factors for further surgery after lumbar decompression.ResultsThe rate of further surgery was 37.9% during an average of 48.8 months of follow-up. Cox proportional hazard analysis demonstrated that the presence of fewer than three segments of non-operated mobile caudal segments was an independent predictor for further surgery (including both the same and adjacent levels) after lumbar decompression (adjusted hazard ratio 2.53, 95%CI [1.12–5.70]).ConclusionsL-DISH patients with fewer than three mobile caudal segments besides index decompression levels are at a high risk of further surgery. Ankylosis status of the residual lumbar segments and SIJ should be thoroughly evaluated using CT during preoperative planning.

IntroductionTo delineate the MRI characteristics of Hyperostosis Frontalis Interna (HFI) and evaluate its imaging features to aid in accurate diagnosis and differentiation from other pathologies, particularly metastatic disease.MethodsA retrospective analysis of 74 patients with HFI was conducted. MRI sequences, including pre-contrast T1-weighted, post-contrast T1-weighted, and T2-weighted fat-saturation imaging, were evaluated. Quantitative and qualitative assessments of HFI signal intensity and enhancement patterns were performed. The Hershkovitz classification categorized the extent of HFI.ResultsPre-contrast T1-weighted imaging showed varied signal intensities, with hypointense regions more common in advanced HFI stages. Hypointense HFI was associated with a lower likelihood (OR: 0.303, CI: 0.113–0.808) and heterogeneous distribution was associated with a higher likelihood (OR: 5.128, CI: 1.982–13.265) of higher Hershkovitz classification (P = 0.0008). Post-contrast T1-weighted imaging revealed that 31% of subjects demonstrated enhancement, with focal geographic enhancement associated with lower CT attenuation values (P = 0.0138), indicating higher fat content. T2-weighted fat-saturation imaging supported the correlation between hypointense signals on pre-contrast T1 imaging and lower T2 signal intensities (P = 0.0022). No significant differences were found in enhancement patterns between different MRI sequences (P > 0.1326).ConclusionsHFI demonstrates varying appearances on pre- and post-contrast MRI sequences, crucial for differentiating benign HFI from metastatic lesions. Understanding these imaging characteristics can enhance diagnostic accuracy, reduce the risk of misdiagnosis, and improve patient management. Future studies should focus on larger, more diverse populations and explore advanced MRI techniques to further understand HFI.

Purpose Studies investigating hyperostosis frontalis interna (HFI) in acromegaly are limited. We aimed to investigate HFI and the association of disease control with frontal bone thickness (FBT) in acromegaly. Methods Adult patients with acromegaly were grouped according to the presence of HFI on the baseline MRI: Group 1 absent, Group 2 present. We measured FBT, parietal bone thickness (PBT) and occipital bone thickness (OBT) in the mid-sagittal plane on MRI. The changes between first and last measurements were analyzed. We grouped the patients as controlled vs. uncontrolled acromegaly, and as established disease control for at least 5-year vs. 1-5-years. Results Group 1/Group 2 comprised of 23/29 patients, female/male ratio was 34/18, and mean age 55.41(± 14.21) years. Median follow-up duration was 108 months (6-408). FBT first ( p  = 0.001), FBT last ( p  < 0.001), PBT last ( p  = 0.025), and OBT last ( p  = 0.028) were higher in Group 2 than in Group 1. FBT change , PBT change , and OBT change were positive in Group 2 ( p  < 0.001, p  = 0.008, and p  = 0.008; respectively). The ratio of patients with FBT(increased) was higher in Group 2 than in Group 1 ( p  = 0.001). FBT first , FBT last , PBT first , PBT last , OBT first , OBT last , FBT change , PBT change and OBT change were similar in controlled or uncontrolled acromegaly groups. FBT change and OBT change were positive in patients with disease control established for at least 5 years ( n  = 30) ( p  = 0.027 and p  = 0.002, respectively). Conclusion HFI was common in patients with acromegaly. HFI is associated with a continuous increase in FBT, PBT and OBT. HFI, bone thickness, or increase in bone thickness seems independent of disease activity. Since headaches can be related to an increase in bone thickness, patients should be evaluated and graded during baseline imaging.

Background Computed tomography (CT) analyses have reported that the prevalence of diffuse idiopathic skeletal hyperostosis (DISH) in Japan is 8.7–27.1%. However, these data were obtained using chest-abdominal CT, and no evaluations of sagittal, coronal, and axial images using whole-spine CT have been reported. The aim of this study was to investigate the prevalence and characteristic of DISH by whole spinal CT. Methods Participants were patients who had experienced trauma who had undergone whole-spine CT scanning based on the initial clinical practice guidelines for trauma in our institute from April 2015 to February 2018. The subjects were > 20 years old and 1479 were included in the analysis. The presence and distribution of DISH and clinical parameters such as age and sex were reviewed retrospectively according to the location of DISH. Results The overall prevalence of DISH was 19.5% ( n  = 289). Subjects with DISH were older than those without. DISH was located in the thoracic spine in 65.1% and thoracolumbar spine in 24.2% of patients. More than 80% of ligamentous ossifications associated with DISH occurred at T8 ( n  = 255, 88%), T9 ( n  = 262, 91%), and T10 ( n  = 247, 85%). Most of the ossification occurred to the right anterior of the vertebral body, and there were few ossifications in the areas in contact with the artery and vein. Conclusions The prevalence of DISH based on whole-spine CT was 19.5%. Ossification was noted more often at T8, T9, and T10, and to the right anterior of the vertebral body. It is for the first time report that we have studied the location of ossification in detail using the axial images of whole spine CT. We hope this study will enhance the understanding of the characteristics of DISH.