ABSTRACT
BACKGROUND: Recently, JAKi has also been widely proved to be an effective alternative to conventional treatment for Synovitis acne pustulosis-hyperostosis-osteitis (SAPHO) cases, after failure of multiple drugs including those described above. But what to do when all these treatments fail? We report a case of remission from Tripterygium wilfordii Hook (TwHF) treatment. METHODS: The patient was treated with nonsteroidal anti-inflammatory drugs, oral prednisone, minocycline, bisphosphonate injection, etanercept, and tofacitinib, but the symptoms did not change significantly. Treatment with TwHF (1.0 mg/kg/day, patient weight 60 kg) was started for 24 weeks. RESULTS: After 50 months of unsatisfactory treatment, this patient was finally treated with herbal TwHF, and after 6 months of treatment, the patient's magnetic resonance imaging and inflammatory indexes were significantly improved, indicating that the disease had been better controlled. CONCLUSION: In this study, TwHF was successful in treating a patient with refractory SAPHO syndrome who was refractory to multiple Western medications without significant adverse effects or toxicities, but further follow-up is needed to determine long-term efficacy. More case reports as well as clinical trials are still needed to confirm whether TwHF can effectively treat refractory SAPHO syndrome.
Subject(s)
Acquired Hyperostosis Syndrome , Tripterygium , Humans , Acquired Hyperostosis Syndrome/drug therapy , Female , Adult , Janus Kinase Inhibitors/therapeutic use , Recurrence , Middle Aged , Plant Extracts/therapeutic use , Treatment Outcome , MaleSubject(s)
Acquired Hyperostosis Syndrome , Clavicle , Lumbar Vertebrae , Humans , Lumbar Vertebrae/diagnostic imaging , Clavicle/injuries , Clavicle/diagnostic imaging , Acquired Hyperostosis Syndrome/diagnosis , Female , Treatment Outcome , Fractures, Bone/etiology , Fractures, Bone/diagnostic imaging , Middle AgedSubject(s)
Acquired Hyperostosis Syndrome , Piperidines , Pyrimidines , Humans , Female , Pregnancy , Pyrimidines/therapeutic use , Pyrimidines/adverse effects , Piperidines/therapeutic use , Adult , Acquired Hyperostosis Syndrome/drug therapy , Acquired Hyperostosis Syndrome/diagnosis , Treatment Outcome , Protein Kinase Inhibitors/therapeutic use , Protein Kinase Inhibitors/adverse effects , Janus Kinase Inhibitors/therapeutic use , Live Birth , Pyrroles/therapeutic use , Pyrroles/adverse effectsABSTRACT
Synovitis-acne-pustulosis-hyperostosis-osteomyelitis (SAPHO) syndrome is characterised by aseptic osteitis and is often complicated by pustular dermatitis, such as palmoplantar pustulosis or acne. Although bone lesions are most found in the anterior thoracic region or spine, femoral lesions are not well documented in the literature. There is no established treatment for this condition, and few reports have described its long-term course. Here, we describe two cases of SAPHO syndrome involving the femur and discuss their long-term follow-up. A 40-year-old man (Case 1) presented with right thigh pain. Fifteen years after the initial diagnosis, the pain could be controlled with minomycin, salazosulfapyridine, and methotrexate. X-rays of the femur showed gradual cortical thickening. Although there were waves of pain, it gradually improved with the adjustment of drugs 25 years following the initial diagnosis. A 35-year-old man (Case 2) with right thigh pain was prescribed salazosulfapyridine and methotrexate; however, these were ineffective. Alendronate and guselkumab also proved ineffective. Ultimately, infliximab was started 9 years following disease onset, and pain became manageable. X-rays of the femur showed cortical thickening. SAPHO syndrome can be managed with drug therapies, such as nonsteroidal anti-inflammatory drugs, methotrexate, and conventional synthetic disease-modifying antirheumatic drugs; however, there are occasional treatment-resistant cases.
Subject(s)
Acquired Hyperostosis Syndrome , Femur , Humans , Male , Adult , Acquired Hyperostosis Syndrome/diagnosis , Acquired Hyperostosis Syndrome/complications , Acquired Hyperostosis Syndrome/drug therapy , Femur/pathology , Femur/diagnostic imaging , Treatment Outcome , Methotrexate/therapeutic use , Methotrexate/administration & dosageSubject(s)
Acquired Hyperostosis Syndrome , Pulmonary Arterial Hypertension , Humans , Acquired Hyperostosis Syndrome/diagnosis , Acquired Hyperostosis Syndrome/complications , Acquired Hyperostosis Syndrome/drug therapy , Pulmonary Arterial Hypertension/etiology , Pulmonary Arterial Hypertension/diagnosis , Pulmonary Arterial Hypertension/drug therapy , Pulmonary Arterial Hypertension/physiopathology , Treatment Outcome , Female , Pulmonary Artery/diagnostic imaging , Antihypertensive Agents/therapeutic use , Arterial Pressure/drug effects , Middle AgedSubject(s)
Acquired Hyperostosis Syndrome , Humans , Retrospective Studies , Acquired Hyperostosis Syndrome/drug therapy , Acquired Hyperostosis Syndrome/diagnosis , Acquired Hyperostosis Syndrome/therapy , Female , Male , Treatment Outcome , Adult , Middle Aged , Antirheumatic Agents/therapeutic useABSTRACT
OBJECTIVE: To report a statistical evaluation of symptomatology based on 56 cases of SAPHO syndrome and 352 non-SAPHO involvement cases, to propose a symptomatic scoring system in consideration of early warning for SAPHO syndrome. METHODS: A cohort comprising 56 subjects diagnosed with SAPHO syndrome was reported, as well as 352 non-SAPHO involvement cases, including their chief complaints, skin manifestations, radiological findings, and laboratory tests. We systematically reviewed previous published five representative huge cohorts from different countries to conclude several specific features of SAPHO by comparing with our case series. The score of each specific index is based on respective incidence and comparison of two cohorts was performed. RESULT: In terms of complaint rates, all subjects of two cohorts suffered from osseous pain, which appeared in the anterior chest wall, spine, and limb which were calculated. In respect to dermatological lesions, SAPHO patients suffered from severe acne, and other patients (82.14%) accompanied with palmoplantar pustulosis. Having received radiological examinations, most SAPHO subjects rather than non-SAPHO involvement cases showed abnormal osteoarticular lesions under CT scanning and more detailed information under whole-body bone scintigraphy. Differences also emerged in elevation of inflammation values and rheumatic markers like HLA-B27. Based on our cases and huge cohorts documented, the early warning standard is set to be 5 scores. CONCLUSIONS: SAPHO syndrome case series with 56 subjects were reported and an accumulative scoring system for the early reminder on SAPHO syndrome was proposed. The threshold of this system is set to be 5 points. Key Points ⢠Fifty-six patients diagnosed by SAPHO syndrome with detailed symptoms and radiological findings were reported. ⢠Comparison was made between the 56 SAPHO patients and 352 non-SAPHO involvement cases. ⢠An accumulative scoring system for the early reminder on SAPHO syndrome was proposed and the threshold of this system is set to be five points.
Subject(s)
Acquired Hyperostosis Syndrome , Humans , Acquired Hyperostosis Syndrome/diagnostic imaging , Radionuclide Imaging , Bone and Bones/pathology , Radiography , Spine/pathologyABSTRACT
Synovitis, acne, palmoplantar pustulosis, hyperostosis, and osteitis (SAPHO) syndrome is a rare and refractory autoinflammatory disease, and there is no consensus on its treatment. Stellate ganglion block (SGB) blocks sympathetic nerves, ameliorates immune dysfunction, and alleviates stress response, which has been used to treat various chronic pain syndromes, arrhythmias, and post-traumatic stress disorder (PTSD). Also, the SGB has been reported to be successfully used to treat certain skin diseases, autoinflammatory diseases, and menopausal symptoms. In this study, over 3 years of follow-up, we found that SGB successfully intervened the symptoms of SAPHO syndrome, including sternoclavicular joint arthritis and palmoplantar pustulosis.
Subject(s)
Acquired Hyperostosis Syndrome , Autonomic Nerve Block , Stellate Ganglion , Humans , Acquired Hyperostosis Syndrome/diagnosis , Acquired Hyperostosis Syndrome/therapy , Autonomic Nerve Block/methods , Female , Treatment Outcome , Middle Aged , AdultABSTRACT
INTRODUCTION: The synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome is a rare condition. Its treatment remains a challenge for clinicians, and often yields mixed results. CASE: We report the case of a 51-year-old Caucasian woman who presented with SAPHO syndrome with mainly axial involvement. She had been treated with sulfasalazine and anti-inflammatory drugs for many years without any success. A few weeks after starting treatment with tofacitinib, both clinical and biological parameters dramatically improved. Imaging also showed considerable regression of the vertebral and pelvic lesions. However, tofacitinib had to be discontinued due to the occurrence of pulmonary embolism. Consequently, recurrence of bone pain and biologic inflammation was rapidly observed. CONCLUSIONS: Anti-JAKs are an interesting treatment option in the management of SAPHO syndrome that need further clinical trials and assessment for validating response.
Subject(s)
Acne Vulgaris , Acquired Hyperostosis Syndrome , Hyperostosis , Osteitis , Piperidines , Pyrimidines , Synovitis , Female , Humans , Middle Aged , Acquired Hyperostosis Syndrome/diagnosis , Acquired Hyperostosis Syndrome/drug therapySubject(s)
Acquired Hyperostosis Syndrome , Piperidines , Pyrimidines , Pyrroles , Humans , Piperidines/therapeutic use , Pyrimidines/therapeutic use , Acquired Hyperostosis Syndrome/drug therapy , Acquired Hyperostosis Syndrome/diagnosis , Pyrroles/therapeutic use , Protein Kinase Inhibitors/therapeutic use , Female , Treatment Outcome , Middle Aged , Male , AdultABSTRACT
BACKGROUND: SAPHO syndrome is recognized as a rare entity with damage to skin and bones due to inflammation. Currently, the treatment for SAPHO syndrome is still a challenge in clinical practice. In this study, an integrated transcriptomics and network pharmacology approach was applied to explore the therapeutic effect and mechanism of Wang-Bi tablet (WBT) on SAPHO syndrome. METHODS: The main components of WBT and their targets, as well as the targets of SAPHO syndrome, were collected from databases. Network visualization was performed using Cytoscape software. The GO and KEGG enrichment analysis was executed by David dataset. Then, the molecular mechanism of WBT improving SAPHO syndrome was validated by transcriptomics of peripheral blood neutrophils in SAPHO syndrome. Finally, the above results were validated by molecular docking. RESULTS: The Network Pharmacology results showed there are 152 core targets for WBT treatment on SAPHO syndrome. RNA-seq data showed 442 differentially expressed genes (DEGs) in peripheral blood neutrophils of SAPHO patients. Intriguingly, NIK/NF-kappaB-, MyD88-dependent toll-like receptor-, and MAPK pathway were included in the enrichment results of network pharmacology and RNA-seq. Moreover, we verified that the core components of WBT have good affinity with the core targets of NIK/NF-kappaB-, MyD88-dependent toll-like receptor-, and MAPK pathway by molecular docking. CONCLUSIONS: This study illustrated that the possible mechanisms of WBT against SAPHO syndrome may be related to NIK/NF-kappaB-, MyD88-dependent toll-like receptor-, and MAPK pathway, and further experiments are needed to prove these predictions.
