Assuntos
Síndrome de Hiperostose Adquirida , Neurite Óptica , Humanos , Neurite Óptica/diagnóstico , Neurite Óptica/tratamento farmacológico , Neurite Óptica/etiologia , Resultado do Tratamento , Síndrome de Hiperostose Adquirida/diagnóstico , Síndrome de Hiperostose Adquirida/complicações , Síndrome de Hiperostose Adquirida/tratamento farmacológico , Feminino , Adulto , Glucocorticoides/uso terapêuticoRESUMO
SAPHO syndrome is a complex inflammatory disorder affecting the skin and bones, characterized by osteomyelitis, acne, and pustulosis. Cytokines play a pivotal role in the pathogenesis of SAPHO syndrome, especially in inflammatory responses and immune regulation. This article reviews the cytokines involved in the pathogenesis of SAPHO syndrome, such as tumor necrosis factor α (TNF-α), interleukin 1ß (IL-1ß), IL-6, IL-10, and transforming growth factor-ß (TGF-ß), and discusses their potential as intervention points for treatment. These findings elucidate the intricate immune regulatory network of SAPHO syndrome and provide a theoretical foundation for the development of new targeted therapeutic strategies.
Assuntos
Síndrome de Hiperostose Adquirida , Citocinas , Síndrome de Hiperostose Adquirida/imunologia , Humanos , Citocinas/metabolismo , AnimaisAssuntos
Síndrome de Hiperostose Adquirida , Esterno , Humanos , Esterno/lesões , Esterno/diagnóstico por imagem , Síndrome de Hiperostose Adquirida/diagnóstico , Síndrome de Hiperostose Adquirida/complicações , Resultado do Tratamento , Feminino , Pessoa de Meia-Idade , Tomografia Computadorizada por Raios X , MasculinoAssuntos
Acne Vulgar , Síndrome de Hiperostose Adquirida , Doenças Mandibulares , Osteomielite , Humanos , Acne Vulgar/complicações , Doenças Mandibulares/patologia , Doenças Mandibulares/complicações , Doenças Mandibulares/diagnóstico , Osteomielite/complicações , Osteomielite/diagnóstico , Síndrome de Hiperostose Adquirida/complicações , Síndrome de Hiperostose Adquirida/diagnóstico , Masculino , Esclerose , Feminino , Sinovite/complicações , Sinovite/diagnósticoAssuntos
Síndrome de Hiperostose Adquirida , Inibidores de Janus Quinases , Humanos , Inibidores de Janus Quinases/uso terapêutico , Síndrome de Hiperostose Adquirida/tratamento farmacológico , Síndrome de Hiperostose Adquirida/diagnóstico , Resultado do Tratamento , Feminino , Pessoa de Meia-Idade , Masculino , Pirimidinas/uso terapêutico , Adulto , PiperidinasRESUMO
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.
Assuntos
Síndrome de Hiperostose Adquirida , Tripterygium , Humanos , Síndrome de Hiperostose Adquirida/tratamento farmacológico , Feminino , Adulto , Inibidores de Janus Quinases/uso terapêutico , Recidiva , Pessoa de Meia-Idade , Extratos Vegetais/uso terapêutico , Resultado do Tratamento , MasculinoAssuntos
Síndrome de Hiperostose Adquirida , Poliarterite Nodosa , Talidomida , Humanos , Talidomida/uso terapêutico , Talidomida/análogos & derivados , Poliarterite Nodosa/tratamento farmacológico , Poliarterite Nodosa/complicações , Síndrome de Hiperostose Adquirida/tratamento farmacológico , Síndrome de Hiperostose Adquirida/complicações , Feminino , Anti-Inflamatórios não Esteroides/uso terapêutico , Resultado do Tratamento , Pessoa de Meia-IdadeAssuntos
Síndrome de Hiperostose Adquirida , Clavícula , Vértebras Lombares , Humanos , Vértebras Lombares/diagnóstico por imagem , Clavícula/lesões , Clavícula/diagnóstico por imagem , Síndrome de Hiperostose Adquirida/diagnóstico , Feminino , Resultado do Tratamento , Fraturas Ósseas/etiologia , Fraturas Ósseas/diagnóstico por imagem , Pessoa de Meia-IdadeAssuntos
Síndrome de Hiperostose Adquirida , Síndrome do Desfiladeiro Torácico , Humanos , Síndrome do Desfiladeiro Torácico/etiologia , Síndrome do Desfiladeiro Torácico/diagnóstico , Síndrome de Hiperostose Adquirida/complicações , Síndrome de Hiperostose Adquirida/diagnóstico , Resultado do Tratamento , Feminino , Pessoa de Meia-IdadeAssuntos
Síndrome de Hiperostose Adquirida , Piperidinas , Pirimidinas , Humanos , Feminino , Gravidez , Pirimidinas/uso terapêutico , Pirimidinas/efeitos adversos , Piperidinas/uso terapêutico , Adulto , Síndrome de Hiperostose Adquirida/tratamento farmacológico , Síndrome de Hiperostose Adquirida/diagnóstico , Resultado do Tratamento , Inibidores de Proteínas Quinases/uso terapêutico , Inibidores de Proteínas Quinases/efeitos adversos , Inibidores de Janus Quinases/uso terapêutico , Nascido Vivo , Pirróis/uso terapêutico , Pirróis/efeitos adversosAssuntos
Síndrome de Hiperostose Adquirida , Vacinas contra COVID-19 , COVID-19 , Humanos , Pessoa de Meia-Idade , Síndrome de Hiperostose Adquirida/diagnóstico , COVID-19/prevenção & controle , Vacinas contra COVID-19/efeitos adversos , SARS-CoV-2/imunologia , Resultado do Tratamento , Vacinação/efeitos adversosAssuntos
Síndrome de Hiperostose Adquirida , Hipertensão Arterial Pulmonar , Humanos , Síndrome de Hiperostose Adquirida/diagnóstico , Síndrome de Hiperostose Adquirida/complicações , Síndrome de Hiperostose Adquirida/tratamento farmacológico , Hipertensão Arterial Pulmonar/etiologia , Hipertensão Arterial Pulmonar/diagnóstico , Hipertensão Arterial Pulmonar/tratamento farmacológico , Hipertensão Arterial Pulmonar/fisiopatologia , Resultado do Tratamento , Feminino , Artéria Pulmonar/diagnóstico por imagem , Anti-Hipertensivos/uso terapêutico , Pressão Arterial/efeitos dos fármacos , Pessoa de Meia-IdadeRESUMO
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.
Assuntos
Síndrome de Hiperostose Adquirida , Fêmur , Humanos , Masculino , Adulto , Síndrome de Hiperostose Adquirida/diagnóstico , Síndrome de Hiperostose Adquirida/complicações , Síndrome de Hiperostose Adquirida/tratamento farmacológico , Fêmur/patologia , Fêmur/diagnóstico por imagem , Resultado do Tratamento , Metotrexato/uso terapêutico , Metotrexato/administração & dosagemAssuntos
Síndrome de Hiperostose Adquirida , Neoplasias Ósseas , Osteíte , Osteossarcoma , Humanos , Síndrome de Hiperostose Adquirida/diagnóstico , Osteossarcoma/diagnóstico , Diagnóstico Diferencial , Osteíte/diagnóstico por imagem , Osteíte/diagnóstico , Neoplasias Ósseas/diagnóstico , Feminino , AdultoAssuntos
Síndrome de Hiperostose Adquirida , Humanos , Estudos Retrospectivos , Síndrome de Hiperostose Adquirida/tratamento farmacológico , Síndrome de Hiperostose Adquirida/diagnóstico , Síndrome de Hiperostose Adquirida/terapia , Feminino , Masculino , Resultado do Tratamento , Adulto , Pessoa de Meia-Idade , Antirreumáticos/uso terapêuticoRESUMO
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.
Assuntos
Síndrome de Hiperostose Adquirida , Humanos , Síndrome de Hiperostose Adquirida/diagnóstico por imagem , Cintilografia , Osso e Ossos/patologia , Radiografia , Coluna Vertebral/patologiaAssuntos
Acne Vulgar , Síndrome de Hiperostose Adquirida , Hiperostose , Osteíte , Sinovite , Humanos , Síndrome de Hiperostose Adquirida/complicações , Síndrome de Hiperostose Adquirida/diagnóstico , Síndrome de Hiperostose Adquirida/tratamento farmacológico , Acne Vulgar/diagnóstico , Acne Vulgar/tratamento farmacológicoAssuntos
Acne Vulgar , Síndrome de Hiperostose Adquirida , Osteíte , Osteomielite , Sinovite , Humanos , Síndrome de Hiperostose Adquirida/complicações , Síndrome de Hiperostose Adquirida/diagnóstico , Síndrome de Hiperostose Adquirida/tratamento farmacológico , Osteíte/diagnóstico por imagem , Osteíte/tratamento farmacológicoRESUMO
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.