SAPHO syndrome with a pathological fracture of clavicle: a case report

SAPHO syndrome with a pathological fracture of clavicle: a case report | Research Square window.SnipcartSettings = { analytics: { enabled: false } }; (function() { var accessVector = localStorage.getItem('access_vector') || ''; window.dataLayer = window.dataLayer || []; if (accessVector) { window.dataLayer.push({ user: { profile: { profileInfo: { snid: accessVector } } } }); } })(); (function(w,d,s,l,i){w[l]=w[l]||[];w[l].push({'gtm.start':new Date().getTime(),event:'gtm.js'});var f=d.getElementsByTagName(s)[0],j=d.createElement(s),dl=l!='dataLayer'?'&l='+l:'';j.async=true;j.src='https://www.googletagmanager.com/gtm.js?id='+i+dl;f.parentNode.insertBefore(j,f);})(window,document,'script','dataLayer','GTM-K279D39R'); Browse Preprints In Review Journals COVID-19 Preprints AJE Video Bytes Research Tools Research Promotion AJE Professional Editing AJE Rubriq About Preprint Platform In Review Editorial Policies Our Team Advisory Board Help Center Sign In Submit a Preprint Cite Share Download PDF Case Report SAPHO syndrome with a pathological fracture of clavicle: a case report WeiJie Shao, Zhipeng Wu, Xinyang Shou, Xinzheng Zhao, Haojing Zhou, and 3 more This is a preprint; it has not been peer reviewed by a journal. https://doi.org/ 10.21203/rs.3.rs-3857085/v1 This work is licensed under a CC BY 4.0 License Status: Posted Version 1 posted You are reading this latest preprint version Abstract Background Synovitis-acne-pustulosis-hyperostosis-osteomyelitis (SAPHO) syndrome is a rare disease characterized by combined osteoarticular and cutaneous manifestations. The diagnosis of SAPHO syndrome remains difficult due to its clinical heterogeneity and often requires the identification of infectious neoplasm and other bone abnormalities. Surgical treatment of pathological fractures caused by SAPHO syndrome is extremely rare. We report the first surgical case of a pathological clavicle fracture with SAPHO syndrome. Case presentation: A 53-year-old male patient came to our Orthopedic Surgery Department in 2022, complaining of right shoulder pain for 4 months, aggravated with activity limitation for 3 days. And the patient presented with cutaneous lesions on the right upper extremity. Based on the clinical manifestations, imaging findings, bone scintigraphy findings and bone puncture pathology, the diagnosis of SAPHO syndrome was made. Notably, the patient also suffered pathological fracture of the ipsilateral clavicle. We treated the clavicle with open reduction and internal fixation and found satisfactory results at the subsequent 6-month follow-up. Conclusions This case shows the efficacy and safety of open reduction and internal fixation in the treatment of pathological fractures caused by SAPHO syndrome. We hope to provide valuable information regarding the treatment of SAPHO syndrome in this case which makes clinical data more comprehensive. SAPHO syndrome clavicle pathological fracture surgical treatment case report Figures Figure 1 Figure 2 Figure 3 Figure 4 Figure 5 Figure 6 Background Synovitis-acne-pustulosis-hyperostosis-osteomyelitis (SAPHO) syndrome is a rare disease which is difficult to diagnose due to the diversity of clinical manifestations as well as the insidious nature of onset. Frequently, osteoarticular disorders and osteoarthropathy simultaneous occurrence is infrequent, although they are described as SAPHO's primary expression( 1 ). It is necessary to meet at least one of the following three diagnosis principles: ( 1 ) Chronic recurrent multifocal sterile and axial osteomyelitis, with or without dermatosis; ( 2 ) Acute, subacute, or chronic arthritis associated with palmoplantar pustulosis, pustular psoriasis, or severe acne; ( 3 ) Any sterile osteitis associated with palmoplantar pustulosis, pustular psoriasis, or severe acne( 2 , 3 ). In addition, there are no authoritative clinical treatment guidelines for SAPHO, neither oral medication nor surgical treatment. The oral medication treatment mainly includes nonsteroidal anti-inflammatory drugs, antirheumatic drugs, corticosteroids, bisphosphonates, biologics and antibiotics. Surgical treatment required SAPHO syndrome are rarely reported. Some studies even suggest surgical treatment should be avoided( 4 ).This study reported a patient diagnosed with SAPHO syndrome with pathological fracture of the clavicle and successfully received surgical treatment. Case presentation A 53-year-old male complained of the right shoulder pain for 4 months, aggravated with activity limitation for 3 days without any clear predisposing cause. He took analgesic medicine (an unknown analgesic) himself but ineffective. Physical examinations on admission revealed pustules on the palms and multi-erythematous nodules on the lower legs. There was also redness, swelling and tenderness in the right sternoclavicular joint area and acromioclavicular joint. Meanwhile, right shoulder forward bends, outreach and extorsion activities significantly limited and right upper limb skin visible subcutaneous ecchymosis. Four months ago, the patient was admitted to another hospital for right shoulder pain and underwent a pathological puncture of the right clavicle for further diagnosis. The pathological puncture revealed calcification of bone tissue in the right clavicle, hyperplasia of fibrous tissue in the intertrabecular bone, proliferation of osteoblasts around the trabecular bone, and accumulation of dead bone, focal hemorrhage and plasma cells infiltration. The clavicle radiographs a month ago at our hospital suggested the possibility of bilateral chronic clavicular osteomyelitis and pathological fracture of the right clavicle. This patient's right shoulder pain got worse 3 days before admission of our hospital, due to heavy lifting. A more in-depth patient history revealed that he had no alcohol or addictive drug use history, but did have the tobacco use history for 30 years (about 20 cigarettes per day). He also had no infectious diseases, inflammatory arthritis, malignancy or genetic disease history. Laboratory assays were as follows: C-reactive Protein 24.70 mg/L (normal range 0.00–8.00 mg/L); Hemoglobin 120 g/L (normal range 130–175 g/L); Fibrinogen 5.43 g/L (2.00–4.00 g/L); D-Dimer 0.90 mg/L FEU (normal range 0.00-0.55 mg/L FEU); Osteocalcin 28.05 µg/L (normal range 6.00-24.66 µg/L); Serum tumor markers were normal. X-rays of the right clavicle showed expansive bone changes near the interrupted right clavicle, with uneven internal density and multiple low-density shadows (Fig. 1 ). CT scan and three-dimensional reconstruction of the sacroiliac joint showed mild bone hyperplasia at the edge of bilateral sacroiliac joints, normal joint space, local roughness of the joint surface, and no obvious abnormal density in the surrounding soft tissue (Fig. 2 ). Bone scintigraphy revealed that the tracer distribution was concentrated in the double clavicle, sternum, double first to third anterior ribs and pubic symphysis. The tracer distribution was slightly concentrated in bilateral scapula, right 6th posterior rib, thoracic vertebra 4th, 11th, right femur, right sacroiliac joint, right shoulder joint, and left ischium. The other bones did not show abnormal concentration or defect of tracer distribution. The spine was slightly curved. The remaining scan was normal (Fig. 3 ). The aforementioned findings, including skin manifestations, imaging features, and pathological puncture results, indicated that the patient likely experienced a pathologic fracture of the right clavicle attributed to SAPHO syndrome. A 12 cm long incision was made in front of the right clavicle, after preoperative contraindications were excluded. The skin and subcutaneous tissue were cut open and the fracture was exposed by retracting. During the surgical procedure, there was observed swelling of the right clavicle along with a mid-segment fracture and partial displacement of the fractured end, accompanied by firm adjacent soft tissue and heightened inflammatory hyperplasia (Fig. 4 ). The midsection of the right clavicle was slated, and the inflammatory tissue in the medullary cavity was removed with a scraping scoop (Fig. 4 ). Intraoperative tissues were sent for pathological examination. The intraoperative frozen section pathology showed fibromatous hyperplasia of the right clavicle and new bone trabecula formation accompanied by chronic inflammatory cell infiltration (Fig. 5 ). The artificial bone was pressed into the medullary cavity for bone grafting. And a bone block about 3 cm × 1 cm long was taken from the right iliac bone. Structural bone grafting was performed at the right clavicle slot after the bone block was pruning (Fig. 4 ). Plates of appropriate length were fixed with 6 screws on the right clavicle. The operation was performed without any intraoperative challenges. The immunohistochemical results have shown the evidence of broken bone with hemorrhage, necrosis, inflammatory cell infiltration, and fibrous tissue hyperplasia. Bone marrow histopathology revealed proliferation of immature braided bone and spindle cells (Fig. 5 ). Antibiotics were administered for 24h (Cefuroxime sodium 1.5g i.v.g.t.t bid) to prevent infection. The patient recovered appropriately post-operatively and the plain radiograph showed internal fixation devices were in good position and alignment (Fig. 6 ). The wound healed well after surgery and no major local or systemic complication was recorded during the 6-month follow-up. Nonsteroidal anti-inflammatory drugs (NSAIDS) were stopped at 1 week after surgery while pain visual analogue scale (VAS) was 2/10 at 2 weeks, 0/10 at 3 months. The patient regained full active and passive range of motion (ROM) all planes at 4 months. X-rays imaging at 6 months after surgery showed fracture healing with callus formation (Fig. 6 ). Discussion SAPHO syndrome is a chronic aseptic inflammatory disease characterized by skin and bone involvement. SAPHO syndrome has a high rate of misdiagnosis and missed diagnosis during clinical practice. The pathogenesis of SAPHO syndrome has not been clear yet. Immune dysfunction, microbial infection, and genetic factors may all contribute to SAPHO syndrome( 5 , 6 ). In this case, we also did not find the cause of SAPHO syndrome in this patient. SAPHO syndrome diagnosis is extremely challenging and usually requires an complexing exclusive diagnosis( 1 , 7 ). With previous clavicular biopsy results, we basically ruled out the possibility of chronic bacterial osteomyelitis, which usually developed from acute osteomyelitis with a history of trauma or surgery or the presence of an vivo infection ( 8 , 9 ). This patient had no similar medical use history or any bacteria though the puncture. Codman triangle was not found in the imaging of this middle-aged patient, neither. Tumor cells did not existed in the biopsy. We essentially excluded Ewing sarcoma and bone tumors. Bone scintigraphy was performed ultimately, which revealed the “bullhead sign” with the manubrium sterni representing the upper skull and the inflamed sternoclavicular joint with the double claviculae forming the horns. Increased multiple tracer uptake in the right sacroiliac joint, pubic union, and ribs was also highly suspected to be caused by SAPHO syndrome. Meanwhile, some studies ( 1 , 10 ) show that the histological progression of the disease presents in bone remodeling and chronic inflammation in the advanced stage. Bone remodeling and chronic inflammation lead to trabecular bone sclerosis and significant myelofibrosis, and the histopathology of this patient fits this description( 11 ). Clinical presentation, imaging findings, and histopathology analysis ultimately support the diagnosis of SAPHO syndrome. SAPHO syndrome causes the change of bone density and the increase of bone fragility, while lifting heavy objects as indirect violence leads to pathological fracture. There was no treatment or diagnostic consensus about SAPHO syndrome published. NSAIDs, disease-modifying anti-rheumatic drugs and bisphosphonates were the first line of treatment for patients with SAPHO syndrome( 12 ). TNF blockers, IL-1 inhibition, Tripterygium-wilfordii-Hook-F and small molecule compounds, such as JAK inhibitors and phosphodiesterase 4 inhibitors can also play a certain role( 13 ). The treatment of SAPHO syndrome combined with clavicle fractures has been reported. Miyamoto et al( 14 ) mentioned a case of midshaft clavicle fracture associated with osteogenesis of sternum and clavicle. The patient underwent conservative treatment of the clavicle band for 6 weeks and the fracture healed successfully. Kuroda et al( 15 ) also reported a similar case and the fracture heal. However, we retrieved no cases of surgical treatment. Nonetheless, the patient was going through severe pain, limited mobility, and had a strong willingness to receive surgical treatment. We also believe that strong internal fixation can have a good effect and therefore arrange surgery for this patient. There was no local or systemic inflammatory reaction in the patient postoperatively, nor nonunion or malunion of the fracture, which indicated that internal fixation was safe and reliable. To the best of our knowledge, this is the first surgical treatment case of SAPHO syndrome with clavicle pathological fracture. During the operation, the using of autologous iliac bone for bone grafting avoids rejection. Simultaneously, we cleared the visible inflammatory tissue as much as possible. The clearance of inflamed tissue was required. Cheng et al( 16 ) used percutaneous vertebroplasty for SAPHO syndrome with vertebral destruction, where the heat generated during PVP can destroy inflammatory factors. Yeo et al( 17 ) also thoroughly removed the inflamed tissue during a total hip replacement in patients with SAPHO syndrome. As an independent case, it can enrich our knowledge about SAPHO syndrome, especially in terms of treatment. Internal fixation operation met our expectations but more cases are needed to support its safety and efficacy. Conclusion Patients with SAPHO syndrome require more defined diagnostic process as well as individualized treatment modalities. Invasive surgery is not an absolute contraindication of SAPHO syndrome. For SAPHO syndrome patients with combined fractures, internal fixation therapy may be considered. Abbreviations SAPHO Synovitis-acne-pustulosis-hyperostosis-osteomyelitis Ivgtt intravenously guttae BID Twice a day NSAID Nonsteroidal anti-inflammatory drugs VAS Visual analogue scale CT Computed Tomography ROM Range of motion TNF Tumor necrosis factor IL-1 Interleukin-1 JAK Janus kinase Declarations Acknowledgements The authors would like to thank the participating patient and colleagues who made this work possible. Funding Not applicable. Availability of data and materials Data sharing is not applicable to this case report as no datasets were generated or analyzed during the current study. Authors’ contributions WS reviewed the literature and drafted the manuscript. ZW and PT decided about final diagnosis and operated on the patient. XZ, HZ, HS participated in the follow-up of the patient. XS and HP revised the paper. All authors read and approved the final manuscript. Ethics approval and consent to participate The need for approval was waived by the institutional ethics of the First Affiliated Hospital of Zhejiang Traditional Chinese Medicine University for using medical records obtained from previous clinical diagnosis and treatment. Consent for publication A written informed consent was obtained from the patient. Competing interests The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. References Zimmermann P, Curtis N. Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome - A challenging diagnosis not to be missed. J Infect. 2016;72(Suppl):106–S14. Kahn MF, Khan MA. The SAPHO syndrome. Baillieres Clin Rheumatol. 1994;8(2):333–62. Freira S, Fonseca H, Ferreira PD, Vasconcelos P, Fonseca JE. SAPHO syndrome in an adolescent: a clinical case with unusual severe systemic impact. J Adolesc Health. 2014;55(2):304–6. Müller-Rath R, Mumme T, Zilkens K, Spüntrup E. Isolierte, bilaterale sternokostoklavikuläre Hyperostose (SCCH) mit pathologischer Klavikulafraktur - Ein Verlauf über 10 Jahre. Z Orthop Grenzgeb. 2005;143(06):691–3. Depasquale R, Kumar N, Lalam RK, Tins BJ, Tyrrell PNM, Singh J, et al. SAPHO: What radiologists should know. Clin Radiol. 2012;67(3):195–206. Leone A, Cassar-Pullicino VN, Casale R, Magarelli N, Semprini A, Colosimo C. The SAPHO syndrome revisited with an emphasis on spinal manifestations. Skeletal Radiol. 2015;44(1). Cianci F, Zoli A, Gremese E, Ferraccioli G. Clinical heterogeneity of SAPHO syndrome: challenging diagnose and treatment. Clin Rheumatol. 2017;36(9):2151–8. Bury DC, Rogers TS, Dickman MM. Osteomyelitis: Diagnosis and Treatment. Am Fam Physician. 2021;104(4):395–402. King SM, Laxer RM, Manson D, Gold R. Chronic recurrent multifocal osteomyelitis: a noninfectious inflammatory process. Pediatr Infect Dis J. 1987;6(10):907–11. Reith JD, Bauer TW, Schils JP. Osseous Manifestations of SAPHO (Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis) Syndrome. Am J Surg Pathol. 1996;20(11). Qiu C, Cheng L, Hou H, Liu T, Xu B, Xiao X, et al. Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome of femoral neoplasm-like onset: a case-based review. J Int Med Res. 2021;49(12):3000605211065314. Huang H, Zhang Z, Zhao J, Hao Y, Zhou W. The effectiveness of treatments for patients with SAPHO syndrome: a follow-up study of 24 cases from a single center and review of literature. Clin Rheumatol. 2021;40(3):1131–9. Cheng W, Li F, Tian J, Xie X, Chen JW, Peng XF, et al. New Insights in the Treatment of SAPHO Syndrome and Medication Recommendations. J Inflamm Res. 2022;15:2365–80. Miyamoto S, Otsuka M, Hasue F, Fujiyoshi T, Kamiya K, Kiuchi H, et al. Stress fracture of the midshaft clavicle associated with sternocostoclavicular hyperostosis-Case report. Int J Surg Case Rep. 2019;58:121–6. Kuroda T, Ehara S, Murakami H. Stress fracture of the clavicle associated with sternocostoclavicular hyperostosis. Skeletal Radiol. 2005;34(7):424–6. Cheng Y, Feng H, Mu J, Chen J, Wu H. Percutaneous vertebroplasty for SAPHO syndrome with vertebral destruction: a case report and literature review. Front Med (Lausanne). 2023;10:1175787. Yeo I, Cha HS, Yoon YC, Park YS, Lim SJ. A case report of severely damaged hip joint caused by SAPHO syndrome treated with 2-stage total hip arthroplasty. Med (Baltim). 2016;95(27):e4168. Additional Declarations No competing interests reported. Cite Share Download PDF Status: Posted Version 1 posted You are reading this latest preprint version Research Square lets you share your work early, gain feedback from the community, and start making changes to your manuscript prior to peer review in a journal. As a division of Research Square Company, we’re committed to making research communication faster, fairer, and more useful. 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Also discoverable on Platform About Our Team In Review Editorial Policies Advisory Board Help Center Resources Author Services Accessibility API Access RSS feed Manage Cookie Preferences © Research Square 2026 | ISSN 2693-5015 (online) Privacy Policy Terms of Service Do Not Sell My Personal Information {"props":{"pageProps":{"initialData":{"identity":"rs-3857085","acceptedTermsAndConditions":true,"allowDirectSubmit":true,"archivedVersions":[],"articleType":"Case Report","associatedPublications":[],"authors":[{"id":268380125,"identity":"ddeaf750-a21b-474f-afbf-33c98b6ebb39","order_by":0,"name":"WeiJie Shao","email":"","orcid":"","institution":"Zhejiang Chinese Medical University","correspondingAuthor":false,"prefix":"","firstName":"WeiJie","middleName":"","lastName":"Shao","suffix":""},{"id":268380126,"identity":"5e9b07c5-292f-4170-b5c7-c17054f927da","order_by":1,"name":"Zhipeng Wu","email":"","orcid":"","institution":"The First Afliated Hospital of Zhejiang Chinese Medical 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2","display":"","copyAsset":false,"role":"figure","size":469545,"visible":true,"origin":"","legend":"\u003cp\u003eSacroiliac joint CT scan and three-dimensional reconstruction examination shows bilateral sacroiliac joint hyperplasia\u003c/p\u003e","description":"","filename":"Figure2.png","url":"https://assets-eu.researchsquare.com/files/rs-3857085/v1/fa2796f450dddfe86f0e118b.png"},{"id":50042244,"identity":"bbad5fe1-fd71-4764-81d5-399d9e87e9d6","added_by":"auto","created_at":"2024-01-23 15:45:41","extension":"png","order_by":3,"title":"Figure 3","display":"","copyAsset":false,"role":"figure","size":274280,"visible":true,"origin":"","legend":"\u003cp\u003eBone scintigraphy demonstrated increased tracer uptake in the double clavicle, sternum, double 1-3th anterior ribs and pubic symphysis\u003c/p\u003e","description":"","filename":"Figure3.png","url":"https://assets-eu.researchsquare.com/files/rs-3857085/v1/3e2bf133be925a13ab88ad3c.png"},{"id":50046520,"identity":"5c7c1ee0-6695-44df-b347-b8aaab108b7f","added_by":"auto","created_at":"2024-01-23 16:01:40","extension":"png","order_by":4,"title":"Figure 4","display":"","copyAsset":false,"role":"figure","size":1319655,"visible":true,"origin":"","legend":"\u003cp\u003ePicture of intraoperative operation: \u003cstrong\u003ea \u003c/strong\u003eInflammatory tissue hyperplasia of right clavicle fracture,\u003cstrong\u003e b \u003c/strong\u003eRight collarbone fracture block,\u003cstrong\u003e c \u003c/strong\u003eRemove inflammatory tissue from the medullary cavity,\u003cstrong\u003e d\u003c/strong\u003e bone grafting\u003c/p\u003e","description":"","filename":"Figure4.png","url":"https://assets-eu.researchsquare.com/files/rs-3857085/v1/2e7c064c45dd9796f0455397.png"},{"id":50044578,"identity":"1c782a82-6d54-4175-977c-56648566e277","added_by":"auto","created_at":"2024-01-23 15:53:40","extension":"png","order_by":5,"title":"Figure 5","display":"","copyAsset":false,"role":"figure","size":670242,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cstrong\u003ea \u003c/strong\u003eIntraoperative frozen section pathology revealed fibromatous hyperplasia and new trabecular bone with chronic inflammatory cell infiltration and \u003cstrong\u003eb \u003c/strong\u003eImmunohistochemical results（H\u0026amp;E, 400×）\u003c/p\u003e","description":"","filename":"Figure5.png","url":"https://assets-eu.researchsquare.com/files/rs-3857085/v1/ce21228f3a933408fd3c5fa8.png"},{"id":50044580,"identity":"4194897c-c134-40d7-a897-65ad08defc17","added_by":"auto","created_at":"2024-01-23 15:53:41","extension":"png","order_by":6,"title":"Figure 6","display":"","copyAsset":false,"role":"figure","size":207450,"visible":true,"origin":"","legend":"\u003cp\u003e\u003cstrong\u003ea \u003c/strong\u003ePost-operative X-rays imaging and \u003cstrong\u003eb \u003c/strong\u003eX-rays Imaging of the right clavicle at 6 months after surgery\u003c/p\u003e","description":"","filename":"Figure6.png","url":"https://assets-eu.researchsquare.com/files/rs-3857085/v1/65113a3cb172985284fa4e58.png"},{"id":53996946,"identity":"b9f3261a-ffa0-403d-92cc-fceb7c726598","added_by":"auto","created_at":"2024-04-03 07:26:24","extension":"pdf","order_by":0,"title":"","display":"","copyAsset":false,"role":"manuscript-pdf","size":4948025,"visible":true,"origin":"","legend":"","description":"","filename":"manuscript.pdf","url":"https://assets-eu.researchsquare.com/files/rs-3857085/v1/9a28ef96-0c7d-4407-9c63-a1fd7e1579e8.pdf"}],"financialInterests":"No competing interests reported.","formattedTitle":"SAPHO syndrome with a pathological fracture of clavicle: a case report","fulltext":[{"header":"Background","content":"\u003cp\u003eSynovitis-acne-pustulosis-hyperostosis-osteomyelitis (SAPHO) syndrome is a rare disease which is difficult to diagnose due to the diversity of clinical manifestations as well as the insidious nature of onset. Frequently, osteoarticular disorders and osteoarthropathy simultaneous occurrence is infrequent, although they are described as SAPHO's primary expression(\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e). It is necessary to meet at least one of the following three diagnosis principles: (\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e) Chronic recurrent multifocal sterile and axial osteomyelitis, with or without dermatosis; (\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e) Acute, subacute, or chronic arthritis associated with palmoplantar pustulosis, pustular psoriasis, or severe acne; (\u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e) Any sterile osteitis associated with palmoplantar pustulosis, pustular psoriasis, or severe acne(\u003cspan citationid=\"CR2\" class=\"CitationRef\"\u003e2\u003c/span\u003e, \u003cspan citationid=\"CR3\" class=\"CitationRef\"\u003e3\u003c/span\u003e). In addition, there are no authoritative clinical treatment guidelines for SAPHO, neither oral medication nor surgical treatment. The oral medication treatment mainly includes nonsteroidal anti-inflammatory drugs, antirheumatic drugs, corticosteroids, bisphosphonates, biologics and antibiotics. Surgical treatment required SAPHO syndrome are rarely reported. Some studies even suggest surgical treatment should be avoided(\u003cspan citationid=\"CR4\" class=\"CitationRef\"\u003e4\u003c/span\u003e).This study reported a patient diagnosed with SAPHO syndrome with pathological fracture of the clavicle and successfully received surgical treatment.\u003c/p\u003e"},{"header":"Case presentation","content":"\u003cp\u003eA 53-year-old male complained of the right shoulder pain for 4 months, aggravated with activity limitation for 3 days without any clear predisposing cause. He took analgesic medicine (an unknown analgesic) himself but ineffective. Physical examinations on admission revealed pustules on the palms and multi-erythematous nodules on the lower legs. There was also redness, swelling and tenderness in the right sternoclavicular joint area and acromioclavicular joint. Meanwhile, right shoulder forward bends, outreach and extorsion activities significantly limited and right upper limb skin visible subcutaneous ecchymosis. Four months ago, the patient was admitted to another hospital for right shoulder pain and underwent a pathological puncture of the right clavicle for further diagnosis. The pathological puncture revealed calcification of bone tissue in the right clavicle, hyperplasia of fibrous tissue in the intertrabecular bone, proliferation of osteoblasts around the trabecular bone, and accumulation of dead bone, focal hemorrhage and plasma cells infiltration. The clavicle radiographs a month ago at our hospital suggested the possibility of bilateral chronic clavicular osteomyelitis and pathological fracture of the right clavicle. This patient's right shoulder pain got worse 3 days before admission of our hospital, due to heavy lifting. A more in-depth patient history revealed that he had no alcohol or addictive drug use history, but did have the tobacco use history for 30 years (about 20 cigarettes per day). He also had no infectious diseases, inflammatory arthritis, malignancy or genetic disease history.\u003c/p\u003e \u003cp\u003eLaboratory assays were as follows: C-reactive Protein 24.70 mg/L (normal range 0.00\u0026ndash;8.00 mg/L); Hemoglobin 120 g/L (normal range 130\u0026ndash;175 g/L); Fibrinogen 5.43 g/L (2.00\u0026ndash;4.00 g/L); D-Dimer 0.90 mg/L FEU (normal range 0.00-0.55 mg/L FEU); Osteocalcin 28.05 \u0026micro;g/L (normal range 6.00-24.66 \u0026micro;g/L); Serum tumor markers were normal. X-rays of the right clavicle showed expansive bone changes near the interrupted right clavicle, with uneven internal density and multiple low-density shadows (Fig.\u0026nbsp;\u003cspan refid=\"Fig1\" class=\"InternalRef\"\u003e1\u003c/span\u003e). CT scan and three-dimensional reconstruction of the sacroiliac joint showed mild bone hyperplasia at the edge of bilateral sacroiliac joints, normal joint space, local roughness of the joint surface, and no obvious abnormal density in the surrounding soft tissue (Fig.\u0026nbsp;\u003cspan refid=\"Fig2\" class=\"InternalRef\"\u003e2\u003c/span\u003e). Bone scintigraphy revealed that the tracer distribution was concentrated in the double clavicle, sternum, double first to third anterior ribs and pubic symphysis. The tracer distribution was slightly concentrated in bilateral scapula, right 6th posterior rib, thoracic vertebra 4th, 11th, right femur, right sacroiliac joint, right shoulder joint, and left ischium. The other bones did not show abnormal concentration or defect of tracer distribution. The spine was slightly curved. The remaining scan was normal (Fig.\u0026nbsp;\u003cspan refid=\"Fig3\" class=\"InternalRef\"\u003e3\u003c/span\u003e). The aforementioned findings, including skin manifestations, imaging features, and pathological puncture results, indicated that the patient likely experienced a pathologic fracture of the right clavicle attributed to SAPHO syndrome.\u003c/p\u003e \u003cp\u003eA 12 cm long incision was made in front of the right clavicle, after preoperative contraindications were excluded. The skin and subcutaneous tissue were cut open and the fracture was exposed by retracting. During the surgical procedure, there was observed swelling of the right clavicle along with a mid-segment fracture and partial displacement of the fractured end, accompanied by firm adjacent soft tissue and heightened inflammatory hyperplasia (Fig.\u0026nbsp;\u003cspan refid=\"Fig4\" class=\"InternalRef\"\u003e4\u003c/span\u003e). The midsection of the right clavicle was slated, and the inflammatory tissue in the medullary cavity was removed with a scraping scoop (Fig.\u0026nbsp;\u003cspan refid=\"Fig4\" class=\"InternalRef\"\u003e4\u003c/span\u003e). Intraoperative tissues were sent for pathological examination. The intraoperative frozen section pathology showed fibromatous hyperplasia of the right clavicle and new bone trabecula formation accompanied by chronic inflammatory cell infiltration (Fig.\u0026nbsp;\u003cspan refid=\"Fig5\" class=\"InternalRef\"\u003e5\u003c/span\u003e). The artificial bone was pressed into the medullary cavity for bone grafting. And a bone block about 3 cm \u0026times; 1 cm long was taken from the right iliac bone. Structural bone grafting was performed at the right clavicle slot after the bone block was pruning (Fig.\u0026nbsp;\u003cspan refid=\"Fig4\" class=\"InternalRef\"\u003e4\u003c/span\u003e).\u003c/p\u003e \u003cp\u003ePlates of appropriate length were fixed with 6 screws on the right clavicle. The operation was performed without any intraoperative challenges. The immunohistochemical results have shown the evidence of broken bone with hemorrhage, necrosis, inflammatory cell infiltration, and fibrous tissue hyperplasia. Bone marrow histopathology revealed proliferation of immature braided bone and spindle cells (Fig.\u0026nbsp;\u003cspan refid=\"Fig5\" class=\"InternalRef\"\u003e5\u003c/span\u003e). Antibiotics were administered for 24h (Cefuroxime sodium 1.5g i.v.g.t.t bid) to prevent infection. The patient recovered appropriately post-operatively and the plain radiograph showed internal fixation devices were in good position and alignment (Fig.\u0026nbsp;\u003cspan refid=\"Fig6\" class=\"InternalRef\"\u003e6\u003c/span\u003e). The wound healed well after surgery and no major local or systemic complication was recorded during the 6-month follow-up. Nonsteroidal anti-inflammatory drugs (NSAIDS) were stopped at 1 week after surgery while pain visual analogue scale (VAS) was 2/10 at 2 weeks, 0/10 at 3 months. The patient regained full active and passive range of motion (ROM) all planes at 4 months. X-rays imaging at 6 months after surgery showed fracture healing with callus formation (Fig.\u0026nbsp;\u003cspan refid=\"Fig6\" class=\"InternalRef\"\u003e6\u003c/span\u003e).\u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003e \u003c/p\u003e \u003cp\u003e \u003c/p\u003e"},{"header":"Discussion","content":"\u003cp\u003eSAPHO syndrome is a chronic aseptic inflammatory disease characterized by skin and bone involvement. SAPHO syndrome has a high rate of misdiagnosis and missed diagnosis during clinical practice. The pathogenesis of SAPHO syndrome has not been clear yet. Immune dysfunction, microbial infection, and genetic factors may all contribute to SAPHO syndrome(\u003cspan citationid=\"CR5\" class=\"CitationRef\"\u003e5\u003c/span\u003e, \u003cspan citationid=\"CR6\" class=\"CitationRef\"\u003e6\u003c/span\u003e). In this case, we also did not find the cause of SAPHO syndrome in this patient.\u003c/p\u003e \u003cp\u003eSAPHO syndrome diagnosis is extremely challenging and usually requires an complexing exclusive diagnosis(\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e, \u003cspan citationid=\"CR7\" class=\"CitationRef\"\u003e7\u003c/span\u003e). With previous clavicular biopsy results, we basically ruled out the possibility of chronic bacterial osteomyelitis, which usually developed from acute osteomyelitis with a history of trauma or surgery or the presence of an vivo infection (\u003cspan citationid=\"CR8\" class=\"CitationRef\"\u003e8\u003c/span\u003e, \u003cspan citationid=\"CR9\" class=\"CitationRef\"\u003e9\u003c/span\u003e). This patient had no similar medical use history or any bacteria though the puncture. Codman triangle was not found in the imaging of this middle-aged patient, neither. Tumor cells did not existed in the biopsy. We essentially excluded Ewing sarcoma and bone tumors. Bone scintigraphy was performed ultimately, which revealed the \u0026ldquo;bullhead sign\u0026rdquo; with the manubrium sterni representing the upper skull and the inflamed sternoclavicular joint with the double claviculae forming the horns. Increased multiple tracer uptake in the right sacroiliac joint, pubic union, and ribs was also highly suspected to be caused by SAPHO syndrome. Meanwhile, some studies (\u003cspan citationid=\"CR1\" class=\"CitationRef\"\u003e1\u003c/span\u003e, \u003cspan citationid=\"CR10\" class=\"CitationRef\"\u003e10\u003c/span\u003e) show that the histological progression of the disease presents in bone remodeling and chronic inflammation in the advanced stage. Bone remodeling and chronic inflammation lead to trabecular bone sclerosis and significant myelofibrosis, and the histopathology of this patient fits this description(\u003cspan citationid=\"CR11\" class=\"CitationRef\"\u003e11\u003c/span\u003e). Clinical presentation, imaging findings, and histopathology analysis ultimately support the diagnosis of SAPHO syndrome. SAPHO syndrome causes the change of bone density and the increase of bone fragility, while lifting heavy objects as indirect violence leads to pathological fracture.\u003c/p\u003e \u003cp\u003eThere was no treatment or diagnostic consensus about SAPHO syndrome published. NSAIDs, disease-modifying anti-rheumatic drugs and bisphosphonates were the first line of treatment for patients with SAPHO syndrome(\u003cspan citationid=\"CR12\" class=\"CitationRef\"\u003e12\u003c/span\u003e). TNF blockers, IL-1 inhibition, Tripterygium-wilfordii-Hook-F and small molecule compounds, such as JAK inhibitors and phosphodiesterase 4 inhibitors can also play a certain role(\u003cspan citationid=\"CR13\" class=\"CitationRef\"\u003e13\u003c/span\u003e). The treatment of SAPHO syndrome combined with clavicle fractures has been reported. Miyamoto et al(\u003cspan citationid=\"CR14\" class=\"CitationRef\"\u003e14\u003c/span\u003e) mentioned a case of midshaft clavicle fracture associated with osteogenesis of sternum and clavicle. The patient underwent conservative treatment of the clavicle band for 6 weeks and the fracture healed successfully. Kuroda et al(\u003cspan citationid=\"CR15\" class=\"CitationRef\"\u003e15\u003c/span\u003e) also reported a similar case and the fracture heal. However, we retrieved no cases of surgical treatment. Nonetheless, the patient was going through severe pain, limited mobility, and had a strong willingness to receive surgical treatment. We also believe that strong internal fixation can have a good effect and therefore arrange surgery for this patient. There was no local or systemic inflammatory reaction in the patient postoperatively, nor nonunion or malunion of the fracture, which indicated that internal fixation was safe and reliable.\u003c/p\u003e \u003cp\u003eTo the best of our knowledge, this is the first surgical treatment case of SAPHO syndrome with clavicle pathological fracture. During the operation, the using of autologous iliac bone for bone grafting avoids rejection. Simultaneously, we cleared the visible inflammatory tissue as much as possible. The clearance of inflamed tissue was required. Cheng et al(\u003cspan citationid=\"CR16\" class=\"CitationRef\"\u003e16\u003c/span\u003e) used percutaneous vertebroplasty for SAPHO syndrome with vertebral destruction, where the heat generated during PVP can destroy inflammatory factors. Yeo et al(\u003cspan citationid=\"CR17\" class=\"CitationRef\"\u003e17\u003c/span\u003e) also thoroughly removed the inflamed tissue during a total hip replacement in patients with SAPHO syndrome.\u003c/p\u003e \u003cp\u003eAs an independent case, it can enrich our knowledge about SAPHO syndrome, especially in terms of treatment. Internal fixation operation met our expectations but more cases are needed to support its safety and efficacy.\u003c/p\u003e"},{"header":"Conclusion","content":"\u003cp\u003ePatients with SAPHO syndrome require more defined diagnostic process as well as individualized treatment modalities. Invasive surgery is not an absolute contraindication of SAPHO syndrome. For SAPHO syndrome patients with combined fractures, internal fixation therapy may be considered.\u003c/p\u003e"},{"header":"Abbreviations","content":"\u003cdiv class=\"DefinitionList\"\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eSAPHO\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003eSynovitis-acne-pustulosis-hyperostosis-osteomyelitis\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eIvgtt\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003eintravenously guttae\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eBID\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003eTwice a day\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eNSAID\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003eNonsteroidal anti-inflammatory drugs\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eVAS\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003eVisual analogue scale\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eCT\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003eComputed Tomography\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eROM\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003eRange of motion\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eTNF\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003eTumor necrosis factor\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eIL-1\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003eInterleukin-1\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003cdiv class=\"DefinitionListEntry\"\u003e \u003cdiv class=\"Term\"\u003eJAK\u003c/div\u003e \u003cdiv class=\"Description\"\u003e \u003cp\u003eJanus kinase\u003c/p\u003e \u003c/div\u003e \u003c/div\u003e \u003c/div\u003e"},{"header":"Declarations","content":"\u003cp\u003eAcknowledgements\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eThe authors would like to thank the participating patient and colleagues who made this work possible.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eFunding\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eNot applicable.\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eAvailability of data and materials\u003c/p\u003e\n\u003cp\u003eData sharing is not applicable to this case report as no datasets were generated or analyzed during the current study.\u003c/p\u003e\n\u003cp\u003eAuthors\u0026rsquo; contributions\u003c/p\u003e\n\u003cp\u003eWS reviewed the literature and drafted the manuscript. ZW and PT decided about final diagnosis and operated on the patient. XZ, HZ, HS participated in the follow-up of the patient. XS and HP revised the paper. All authors read and approved the final manuscript.\u003c/p\u003e\n\u003cp\u003eEthics approval and consent to participate\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eThe need for approval was waived by the institutional ethics of the First Affiliated Hospital of Zhejiang Traditional Chinese Medicine University for using medical records obtained from previous clinical diagnosis and treatment.\u003c/p\u003e\n\u003cp\u003eConsent for publication\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eA written informed consent was obtained from the patient.\u003c/p\u003e\n\u003cp\u003eCompeting interests\u0026nbsp;\u003c/p\u003e\n\u003cp\u003eThe authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.\u003c/p\u003e\n\u003cp\u003e\u003cbr\u003e\u003c/p\u003e"},{"header":"References","content":"\u003col\u003e\u003cli\u003e\u003cspan\u003eZimmermann P, Curtis N. Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome - A challenging diagnosis not to be missed. J Infect. 2016;72(Suppl):106\u0026ndash;S14.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eKahn MF, Khan MA. The SAPHO syndrome. Baillieres Clin Rheumatol. 1994;8(2):333\u0026ndash;62.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eFreira S, Fonseca H, Ferreira PD, Vasconcelos P, Fonseca JE. SAPHO syndrome in an adolescent: a clinical case with unusual severe systemic impact. J Adolesc Health. 2014;55(2):304\u0026ndash;6.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eM\u0026uuml;ller-Rath R, Mumme T, Zilkens K, Sp\u0026uuml;ntrup E. Isolierte, bilaterale sternokostoklavikul\u0026auml;re Hyperostose (SCCH) mit pathologischer Klavikulafraktur - Ein Verlauf \u0026uuml;ber 10 Jahre. Z Orthop Grenzgeb. 2005;143(06):691\u0026ndash;3.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eDepasquale R, Kumar N, Lalam RK, Tins BJ, Tyrrell PNM, Singh J, et al. SAPHO: What radiologists should know. Clin Radiol. 2012;67(3):195\u0026ndash;206.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eLeone A, Cassar-Pullicino VN, Casale R, Magarelli N, Semprini A, Colosimo C. The SAPHO syndrome revisited with an emphasis on spinal manifestations. Skeletal Radiol. 2015;44(1).\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eCianci F, Zoli A, Gremese E, Ferraccioli G. Clinical heterogeneity of SAPHO syndrome: challenging diagnose and treatment. Clin Rheumatol. 2017;36(9):2151\u0026ndash;8.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eBury DC, Rogers TS, Dickman MM. Osteomyelitis: Diagnosis and Treatment. Am Fam Physician. 2021;104(4):395\u0026ndash;402.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eKing SM, Laxer RM, Manson D, Gold R. Chronic recurrent multifocal osteomyelitis: a noninfectious inflammatory process. Pediatr Infect Dis J. 1987;6(10):907\u0026ndash;11.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eReith JD, Bauer TW, Schils JP. Osseous Manifestations of SAPHO (Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis) Syndrome. Am J Surg Pathol. 1996;20(11).\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eQiu C, Cheng L, Hou H, Liu T, Xu B, Xiao X, et al. Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome of femoral neoplasm-like onset: a case-based review. J Int Med Res. 2021;49(12):3000605211065314.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eHuang H, Zhang Z, Zhao J, Hao Y, Zhou W. The effectiveness of treatments for patients with SAPHO syndrome: a follow-up study of 24 cases from a single center and review of literature. Clin Rheumatol. 2021;40(3):1131\u0026ndash;9.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eCheng W, Li F, Tian J, Xie X, Chen JW, Peng XF, et al. New Insights in the Treatment of SAPHO Syndrome and Medication Recommendations. J Inflamm Res. 2022;15:2365\u0026ndash;80.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eMiyamoto S, Otsuka M, Hasue F, Fujiyoshi T, Kamiya K, Kiuchi H, et al. Stress fracture of the midshaft clavicle associated with sternocostoclavicular hyperostosis-Case report. Int J Surg Case Rep. 2019;58:121\u0026ndash;6.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eKuroda T, Ehara S, Murakami H. Stress fracture of the clavicle associated with sternocostoclavicular hyperostosis. Skeletal Radiol. 2005;34(7):424\u0026ndash;6.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eCheng Y, Feng H, Mu J, Chen J, Wu H. Percutaneous vertebroplasty for SAPHO syndrome with vertebral destruction: a case report and literature review. Front Med (Lausanne). 2023;10:1175787.\u003c/span\u003e\u003c/li\u003e \u003cli\u003e\u003cspan\u003eYeo I, Cha HS, Yoon YC, Park YS, Lim SJ. A case report of severely damaged hip joint caused by SAPHO syndrome treated with 2-stage total hip arthroplasty. Med (Baltim). 2016;95(27):e4168.\u003c/span\u003e\u003c/li\u003e\u003c/ol\u003e"}],"fulltextSource":"","fullText":"","funders":[],"hasAdminPriorityOnWorkflow":false,"hasManuscriptDocX":true,"hasOptedInToPreprint":true,"hasPassedJournalQc":"","hasAnyPriority":false,"hideJournal":true,"highlight":"","institution":"","isAcceptedByJournal":false,"isAuthorSuppliedPdf":false,"isDeskRejected":"","isHiddenFromSearch":false,"isInQc":false,"isInWorkflow":false,"isPdf":false,"isPdfUpToDate":true,"isWithdrawnOrRetracted":false,"journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true},"keywords":"SAPHO syndrome, clavicle, pathological fracture, surgical treatment, case report","lastPublishedDoi":"10.21203/rs.3.rs-3857085/v1","lastPublishedDoiUrl":"https://doi.org/10.21203/rs.3.rs-3857085/v1","license":{"name":"CC BY 4.0","url":"https://creativecommons.org/licenses/by/4.0/"},"manuscriptAbstract":"\u003ch2\u003eBackground\u003c/h2\u003e \u003cp\u003eSynovitis-acne-pustulosis-hyperostosis-osteomyelitis (SAPHO) syndrome is a rare disease characterized by combined osteoarticular and cutaneous manifestations. The diagnosis of SAPHO syndrome remains difficult due to its clinical heterogeneity and often requires the identification of infectious neoplasm and other bone abnormalities. Surgical treatment of pathological fractures caused by SAPHO syndrome is extremely rare. We report the first surgical case of a pathological clavicle fracture with SAPHO syndrome.\u003c/p\u003e\u003ch2\u003eCase presentation:\u003c/h2\u003e \u003cp\u003eA 53-year-old male patient came to our Orthopedic Surgery Department in 2022, complaining of right shoulder pain for 4 months, aggravated with activity limitation for 3 days. And the patient presented with cutaneous lesions on the right upper extremity. Based on the clinical manifestations, imaging findings, bone scintigraphy findings and bone puncture pathology, the diagnosis of SAPHO syndrome was made. Notably, the patient also suffered pathological fracture of the ipsilateral clavicle. We treated the clavicle with open reduction and internal fixation and found satisfactory results at the subsequent 6-month follow-up.\u003c/p\u003e\u003ch2\u003eConclusions\u003c/h2\u003e \u003cp\u003eThis case shows the efficacy and safety of open reduction and internal fixation in the treatment of pathological fractures caused by SAPHO syndrome. We hope to provide valuable information regarding the treatment of SAPHO syndrome in this case which makes clinical data more comprehensive.\u003c/p\u003e","manuscriptTitle":"SAPHO syndrome with a pathological fracture of clavicle: a case report","msid":"","msnumber":"","nonDraftVersions":[{"code":1,"date":"2024-01-23 15:45:35","doi":"10.21203/rs.3.rs-3857085/v1","editorialEvents":[{"type":"communityComments","content":0}],"status":"published","journal":{"display":true,"email":"[email protected]","identity":"researchsquare","isNatureJournal":false,"hasQc":true,"allowDirectSubmit":true,"externalIdentity":"","sideBox":"","snPcode":"","submissionUrl":"/submission","title":"Research Square","twitterHandle":"researchsquare","acdcEnabled":true,"dfaEnabled":false,"editorialSystem":"","reportingPortfolio":"","inReviewEnabled":false,"inReviewRevisionsEnabled":true}}],"origin":"","ownerIdentity":"df75b9ba-7d5a-4724-81b3-0024f641a136","owner":[],"postedDate":"January 23rd, 2024","published":true,"recentEditorialEvents":[],"rejectedJournal":[],"revision":"","amendment":"","status":"posted","subjectAreas":[],"tags":[],"updatedAt":"2024-04-03T07:18:16+00:00","versionOfRecord":[],"versionCreatedAt":"2024-01-23 15:45:35","video":"","vorDoi":"","vorDoiUrl":"","workflowStages":[]},"version":"v1","identity":"rs-3857085","journalConfig":"researchsquare"},"__N_SSP":true},"page":"/article/[identity]/[[...version]]","query":{"redirect":"/article/rs-3857085","identity":"rs-3857085","version":["v1"]},"buildId":"qtupq5eGEP_6zYnWcrvyt","isFallback":false,"isExperimentalCompile":false,"dynamicIds":[84888],"gssp":true,"scriptLoader":[]}