Berlin, Grace, DO

Erickson, Kourtney, DO

Mishriki, Yehia Y, MD

English

Lehigh Valley Health Network: LVHN Scholarly Works

Lehigh Valley Health NetworkLVHN Scholarly WorksDepartment of MedicineSAPHO: A Challenging Diagnosis in a YoungWoman With Recurrent OsteomyelitisKourtney Erickson DOLehigh Valley Health Network, Kourtney.Erickson@lvhn.orgGrace Berlin DOLehigh Valley Health Network, grace.berlin@lvhn.orgYehia Y. Mishriki MDLehigh Valley Health Network, Yehia.Mishriki@lvhn.orgFollow this and additional works at: https://scholarlyworks.lvhn.org/medicinePart of the Internal Medicine Commons, and the Rheumatology CommonsThis Poster is brought to you for free and open access by LVHN Scholarly Works. It has been accepted for inclusion in LVHN Scholarly Works by anauthorized administrator. For more information, please contact LibraryServices@lvhn.org.Published In/Presented AtErickson, K. Berlin, G. Mishriki, Y. (2018, November 10). SAPHO: A Challenging Diagnosis in a Young Woman With RecurrentOsteomyelitis. Poster Presented at: The American College of Physicians Regional Conference (ACP), Scranton, PA.© 2018 Lehigh Valley Health NetworkSAPHO: A Challenging Diagnosis in a  Young Woman With Recurrent Osteomyelitis Kourtney Erickson, DO, G. Berlin, DO, Y. Mishriki, MDLehigh Valley Health Network, Allentown, Pa.INTRODUCTIONSAPHO, known as synovitis, acne, pustulosis, hyperostosis and osteitis is a rare syndrome and has been estimated to occur in 1 in 10,000 in the Caucasian population. It is a disease of unknown pathogenesis which can present with swelling and bony tenderness as well as osteitis affecting multiple bones. CASE DESCRIPTIONA 25-year-old female with a history significant for severe hidradenitis suppurativa requiring multiple drainages and frequent antibiotics, mandibular osteomyelitis due to actinomyces, and culture negative osteomyelitis of the clavicle presented with left elbow pain and swelling. This was evaluated with MRI demonstrating a septic joint with surrounding myositis and uncertain osteomyelitis. Incision and drainage including bone biopsy was performed; pathology demonstrated osteomyelitis but cultures were negative. The patient was treated with 4 weeks of intravenous antibiotics. After discharge, the patient returned with recurrent left elbow pain; MRI showed worsening osteomyelitis and blood work showed increasing inflammatory markers. There was concern for osteosarcoma; however pathology again revealed acute osteomyelitis with recurrent negative cultures. With the constellation of symptoms including hidradenitis suppurativa and recurrent culture-negative osteomyelitis particularly including the clavicle, the diagnosis of SAPHO was established. The patient was started on anti-inflammatory medications and methotrexate with eventual progression to adalimumab.REFERENCES1  Rukavina, I. (2015). SAPHO syndrome: a review. Journal of Children’s Orthopaedics. 9:1; 19-27. Retrieved from https://doi.org/10.1007/s11832-014-0627-72  Merola, J.F. (2017). SAPHO (synovitis, acne, pustulosis, hyperostosis, osteitis) syndrome. In Romain P.L., Ofori, A.O. (Ed.). UpToDate. Retrieved on August 09, 2018.) from https://www.uptodate.com/contents/sapho-synovitis-acne-pustulosis-hyperostosis-osteitis-syndrome3  Matzaroglou, C., Velissaris, D., Karageorgos, A., Marangos, M., Panagiotopoulos, E., & Karanikolas, M. (2009). SAPHO Syndrome Diagnosis and Treatment: Report of Five Cases and Review of the Literature. The open orthopaedics journal,3, 100-6. doi:10.2174/18743250009030101004  Khan, S.H.M, Bloem, H.L. (2015). Appendicular infections. In Musculoskeletal Imaging. (Chapter 65, pp 779-790). Retrieved from https://www.clinicalkey.com5  Bois, A.J., Wirth., M.A, Rockwood, C.A. (2017). Disorders of the Sternoclavicular Joint in Rockwood and Matsen’s The Shoulder. (Chapter 10, pp 453-491). Retrieved from https://www.clinicalkey.comDISCUSSIONSAPHO is a diagnosis of exclusion once infectious osteomyelitis, malignancy, and other autoimmune conditions have been ruled out. After these exclusions, the diagnosis can be made if the patient has severe acne, palmoplantar pustulosis, or any case of hyperostosis or recurrent multifocal osteomyelitis with or without dermatologic features. Most commonly affected bones are the anterior chest wall (sternum, clavicle, or sternocostoclavicular joints), the vertebrae, sacroiliac joints and long bones. There are no specific tests for SAPHO, but elevated inflammatory markers may be seen. Plain films and bone scan may help identify additional areas of involvement. Tissue sampling and joint aspiration can help rule out infectious osteomyelitis. Treatment consists of NSAIDs and for refractory cases: prednisone, methotrexate, bisphosphonates and biologics. Increased awareness of SAPHO can help clinicians arrive at a faster diagnosis with hope of reducing the incidence of osteomyelitis and decreasing unnecessary antibiotic administration and surgical procedures.X-ray of the left elbow of our patient read as significant soft tissue calcifications.Palmoplantar pustulosis2Diagnostic criteria proposed by Benhamou for SAPHO syndrome diagnosis1,21.  Osteoarticular manifestations in severe acne2.  Osteoarticular manifestations in palmoplantar pustulosis3.  Hyperostosis with or without dermatosis and4.  Recurrent multifocal chronic osteomyelitis involving the axial or peripheral skeleton, with or without dermatosisSAPHO: synovitis, acne, pustulosis, hyperostosis osteitis

SAPHO: A Challenging Diagnosis in a Young Woman With Recurrent Osteomyelitis

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SAPHO: A Challenging Diagnosis in a Young Woman With Recurrent Osteomyelitis

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