HSS What's the Diagnosis Case 150

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What’s the Diagnosis – Case 150

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Findings The radiographs show mild degenerative disc disease and otherwise are not particularly helpful in this case. The MRI demonstrates areas of prominent edema and subtle fatty infiltration about the corners of multiple vertebrae. A spiculated type configuration is present on the axial images. Areas of focal edema and fatty infiltration are present of the iliac aspect of both sacroiliac joints. The bone scan demonstrates abnormal radiotracer uptake about the left sternoclavicular (SC) joint and the manubriosternal (MS) articulation.

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Diagnosis: SAPHO Syndrome SAPHO is an acronym of synovitis, acne, pustulosis, hyperostosis, and osteitis. A still somewhat vague entity which is not completely understood. It is an inflammatory/auto immune process which many feels represent the adult form of CRMO although it does have differences. Often SAPHO also incorporates more so findings of seronegative arthropathies than CRMO. SAPHO typically will cause lesions of the vertebrae at the corners thought related to enthesopathic changes at the Sharpey fibers attachment and longitudinal ligament attachments. In the active phase, edema is precipitated and over time fatty replacement ensues. This can produce a spiculated architecture of the vertebrae on axial images. As compared to a metastatic process with random distribution, these findings are found at the corners of the vertebrae. Not shown in this example, but an inflammatory spondylodiscitis similar to an Andersson lesion of ankylosing spondylitis can occur. SAPHO very characteristically causes inflammation of the SC and MS joints which in this case produced the increased uptake on the bone scan. It can cause an inflammatory sacroiliitis or as seen in this case areas of focal bony abnormality of the iliac bones adjacent to the SI joints as these areas are metaphyseal equivalents which frequently show focal abnormalities. This diagnosis frequently is delayed as often the constellation of findings is needed to come to the correct diagnosis.

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References SAPHO Syndrome: Imaging Findings of Vertebral Involvement. A.M. McGauvran, A.L. Kotsenas, F.E. Diehn, J.T. Wald, C.M. Carr and J.M. Morris American Journal of Neuroradiology August 2016, 37 (8) 1567-1572; DOI: https://doi.org/10.3174/ajnr.A4736 Imaging of Chronic Recurrent Multifocal Osteomyelitis. Geetika Khanna, MD, MS, Takashi S. P. Sato, MD, Polly Ferguson, MD. Radiographics July-August 2009, Vol 29, No. 4 1159-1177 Case 181: Synovitis Acne Pustulosis Hyperostosis Osteitis (SAPHO) Syndrome. Sarah A. Sweeney, MD, Vinodh A. Kumar, MD, Jean Tayar, MD, Donna M. Weber, MD, Amar Safdar, MD, Carol Alonso, MD, Sharon Hymes, MD. Radiology. Vol 263, No. 2, May 2012 Synovitis, acne, pustulosis, hyperostosis, and osteitis syndrome: review and update. Shuang Liu, Mingwei Tang, Yihan Cao and Chen Li. Therapeutic Advances in Musculoskeletal Disease. 2020, Vol. 12: 1–14 DOI: 10.1177/1759720X20912865

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