School of Medicine and Health Sciences Poster Presentations
Retinal Vasculitis as an Initial Presentation of Synovitis, Acne, Pustulosis, Hyperostosis, Osteomyelitis/Chronic Recurrent Multifocal Osteomyelitis (SAPHO/CRMO)
Poster Number
202
Document Type
Poster
Publication Date
3-2016
Abstract
Background and Purpose:
This is a case of a patient with chronic sternoclavicular pain presenting with retinal vasculitis found to have synovitis, acne, pustulosis, hyperostosis, osteomyelitis (SAPHO)/chronic recurrent multifocal osteomyelitis (CRMO) spectrum disorder. SAPHO syndrome is a rare chronic condition commonly involving the bones of the anterior chest with concomitant dermatological manifestations. CRMO is an aseptic inflammatory bone disease similar to SAPHO syndrome but lacks joint and cutaneous involvement. In more recent literature, SAPHO syndrome and CRMO are often described as a single clinical spectrum. This case illustrates the potential of a rare disease to masquerade as common process, thus delaying adequate treatment.
Methods/Case Description:
The patient is a 37-year-old female who presented with years of recurrent episodes of sternoclavicular pain responsive to non-steroidal anti-inflammatory drugs (NSAIDs). Initial biopsy revealed reactive bone and fibrosis and she was told she had “costochondritis.” She began experiencing floaters in her left eye and initial workup revealed elevated C-reactive protein (CRP) and estimated sedimentation rate (ESR), normocytic anemia, and regions of vascular leakage on fluorescent angiography. Further investigation included a CT showing sternal hyperostosis and sclerosis and a PET/CT showing sclerotic and partially lytic lesions in the sternum and bilateral clavicular heads. This was particularly concerning for a neoplastic process but a re-biopsy showed chronic inflammation without evidence of malignancy. Sternoclavicular pain continued and months later, the patient reported an episode of pustular skin lesions in a dermatomal distribution on the right chest wall.
Results/Case Discussion:
Given the presence of pustulosis, hyperostosis, and chronic inflammation on biopsy, a diagnosis of SAPHO/CRMO spectrum disorder was made and immunosuppressive therapy was initiated. Following treatment with oral prednisone 40mg, her symptoms improved and CRP and ESR levels returned to normal. Decreasing the prednisone to 10mg resulted in the return of sternal pain and the cessation of prednisone completely resulted in a re-elevation of inflammatory markers. Pustular skin lesions and retinal vasculitis have not returned.
Conclusion/Significance:
While bone pain can be a common complaint, serologic and radiographic evidence of inflammation should prompt one to exclude a malignant process prior to initiating treatment. This case demonstrates a unique scenario where a mundane process reveals to be something more sinister. SAPHO/CRMO spectrum disorder describes the clinical spectrum of sterile inflammatory bone disease with the commonly concurring disorders of synovitis, acne, pustulosis, hyperostosis, and osteomyelitis. In addition to the typical manifestations, this presentation is particularly unique involving retinal vasculitis.
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Open Access
1
Retinal Vasculitis as an Initial Presentation of Synovitis, Acne, Pustulosis, Hyperostosis, Osteomyelitis/Chronic Recurrent Multifocal Osteomyelitis (SAPHO/CRMO)
Background and Purpose:
This is a case of a patient with chronic sternoclavicular pain presenting with retinal vasculitis found to have synovitis, acne, pustulosis, hyperostosis, osteomyelitis (SAPHO)/chronic recurrent multifocal osteomyelitis (CRMO) spectrum disorder. SAPHO syndrome is a rare chronic condition commonly involving the bones of the anterior chest with concomitant dermatological manifestations. CRMO is an aseptic inflammatory bone disease similar to SAPHO syndrome but lacks joint and cutaneous involvement. In more recent literature, SAPHO syndrome and CRMO are often described as a single clinical spectrum. This case illustrates the potential of a rare disease to masquerade as common process, thus delaying adequate treatment.
Methods/Case Description:
The patient is a 37-year-old female who presented with years of recurrent episodes of sternoclavicular pain responsive to non-steroidal anti-inflammatory drugs (NSAIDs). Initial biopsy revealed reactive bone and fibrosis and she was told she had “costochondritis.” She began experiencing floaters in her left eye and initial workup revealed elevated C-reactive protein (CRP) and estimated sedimentation rate (ESR), normocytic anemia, and regions of vascular leakage on fluorescent angiography. Further investigation included a CT showing sternal hyperostosis and sclerosis and a PET/CT showing sclerotic and partially lytic lesions in the sternum and bilateral clavicular heads. This was particularly concerning for a neoplastic process but a re-biopsy showed chronic inflammation without evidence of malignancy. Sternoclavicular pain continued and months later, the patient reported an episode of pustular skin lesions in a dermatomal distribution on the right chest wall.
Results/Case Discussion:
Given the presence of pustulosis, hyperostosis, and chronic inflammation on biopsy, a diagnosis of SAPHO/CRMO spectrum disorder was made and immunosuppressive therapy was initiated. Following treatment with oral prednisone 40mg, her symptoms improved and CRP and ESR levels returned to normal. Decreasing the prednisone to 10mg resulted in the return of sternal pain and the cessation of prednisone completely resulted in a re-elevation of inflammatory markers. Pustular skin lesions and retinal vasculitis have not returned.
Conclusion/Significance:
While bone pain can be a common complaint, serologic and radiographic evidence of inflammation should prompt one to exclude a malignant process prior to initiating treatment. This case demonstrates a unique scenario where a mundane process reveals to be something more sinister. SAPHO/CRMO spectrum disorder describes the clinical spectrum of sterile inflammatory bone disease with the commonly concurring disorders of synovitis, acne, pustulosis, hyperostosis, and osteomyelitis. In addition to the typical manifestations, this presentation is particularly unique involving retinal vasculitis.
Comments
Presented at: GW Research Days 2016