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ARTICLE IN PRESS
Diagnostic and Interventional Imaging (2014) xxx, xxx—xxx
LETTER / Musculoskeletal imaging SAPHO syndrome revealed by sclerosing mandibular osteomyelitis Keywords: SAPHO syndrome; Sclerosing osteomyelitis mandibular; Osteitis chronic mandibular Case observation A 55-year-old female patient whose main medical history consisted of an aortic coarctation operated on when she was 16 years old, consulted in 1999 for neuralgic facial pain of the right mandible. The initial imaging examinations (CT, MRI) gave normal results. In September 2011, the patient was referred with a clinical picture of right dental cellulitis: the pain, increased relative to the previous episode, was associated with trismus and swelling of the adjacent cheek. There was no generalized inflammatory syndrome. There were no associated general signs. In the CT examination of the facial skeleton (Fig. 1), the appearance was of osteitis of the right horizontal mandibular ramus with a radicular dental cyst projecting from the root of tooth no 45. The patient then underwent percutaneous bone biopsy but the bacteriological and histopathological examinations were negative. Tooth no 45 were extracted and a trial antibiotic treatment initiated for 6 weeks. After 8 months of clinical improvement, there was recurrence, with the appearance of a moderate biological inflammatory syndrome with CRP at 27 mg/L and moderate hyperleukocytosis of 15,000/mm3 . The CT scan performed found osteosclerosis of ground glass appearance with indistinct margins of the horizontal ramus of the right mandible with enlargement of the bone with no periosteal apposition (Fig. 2). The MRI performed a month later found a considerable inflammatory reaction in the muscles and subcutaneous tissues in contact, but with no collection or abscess (Fig. 3). Another biopsy was performed and was positive for Propionibacterium acnes. A new appropriate dual antibiotic treatment was administered, to no effect. Clinical examination of the patient at the time of another flare-up of mandibular inflammation found plantar pustulosis associated with Achilles tendon pain. In addition to right mandibular fixation, bone scintigraphy found bilateral calcaneal hyperfixation suggesting
Figure 1. Initial CT scan of the lower jaw. Appearance of right mandibular osteitis: demineralization of the cortical bone showing lacunae associated with medullary osteosclerosis (asterisk) and unilamellar periosteal appositions (dotted arrows).
enthesis compatible with the specific effects of spondyloarthropathy. The association of mandibular osteitis with Propionibacterium acnes and cutaneous signs meant that a diagnosis could be made, according to the Kahn criteria, of SAPHO. The patient was then treated in a specialized department to begin anti-inflammatory treatment combined with methotrexate. At 8 months, due to only partial improvement and still considerable clinical repercussions, treatment was initiated with anti-TNF␣, resulting in the disappearance of the mandibular pain. Discussion SAPHO, an acronym of Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis, is a syndrome, characterized by a set of clinical, radiological and anatomical symptoms, which can be diagnosed according to defined osteoarticular and cutaneous criteria (Table 1). Bone involvement is inflammatory
2211-5684/$ — see front matter © 2014 Éditions franc ¸aises de radiologie. Published by Elsevier Masson SAS. All rights reserved. http://dx.doi.org/10.1016/j.diii.2014.02.004
Please cite this article in press as: Pottecher P, et al. SAPHO syndrome revealed by sclerosing mandibular osteomyelitis. Diagnostic and Interventional Imaging (2014), http://dx.doi.org/10.1016/j.diii.2014.02.004
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Letter
Figure 2. CT scan of the lower jaw: a: axial slice: showing chronic sclerosing osteitis as osteosclerosis of the horizontal ramus and the mandibular angle with indistinct margins, enlarging the bone without affecting the dental canal, with the addition of periosteal appositions contributing to bone hypertrophy; b: coronal slice: osteosclerosis reaching the mandibular angle and the vertical ramus but not affecting the condyle.
and certain biopsies can come back positive for P. acnes [1]. It is rare for the clinical picture to be complete, especially at onset of the disease. This syndrome is noted for radiological polymorphism with bone involvement presenting specific topography [2]: • the anterior thoracic wall, marked by hyperostosis, is affected in 60—95% of the cases, with sternoclavicular, chondrocostal or manubriosternal involvement; • the spine is affected in 32—52% of cases, seen as an ivory vertebra sign, paravertebral ossification and/or spondylodiscitis [3]; • in two cases out of three, there is only vertebral involvement and of varying extent;
• the long bones are affected in 30% of the cases, especially in children, with metaphyseal/diaphyseal involvement (proximal and distal femur and distal tibia); • the flat bones (iliac bone, mandible) are involved in 8% of cases. SAPHO syndrome, identified in 1987, was associated in the literature with mandibular sclerosing osteitis in 1994 through the 7 cases identified by Kahn et al. [4]. Usually only one side is involved and other bone sites may be associated. Extension to the temporomandibular joint is described [5], evolving in rare cases to mandibular ankylosis [6].
Figure 3. MRI of the lower jaw. Axial T1-weighted slices with fat saturation after injection of gadolinium, showing considerable enhancement of the masticator spaces and right parotid gland (solid asterisk), with a mass effect concerning the parapharyngeal space (arrow) (a), and the pseudo-tumoral infiltrated appearance of the right medial pterygoid (hollow asterisk) and masseter muscles (solid asterisk) (b), with no visible collection.
Please cite this article in press as: Pottecher P, et al. SAPHO syndrome revealed by sclerosing mandibular osteomyelitis. Diagnostic and Interventional Imaging (2014), http://dx.doi.org/10.1016/j.diii.2014.02.004
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Letter Table 1
3 SAPHO diagnostic criteria.
SAPHO: diagnostic criteria Principal criterion
Aseptic multifocal osteomyelitis or caused by Propionibacterium acnes
Lesser criteria
1—Osteoarticular
Acute or chronic joint involvement Monostotic or polyostotic aseptic osteitis Palmoplantar pustulosis Severe acne Hidradenitis suppurativa
2—Cutaneous
SAPHO is diagnosed if the clinical radiological examination finds: one or other form of the principal criterion or an association of 2 lesser criteria Kahn MF, Khan MA. Baillieres. Clin Rheumatol 1994;8(2):333—62. The SAPHO syndrome
Its clinical picture is characterized by chronic progression in flare-ups evolving over a few weeks or months, but there is never any suppuration [7]. The appearance in CT also changes over time with osteosclerosis becoming more pronounced as the disease progresses. In the early stage, the appearance is of subacute osteitis, in which both cortical bone (demineralization, lacunae, unilamellar periosteal apposition) and the spinal cord (osteosclerosis) is involved. As the disease progresses, the bone volume increases through medullary extension and integration of periosteal apposition. In our case, as in a retrospective study of mandibular osteitis in SAPHO syndrome, the dental canal was not affected [7]. If the diagnosis is suspected, multifocal involvement can be sought using bone scintigraphy. Conclusion Diffuse sclerosing mandibular osteomyelitis in SAPHO is a rare clinical radiological entity, which should be suggested whenever there is osteitis of unknown etiology. Chronic evolution and the absence of suppuration or fistulization are the key diagnostic features. Bone biopsy is essential to eliminate the differential diagnoses (infectious osteitis, Ewing’s sarcoma, Paget’s disease, lymphoma). Disclosure of interest The authors declare that they have no conflicts of interest concerning this article. References [1] Hustache-Mathieu L, Brousse A, Lohse A, et al. Infectious osteoarthritis due to Propionibacterium acnes. Two new cases. Rev Med Interne 2000;21:547—9.
[2] Depasquale R, Kumar N, Lalam RK, et al. SAPHO: what radiologists should know. Clin Radiol 2011;67:195—206. [3] Parlier-Cuau C, Laredo JD. Vertebral involvement in SAPHO syndrome. J Radiol 2010;91:1068—78. [4] Kahn MF, Hayem F, Hayem G, Grossin M. Is diffuse sclerosing osteomyelitis of the mandible part of the synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome? Analysis of seven cases. Oral Surg Oral Med Oral Pathol 1994;78: 594—8. [5] Marsot-Dupuch K, Doyen JE, Grauer WO, et al. SAPHO syndrome of the temporomandibular joint associated with sudden deafness. AJNR Am J Neuroradiol 1999;20:902—5. [6] Müller-Richter UDA, Roldan JC, Mörtl M, Behr M, Reichert TE, Drieml O. SAPHO syndrome with ankylosis of the temporomandibular joint. Int J Oral Maxillofac Surg 2009;38: 1335—41. [7] Fleuridas G, Teysseres N, Ragot JP, Chikhani L, FavreDauvergne E. Diffuse sclerosing osteomyelitis of the mandible and SAPHO syndrome. Rev Stomatol Chir Maxillofac 2002;103: 96—104.
P. Pottecher a,∗ , R. Loffroy a , L. Estivalet a , P. Thouant a , P. Ornetti b , F. Ricolfi a , D. Krausé a a
Department of Radiodiagnostics and Diagnostic and Therapeutic Medical Imaging, CHU de Dijon, 2, boulevard Maréchal-de-Lattre-de-Tassigny, BP 77908, 21079 Dijon cedex, France b Department of Rheumatology, Hôpital Général, 3, rue du Faubourg-Raines, 21000 Dijon, France ∗ Corresponding author. E-mail address: [email protected] (P. Pottecher)
Please cite this article in press as: Pottecher P, et al. SAPHO syndrome revealed by sclerosing mandibular osteomyelitis. Diagnostic and Interventional Imaging (2014), http://dx.doi.org/10.1016/j.diii.2014.02.004
ARTICLE IN PRESS
Diagnostic and Interventional Imaging (2014) xxx, xxx—xxx
LETTER / Musculoskeletal imaging SAPHO syndrome revealed by sclerosing mandibular osteomyelitis Keywords: SAPHO syndrome; Sclerosing osteomyelitis mandibular; Osteitis chronic mandibular Case observation A 55-year-old female patient whose main medical history consisted of an aortic coarctation operated on when she was 16 years old, consulted in 1999 for neuralgic facial pain of the right mandible. The initial imaging examinations (CT, MRI) gave normal results. In September 2011, the patient was referred with a clinical picture of right dental cellulitis: the pain, increased relative to the previous episode, was associated with trismus and swelling of the adjacent cheek. There was no generalized inflammatory syndrome. There were no associated general signs. In the CT examination of the facial skeleton (Fig. 1), the appearance was of osteitis of the right horizontal mandibular ramus with a radicular dental cyst projecting from the root of tooth no 45. The patient then underwent percutaneous bone biopsy but the bacteriological and histopathological examinations were negative. Tooth no 45 were extracted and a trial antibiotic treatment initiated for 6 weeks. After 8 months of clinical improvement, there was recurrence, with the appearance of a moderate biological inflammatory syndrome with CRP at 27 mg/L and moderate hyperleukocytosis of 15,000/mm3 . The CT scan performed found osteosclerosis of ground glass appearance with indistinct margins of the horizontal ramus of the right mandible with enlargement of the bone with no periosteal apposition (Fig. 2). The MRI performed a month later found a considerable inflammatory reaction in the muscles and subcutaneous tissues in contact, but with no collection or abscess (Fig. 3). Another biopsy was performed and was positive for Propionibacterium acnes. A new appropriate dual antibiotic treatment was administered, to no effect. Clinical examination of the patient at the time of another flare-up of mandibular inflammation found plantar pustulosis associated with Achilles tendon pain. In addition to right mandibular fixation, bone scintigraphy found bilateral calcaneal hyperfixation suggesting
Figure 1. Initial CT scan of the lower jaw. Appearance of right mandibular osteitis: demineralization of the cortical bone showing lacunae associated with medullary osteosclerosis (asterisk) and unilamellar periosteal appositions (dotted arrows).
enthesis compatible with the specific effects of spondyloarthropathy. The association of mandibular osteitis with Propionibacterium acnes and cutaneous signs meant that a diagnosis could be made, according to the Kahn criteria, of SAPHO. The patient was then treated in a specialized department to begin anti-inflammatory treatment combined with methotrexate. At 8 months, due to only partial improvement and still considerable clinical repercussions, treatment was initiated with anti-TNF␣, resulting in the disappearance of the mandibular pain. Discussion SAPHO, an acronym of Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis, is a syndrome, characterized by a set of clinical, radiological and anatomical symptoms, which can be diagnosed according to defined osteoarticular and cutaneous criteria (Table 1). Bone involvement is inflammatory
2211-5684/$ — see front matter © 2014 Éditions franc ¸aises de radiologie. Published by Elsevier Masson SAS. All rights reserved. http://dx.doi.org/10.1016/j.diii.2014.02.004
Please cite this article in press as: Pottecher P, et al. SAPHO syndrome revealed by sclerosing mandibular osteomyelitis. Diagnostic and Interventional Imaging (2014), http://dx.doi.org/10.1016/j.diii.2014.02.004
+Model DIII-420; No. of Pages 3
ARTICLE IN PRESS
2
Letter
Figure 2. CT scan of the lower jaw: a: axial slice: showing chronic sclerosing osteitis as osteosclerosis of the horizontal ramus and the mandibular angle with indistinct margins, enlarging the bone without affecting the dental canal, with the addition of periosteal appositions contributing to bone hypertrophy; b: coronal slice: osteosclerosis reaching the mandibular angle and the vertical ramus but not affecting the condyle.
and certain biopsies can come back positive for P. acnes [1]. It is rare for the clinical picture to be complete, especially at onset of the disease. This syndrome is noted for radiological polymorphism with bone involvement presenting specific topography [2]: • the anterior thoracic wall, marked by hyperostosis, is affected in 60—95% of the cases, with sternoclavicular, chondrocostal or manubriosternal involvement; • the spine is affected in 32—52% of cases, seen as an ivory vertebra sign, paravertebral ossification and/or spondylodiscitis [3]; • in two cases out of three, there is only vertebral involvement and of varying extent;
• the long bones are affected in 30% of the cases, especially in children, with metaphyseal/diaphyseal involvement (proximal and distal femur and distal tibia); • the flat bones (iliac bone, mandible) are involved in 8% of cases. SAPHO syndrome, identified in 1987, was associated in the literature with mandibular sclerosing osteitis in 1994 through the 7 cases identified by Kahn et al. [4]. Usually only one side is involved and other bone sites may be associated. Extension to the temporomandibular joint is described [5], evolving in rare cases to mandibular ankylosis [6].
Figure 3. MRI of the lower jaw. Axial T1-weighted slices with fat saturation after injection of gadolinium, showing considerable enhancement of the masticator spaces and right parotid gland (solid asterisk), with a mass effect concerning the parapharyngeal space (arrow) (a), and the pseudo-tumoral infiltrated appearance of the right medial pterygoid (hollow asterisk) and masseter muscles (solid asterisk) (b), with no visible collection.
Please cite this article in press as: Pottecher P, et al. SAPHO syndrome revealed by sclerosing mandibular osteomyelitis. Diagnostic and Interventional Imaging (2014), http://dx.doi.org/10.1016/j.diii.2014.02.004
+Model
ARTICLE IN PRESS
DIII-420; No. of Pages 3
Letter Table 1
3 SAPHO diagnostic criteria.
SAPHO: diagnostic criteria Principal criterion
Aseptic multifocal osteomyelitis or caused by Propionibacterium acnes
Lesser criteria
1—Osteoarticular
Acute or chronic joint involvement Monostotic or polyostotic aseptic osteitis Palmoplantar pustulosis Severe acne Hidradenitis suppurativa
2—Cutaneous
SAPHO is diagnosed if the clinical radiological examination finds: one or other form of the principal criterion or an association of 2 lesser criteria Kahn MF, Khan MA. Baillieres. Clin Rheumatol 1994;8(2):333—62. The SAPHO syndrome
Its clinical picture is characterized by chronic progression in flare-ups evolving over a few weeks or months, but there is never any suppuration [7]. The appearance in CT also changes over time with osteosclerosis becoming more pronounced as the disease progresses. In the early stage, the appearance is of subacute osteitis, in which both cortical bone (demineralization, lacunae, unilamellar periosteal apposition) and the spinal cord (osteosclerosis) is involved. As the disease progresses, the bone volume increases through medullary extension and integration of periosteal apposition. In our case, as in a retrospective study of mandibular osteitis in SAPHO syndrome, the dental canal was not affected [7]. If the diagnosis is suspected, multifocal involvement can be sought using bone scintigraphy. Conclusion Diffuse sclerosing mandibular osteomyelitis in SAPHO is a rare clinical radiological entity, which should be suggested whenever there is osteitis of unknown etiology. Chronic evolution and the absence of suppuration or fistulization are the key diagnostic features. Bone biopsy is essential to eliminate the differential diagnoses (infectious osteitis, Ewing’s sarcoma, Paget’s disease, lymphoma). Disclosure of interest The authors declare that they have no conflicts of interest concerning this article. References [1] Hustache-Mathieu L, Brousse A, Lohse A, et al. Infectious osteoarthritis due to Propionibacterium acnes. Two new cases. Rev Med Interne 2000;21:547—9.
[2] Depasquale R, Kumar N, Lalam RK, et al. SAPHO: what radiologists should know. Clin Radiol 2011;67:195—206. [3] Parlier-Cuau C, Laredo JD. Vertebral involvement in SAPHO syndrome. J Radiol 2010;91:1068—78. [4] Kahn MF, Hayem F, Hayem G, Grossin M. Is diffuse sclerosing osteomyelitis of the mandible part of the synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome? Analysis of seven cases. Oral Surg Oral Med Oral Pathol 1994;78: 594—8. [5] Marsot-Dupuch K, Doyen JE, Grauer WO, et al. SAPHO syndrome of the temporomandibular joint associated with sudden deafness. AJNR Am J Neuroradiol 1999;20:902—5. [6] Müller-Richter UDA, Roldan JC, Mörtl M, Behr M, Reichert TE, Drieml O. SAPHO syndrome with ankylosis of the temporomandibular joint. Int J Oral Maxillofac Surg 2009;38: 1335—41. [7] Fleuridas G, Teysseres N, Ragot JP, Chikhani L, FavreDauvergne E. Diffuse sclerosing osteomyelitis of the mandible and SAPHO syndrome. Rev Stomatol Chir Maxillofac 2002;103: 96—104.
P. Pottecher a,∗ , R. Loffroy a , L. Estivalet a , P. Thouant a , P. Ornetti b , F. Ricolfi a , D. Krausé a a
Department of Radiodiagnostics and Diagnostic and Therapeutic Medical Imaging, CHU de Dijon, 2, boulevard Maréchal-de-Lattre-de-Tassigny, BP 77908, 21079 Dijon cedex, France b Department of Rheumatology, Hôpital Général, 3, rue du Faubourg-Raines, 21000 Dijon, France ∗ Corresponding author. E-mail address: [email protected] (P. Pottecher)
Please cite this article in press as: Pottecher P, et al. SAPHO syndrome revealed by sclerosing mandibular osteomyelitis. Diagnostic and Interventional Imaging (2014), http://dx.doi.org/10.1016/j.diii.2014.02.004
