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Editorial Manager(tm) for Spine Journal Manuscript Draft Manuscript Number: SPINE 110855 Title: Spontaneous Clinical and

Radiological Resolution of Vertebral Sarcoidosis: A Case Report Article Type: Case Report Keywords: sarcoidosis, cervical, thoracic, lumbar, vertebral mass, enhancing lesion Corresponding Author: Jason Michael Johnson, M.D. Corresponding Author's Institution: Fletcher Allen Health Care / University of Vermont First Author: Alisa K Johnson, M.D. Order of Authors: Alisa K Johnson, M.D.;Jason Michael Johnson, M.D.;Elizabeth Ames, M.D.;Christopher G Filippi, M.D.

Title Page

Vertebral Sarcoidosis

Spontaneous Clinical and Radiological Resolution of Vertebral Sarcoidosis: A Case Report

Alisa K. Johnson, MD1; Jason M. Johnson, MD1; Elizabeth Ames, MD2; Christopher Filippi, MD1

Section of Neuroradiology, Department of Radiology, Fletcher Allen Health Care / University of Vermont, Burlington, Vermont. 2 Department of Orthopedic Surgery, Fletcher Allen Health Care / University of Vermont, Burlington, Vermont.

All authors share the same address with the corresponding author.

Retrospective case review exempt from local IRB review.

Correspondence to: Alisa K. Johnson, MD, Department of Radiology, 111 Colchester Ave., Burlington, VT 05401. Phone: 802-847-2700; FAX: 802-847-4822. E-mail: Alisa.Johnson@vtmednet.org

Vertebral Sarcoidosis The manuscript submitted does not contain information about medical device(s)/drug(s). No funds were received in support of this work. No benefits in any form have been or will be received from a commercial party related directly or indirectly to the subject of this manuscript.

*Structured Abstract (300 words)

Vertebral Sarcoidosis Abstract Study Design: Case report of occult presentation of asymptomatic vertebral sarcoidosis.

Objective: To illustrate a case of vertebral sarcoidosis with occult presentation and subsequent spontaneous resolution.

Summary of Background Data: Vertebral sarcoidosis is an uncommon disorder characterized by noncaseating granulomatous infiltration of the spine. It most commonly affects the thoracic spine. The disc spaces are usually preserved, clinical presentation and imaging characteristics are variable.

Methods: Case presentation and report.

Results: A 41-year-old man with classic pulmonary sarcoidosis presented after a work related back injury. MRI of his spine demonstrated multiple enhancing lesions throughout the thoracic and lumbar vertebra. Classic granulomas were identified on subsequently obtained core biopsy specimens and the diagnosis of vertebral sarcoidosis was made.

Conclusion: To our knowledge this is the first case of concomitant pulmonary and asymptomatic vertebral sarcoidosis with spontaneous resolution of the vertebral manifestations without definitive treatment. A broad differential diagnosis should be entertained for new enhancing spine lesions including non-infectious inflammatory diseases such as sarcoidosis.

Vertebral Sarcoidosis Key words: sarcoidosis, cervical, thoracic, lumbar, vertebral mass, enhancing lesion

*Key Points (3-5 main points of the article)

Vertebral Sarcoidosis KEY POINTS

1. Sarcoidosis is a multisystem disorder with the primary manifestation being pulmonary. 2. Osseous involvement of the vertebra is uncommon and can present with appearance similar to that of multifocal metastatic disease. 3. The diagnosis for multiple enhancing vertebral lesions should include sarcoidosis, especially given typical lung findings or medical history of the disease.

*Mini Abstract (50 words)

Vertebral Sarcoidosis Mini Abstract

A 41-year-old man with pulmonary sarcoidosis presented after a work related back injury. MRI of his spine demonstrated multiple enhancing lesions throughout the thoracic and lumbar vertebra. Classic granulomas were identified on subsequently obtained core biopsy specimens and the diagnosis of vertebral sarcoidosis was made. The lesions subsequently spontaneously resolved without directed therapy.

*Manuscript Text (must include page numbers)

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Vertebral Sarcoidosis

Sarcoidosis is a multi-organ granulomatous inflammatory disease that primarily affects the lungs. The etiology of sarcoidosis is thought to be multifactorial, involving a complex interplay of multiple genetic loci1 and an exaggerated T-cell response to an airborne antigen.2 This multisystem disorder is characterized by noncaseating granulomatous infiltration affecting multiple organ system. The pulmonic system is affected in more than 90% of patients, whereas osseous involvement is estimated to only affect 1 13%.3,4 Bone involvement, when present, is thought to typically affect the hands and feet. Vertebral sarcoidosis is thought to be rare with only a handful of cases reported in the literature.5 This report presents a case of vertebral sarcoidosis, which was incidentally detected during the evaluation of radiculopathy.

Case report A 41-year old male patient presented to his primary care physician for shoulder and neck pain after a work-related injury that resulted in left-sided neck and shoulder pain, with radiation to the left upper extremity. Radiographic evaluation of the cervical spine revealed mild degenerative changes. Due to persistence of cervical radiculopathy, an MRI was performed to exclude disc related pathology. The MR showed that there was no significant neruoforaminal or central stenosis. However, multiple T1 low-signal lesions throughout the upper thoracic vertebral bodies were noted. A dedicated MRI of the thoracic and lumber spine for further evaluation showed similar pattern of abnormality with multiple small lesions throughout the thoracic and lumbar vertebral bodies and posterior elements which were bright on T2, low signal on T1, and showed enhancement after gadolinium administration (Figure 1).

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Vertebral Sarcoidosis A broad differential diagnosis was entertained including metastatic disease, myeloma, atypical hemangiomas, disseminated infection and vertebral sarcoidosis. A bone scan showed normal osseous activity. A CT-guided percutaneous biopsy of enhancing lesion in T12 and L3 was preformed. The pathology demonstrated focal granulomas without evidence of metastatic disease or other pathology. These findings were felt consistent with extra pulmonary sarcoidosis given the patients clinical history of pulmonary sarcoidosis. The patients presenting symptoms were not felt to be due to the vertebral sarcoidosis so a recommendation to have a follow up scan was given. An 18-month follow-up CT examination of the chest revealed near complete resolution of the peribronchovascular nodules and ground glass opacities (Figure 2). Six months after the chest CT the recommended follow up MRI of the spine was performed which showed complete resolution of the areas of marrow signal abnormality (Figure 3).

Discussion

Sarcoidosis is a multisystem disorder of unknown etiology with the primary manifestation being pulmonary. Many cases of osseous sarcoidosis are asymptomatic. However, cases may present with pain related to granulomatous infiltration. Osseous sarcoidosis typically presents in the setting of typical findings on chest radiography and also with cutaneous involvement. In our case, there were typical chest findings but no cutaneous stigmata were identified.3

Radiographic findings in osseous sarcoidosis is variable and can present as lytic, mixed lytic and sclerotic and predominantly sclerotic lesions mimicking blastic metastasis.6 The thoracic, lumbar and, less commonly, the cervical vertebrae, may be involved. The disk spaces are usually

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Vertebral Sarcoidosis preserved, as in our case. Extension into the posterior elements has been reported, but isolated involvement of the pedicles is rare. Bone scintigraphy, although nonspecific, may be a sensitive marker for the extent of osseous sarcoidosis. Scintigraphy also has the potential utility in that it can localize additional sites for biopsy if the clinical area is not readily accessible and can also be used as a tool for monitoring disease activity.4

MRI in vertebral sarcoidosis is non-specific, as in our case, and can present as a single, multiple focal or diffuse lesions that are hypointense on T1 and hyperintense on fluid sensitive sequences (T2 and STIR). Lesions that are predominantly sclerotic on radiography may be hypointense on all pulse sequences. The intensity of the signal is enhanced after gadolinium administration.7 It is not possible to distinguish infectious from granulomatous discitis by MRI or other imaging studies making biopsy necessary for diagnosis in certain situations.

Sarcoidosis is generally only treated if symptomatic, at which point a weight based regimen of oral prednisone is typically prescribed. However, 70% of patients with lung manifestations of sarcoidosis will experience regressesion within two years of the initial onset regardless of treatment. In some patients, sarcoidosis progresses relentlessly and these patients may require long-term medication.2 To our knowledge this is the first described case of spontaneous resolution of vertebral sarcoidosis. A broad differential diagnosis should be entertained for new vertebral lesions and a history of granulomatous disease should be considered.

*References (cited in order of appearance)

Vertebral Sarcoidosis REFERENCES

1. Rybicki BA, Levin AM, McKeigue P, et al. A genome-wide admixture scan for ancestrylinked genes predisposing to sarcoidosis in African-Americans. Genes Immun 2011;12:67-77. 2. Statement on sarcoidosis. Joint Statement of the American Thoracic Society (ATS), the European Respiratory Society (ERS) and the World Association of Sarcoidosis and Other Granulomatous Disorders (WASOG) adopted by the ATS Board of Directors and by the ERS Executive Committee, February 1999. Am J Respir Crit Care Med 1999;160:736-55. 3. James DG, Neville E, Carstairs LS. Bone and joint sarcoidosis. Semin Arthritis Rheum 1976;6:53-81. 4. Shorr AF, Murphy FT, Kelly WF, Kaplan KJ, Gilliland WR, Shapeero LG. Osseous Sarcoidosis Clinical, Radiographic, and Therapeutic Observations. J Clin Rheumatol 1998;4:186-92. 5. Rua-Figueroa I, Gantes MA, Erausquin C, Mhaidli H, Montesdeoca A. Vertebral sarcoidosis: clinical and imaging findings. Semin Arthritis Rheum 2002;31:346-52. 6. Resnick D. Diagnosis of bone and joint disorders. 4th ed. Philadelphia: Saunders; 2002. 7. Ginsberg LE, Williams DW, Stanton C. MRI of vertebral sarcoidosis. J Comput Assist Tomogr 1993;17:158-9.

Figure Legends

Spontaneous Clinical and Radiological Resolution of Vertebral Sarcoidosis: A Case Report

FIGURE LEGEND

Figure 1. 41-year-old male with vertebralsarcoiosis.T1, T2 and T1 fat saturated post gadolinium sagittal MR images of the thoracic spine demonstrates multiple discrete T1 hypointense, T2 hyperintense and post gadolinium enhancementof marrow space lesions.

Figure 2. 41-year-old male with vertebralsarcoiosis.Axial HRCT image shows predominantly peripheral areas of scarring without evidence of significant groundglass opacities or lymphadenopathy.

Figure 3. 41-year-old male with vertebralsarcoiosis.T1, T2 and T1 fat saturated post gadolinium sagittal MR images of the thoracic spine demonstratescomplete resolution of the multiple discrete lesions previously identified. No abnormal enhancement is seen on this repeat MR study.

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