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Progressive Occipitocervical Subluxation Despite Biologic Agent Therapy in a Patient with Advanced Rheumatoid Arthritis

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Received:April 1, 2017, Revised:(1st) April 24, 2017, (2nd) May 22, 2017, Accepted:May 23, 2017

Corresponding to:Kichul Shin, Division of Rheumatology, Department of Internal Medicine, SMG-SNU Boramae Medical Center, 20 Boramae-ro 5-gil, Dongjak-gu, Seoul 07061, Korea. E-mail:[email protected]

Copyright ⓒ 2017 by The Korean College of Rheumatology. All rights reserved.

This is a Open Access article, which permits unrestricted non-commerical use, distribution, and reproduction in any medium, provided the original work is properly cited.

Clinical Image

pISSN: 2093-940X, eISSN: 2233-4718

Journal of Rheumatic Diseases Vol. 24, No. 3, June, 2017 https://doi.org/10.4078/jrd.2017.24.3.169

Figure 1. (A) There are multiple erosions (arrowheads) at the base and neck of the odontoid process of axis (open mouth view).

Note the symmetrical joint space narrowing (arrows) of lateral atlantoaxial joints, which is a typical finding in rheumatoid arthritis.

(B) Anterior subluxation of the atlantoaxial joint is evident at flexion. The atlantodental interval is 7 mm (double-headed arrow).

(C) The Ranawat C1∼C2 index (double-headed arrow) is 12 mm, just below the normal range.

Progressive Occipitocervical Subluxation Despite Biologic Agent Therapy in a Patient with Advanced Rheumatoid Arthritis

Yoon-Jeong Oh1, Jee Won Chai2, Kichul Shin1

1Division of Rheumatology, Department of Internal Medicine, SMG-SNU Boramae Medical Center, 2Department of Radiology, SMG-SNU Boramae Medical Center, Seoul National University College of Medicine, Seoul, Korea

Rheumatoid arthritis (RA) is a chronic systemic in- flammatory disease that can affect the cervical (c-) spine in up to 80% of the patients [1,2]. Although c-spine in- volvement in RA is often asymptomatic, uncontrolled high disease activity eventually becomes a major risk fac- tor for future spinal instability [3]. Adequate treatment with conventional or biologic disease-modifying anti- rheumatic drugs (DMARDs) can minimize cervical in- stability, but it does not halt the progression of pre-exist- ing joint damage [3]. Furthermore, progressive atlan- toaxial instability can induce intractable neck pain and can cause neurological deficits [4]. We hereby report a

case of a 68-year-old woman who presented with occipital headache for 10 weeks. She was diagnosed with sero- positive RA in 2003. She was first treated conservatively according to her own wish. She shortly developed pain in her knees, and eventually received total joint replacement arthroplasty for her left (2006) and right (2013) knees. A preoperative c-spine evaluation performed in 2013 re- vealed bony erosions at both C1 and C2 facet joints and anterior atlantoaxial subluxation (Figure 1). The Ranawat C1∼C2 index, which is used to evaluate vertical sub- luxation, was 12 mm, and it was determined by assessing the length between the center of the C2 pedicle and a line

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Yoon-Jeong Oh et al.

170 J Rheum Dis Vol. 24, No. 3, June, 2017

Figure 2. (A) Three years later, the Ranawat C1∼C2 index (double-headed arrow) is markedly decreased, suggesting basilar impression. Note the odontoid process (asterisk) is protruded beyond the horizontal axis of atlas (dashed line). (B) The reformatted sagittal computed tomography (CT) scan shows that the odontoid process is protruded above the level of foramen magnum (McRae line; dashed line). There are multiple erosions (arrows) in the C4, C5 discovertebral area. (C) A reformatted sagittal CT scan of a 25-year-old healthy female. The odontoid process is under the McRae line (dashed line).

connecting the midpoint of the anterior and posterior arches of C1 (normal value; male >15 mm, female >13 mm) [5]. This index is useful because it does not require visualization of the tip of the odontoid process nor the hard palate (as in the McGregor index). She finally began receiving conventional DMARDs in April 2014 after de- veloping intractable joint pain in her hands, shoulders, and knees. Her disease activity score (DAS)28 soared up to 5.8. Intravenous tocilizumab therapy without metho- trexate was initiated in May 2015. After the third in- fusion, her joint symptoms were alleviated and DAS28 was substantially reduced to 2.0. Nevertheless, the image obtained in August 2016 showed vertical atlantoaxial in- stability which had worsened than before; she developed severe occipital headache. The Ranawat C1∼C2 index was significantly reduced to 4.3 mm (Figure 2A). Bony erosions in the discovertebral area of her c-spine were al- so prominent (Figure 2B). In summary, this case presents a patient with advanced RA who showed progressive occi- pitocervical subluxation, regardless of one-year long treatment with biologics.

CONFLICT OF INTEREST

No potential conflict of interest relevant to this article was reported.

REFERENCES

1. Joaquim AF, Appenzeller S. Cervical spine involvement in rheumatoid arthritis--a systematic review. Autoimmun Rev 2014;13:1195-202.

2. Ahn JK, Hwang JW, Oh JM, Lee J, Lee YS, Jeon CH, et al.

Risk factors for development and progression of atlantoax- ial subluxation in Korean patients with rheumatoid arthritis. Rheumatol Int 2011;31:1363-8.

3. Imagama S, Oishi Y, Miura Y, Kanayama Y, Ito Z, Wakao N, et al. Predictors of aggravation of cervical spine instability in rheumatoid arthritis patients: the large joint index. J Orthop Sci 2010;15:540-6.

4. Joaquim AF, Ghizoni E, Tedeschi H, Appenzeller S, Riew KD. Radiological evaluation of cervical spine involvement in rheumatoid arthritis. Neurosurg Focus 2015;38:E4.

5. Han MH, Ryu JI, Kim CH, Kim JM, Cheong JH, Bak KH, et al. Factors that predict risk of cervical instability in rheuma- toid arthritis patients. Spine (Phila Pa 1976) 2016 Oct 25 [Epub]. DOI: 10.1097/BRS.0000000000001942.

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