Osteolysis of the cervical spine and mandible in systemic sclerosis: a case report with computed tomography and magnetic resonance imaging findings

R. Benitha, M. Modi1 and M. Tikly

Departments of Medicine
1 Radiology, Chris Hani Baragwanath Hospital and University of the Witwatersrand, Johannesburg, South Africa

SIR, Systemic sclerosis (SSc) is a multisystem rheumatic disorder characterized by excessive fibrosis of the skin and visceral organs, vasculopathy and circulating autoantibodies. A wide spectrum of bone, joint and soft-tissue manifestations occur in 46–97% of all SSc patients and as the presenting feature in 12–65% of patients [1, 2]. Osseous resorption or osteolysis is a rare, but perhaps the most characteristic, radiographic feature of SSc. The terminal digital tufts of the phalanges are most commonly affected, but osteolysis has also been observed in other skeletal sites, including the distal radius and ulna, ribs, the mandible, the distal clavicle and cervical spine. We report a case of extensive osteolysis involving the terminal phalanges, ribs, mandible and posterior elements of the cervical spine in a patient with diffuse cutaneous SSc (dcSSc).

Ms AK, a 54-yr-old black patient with a 16-yr history of SSc, complained of progressive worsening of hand deformities, difficulty biting and chewing food, and neck pain over a 2-yr period. There were no symptoms suggestive of trigeminal neuralgia. She had previously been documented to have dcSSc, characterized by severe Raynaud's phenomenon (present throughout the year), oesophageal dysmotility, telangiectasia, calcinosis, ‘salt and pepper’ depigmentation of the face and trunk, ischaemic pits of the pulp spaces of the fingers, and resorption of the terminal phalanges. There was no history of exposure to silica or any chemicals. Investigations showed that she was ANA-positive and positive for anti-topoisomerase-1 and U3 RNP (fibrillarin) antibodies. The radiographs of the hand confirmed resorption of the distal phalangeal tufts. Lung function tests revealed a restrictive pattern. A high-resolution computed tomography (CT) scan of the lungs was normal. She has been treated with omeprazole for reflux oesophagitis and a combination of nifedipine and perindopril for the Raynaud's phenomenon. In the past she had been treated for approximately 3 yr with d-penicillamine, which was stopped about 5 yr ago because of no noticeable improvement in the skin.

On examination she had extensive skin thickening with a modified Rodnan's total skin score of 18 [3]. Apart from the features noted previously, she had severe acro-osteolysis of the fingers resembling arthritis mutilans and obvious retrognathia. She was tender over the posterior aspect of the neck and there was cervical scoliosis to the left. There were no cranial nerve abnormalities or pyramidal tract signs.

Plain radiographs of the neck confirmed the scoliosis as well as extensive resorption of the posterior spinous processes. The chest radiograph showed resorption of the clavicle and ribs 2, 3, 4 and 5 bilaterally. A CT scan of the neck revealed almost 45° rotation of the bodies of the cervical vertebrae, except for C1 (Figs 1 and 2GoGo). In addition, there was variable osteolysis of the laminae of all the cervical vertebrae and mandible. The sagittal T2-weighted magnetic resonance imaging (MRI) similarly showed striking osteolysis of the posterior elements of C2–C5, including the laminae and spinous processes (Fig. 3Go). The spinal canal was patent and there was secondary ectasia of the thecal sac due to loss of the posterior bony elements. The neural exit foramina were unimpeded. Serum biochemistry, including calcium, phosphate and parathyroid hormone levels, was within the normal limits.



View larger version (110K):
[in this window]
[in a new window]
 
FIG. 1. Axial CT scan image at the level of C1 demonstrating the relatively normal position of the body of C1 with complete loss of its posterior bony elements and variable resorption of the mandible.

 


View larger version (102K):
[in this window]
[in a new window]
 
FIG. 2. Axial CT scan image at the level of the body of C2 demonstrating almost 45° anticlockwise rotation of the body of C2 with respect to C1, with loss of the laminae of the cervical vertebral bodies and variable resorption of the mandible.

 


View larger version (103K):
[in this window]
[in a new window]
 
FIG. 3. Midline sagittal T2-weighted MRI of the cervical spine demonstrating marked osteolysis of the posterior spinous processes of C2–C5 with secondary ectasia of the thecal sac.

 
Osteolysis is a well-characterized skeletal feature of SSc [4]. Acro-osteolysis, with resorption of the terminal phalanges, is the commonest presentation of this process, occurring in 40–80% of patients [5]. Another common site of osteolysis is the periodontal membrane, which is initially thickened in up to 20% of patients [6]; this leads eventually to loss of the lamina dura and loosening of dentition. Trigeminal neuralgia, which is the commonest cranial nerve abnormality in SSc [7], is thought to be the result of this process [8]. Extensive mandibular resorption, as in our case, is an extremely rare manifestation, and is usually detected between the fifth and seventh years after diagnosis [9, 10]. Rib resorption, as in our case, typically affects superior surfaces of the posterior parts of the third to sixth ribs [11].

Only three cases of osteolysis of the cervical spine have been reported previously. All of these patients showed extensive osteolysis at other sites, including the digits, distal ulna and radius. All except one had associated mandibular resorption. Two of the patients had associated pyramidal tract signs and one patient developed a neuropathic arthritis due to nerve entrapment [9]. Spinal cord compression is less likely to occur in our patient in the face of almost complete osteolysis of the laminae and spinous processes.

CT or MRI features of osteolysis of the cervical spine and mandible have not been reported previously. Apart from showing marked bone resorption, both the CT and MRI images demonstrated rotation of the cervical vertebral bodies, for which there is no obvious explanation. We postulate that osteolysis at the sites of ligamentous insertion resulted in ligamentous instability, allowing uninhibited rotatory contraction of the paraspinal muscles and leading to rotatory scoliosis of the cervical spine.

The pathogenesis of osteolysis is poorly understood, although the condition is frequently associated with severe Raynaud's phenomenon and dcSSc [12]. The condition can occur at any time during the course of the disease, and in some instances, as in our patient, can accelerate many years after the onset of SSc. The current hypothesis is that bone resorption is the result of ischaemia, due to a combination of the microvasculopathy of SSc and pressure ischaemia secondary to skin tightening and muscular atrophy. No humoral factors have been reported, but it is noteworthy that acro-osteolysis has been reported in one study to be more common in patients who are positive for anti-topoisomerase-1 antibody [12]. Bone turnover has not been studied specifically in this subgroup of SSc patients, but there is evidence of increased bone turnover in SSc generally [13]. Bone collagen breakdown products were not measured in our case due to the unavailability of the service.

SSc, especially the dcSSc subset, can be associated with severe physical disability for a variety of reasons, including skin induration, joint contractures, inflammatory arthritis, myopathy and osseous resorption. In our patient the last feature has resulted in progressively worsening hand function and severe difficulty with mastication, but so far there have been no neurological sequelae. Antiresorptive therapy with alendronate has been initiated in an attempt to halt the resorptive process. Reconstructive surgery for the mandible, in an attempt to improve mastication, is being considered.

Notes

Correspondence to: M Tikly, Division of Rheumatology, Department of Medicine, Chris Hani Baragwanath Hospital, P.O. Bertsham 2013, South Africa. Back

References

  1. Tuffanelli DL, Winkelman RK. Systemic scleroderma: Clinical study of 727 cases. Arch Dermatol 1961;84:359–71.[ISI]
  2. Baron M, Lee P, Keystone EC. The articular manifestations of progressive systemic sclerosis (scleroderma). Ann Rheum Dis 1982;41:147–52.[Abstract]
  3. Brennan P, Silman A, Black C et al. Reliability of skin involvement measures in scleroderma. The UK Scleroderma Study Group. Br J Rheumatol 1992;31:457–60.[ISI][Medline]
  4. Rodnan GP. The nature of joint involvement in progressive systemic sclerosis. Ann Intern Med 1962;56:422–39.[ISI][Medline]
  5. Blocka K. Organ involvement: musculoskeletal. In: Clements PJ, Furst DE, eds. Systemic sclerosis. Baltimore: Williams and Wilkins, 1996:409–24.
  6. Rowell NR, Hopper FE. The periodontal membrane in systemic sclerosis. Br J Dermatol 1975;93 (Suppl. II):23.[ISI]
  7. Farrell DA, Medsger TA. Trigeminal neuropathy in progressive systemic sclerosis. Am J Med 1982;73:57–62.[ISI][Medline]
  8. Fischoff DK, Sirois D. Painful trigeminal neuropathy caused by severe mandibular resorption and nerve compression in a patient with systemic sclerosis: case report and literature review. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2000;90:456–9.[ISI][Medline]
  9. Haers PE, Sailer HF. Mandibular resorption due to systemic sclerosis. Case report of surgical correction of a secondary open bite deformity. Int J Oral Maxillofac Surg 1995;24:261–7.[ISI][Medline]
  10. Rout PGJ, Hamburger J, Potts AJC. Orofacial radiological manifestations of systemic sclerosis. Dentomaxillofac Radiol 1996;25:193–6.[Abstract]
  11. Keats TE. Rib erosions in scleroderma. Am J Roentgenol 1967;100:530–2.
  12. Jacobsen S, Halberg P, Ullman S et al. Clinical features and serum antinuclear antibodies in 230 Danish patients with systemic sclerosis. Br J Rheumatol 1998;37:39–45.[ISI][Medline]
  13. La Montagna G, Baruffo A, Abbadessa S, Maja L, Tirri R. Evidence for bone resorption in systemic sclerosis. J Rheumatol 1995;22:797–9.[ISI][Medline]
Accepted 8 April 2002





This Article
Full Text (PDF)
Alert me when this article is cited
Alert me if a correction is posted
Services
Email this article to a friend
Similar articles in this journal
Similar articles in ISI Web of Science
Similar articles in PubMed
Alert me to new issues of the journal
Add to My Personal Archive
Download to citation manager
Disclaimer
Request Permissions
Google Scholar
Articles by Benitha, R.
Articles by Tikly, M.
PubMed
PubMed Citation
Articles by Benitha, R.
Articles by Tikly, M.
Related Collections
Other Rheumatology