PEDIATRICS Vol. 118 No. 5 November 2006, pp. e1569-e1571 (doi:10.1542/10.1542/peds.2006-1089)
EXPERIENCE & REASON |
Cervical Spine and Temporomandibular Joint Arthritis in a Child With Kawasaki Disease
a Divisions of General Pediatrics
c Rheumatology, Department of Pediatrics
b Department of Radiology, Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania
d Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania
ABSTRACT
Severe neck pain, stiffness, and torticollis in a child with Kawasaki disease commonly represent aseptic meningitis or lymphadenitis. Here we present an unusual case of severe cervical spine and bilateral temporomandibular joint arthritis in a 5-year-old boy with a relapse of Kawasaki disease and coronary artery ectasia. The patient had a favorable response to a second course of intravenous immunoglobulin G and indomethacin therapy, with complete resolution of his symptoms.
Key Words: Kawasaki disease • arthritis • child
Abbreviations: KD, Kawasaki disease • WBC, white blood cell • Ig, immunoglobulin • IVIG, intravenous immunoglobulin G
Kawasaki disease (KD) is an idiopathic systemic vasculitis characterized by fever, lymphadenopathy, nonexudative conjunctivitis, inflammation of the lips and oral mucosa, edema of the dorsal surfaces of the hands and feet, polymorphous exanthem, and, in some cases, dilation and aneurysms of the coronary arteries.1 Arthritis is commonly observed but may represent a diagnostic challenge because of the young age of children with KD, the irritability that is so characteristic of the condition, and the presence of edema in the upper and lower extremities. Arthritis of the cervical spine and temporomandibular joints, which is rare among children without juvenile idiopathic arthritis, represents a specific diagnostic challenge because of the depth of the involved joints and the anatomic complexity of the region. Here we present the first reported case of severe and transient arthritis of the cervical spine and temporomandibular joints in the setting of a KD relapse.
CASE REPORT
A previously healthy 5-year-old boy presented to our hospital with 3 days of fever, sore throat, abdominal pain, and rash. On admission, his temperature was 38.5°C, and he had conjunctivitis with limbic sparing, cracked erythematous lips, a strawberry tongue, edematous and erythematous extremities, marked cervical lymphadenopathy, and a generalized erythematous maculopapular rash. He was tachycardic, and his abdomen was tender to palpation without rebound tenderness. Laboratory investigations revealed an elevated C-reactive protein level of 10.4 mg/dL, an erythrocyte sedimentation rate of 68 mm/hour, and mildly elevated hepatic transaminase levels. The throat culture was negative for group A ß-hemolytic Streptococcus. His white blood cell (WBC) count was 13300/mm3 (5% bands, 78% segmented neutrophils, 10% lymphocytes, 7% monocytes), hemoglobin level was 11.4 g/dL, and platelets were 333000/mm3. A clean-catch urinalysis showed no pyuria. An echocardiogram revealed ectasia of the right coronary artery (0.29 cm, z = 2.74). On the fourth day of illness, he was treated with intravenous immunoglobulin G (IVIG, 2 g/kg) and high-dose aspirin (100 mg/kg per day) for the KD. The fever resolved rapidly, and he was discharged the following day on low-dose aspirin.
He was readmitted 5 days later with 2 days of fever to 38.6°C and an acute onset of severe neck pain and stiffness. Physical examination revealed torticollis and an inability to extend the neck, bilateral conjunctival injection, cracked lips, shotty cervical lymphadenopathy, erythema of the urethral meatus, perirectal desquamation, and loss of range of motion of the left elbow and right knee without effusions. He was unable to fully open his mouth. The WBC count was 15600/mm3, his hemoglobin level was 10.2 g/dL, and the platelet count had risen to 884000/mm3. His C-reactive protein level remained elevated at 4.3 mg/dL. Cerebrospinal fluid was sterile, with a WBC count of 2 cells per mm3 with glucose and protein concentrations of 57 mg/dL (reference range: 32–82 mg/dL) and 62 mg/dL (reference range: 15–40 mg/dL), respectively.
MRI of the cervical spine with gadolinium enhancement was obtained to rule out arthritis or an invasive bacterial infection such as osteomyelitis or a retropharyngeal or spinal epidural abscess. The MRI showed effusions of the bilateral atlanto-occipital, atlanto-axial, and temporomandibular joints (Figs 1 and 2). A repeat echocardiogram revealed minimal progression of the right coronary artery lesion (0.33 cm, z = 3.80). He was treated with IVIG (2 g/kg) and indomethacin (3 mg/kg per day) in addition to low-dose aspirin. The fever resolved, and his pain improved, with recovery of the full range of motion of his neck and temporomandibular joints over 48 hours. Testing for human leukocyte antigen-B27 was negative. Three weeks later, he remained asymptomatic, and the indomethacin was slowly discontinued.
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DISCUSSION
This is the first report of acute arthritis affecting the cervical spine and temporomandibular joints in KD. Our patient fulfilled criteria for the diagnosis of KD and had documented coronary artery ectasia, which argues against a diagnosis of postenteric or posturethritic reactive arthritis.2 His fever pattern, conjunctivitis, rash, desquamation, and clinical course were not consistent with systemic juvenile idiopathic arthritis, which may also be associated with coronary artery dilation.3
The incidence of arthritis in KD is as high as 45%.4 Two forms of arthritis have been described, and they both resolve without sequelae.1 Arthritis or arthralgia found in the acute stage is characterized by polyarticular involvement of large and small joints. Arthritis in the subacute phase of KD tends to involve the hips, knees, and ankles. The frequency of late-onset arthritis may be less common since the introduction of IVIG therapy and was as low as 2% in a recent report.5
The joint manifestations observed in KD are most consistent with either a virus-induced or reactive arthritis. Epidemiologic and immunologic studies of KD suggest an infectious trigger, but the cause of KD remains elusive.1 Patients with KD have oligoclonal IgA plasma cell infiltration of coronary arteries and the upper respiratory tract. The presence of macrophages and CD8+ T cells in the inflamed respiratory mucosa and intracytoplasmic inclusion bodies in respiratory epithelial cells suggests that KD may be caused by an intracellular pathogen.6,7 Pathophysiologic mechanisms of arthritis related to infection include invasion of organisms into the joint space, infection of synoviocytes, molecular mimicry, and formation of immune complexes. Septic, reactive, and virus-induced arthritis of the cervical spine and temporomandibular joint have been described.8–10
This case emphasizes the importance of considering arthritis of the temporomandibular joints or cervical spine in addition to meningitis and lymphadenitis in a patient with KD with severe neck or jaw pain. Our patient responded well to re-treatment with IVIG and indomethacin, a potent nonsteroidal antiinflammatory drug.
ACKNOWLEDGMENTS
We thank Drs David D. Sherry and Randy Q. Cron and Samir S. Shah for helpful critiques of the manuscript.
FOOTNOTES
Accepted Jun 1, 2006.
Address correspondence to Jon M. Burnham, MD, MSCE, Children's Hospital of Philadelphia, Room 1579 CHOP North, 3535 Market St, Philadelphia, PA 19104. E-mail: burnhams{at}email.chop.edu
The authors have indicated they have no financial relationships relevant to this article to disclose.
This case report was approved by the Children's Hospital of Philadelphia Institutional Review Board.
REFERENCES
- Newburger JW, Takahashi M, Gerber MA, et al. Diagnosis, treatment, and long-term management of Kawasaki disease: a statement for health professionals from the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association [published correction appears in Pediatrics. 2005;115(4):1118].
Pediatrics. 2004;114
:1708
–1733
[Abstract/Free Full Text] - Bauman C, Cron RQ, Sherry DD, Francis JS. Reiter syndrome initially misdiagnosed as Kawasaki disease. J Pediatr. 1996;128 :366 –369[CrossRef][Web of Science][Medline]
- Binstadt BA, Levine JC, Nigrovic PA, et al. Coronary artery dilation among patients presenting with systemic-onset juvenile idiopathic arthritis. Pediatrics. 2005;116(1) . Available at: www.pediatrics.org/cgi/content/full/116/1/e89
- Duzova A, Topaloglu R, Keskin M, Ozcelik U, Secmeer G, Tokgozoglu AM. An unusual pattern of arthritis in a child with Kawasaki syndrome. Clin Rheumatol. 2004;23 :73 –75[CrossRef][Web of Science][Medline]
- Lee KY, Oh JH, Han JW, Lee JS, Lee BC. Arthritis in Kawasaki disease after responding to intravenous immunoglobulin treatment. Eur J Pediatr. 2005;164 :451 –452[CrossRef][Web of Science][Medline]
- Rowley AH, Baker SC, Shulman ST, et al. Cytoplasmic inclusion bodies are detected by synthetic antibody in ciliated bronchial epithelium during acute Kawasaki disease. J Infect Dis. 2005;192 :1757 –1766[CrossRef][Web of Science][Medline]
- Rowley AH, Shulman ST, Spike BT, Mask CA, Baker SC. Oligoclonal IgA response in the vascular wall in acute Kawasaki disease.
J Immunol. 2001;166
:1334
–1343
[Abstract/Free Full Text] - Guillaume MP, Hermanus N, Peretz A. Unusual localisation of chronic arthropathy in lumbar facet joints after parvovirus B19 infection. Clin Rheumatol. 2002;21 :306 –308[CrossRef][Web of Science][Medline]
- Henry CH, Hughes CV, Gerard HC, Hudson AP, Wolford LM. Reactive arthritis: preliminary microbiologic analysis of the human temporomandibular joint. J Oral Maxillofac Surg. 2000;58 :1137 –1142; discussion 1143–1144[CrossRef][Web of Science][Medline]
- Nishimura Y, Ishida H, Shin N, et al. Streptococcus pneumoniae-associated arthritis of a lumbar facet joint in a 4-year-old boy. Pediatr Int. 2004;46 :101 –103[CrossRef][Web of Science][Medline]
PEDIATRICS (ISSN 1098-4275). ©2006 by the American Academy of Pediatrics
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