Juvenile dermatomyositis and Sjögren's syndrome occurring concurrently in an adolescent male

Letters to the Editor

D. Cody and J. Davidson

Department of Rheumatology, Alder Hey Children's Hospital, Eaton Road, Liverpool 12, UK

SIR, Sjögren's syndrome has not been well studied in childhood but is uncommon. It may occur as a primary disorder but is more commonly secondary, occurring in association with some other autoimmune disease. Juvenile dermatomyositis is an inflammatory muscle disorder usually affecting children aged 5–15 yr. There are approximately 3 cases per million population per year, with a 2:1 female:male ratio. The causation is unknown but is likely to be multifactorial [1]. An association with Sjögren's syndrome has not been reported previously.

We describe here a 14-yr-old male who presented with a left pre-auricular swelling that had been present for the previous 3–4 weeks. There was no evidence of facial nerve weakness. He had no other significant medical history and no family history of autoimmune disease. The initial impression was of a viral sialadenitis but, following review in the maxillofacial out-patients department, a magnetic resonance imaging (MRI) scan of his parotid glands and a sialogram was arranged.

Prior to these investigations, he returned with further complaints of general malaise, weight loss and joint pains affecting his hands, feet and wrists. A photosensitive scaly rash had developed over the preceding week, affecting his hands and neck. This hadn't cleared despite treatment with topical hydrocortisone. On referral to the rheumatology service, he was found to have scaly erythematous plaques on his knuckles, elbows and knees (Fig. 1Go). A violaceous rash had developed around his eyes. There was no discernible muscle weakness and he denied any muscle pain. He had a left parotid swelling. The clinical impression was of a connective tissue disorder, most probably juvenile dermatomyositis. The significance of the parotid swelling was uncertain but the possibility of associated Sjögren's syndrome was considered.



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FIG. 1.  Typical rash of juvenile dermatomyositis with Gottron's papules.

 
Investigations revealed a moderate lymphopenia of 1.63x109/l. The erythrocyte sedimentation rate and C-reactive protein concentration were normal. Serum transaminases were elevated, with an aspartate aminotransferase concentration of 73 (1–37) µmol/l and alanine aminotransferase of 115 (1–40) U/l. This, together with a mildly raised creatine kinase of 255 (24–195) U/l, was compatible with myositis. Serum immunoglobulins and complement were normal. The patient was negative for rheumatoid factor, antinuclear antibodies and anti-Ro and La antibodies. MRI of muscle was normal. A muscle biopsy demonstrated abnormal major histocompatibility complex class 1 staining on the surface of the muscle fibres, consistent with the early changes associated with an inflammatory myopathy. Echocardiography and lung function tests were normal. Further imaging of the parotid gland was arranged. MRI and ultrasound both demonstrated diffuse swelling of the left parotid gland (Fig. 2Go). Colour and power Doppler ultrasonography showed a marked increase in vascularity. Left parotid sialography showed pooling of contrast in multiple tiny cavities with appearances typical of punctate sialectasis. Ophthalmology assessment demonstrated keratoconjunctivitis sicca with significantly reduced tear function in both eyes and a reduced tear meniscus and break-up time.



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FIG. 2.  Left parotid ultrasound scan, showing inflamed lobules containing an echogenic central part surrounded by an echolucent rim, separated by echogenic septa.

 
All results were in keeping with a diagnosis of juvenile dermatomyositis with secondary Sjögren's syndrome. Treatment was commenced with a course of intravenous methylprednisolone, to which he responded well. He was discharged home on oral prednisolone, but on dose reduction his symptoms returned and methotrexate was added to his treatment regime. He is currently well on methotrexate alone.

Parotid swelling is common in childhood and may be due to a variety of causes, including mumps, acute bacterial infection and salivary stones. Rarer causes include tumours, HIV, tuberculosis and dental conditions [2]. Parotitis is suggested to be the most common presenting symptom in Sjögren's syndrome in children compared with adults [3], and this was the case with our patient.

Sjögren's syndrome is a rare diagnosis in both childhood and adolescence [4]. The incidence in adults is suggested at 0.2% but the incidence in childhood is unknown. The clinical features are due to lymphocytic infiltration of the lachrymal and parotid glands. Secondary Sjögren's syndrome is found in association with other autoimmune disorders, including systemic lupus erythematosus and rheumatoid arthritis.

The criteria required for the diagnosis of Sjögren's syndrome remain controversial. In adults, the San Diego criteria include objective keratoconjunctivitis, xerostomia and either a minor salivary gland biopsy or characteristic antibodies. In contrast, the European criteria can be satisfied by a history of sicca symptoms and abnormal salivary gland scintigraphy [5, 6]. The situation in the paediatric population is even less clear. One recent study included clinical symptoms of keratoconjunctivitis sicca as well as sialography, scintigraphy and lip histology in its classification [7].

In our patient, the diagnosis of Sjögren's syndrome, which was suggested initially by the painless parotid swelling, was confirmed by the positive radiological findings on parotid imaging and the objective evidence of dry eyes. In our experience, ultrasound gives superior information compared with MRI and sialography and is the investigation of choice in children. Lip biopsy is unacceptable to most children.

Notes

Correspondence to: D. Cody. Back

References

  1. Pachman L. Juvenile dermatomyositis. Pathophysiology and disease expression. Pediatr Clin North Am1995;42:1071–98.[ISI][Medline]
  2. Forfar and Arneil's textbook of paediatrics, edn 5. In: Cartilage and bone, Campbell AGM, McIntosh, eds. Edinburgh: Churchill Livingstone, 1998. pp. 425 and 1372.
  3. Anaya JM, Ogawa N, Talal N. Sjögren's syndrome in childhood. J Rheumatol1995;22:1152–8.[ISI][Medline]
  4. Bernstein B, Koster-King K, Singsen S, Kornreich HK, Hanson V. Sjögren's syndrome in childhood. Arthritis Rheum1977;20(Suppl.):361–2.
  5. Fox RI. Clinical features, pathogenesis and treatment of Sjögren's syndrome. Curr Opin Rheumatol1996;8:438–45.[Medline]
  6. Fox RI, Tornwall J, Maruyama T, Stern M. Evolving concepts of diagnosis, pathogenesis and therapy of Sjögren's syndrome. Curr Opin Rheumatol1998;10:446–56.[Medline]
  7. Bartunkova J, Sediva A, Vencovsky V, Tesar V. Primary Sjögren's syndrome in children and adolescents: proposal for diagnostic criteria. Clin Exp Rheumatol1999;17:381–6.[ISI][Medline]
Accepted 28 December 2001





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Myositis and Muscle Disease
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