Primary Sjögren’sSyndrome in a Child

Authors: A. Šedivá;  J. Bartůňková;  J. Janda 1;  A. Zumrová 2;  K. Dlask 3
Authors‘ workplace: Ústav imunologie, 1 I. dětská klinika, 2 Klinika dětské neurologie, 3 Klinika anestezie a resuscitace
Published in: Čes. Revmatol., , 2000, No. 3, p. 109-111.


Primary Sjögren’s syndrome is a very rare disease during childhood. The affectedchildren escape the correct diagnostic process due to atypical clinical presentation in children incomparison with classical sicca syndrome in adults. The submitted case report demonstratesa severe clinical course of primary Sjögren’s syndrome in a 14-year-old girl, who presented with firstsigns of the disease at the age of ten. Case report: The initial clinical problems of the patient werenon-specific complaints of fatigue, loss of appetite, consequent weight reduction and low back pain.Within five months the clinical picture progressed until metabolic collapse, hypokalaemia, paralyticileus and both central and peripheral nervous system involvement required intensive care of thepatient. The diagnosis of primary Sjögren’s syndrome was in this stage of the disease based onlaboratory investigation, with marked hyperimmunoglobulinaemia, positivity of antinuclear anti-bodies, positivity of anti SS-A and SS-B antibodies connected with positive anti SS-A in cerebrospinalfluid. Renal damage was represented by severe hypokalaemia, hypophosphataemia due to extremehigh phosphate excretion, decreased renal concentration capacity, slightly decreased glomerularfiltration rate and moderate tubular acidosis. Therapy was initiated with pulses of corticosteroidswith subsequent combined immunosuppression with cyclosporin A. The recovery from quadrupa-resis and metabolic crisis was very slow despite the aggressive treatment. The long-time course wascomplicated by relapses of the disease. The most severe problem making the overall prognosis ofthe patient uncertain was represented by the subsequent attacks of retrobulbar neuritis with partialloss of vision. Conclusion: Primary Sjögren’s syndrome can occur during childhood. The childrenpresented with a wide spectrum of clinical signs, different from typical sicca syndrome of adults.The demonstrated patient represents the extreme pole of severe systemic involvement in primarySjögren’s syndrome.

Key words:
autoimunity, Sjögren’s syndrome, autoantibodies, children, SS-A, SS-B

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Dermatology & STDs Paediatric rheumatology Rheumatology
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