Pericarditis as an initial manifestation of Sjögren syndrome in a child

Abstract

Abstract Introduction: Primary Sjögren syndrome (SS) is a chronic systemic autoimmune disease characterized by lymphocytic inflammation of exocrine glands and epithelia in multiple sites. SS is usually difficult to diagnose particularly in children because SS is rare for this age group, and various clinical signs and symptoms, without typical sicca symptoms, are shown as initial manifestations in pediatric SS patients. There have been no reports on cases of pediatric primary SS with symptomatic pericarditis being the initial manifestation. We report a case of primary SS in a pediatric patient who developed symptomatic pericarditis. Patient concerns: An 11-year-old girl presented with a 2-day history of fever, and chest and left shoulder pains. The pain was exacerbated when she was in a supine position. Diffuse ST-segment elevations on the electrocardiogram, presence of pericardial friction sounds, exacerbation of diastolic pericardial effusion on the echocardiogram, and continuous chest pain indicated acute symptomatic pericarditis. Diagnosis: The results of the blood antibody test, salivary gland scintigram, and salivary gland biopsy led to the diagnosis of SS. Also, this patient has no hypocomplementemia, and classification criteria of American College of Rheumatology of systemic lupus erythematosus and mixed connective tissue disease were not fulfilled. Intervention: Prednisolone therapy relieved her symptoms. Subclinical Hashimoto's thyroiditis, a well-known complication of primary SS, was observed; however, prescriptions were not needed. Outcomes: The clinical signs and symptoms of systemic lupus erythematosus and mixed connective tissue disease were not observed when prednisolone was tapered. During the 1-year treatment and follow-up period, the patient had no symptom recurrence. Conclusions: In pediatric primary SS patients, clinically silent heart changes are common, but obvious heart involvement is extremely rare. Unusual initial symptoms, such as symptomatic pericarditis, should not be missed in childhood-onset primary SS.