Primary Sjögren's Syndrome in Dizygotic Adolescent Twins: One Case with Lymphocytic Interstitial Pneumonia
KRISTIN M. HOUGHTON, DAVID A. CABRAL, ROSS E. PETTY, and LORI B. TUCKER
ABSTRACT.
Primary Sjögren's syndrome (pSS) is uncommonly recognized in childhood, and familial cases are rare. Pulmonary involvement in pediatric pSS is infrequently reported. In adults, asymptomatic pulmonary involvement is increasingly recognized, manifest by pulmonary function test abnormalities and changes on high resolution computerized tomographic scan. We describe a case of pSS in a 14-year-old Vietnamese-Canadian girl who presented with pulmonary symptoms, radiologic changes, and biopsy confirmation of lymphocytic interstitial pneumonia. Her dizygotic twin sister has primary SS without extraglandular manifestations. To our knowledge this is the first report of pediatric pSS with lymphocytic interstitial pneumonia and multiple pulmonary nodules on chest radiograph. We review the literature on pulmonary involvement and familial cases of pSS in childhood.
(J Rheumatol 2005;32:1603-6)
Key Indexing Terms:
CHILD
SJÖGREN'S SYNDROME
LYMPHOCYTIC INTERSTITIAL PNEUMONIA
TWINS
From the Division of Rheumatology, British Columbia's Children's Hospital and University of British Columbia, Vancouver, British Columbia, Canada.
K.M. Houghton, MD, FRCPC; D.A. Cabral, MBBS; R.E. Petty, MD, Division of Rheumatology, Department of Pediatrics, BC Children's Hospital and University of BC; L.B. Tucker, MD, Division of Rheumatology, Department of Pediatrics, Center for Community Child Health Research, BC Children's Hospital and University of BC.
Address reprint requests to Dr. K. Houghton, K4-119 Ambulatory Care Building, BC Children's Hospital, 4480 Oak Street, Vancouver, BC V6H 3V4. E-mail: khoughton@cw.bc.ca
Accepted for publication April 18, 2005.
