Abstract
BackgroundSjögren syndrome (SS) is a rare disease in pediatrics, and little attention has been paid to the clinical feature in these patients. To date, there are few cases concern about neurological and nephrological disorders in childhood Sjögren syndrome. We describe a case of Sjögren syndrome in a 12-year-old girl who developed neurological disorders and interstitial nephritis and review the literature currently available on this topic.Case presentationA 12-year-old girl was admitted to our hospital for arthritis and glucosuria. She was required to do labial gland and renal biopsy, because the positive for anti-nuclear antibody and anti-Sjögren syndrome B (anti-SSB) antibody. Then the biopsy was performed revealing the lymphocytic infiltrate in the small area and renal tubular interstitial damage,thus the diagnosis of Sjögren syndrome with tubular interstitial damage was made. Three months later, she presented again with headache, fever, nausea, vomiting and was recovered without drug therapy. Based on the patient’s medical history, laboratory and imaging examination, and treatment, we speculate that the disorders of the nervous system were caused by the Sjögren syndrome. The girl has stable renal function and no residual nervous system damage in the next 1.5 years, but she underwent low dose prednisone therapy because of persistent renal glucosuria.ConclusionsNephrological disorders and neurological involvement are rare manifestations of Sjögren syndrome in children, and rarely presented as the initial symptoms. It should be suspected in children presenting with unexplained renal diseases, neurological abnormalities, or unexplained fever. Although there is no guidelines on the diagnosis and treatment of children Sjögren syndrome are currently available, early recognition and the appropriate treatment of renal damage and neurologic involvement would improve prognosis and prevent complications.
Highlights
Sjögren syndrome (SS) is a rare disease in pediatrics, and little attention has been paid to the clinical feature in these patients
Nephrological disorders and neurological involvement are rare manifestations of Sjögren syndrome in children, and rarely presented as the initial symptoms. It should be suspected in children presenting with unexplained renal diseases, neurological abnormalities, or unexplained fever
There is no guidelines on the diagnosis and treatment of children Sjögren syndrome are currently available, early recognition and the appropriate treatment of renal damage and neurologic involvement would improve prognosis and prevent complications
Summary
Sjögren syndrome (SS) is a rare disease in pediatrics, and little attention has been paid to the clinical feature in these patients. Case presentation: A 12-year-old girl was admitted to our hospital for arthritis and glucosuria She was required to do labial gland and renal biopsy, because the positive for anti-nuclear antibody and anti-Sjögren syndrome B (antiSSB) antibody. Eventually,she was diagnosed as having renal glucosuria (RG) with normal glucose tolerance and normal HbA1c She tested positive for anti-nuclear antibody (ANA) (1:100), anti-Sjögren syndrome B (antiSSB/Ro), Perinuclear antineutrophil cytoplasmic antibody(P-ANCA)(1:10), and ESR (33 mm/h). Tests for antiSjögren syndrome A (anti-SSA/La), anti-Sm, anti-double stranded DNA (anti-dsDNA), anti-ribonnucleoprotein (anti-RNP), ani-Scl, anti-phospholipid, anti-Jo-1, antimyeloperoxidase antineutrophil cytoplasmic antibody (anti-MPO-ANCA), rheumatoid factor (RF), anti-cyclic citrullinated peptide (anti-CCP) antibody, hepatitis B virus (HBV), hepatitis C virus (HCV), human immunodeficiency virus (HIV), IgG, IgA, IgM, C3, and C4 were negative or normal Ophthalmological assessment revealed her schirmer’s test was positive. Histopathological analysis of minor salivary gland biopsy showed a lymphocytic infiltrate around the ducts and acinus in the small area
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
