Naslov
Acute tubulointerstitial nephritis in pediatric primary Sjogren syndrome

Autori
Trutin, Ivana ; Sitaš, Ingrid ; Perić Marković, Gabrijela ; Vidović, Mandica ;

Izvornik
Pediatric nephrology (0931-041X) 1 (2021); 1, 1

Vrsta, podvrsta i kategorija rada
Radovi u časopisima, članak, znanstveni

Ključne riječi
sjorgen, children, tubulinterstitial nephritis

Sažetak
Introduction: Sjögren syndrome (SS) is a chronic autoimmune disease primarily affecting lachrymal and salivary glands with varying degrees of systemic involvement. Clinical manifestations differ in children and do not meet currently available adult criteria (American European consensus group - AERCG). Recurrent parotid swelling is often observed in children unlike oral and ocular involvement (sicca syndrome) which are specific for adults. Extraglandular involvement ia rare, only 9.9% patients having renal involvement. Pathologic and laboratory findings include lymphocytic infiltration of the exocrine glands, presence of rheumatoid factor (RF) and specific antinuclear antibody (ANA). We present a case of primary SS with renal involvement. Material and methods: A 12-year-old girl with history of recurrent left-sided parotid enlargement episodes and sicca syndrome was admitted to our Department for evaluation. Her mother was diagnosed with systemic lupus in adolescence. Results: Laboratory screening showed highly positive ANA (1:1280), anti-SS-A 122 U/mL, anti-SS-B 176 U/mL and RF (762 U/mL). Schimmer’s test verified moderate hypolacrimation. Salivary gland biopsy confirmed pSS diagnosis. Since there was persistent proteinuria in a 24-hour urine (247 mg/24h) renal biopsy was performed and acute tubulointerstitial nephritis was confirmed by pathohystological evaluation. The patient received pulse corticosteroid therapy. On her last follow there was no proteinuria with low dose prednisone and hydroxichlorochine therapy. mononuclear inflammatory interstitial infiltration (yellow arrow). Courtesy of Prof D.. Ljubanović Conclusions: pSS is rare and often unrecognized condition in children. The lack of pediatric diagnostic criteria and underdiagnosed extraglandular involvement can affect course of disease. Early renal involvement recognition and adequate therapy can prevent progressive disease. Additional studies in children are needed in establishing clasification criteria and therapeutic algoritams to enable further clinical and translational research.

Izvorni jezik
Engleski

POVEZANOST RADA