title: Clinicopathologic characteristics, treatment, and outcomes of tubulointerstitial nephritis and uveitis syndrome in adults: A national retrospective strobe-compliant study.
abstract: Tubulointerstitial nephritis and uveitis (TINU) syndrome is a rare disease, defined by the association of idiopathic acute TINU. The aim of our work was to determine the characteristics of adult TINU syndrome in France, and to assess factors (including treatment) influencing medium-term prognosis.We conducted a nationwide study including 20 French hospitals. Clinical, laboratory, and renal histopathologic data of 41 biopsy-proven TINU syndromes were retrospectively collected. The patients were diagnosed between January 1, 1999 and December 1, 2015.Twenty-five females and 16 males were included (F/M ratio: 1.6:1). The median age at disease onset was 46.8 years (range 16.8-77.4) with a median serum creatinine level at 207 μmol/L (range 100-1687) and a median estimated glomerular filtration rate (eGFR) at 27 mL/min per 1.73 m (range 2-73). Twenty-nine patients (71%) had a bilateral anterior uveitis and 24 (59%) had deterioration in general health at presentation. Moderate proteinuria was found in 32 patients (78%) (median proteinuria 0.52 g/24 h; range 0.10-2.10), aseptic leukocyturia in 25/36 patients (70%). The evaluation of renal biopsies revealed 41 patients (100%) with an acute tubulointerstitial nephritis, 19/39 patients (49%) with light to moderate fibrosis and 5 patients (12%) with an acute tubular necrosis. Thirty-six patients (88%) were treated with oral corticosteroids. After 1 year of follow-up, the median eGFR was 76 mL/min per 1.73 m (range 17-119) and 32% of the patients suffered from moderate to severe chronic kidney disease. Serum creatinine (P < 0.001, r = -0.54), serum bicarbonate and phosphate levels (respectively, P = 0.01, r = 0.53; and P = 0.04, r = 0.46), and age (P = 0.03, r = -0.37) at the 1st symptoms were associated with eGFR after 1 year. During the 1st year 40% of patients had uveitis relapses. The use of oral corticosteroids was not associated with a better kidney function but was associated with fewer uveitis relapses (P = 0.44 and 0.02, respectively).In our study, 32% of patients were suffering from moderate to severe chronic kidney disease after 1 year of follow-up, and 40% had uveitis relapses during this follow-up. This work also suggests that oral corticosteroids are effective for the treatment of TINU syndrome's uveitis.
authors.affiliation: aNephrology Department, Bocage University Hospital and UMR 1098 bInternal Medicine and Systemic Diseases Department, Bocage University Hospital, Dijon cInternal Medicine Department, Edouard Herriot University Hospital and University Claude Bernard Lyon 1, Lyon dInternal Medicine Department, National Referral Center for Rare Autoimmune and Systemic Diseases, Cochin University Hospital and University Paris Descartes, Paris eNephrology Department, Macon Hospital, Mâcon fNephrology Department, La Conception University Hospital and University Aix-Marseille, Marseille gInternal Medicine Department, Ambroise-Paré University Hospital, Paris hNephrology Department, Maison Blanche University Hospital, Reims iInternal Medicine Department, University Hospital Avicenne and University Sorbonne, Paris jNephrology Department, Tenon University Hospital, Paris kNephrology Department, Brabois University Hospital and INSERM CIC-EC CIE6, Nancy lNephrology Department, Dole Hospital, Dôle mNephrology Department, Ambroise-Paré University Hospital, Boulogne-Billancourt nInternal Medicine and Clinical Immunology Department, Pitié Salpetriere University Hospital and DHU Inflammation, Immunopathology, Biotherapy, Paris VI oInternal Medicine Department, University Hospital Clermont Ferrand, Clermont-Ferrand pInternal Medicine Department, Quinze-Vingts Ophthalmogy Hospital, Paris qNephrology Department, University Hospital, Rennes rNephrology Department, University Hospital Claude Huriez, Lille sRheumatology Department, National Referral Center for Rare Autoimmune Diseases, Strasbourg University Hospital, and UMR 1109, Strasbourg, France.
authors.forename: Mathieu
authors.initials: M
authors.lastname: Legendre
authors.forename: Hervé
authors.initials: H
authors.lastname: Devilliers
authors.forename: Laurent
authors.initials: L
authors.lastname: Perard
authors.forename: Matthieu
authors.initials: M
authors.lastname: Groh
authors.forename: Habdelamid
authors.initials: H
authors.lastname: Nefti
authors.forename: Bertrand
authors.initials: B
authors.lastname: Dussol
authors.forename: Salim
authors.initials: S
authors.lastname: Trad
authors.forename: Fatouma
authors.initials: F
authors.lastname: Touré
authors.forename: Sébastien
authors.initials: S
authors.lastname: Abad
authors.forename: Jean-Jacques
authors.initials: JJ
authors.lastname: Boffa
authors.forename: Luc
authors.initials: L
authors.lastname: Frimat
authors.forename: Stéphane
authors.initials: S
authors.lastname: Torner
authors.forename: Alexandre
authors.initials: A
authors.lastname: Seidowsky
authors.forename: Ziad André
authors.initials: ZA
authors.lastname: Massy
authors.forename: David
authors.initials: D
authors.lastname: Saadoun
authors.forename: Virginie
authors.initials: V
authors.lastname: Rieu
authors.forename: Yoland
authors.initials: Y
authors.lastname: Schoindre
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Heron
authors.forename: Thierry
authors.initials: T
authors.lastname: Frouget
authors.forename: Arnaud
authors.initials: A
authors.lastname: Lionet
authors.forename: François
authors.initials: F
authors.lastname: Glowacki
authors.forename: Laurent
authors.initials: L
authors.lastname: Arnaud
authors.forename: Christiane
authors.initials: C
authors.lastname: Mousson
authors.forename: Jean-François
authors.initials: JF
authors.lastname: Besancenot
authors.forename: Jean-Michel
authors.initials: JM
authors.lastname: Rebibou
authors.forename: Philip
authors.initials: P
authors.lastname: Bielefeld
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000368:Aged; D005260:Female; D005602:France; D006801:Humans; D008297:Male; D008875:Middle Aged; D009395:Nephritis, Interstitial / Q000175:diagnosis* / Q000188:drug therapy*; D012189:Retrospective Studies; D016896:Treatment Outcome; D014605:Uveitis / Q000175:diagnosis* / Q000188:drug therapy*; D055815:Young Adult
chemical_list: D000305:Adrenal Cortex Hormones