Tubulointerstitial Nephritis and Uveitis Syndrome: A Systematic Review

Authors

  • Joana Pereira Faculty of Medicine of the University of Porto, Porto, Portugal
  • Paulo Freitas-da-Costa 1- Faculty of Medicine of the University of Porto, Porto, Portugal; 2 - Centro Hospitalar Universitário de São João, Porto, Portugal; 3 - Department of Ophthalmology, Faculty of Medicine of Porto University, Porto, Portugal
  • Luís Figueira 1- Faculty of Medicine of the University of Porto, Porto, Portugal; 2 - Centro Hospitalar Universitário de São João, Porto, Portugal; 3 - Department of Ophthalmology, Faculty of Medicine of Porto University, Porto, Portugal

DOI:

https://doi.org/10.48560/rspo.26970

Keywords:

Nephritis, Interstitial, Syndrome, Uveitis

Abstract

INTRODUCTION : Tubulointerstitial nephritis and uveitis syndrome is a rare disease characterized by the occurrence of tubulointerstitial nephritis and uveitis, in the absence of other explain- able systemic disease. In this review we aim to appraise and to clarify what is acknowledged in order to elucidate the demographics, genetic predisposition, most frequent symptoms and laboratory findings, most adequate treatment and also patient’s prognosis.

METHODS : A systematic review across PubMed, Web of Science and Scopus was performed, according to PRISMA guidelines, in order to identify all relevant articles regarding both tubulointerstitial nephritis and uveitis.

RESULTS: We identified 240 publications, of which 176 were excluded. The average age of Tubulointerstitial nephritis and uveitis syndrome diagnosis was 30.6 years-old, with a female predominance (2:1); according to presentation symptoms, tubulointerstitial nephritis precedes uveitis more commonly and amongst uveitis cases the majority were bilateral anterior uveitis. Moreover, the most common systemic symptoms were fatigue, fever, and weight loss; blood analysis commonly presented with elevated serum creatinine, erythrocyte sedimentation rate, C-reactive protein, blood urea nitrogen level and urinalysis frequently showed proteinuria, glycosuria, and elevated urinary-β2-microglobulin. The majority (78.8%) of patients were putted on corticosteroids, with 21.9% needing an additional immunosuppressor, being mycophenolate mofetil (29.8%) and azathioprine (28.0%) the preferred ones. The mean time until relapse, which occurred in 48.3% of patients, was 89.3 days.

CONCLUSION: Tubulointerstitial nephritis and uveitis syndrome is not just a pediatric syndrome, as once thought. Presentation seems to be variable, although tubulointerstitial nephritis typically presents first, with systemic symptoms such as fatigue. As clinicians becomes more aware of the disease, follow-up after the first symptoms allows for earlier diagnosis. Relatively to treatment, most patients responded to corticosteroids, and despite the relapses, the prognosis was generally favorable.

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Published

2022-09-30

How to Cite

Pereira, J., Freitas-da-Costa, P., & Figueira, L. (2022). Tubulointerstitial Nephritis and Uveitis Syndrome: A Systematic Review. Revista Sociedade Portuguesa De Oftalmologia, 46(3), 160–175. https://doi.org/10.48560/rspo.26970

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Review Article