Tubulointerstitial nephritis and uveitis syndrome in Southern Spain

Abstract

Objective: To examine immunogenetic and clinical features in a series of patients with the idiopathic tubulointerstitial nephritis and uveitis (TINU) syndrome diagnosed at the single referral hospital for a defined population in Southern Spain.

Patients and methods: Retrospective study of the case records of all patients diagnosed with the TINU syndrome in the Departments of Ophthalmology and Medicine of the Valme University Hospital (Seville, Spain) from January 1996 through October 2000. Patients were included in this study if they had a renal biopsy showing interstitial edema and infiltration by lymphocytes, plasma cells, macrophages, eosinophils, and neutrophils. In these cases fibrosis was occasionally seen, but no glomerular changes were found. In addition, a diagnosis of uveitis by expert ophthalmologists was always required. Underlying diseases, which might be responsible for the renal or ocular manifestations, were excluded. Patients were HLA-DRB1 genotyped from DNA by using molecular-based methods.

Results: Six patients (4 females) fulfilled the definitions described above. Four were younger than 18 years. In addition to tubulointerstitial nephritis, non-granulomatous uveitis (anterior or panuveitis) associated with low visual acuity was present at the time of diagnosis. Leukocytosis and increase of acute phase reactants were also commonly observed at the time of diagnosis. Topical and oral corticosteroids were prescribed to all the patients. Cyclosporine A therapy was required in 2 cases. After a 2.5-year median follow-up, visual acuity had improved in all cases. Of note, 4 of 6 patients carried the HLA-DRB1*01 allele.

Conclusion: The TINU syndrome should be considered in the differential diagnosis of patients presenting with visual and renal manifestations. The presence of renal dysfunction in patients with uveitis may be of some help, as a warning sign, for the recognition of patients who require a rapid diagnosis and therapy. In Southern Spain, the TINU syndrome appears to be associated with HLA-DRB1*01 allele.