Lymphoid interstitial lung disease in a patient with acute tubulointerstitial nephritis and uveitis: a new facet of a rare syndrome?

We report for the first time the appearance of lymphoid interstitial lung disease in a patient with tubulointerstitial nephritis and uveitis (TINU) syndrome. A 41-year-old woman with a 9-month history of recurrent bilateral iridocyclitis was referred to our division for examination of renal dysfunction. Renal biopsy showed extensive interstitial CD3+ lymphocytic infiltration; glomerular structures were unaffected. After oral corticosteroid therapy, renal function remained stable during the next few years. Uveitis was controlled by using topical steroids. Five years after first presentation, the patient developed symptoms suggestive of interstitial lung disease, combined with a relapse of uveitis. Lung biopsy showed lymphofollicular bronchiolitis (CD3+) associated with florid alveolitis. There was no evidence for acute infection or systemic disease to be the cause of this pathological state. Lung disease proved to be steroid responsive. A new pulmonary flare-up 3 years later responded to renewed treatment. Accounting for the histological findings, we assume both TINU syndrome and interstitial lung disease to be manifestations of a common autoimmune disorder.