Tubulointerstitial nephritis and renal tubular acidosis of different types are rare but important complications of primary biliary cirrhosis.

Background: A very few cases of biopsy-proven tubulointerstitial nephritis (TIN) in patients with primary biliary cirrhosis (PBC) have been reported. Although the clinical importance of this association has been suggested, information on its clinicopathological features and prognosis is limited.

Methods: We reviewed 5955 renal biopsies processed at our department, and identified four patients with TIN associated with asymptomatic PBC. We evaluated clinicopathological features and outcomes in these patients, and reviewed the previously reported cases of TIN associated with PBC.

Results: Our four patients were female. The patients' age at the time of renal biopsy ranged from 36 to 77. Three patients had been treated with ursodeoxycholic acid. All patients had urinary abnormalities such as proteinuria and elevated levels of urinary β(2)-microglobulin, and three patients had renal insufficiency. All patients had distal renal tubular acidosis (RTA), and two patients also had Fanconi syndrome. Renal biopsy showed severe lymphocyte infiltration in the tubules and interstitium with mild-to-moderate tubular atrophy and fibrosis. All patients responded well to steroid therapy. On review of the previously reported five cases, all patients were female. The patients' age ranged from 42 to 68. Apparent symptoms linked to PBC were not described. All patients had renal insufficiency. Three patients suffering from bone pains or bone fractures also had Fanconi syndrome. Marked or transient improvements were observed after steroid therapy in three patients.

Conclusions: TIN and RTA of different types are extremely rare but one of the important extrahepatic complications of PBC. Steroid therapy can be beneficial in treating PBC patients with these renal complications.