IgG4-related tubulointerstitial nephritis associated with chronic lymphocytic leukemia

Tubulointerstitial nephritis (TIN) is typically seen in association with drug exposure and infection or in autoimmune diseases such as Sjogren's syndrome or systemic lupus erythematosis. The recently described IgG4-related systemic diseases can affect many organ systems including the kidney and typically respond to corticosteroid treatment.

We present a case of IgG4-related TIN in a patient with concomitant chronic lymphocytic leukemia. To our knowledge, IgG4-related TIN has not been associated with any hematological disorder such as chronic lymphocytic leukemia.

We propose that all kidney biopsies with significant plasma cell infiltrate should be stained for IgG4 as response to treatment is common.