Acute interstitial nephritis with symmetric enlargement of the lacrymal and salivary glands and systemic lymphadenopathy.

We describe a 40-year-old man who developed symmetric enlargement of the lacrymal and salivary glands and systemic lymphadenopathy. Laboratory findings included eosinophilia, polyclonal hypergammaglobulinemia, elevated circulating immune complexes, hypocomplementemia, and renal insufficiency. The lymph node pathology was atypical of angioimmunoblastic lymphadenopathy with disproteinemia (AILD), in that it lacked a prominence of arborizing small vessel proliferation. Despite extensive examinations, the cause of the atypical AILD process has not been identified. However, a flow-cytometric analysis of immunophenotypes of lymphoid cells from the lymph node revealed a predominance of activated helper/inducer T cells, indicating a hyperimmune state. Acute interstitial nephritis was secondary to the renal propagation of the atypical AILD process. Thus, atypical AILD can be a new cause of acute interstitial nephritis. A course of combined steroids and immunosuppressive treatment resolved those pathologic disorders for 5 years.