Sjögren's syndrome-like illness associated with the acquired immunodeficiency syndrome-related complex

Three cases of Sjögren's syndrome-like illness occurring in patients with the acquired immunodeficiency syndrome (AIDS)-related complex (ARC) are described. All three patients were male. Positive serologic tests for the human immunodeficiency virus (HIV) were documented in two; the third patient was a prison inmate with a history of drug abuse. The lymphoid infiltrate seen in these cases resembled morphologically the features of persistent generalized lymphadenopathy. One patient complained of dry eyes and arthralgias. Autoimmune phenomena including lupus-like anticoagulant, immune thrombocytopenic purpura, and others have been reported in patients with AIDS and ARC. The occurrence of Sjögren's syndrome in ARC provides further evidence for autoimmune phenomena arising in the severely damaged immune system of ARC patients. Atypical Sjögren's syndrome now requires evaluation for ARC. Proposed criteria for identifying these patients are as follows: 1) young age (less than 40 years); 2) male sex (less than 10 per cent of non-ARC patients are male); 3) homosexuality or bisexuality, or other high-risk groups for AIDS; 4) generalized lymphadenopathy (also seen in rheumatoid arthritis); 5) negative test for rheumatoid factor despite generalized lymphadenopathy; 6) salivary gland lymphoid infiltrate showing features of persistent generalized lymphadenopathy. Patients with such features should be studied for HIV antibodies and other evidence of autoimmune phenomena in order to define more precisely the nature of this new Sjögren's-like illness.