Sjögren's syndrome-like illness associated with the acquired immunodeficiency syndrome-related complex. 1987

R C Ulirsch, and E S Jaffe
Hematopathology Section, National Cancer Institute, Bethesda, MD 20892.

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.

UI MeSH Term Description Entries
D008198 Lymph Nodes They are oval or bean shaped bodies (1 - 30 mm in diameter) located along the lymphatic system. Lymph Node,Node, Lymph,Nodes, Lymph
D008297 Male Males
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D000386 AIDS-Related Complex A prodromal phase of infection with the human immunodeficiency virus (HIV). Laboratory criteria separating AIDS-related complex (ARC) from AIDS include elevated or hyperactive B-cell humoral immune responses, compared to depressed or normal antibody reactivity in AIDS; follicular or mixed hyperplasia in ARC lymph nodes, leading to lymphocyte degeneration and depletion more typical of AIDS; evolving succession of histopathological lesions such as localization of Kaposi's sarcoma, signaling the transition to the full-blown AIDS. ARC,Lymphadenopathy Syndrome,AIDS Related Complex,Complex, AIDS-Related,Lymphadenopathy Syndromes,Syndrome, Lymphadenopathy,Syndromes, Lymphadenopathy
D001706 Biopsy Removal and pathologic examination of specimens from the living body. Biopsies
D012469 Salivary Glands Glands that secrete SALIVA in the MOUTH. There are three pairs of salivary glands (PAROTID GLAND; SUBLINGUAL GLAND; SUBMANDIBULAR GLAND). Gland, Salivary,Glands, Salivary,Salivary Gland
D012859 Sjogren's Syndrome Chronic inflammatory and autoimmune disease in which the salivary and lacrimal glands undergo progressive destruction by lymphocytes and plasma cells resulting in decreased production of saliva and tears. The primary form, often called sicca syndrome, involves both KERATOCONJUNCTIVITIS SICCA and XEROSTOMIA. The secondary form includes, in addition, the presence of a connective tissue disease, usually rheumatoid arthritis. Sicca Syndrome,Sjogren Syndrome,Sjogrens Syndrome,Syndrome, Sicca,Syndrome, Sjogren's

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