Transition from systemic lupus erythematosus to common variable hypogammaglobulinemia.

Abstract:

:A 34-year-old man had polar extremes of B cell dysfunction: systemic lupus erythematosus evolving into common variable hypogammaglobulinemia. He presented in 1974 with seizures and six other criteria for systemic lupus erythematosus; his antinuclear antibody titer was 1:1024 and IgG level, 2870 mg/dL. After 5 months of immunosuppressive treatment, a 79% decrease in serum IgG and 95% decrease in IgA levels occurred and manifestations of systemic lupus erythematosus disappeared. Six years later, he developed panhypogammaglobulinemia, had recurrent sinopulmonary infections, and showed nodular lymphoid hyperplasia on rectal biopsy. For comparison, serum immunoglobulin concentrations were measured serially in 13 other patients with systemic lupus erythematosus. Three developed severe depressions of these levels, two with IgG levels less than 300 mg/dL and one with an IgA level of 8 mg/dL. These decreases were transient, related to treatment, and not associated with infections. Daily high-dose prednisone therapy (60 mg/d) rather than treatment with cytotoxic drugs correlated with decreased immunoglobulin concentrations.

journal_name

Ann Intern Med

authors

Sussman GL,Rivera VJ,Kohler PF

doi

10.7326/0003-4819-99-1-32

subject

Has Abstract

pub_date

1983-07-01 00:00:00

pages

32-5

issue

1

eissn

0003-4819

issn

1539-3704

journal_volume

99

pub_type

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