Antibodies to nucleic acids in congenital immune deficiency states.

The presence of antibodies to native DNA, single-stranded DNA, and double-stranded RNA was determined for 37 patients with selective IgA deficiency, 11 patients with Wiskott-Aldrich syndrome, seven patients with common variable agammaglobulinemia, 14 patients with ataxia telangiectasia, six patients with intestinal lymphangiectasia, and one patient with Nezelof syndrome. Of 37 patients with selective IgA deficiency, 11 had antibodies to at least one nucleic acid; six had antibodies to native DNA, seven had antibodies to single-stranded DNA, and four had antibodies to double-stranded RNA. The only other congenital immune deficiency disease studied in which antibodies to nucleic acids were found was the Wiskott-Aldrich syndrome; in this group three of 11 patients had antibodies to native DNA. Retrospective analysis of our patients with SLE disclosed a 2.6% prevalence of IgA deficiency, a prevalence clearly higher than in the general population. These studies provide further evidence of the association between autoimmunity and abnormalities of IgA production and suggest a relationship between thymic-derived immune regulation and IgA production.

Constrictive pericarditis, intestinal lymphangiectasia, and reversible immunologic deficiency.

A patient with constrictive pericarditis, secondary intestinal lymphangiectasia, and protein-losing enteropathy was demonstrated to have the characteristic immunologic deficiency associated with intestinal lymphangiectasia: hypogammaglobulinemia, lymphocytopenia, cutaneous anergy, impaired allograft rejection. and diminished in vitro lymphocyte proliferative responses. Following surgical correction of the cardiac abnormality, the intestinal lymphangiectasia and protein-losing gastroenteropathy resolved and was accompanied by a slow but progressive return of normal immune function. This documented reversal of the immunologic deficiency in intestinal lymphangiectasia supports the concept that the immune defect in this syndrome is due to the excessive loss of lymphocytes and immunoglobulins into the gastrointestinal tract.