Angioimmunoblastic lymphadenopathy in a child with unusual clinical and immunologic features.

We report a case of angioimmunoblastic lymphadenopathy in a child followed for 13 years. Unusual features include prolonged course, cold urticaria, nonthrombocytopenic purpura, poor wound healing, transfusion reactions, and possible neurologic involvement with cerebritis and epileptic seizures. The patient's serum contained a monoclonal cryoglobulin, immunoglobulin G, kappa light chain type, that activated the classic complement pathway in vitro and mediated passive transfer of the cold urticaria. The patient responded well to corticosteroids and has been in clinical remission for 8 years without specific treatment. There is immunologic evidence of persistent residual disease activity. This case illustrates the remarkable diversity of clinical and immunologic features and the variable prognosis of this disorder.

Immunological parameters and alpha 1-antitrypsinin chronic urticaria.

Serum immunoglobulins, complement and alpha 1-antitrypsin were assayed in forty-eight patients with chronic urticaria. Thirteen cases had chronic cold urticaria and thirty-two had chronic idiopathic urticaria. Elevated mean serum IgM was found in chronic cold urticaria. Seven patients had partial immunoglobulin deficiencies. IgE was elevated in sixteen cases of chronic idiopathic and in two with chronic cold urticaria. Eight patients had depressed serum total haemolytic complement activity. Low C3 and normal C4 serum protein concentrations in four cases suggested alternative complement pathway activation. Twenty of forty-six patients were atopic, although specific allergies responsible for the urticaria were not identified in any of them. alpha 1-antitrypsin levels were normal in all patients. The data suggest that the aetiology and pathogenesis of chronic urticarias in this study are heterogeneous. No evidence of abnormality of the protease inhibitor system in either chronic idiopathic or chronic cold urticaria was found.

Antibody to extractable nuclear antigen in the rheumatic diseases.

Two hundred and eighty-four patients with various rheumatic diseases were studied for the prevalence of antibodies to extractable nuclear antigen (anti-ENA) using an improved haemagglutination technique. Anti-ENA rarely occurred in patients with rheumatoid arthritis, scleroderma, polymyositis, dermatomyositis and Sjögren's syndrome. Seventeen per cent (13/72) of patients fulfilling at least four preliminary ARA criteria for systemic lupus erythematosus (SLE) demonstrated anti-ENA. The predominant antibody was directed at ribo-nuclease-resistant ENA (anti-Sm). Antibodies to ribo-nuclease-sensitive ENA (anti-RNP) occurred in the minority of patients with SLE (2/72) and the mixed connective tissue disease syndrome (MCTD). A trend toward an increased incidence of renal disease in SLE patients with anti-Sm was present. Sequential analysis of anti-Sm in patients with SLE showed a fall in titre paralleling the normalization of anti-DNA antibody titres and serum complement values. No case of unrecognized MCTD was uncovered in our rheumatic disease population.