Serum IgA deficiency induced by prolonged phenytoin treatment.

In a longitudinal study we have evaluated several immunological parameters in thirty three epileptic children and adolescents 4 to 14 years old treated with phenytoin, and matched normal controls. The patients had significantly lower levels than normal controls of IgA (153 +/- 89 vs 236 +/- 128 mg/dL p less than 0.001) and IgM (155 +/- 58 vs 217 +/- 105 mg/dL p less than 0.01). The decrease in serum IgA levels correlated with the length of treatment (r = 0.44, p less than 0.03). Eight of the patients had IgA deficiency. In 7 of these children, T lymphocytes subpopulations were determined. The results did not differ significantly from the matched controls.

T-cell subpopulations in the peripheral blood of patients with connective tissue diseases as determined by flow cytometry using monoclonal antibodies.

We studied by flow cytometry using monoclonal antibodies the T3, T4, and T8 subpopulations of T cells in the peripheral blood of 109 patients with various connective tissue diseases who were not receiving any treatment. Comparison of the results was made with those obtained with normal controls matched for age and sex with each connective tissue disease group. When compared as disease groups, patients with systemic lupus erythematosus (n = 41) had decreased T8 cells, but patients with active disease (n = 17) had all three T-cell subpopulations lower than their controls, whereas those with inactive disease (n = 24) showed no differences. Patients with rheumatoid arthritis (n = 23) had decreased T3 and T8 cells, whereas patients with scleroderma (n = 22) only had decreased T3 cells, and patients with primary Sjögren's syndrome (n = 15) had lower proportions of all three T-cell subpopulations than their matched controls. Patients with mixed connective tissue disease (n = 8) had proportions of all three T-cell subpopulations akin to those of their matched controls, but showed a tendency to have decreased T8 cells that reached statistical significance when compared to the entire control group. Although our findings tend to support the notion that the abnormalities in immunoregulatory T-cell circuits leading to autoimmunity are different in each connective tissue disease, the great variability found in both patients and controls seems to preclude the use of these determinations in individual patients for clinical purposes.

Splenectomy for hemocytopenia in systemic lupus erythematosus. A controlled appraisal.

We compared 15 patients with systemic lupus erythematosus (SLE) treated with splenectomy for thrombocytopenic purpura and/or hemolytic anemia to 15 similar SLE patients treated only medically. There was no significant difference between the splenectomized and the nonsplenectomized patients when their entire course, as well as the presplenectomy and postsplenectomy or their equivalent control periods, were compared by means of an overall severity index. Splenectomized patients, however, had a significantly higher frequency of cutaneous vasculitis after splenectomy than in their own presplenectomy period and a significantly higher frequency of cutaneous vasculitis than the nonsplenectomized patients. Serious infections were more frequent in the postsplenectomy period than in an equivalent period in the nonsplenectomized patients. Splenectomy produced only short-term benefit in the management of hemocytopenic episodes in SLE and seems only warranted as an emergency procedure in patients unresponsive to medical treatment.

Lymphopenia in systemic lupus erythematosus. Clinical, diagnostic, and prognostic significance.

One hundred fifty-eight patients with active, untreated systemic lupus erythematosus (SLE) were studied from the time of diagnosis. Lymphopenia was present in 75%, and another 18% of those patients developed lymphopenia subsequent to disease reactivation. Lymphopenia of less than 1500 cells/microliter occurred more frequently than any of the preliminary criteria for the classification of SLE, and it was the most prevalent initial laboratory abnormality. Lymphocyte counts were significantly lower in lupus than in the other connective tissue diseases except mixed connective tissue disease and polymyositis.

T-lymphocyte subpopulation in untreated SLE. Variations with disease activity.

An active subpopulation of T lymphocytes characterized by their ability to form early rosettes with sheep erythrocytes (active E-RBL) was studied in the blood of 50 patients with untreated systemic lupus erythematosus (SLE) and in 50 normal controls. The findings were related to the absolute number of circulating lymphocytes and total E-receptor-bearing lymphocytes (total E-RBL). Lupus patients with active disease had markedly decreased absolute lymphocyte counts, but the decrease of both the total and the active E-RBL surpassed what would be expected from the lymphopenia. Patients with inactive disease had moderately decreased absolute lymphocyte counts with a marked and disproportionate decrease in total E-RBL and a moderate decrease in active E-RBL, which seemed to reflect only the absolute lymphopenia. Patients with active disease had significantly lower active E-RBL than those with inactive disease. The changes of these and other lymphocyte subpopulations in relation to disease activity in SLE may reflect the influence of factors leading to T-cell depletion and immaturity. Circulating thymic products may be one of those factors.

Hemolytic anemia and thrombocytopenic purpura: two related subsets of systemic lupus erythematosus.

Systemic lupus erythematosus patients who develop hemolytic anemia or thrombocytopenic purpura differ from other lupus patients and are similar enough to be considered two related subsets with a more benign course. Thirty-one lupus patients with either or both these hemocytopenias were found to be significantly younger, more often males, and had less frequent fever, polyarthritis, serositis, cutaneous vasculitis, nephropathy, neurologic manifestations, and persistent hypocomplementemia than 62 lupus patients without any of these hemocytopenias. They also had lower index scores of overall disease severity and required less treatment. It seems important to subdivide lupus patients in subsets for therapeutic and prognostic purposes.