Acute systemic lupus erythematosus in IgA deficiency
John D. Clough, M.D.
Department of Rheumatic Disease, Department of Immunopathology
Treatment of systemic lupus erythematosus (SLE) that has failed to respond to steroids poses a difficult problem. Various regimens involving the long-term use of cytotoxic drugs such as cyclophosphamide,1 azathioprine,2 or chloram-bucil3 have been proposed; such drugs are considered warranted in cases where renal involvement is severe. Occasionally, however, a patient with only mild renal involvement may have an acute attack of SLE that does not respond to moderate doses of steroid. In this situation, a short course of a fast-acting cytotoxic drug followed by more conventional therapy may be useful.
This paper reports on the effectiveness of intravenous nitrogen mustard therapy in subduing an acute attack of SLE which had been unsuccessfully treated with steroid. It also exemplifies the common occurrence of SLE in patients with IgA deficiency4 and the modifications of the usual pathogenic mechanisms this may imply.
Materials and methods
Fluorescent antinuclear antibody tests and lupus erythematosus cell preparations were performed by the method described by Garewal and Deodhar.5 Anti-DNA was determined by passive hemagglutination with a modification of the method of Bank-hurst, using high polymerized DNA* which was heat denatured before coupling with sheep erythrocytes in the presence of chromic chloride. C3 (B1C/B1A) levels were determined by radial immunodiffusion with the use of commercially available materials.† Hemolytic complement titers were measured by the method of Kabat and Mayer.6
Serum content of components of the alternate pathway to C3 activation (properdin system) was assayed as follows: 0.1 mg of zymosan, 0.1 ml of patient's serum, and 106. . .