Circulating immunoglobulin complexes in Wegener's granulomatosis

Abstract

Two cases of Wegener's granulomatosis are reported in which circulating immunoglobulin complexes detected during the active phase of the disease disappeared during induction of remissions of active pulmonary and renal disease by immunosuppressive agents. Elevated antiglobulin activity, urinary immunoglobulin L-chain concentration and evidence of activated coagulation mechanisms were also present during active disease, and returned toward normal with treatment. Studies showed that the serum complexes did not contain deoxyribonucleic acid (DNA) and demonstrated their immunoglobulin nature. Serum complement concentrations were normal, and no cryoglobulins were present. Immunofluorescent staining and electron microscopy of the kidney biopsy specimen of one patient showed marked fibrin deposition but no immunoglobulin or antigen-antibody deposits. Although the role of circulating immunoglobulin complexes in the pathogenesis of Wegener's granulomatosis remains uncertain, serial changes in the described parameters may provide an objective guide to activity of the disease and its response to treatment.