In 10 of 14 patients with an active cytomegalovirus (CMV) infection, distinctive large cells (35-45 microns in diameter) were present in the peripheral blood. Morphologically these cells closely resembled the classic cytomegalic inclusion cells, generally regarded as a diagnostic hallmark of CMV infection. Moreover, these cells were shown to express CMV antigens belonging to all three stages of the viral replication cycle, indicating a productive CMV infection. In addition, immunologic staining with monoclonal antibodies directed against cell differentiation and marker proteins showed that these circulating cytomegalic cells were of endothelial origin. The presence of CMV-infected endothelial cells in the peripheral blood of patients with an active CMV infection indicates that such an infection might be accompanied by widespread occult vascular damage.
To investigate the possible association of crescentic glomerulonephritis (CGN) with autoantibodies to myeloid lysosomal enzymes, we tested sera from 35 consecutive patients with CGN without diagnostic immunohistological findings in their renal biopsy for the presence of antineutrophil cytoplasmic antibodies directed against a 29 kD antigen from azurophilic granules (29 kD-ANCA), against myeloperoxidase (MPO-ANCA) and against elastase (elastase-ANCA), using antigen-catching ELISAs with well-defined monoclonal antibodies. 29 kD-ANCA were present in the sera of all nine patients with CGN as part of biopsy-proven Wegner's granulomatosis (WG), of ten patients with CGN and clinically suspected WG, and of two patients with idiopathic CGN. Sera from the remaining patients with clinically suspected WG (N = 5) or idiopathic CGN (N = 6) were negative for 29 kD-ANCA, but invariably positive for MPO-ANCA. Neither of these antibodies could be detected in sera from patients with CGN of infectious origin (N = 3), different forms of CGN (N = 7), other renal lesions (N = 34), or normal controls (N = 52). None of the sera tested were positive for elastase-ANCA. Our results indicate that both vasculitis-associated CGN and idiopathic CGN are associated with autoantibodies against myeloid lysosomal enzymes. This finding places these disorders within one spectrum of diseases.
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