Anti-neutrophil cytoplasmic antibody vasculitis presenting with bilateral renal vein thrombosis.

We report a case of anti-neutrophil cytoplasmic antibody (ANCA)-associated necrotizing crescentic glomerulonephritis presenting with bilateral renal vein thrombosis and pulmonary emboli in a patient who also had a lupus anticoagulant and anti-cardiolipin antibodies. Although the link between venous thrombosis and ANCA vasculitis is well established, the coexistence of renal vein thrombosis is unusual. Furthermore, despite the positive ANCA, he was initially negative for antibodies to myeloperoxidase (MPO) and proteinase-3 (PR3), illustrating that a positive ANCA may be significant despite a negative test for antibodies to MPO and PR3.

Transferred antigen-specific T(H)17 but not T(H)1 cells induce crescentic glomerulonephritis in mice.

To explore the role of antigen-specific CD4(+) T cells in glomerulonephritis, we administered ovalbumin 323-339 peptide conjugated to glomerular-binding polyclonal antibody and induced disease in RAG1(-/-) mice with CD4(+) T cells from OT2 × RAG1(-/-) mice. These OT2 × RAG1(-/-) mice have a transgenic T-cell receptor specific for this peptide. When CD4(+) T cells were primed in vivo, crescentic glomerulonephritis developed after 21 days in mice given peptide-conjugated glomerular-binding antibody but not unconjugated antibody control.