Pulmonary renal syndrome due to anti-GBM and IgM C-ANCA disease requiring the use of novel therapeutic agents.
There are several known causes for the clinical syndrome of pulmonary hemorrhage and acute renal failure. Here, we report a unique case of a 50-year-old man presenting in this manner. The initial diagnosis was one of antiglomerular basement membrane (anti-GBM) disease that responded well to steroids, cyclophosphamide, and plasma exchange (PE). The pulmonary hemorrhage resolved, but he remained dialysis dependent. However, despite falling anti-GBM titers, the symptoms relapsed and standard therapy was reinitiated with limited success. The anti-GBM antibody titer fell to zero despite clinical deterioration, prompting a search for an alternative diagnosis. He was found to be IgM anti-proteinase-3 antineutrophil cytoplasmic antibody (C-ANCA) positive. The pulmonary hemorrhage responded successfully to the use of intravenous immunoglobulin and the antilymphocyte monoclonal antibody CD52. To our knowledge, this is the first known case of IgM C-ANCA in association with anti-GBM disease. As such, it highlights the predominance of pulmonary hemorrhage in this condition, as well as the need to consider alternative therapies in refractory cases.
O'Riordan
Aisling
A
St. Vincent's University Hospital, Dublin, Ireland. aisling32@ireland.com
Keogh
J A Brian
JA
Watson
Alan
A
D019867:Anti-Glomerular Basement Membrane Disease / Q000276:immunology / Q000473:pathology* / Q000628:therapy*; D000906:Antibodies / Q000032:analysis / Q000276:immunology*; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000032:analysis / Q000276:immunology*; D001323:Autoantibodies; D003131:Combined Modality Therapy; D004636:Emergency Service, Hospital; D005500:Follow-Up Studies; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007676:Kidney Failure, Chronic / Q000276:immunology / Q000473:pathology / Q000628:therapy*; D016030:Kidney Transplantation / Q000379:methods; D008297:Male; D008875:Middle Aged; D006435:Renal Dialysis / Q000379:methods; D018570:Risk Assessment; D012720:Severity of Illness Index; D016896:Treatment Outcome
D000906:Antibodies; D019268:Antibodies, Antineutrophil Cytoplasmic; D001323:Autoantibodies; D016756:Immunoglobulins, Intravenous; C051951:antiglomerular basement membrane antibody