Niles Lab: John L. Niles, MD

Overview

We have shown that antibodies to proteinase 3 and myeloperoxidase are highly specific markers for the spectrum of vasculitis that includes Wegener's Granulomatosis, microscopic polyarteritis (polyangiitis), Churg-Strauss syndrome and pauci-immune necrotizing and crescentic glomerulonephritis with or without lung hemorrhage. By reviewing large numbers of patients and testing large control groups, we have determined the diagnostic value of these tests in a variety of clinical situations. Currently, we are working to further define the role of tests for ANCA and anti-glomerular basement membrane antibodies in the evaluation of pulmonary hemorrhage syndrome.

Another focus of our work is to define the value of serial tests for ANCA in the long term management of patients with the above spectrum of diseases. These patients require long term treatment with immunosuppression. Balancing the treatment between over immunosuppression and recurrence of disease has been a major clinical challenge. ANCA appear to be a highly useful tool in the management of these patients.

In addition to research regarding ANCA associated disease, I run a clinical practice that routinely sees patients with ANCA disease 3 days a week and provides coverage for these patients on a 24 hour per day, 7 day a week basis.

References

1. Niles JL, McCluskey RT, Ahmad MF, Arnaout MA. Wegener's granulomatosis autoantigen is a novel neutrophil serine proteinase. Blood. 1989; 74(6):1888-93.
2. O'Sullivan BP, Erickson LA, Niles JL. Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 30-2002. An eight-year-old girl with fever, hemoptysis, and pulmonary consolidations. N Engl J Med. 2002; 347(13):1009-17.
3. Han WK, Choi HK, Roth RM, McCluskey RT, Niles JL. Serial ANCA titers: useful tool for prevention of relapses in ANCA-associated vasculitis. Kidney Int. 2003; 63(3):1079-85.
4. Rhee EP, Laliberte KA, Niles JL. Rituximab as maintenance therapy for anti-neutrophil cytoplasmic antibody-associated vasculitis. Clin J Am Soc Nephrol. 2010; 5(8):1394-400.
5. McGrath MM, Isakova T, Rennke HG, Mottola AM, Laliberte KA, Niles JL. Contaminated cocaine and antineutrophil cytoplasmic antibody-associated disease. Clin J Am Soc Nephrol. 2011; 6(12):2799-805.
6. Roth AJ, Ooi JD, Hess JJ, van Timmeren MM, Berg EA, Poulton CE, McGregor J, Burkart M, Hogan SL, Hu Y, Winnik W, Nachman PH, Stegeman CA, Niles J, Heeringa P, Kitching AR, Holdsworth S, Jennette JC, Preston GA, Falk RJ. Epitope specificity determines pathogenicity and detectability in ANCA-associated vasculitis. J Clin Invest. 2013; 123(4):1773-83.
7. Pendergraft WF, McGrath MM, Murphy AP, Murphy P, Laliberte KA, Greene MF, Niles JL. Fetal outcomes after rituximab exposure in women with autoimmune vasculitis. Ann Rheum Dis. 2013; 72(12):2051-3.
8. Pendergraft WF, Cortazar FB, Wenger J, Murphy AP, Rhee EP, Laliberte KA, Niles JL. Long-term maintenance therapy using rituximab-induced continuous B-cell depletion in patients with ANCA vasculitis. Clin J Am Soc Nephrol. 2014; 9(4):736-44.
9. Venna N, Niles JL, Lev MH, Frosch MP. Case records of the Massachusetts General Hospital. Case 21-2014. A 68-year-old man with a sudden onset of diplopia. N Engl J Med. 2014; 371(2):162-73.
10. Pendergraft WF, Herlitz LC, Thornley-Brown D, Rosner M, Niles JL. Nephrotoxic effects of common and emerging drugs of abuse. Clin J Am Soc Nephrol. 2014; 9(11):1996-2005.