Abstract
A 69-year-old Caucasian male was admitted to the hospital with progressive dyspnea, decreased urine output, and hemoptysis and was found to have anemia, acute kidney injury, and bilateral pulmonary infiltrates. Antibodies to cytoplasmic antinuclear cytoplasmic antibody (c-ANCA) and proteinase 3 (anti-PR3 Ab) were positive, and perinuclear antinuclear cytoplasmic antibody (p-ANCA) and antibodies to myeloperoxidase (anti-MPO Ab) were negative. A diagnosis of Wegener granulomatosis (WG) was made. His course was complicated by respiratory failure and renal failure requiring hemodialysis. Treatment consisted of cyclophosphamide (CYC), corticosteroids, plasmapheresis, and intravenous immunoglobulin (IVIG). Ultimately, dialysis was discontinued, and serum creatinine level (Cr) was stabilized at 1.6–1.8 g/dL 4 months after presentation. After 6 months, immunosuppression was changed from CYC to azathioprine, and prednisone was tapered and discontinued after 1 year.
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References
Rose B. Serologic tests in the evaluation of nephrotic syndrome. UpToDate [Webpage]. 2011; 18.3. Accessed January 28, 2011.
Jennette J, Falk R. Glomerular clinicopathologic syndromes. In: Greenberg A, editor. Primer on kidney diseases. 4th ed. Philadelphia, PA: Elsevier Saunders; National Kidney Foundation; 2005. p. 150–64.
Madaio MP, Harrington JT. The diagnosis of glomerular diseases: acute glomerulonephritis and the nephrotic syndrome. Arch Intern Med. 2001;161(1):25–34.
Falk RJ, Gross WL, Guillevin L, et al. Granulomatosis with Polyangiitis (Wegener’s): an alternative name for Wegener’s Granulomatosis. Arthritis Rheum. 2011;63(4):863–4.
Saxena R, Bygren P, Arvastson B, Wieslander J. Circulating autoantibodies as serological markers in the differential diagnosis of pulmonary renal syndrome. J Intern Med. 1995;238(2):143–52.
Jara LJ, Vera-Lastra O, Calleja MC. Pulmonary-renal vasculitic disorders: differential diagnosis and management. Curr Rheumatol Rep. 2003;5(2):107–15.
Pusey CD. Anti-glomerular basement membrane disease. Kidney Int. 2003;64(4):1535–50.
Kluth DC, Rees AJ. Anti-glomerular basement membrane disease. J Am Soc Nephrol. 1999;10(11):2446–53.
Brenner B. Brenner and Rector’s the kidney. 8th ed. Philadelphia, PA: Elsevier; 2011.
Hudson BG. The molecular basis of Goodpasture and Alport syndromes: beacons for the discovery of the collagen IV family. J Am Soc Nephrol. 2004;15(10):2514–27.
Pettersson E, Tornroth T, Miettinen A. Simultaneous anti-glomerular basement membrane and membranous glomerulonephritis: case report and literature review. Clin Immunol Immunopathol. 1984;31(2):171–80.
Kalluri R, Weber M, Netzer KO, Sun MJ, Neilson EG, Hudson BG. COL4A5 gene deletion and production of post-transplant anti-alpha 3(IV) collagen alloantibodies in Alport syndrome. Kidney Int. 1994;45(3):721–6.
Levy JB, Hammad T, Coulthart A, Dougan T, Pusey CD. Clinical features and outcome of patients with both ANCA and anti-GBM antibodies. Kidney Int. 2004;66(4):1535–40.
Jayne DR, Marshall PD, Jones SJ, Lockwood CM. Autoantibodies to GBM and neutrophil cytoplasm in rapidly progressive glomerulonephritis. Kidney Int. 1990;37(3):965–70.
O’Donoghue DJ, Short CD, Brenchley PE, Lawler W, Ballardie FW. Sequential development of systemic vasculitis with anti-neutrophil cytoplasmic antibodies complicating anti-glomerular basement membrane disease. Clin Nephrol. 1989;32(6):251–5.
Weber MF, Andrassy K, Pullig O, Koderisch J, Netzer K. Antineutrophil-cytoplasmic antibodies and antiglomerular basement membrane antibodies in Goodpasture’s syndrome and in Wegener’s granulomatosis. J Am Soc Nephrol. 1992;2(7):1227–34.
Kalluri R, Meyers K, Mogyorosi A, Madaio MP, Neilson EG. Goodpasture syndrome involving overlap with Wegener’s granulomatosis and anti-glomerular basement membrane disease. J Am Soc Nephrol. 1997;8(11):1795–800.
Leavitt RY, Fauci AS, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of Wegener’s granulomatosis. Arthritis Rheum. 1990;33(8):1101–7.
Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum. 1994;37(2):187–92.
Masi AT, Hunder GG, Lie JT, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum. 1990;33(8):1094–100.
Choi YH, Im JG, Han BK, Kim JH, Lee KY, Myoung NH. Thoracic manifestation of Churg-Strauss syndrome: radiologic and clinical findings. Chest. 2000;117(1):117–24.
Solans R, Bosch JA, Perez-Bocanegra C, et al. Churg-Strauss syndrome: outcome and long-term follow-up of 32 patients. Rheumatology (Oxford). 2001;40(7):763–71.
Sinico RA, Di Toma L, Maggiore U, et al. Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in Churg-Strauss syndrome. Arthritis Rheum. 2005;52(9):2926–35.
Hellmich B, Gross WL. Recent progress in the pharmacotherapy of Churg-Strauss syndrome. Expert Opin Pharmacother. 2004;5(1):25–35.
Hellmich B, Ehlers S, Csernok E, Gross WL. Update on the pathogenesis of Churg-Strauss syndrome. Clin Exp Rheumatol. 2003;21(6 Suppl 32):S69–77.
Abril A, Calamia KT, Cohen MD. The Churg Strauss syndrome (allergic granulomatous angiitis): review and update. Semin Arthritis Rheum. 2003;33(2):106–14.
Lhote F, Guillevin L. Polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome. Clinical aspects and treatment. Rheum Dis Clin North Am. 1995;21(4):911–47.
Guillevin L, Cohen P, Gayraud M, Lhote F, Jarrousse B, Casassus P. Churg-Strauss syndrome. Clinical study and long-term follow-up of 96 patients. Medicine (Baltimore). 1999;78(1):26–37.
Stone JH. Clinical spectrum of antineutrophil cytoplasmic antibodies. UpToDate [Webpage]. 2011; 18.3. Accessed March 29, 2011.
Hagen EC, Daha MR, Hermans J, et al. Diagnostic value of standardized assays for anti-neutrophil cytoplasmic antibodies in idiopathic systemic vasculitis. EC/BCR Project for ANCA Assay Standardization. Kidney Int. 1998;53(3):743–53.
Jennette JC, Wilkman AS, Falk RJ. Diagnostic predictive value of ANCA serology. Kidney Int. 1998;53(3):796–8.
Finkielman JD, Merkel PA, Schroeder D, et al. Antiproteinase 3 antineutrophil cytoplasmic antibodies and disease activity in Wegener granulomatosis. Ann Intern Med. 2007;147(9):611–9.
Boomsma MM, Stegeman CA, van der Leij MJ, et al. Prediction of relapses in Wegener’s granulomatosis by measurement of antineutrophil cytoplasmic antibody levels: a prospective study. Arthritis Rheum. 2000;43(9):2025–33.
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.
Reichlin M. Antibodies to DNA, Sm, and RNP. UpToDate 2011; 19.1. Accessed April 17, 2011.
Hahn BH. Antibodies to DNA. N Engl J Med. 1998;338(19):1359–68.
ter Borg EJ, Horst G, Hummel EJ, Limburg PC, Kallenberg CG. Measurement of increases in anti-double-stranded DNA antibody levels as a predictor of disease exacerbation in systemic lupus erythematosus. A long-term, prospective study. Arthritis Rheum. 1990;33(5):634–43.
Schur PH, Sandson J. Immunologic factors and clinical activity in systemic lupus erythematosus. N Engl J Med. 1968;278(10):533–8.
Cervera R, Khamashta MA, Font J, et al. Systemic lupus erythematosus: clinical and immunologic patterns of disease expression in a cohort of 1,000 patients. The European Working Party on Systemic Lupus Erythematosus. Medicine (Baltimore). 1993;72(2):113–24.
Bootsma H, Spronk P, Derksen R, et al. Prevention of relapses in systemic lupus erythematosus. Lancet. 1995;345(8965):1595–9.
Nossent JC, Huysen V, Smeenk RJ, Swaak AJ. Low avidity antibodies to double stranded DNA in systemic lupus erythematosus: a longitudinal study of their clinical significance. Ann Rheum Dis. 1989;48(8):677–82.
Deshmukh US, Bagavant H, Fu SM. Role of anti-DNA antibodies in the pathogenesis of lupus nephritis. Autoimmun Rev. 2006;5(6):414–8.
Gladman DD, Urowitz MB, Keystone EC. Serologically active clinically quiescent systemic lupus erythematosus: a discordance between clinical and serologic features. Am J Med. 1979;66(2):210–5.
Smeenk RJ, van den Brink HG, Brinkman K, Termaat RM, Berden JH, Swaak AJ. Anti-dsDNA: choice of assay in relation to clinical value. Rheumatol Int. 1991;11(3):101–7.
Neogi T, Gladman DD, Ibanez D, Urowitz M. Anti-dsDNA antibody testing by Farr and ELISA techniques is not equivalent. J Rheumatol. 2006;33(9):1785–8.
Zhang CQ, Ren L, Gao F, Mu FY, You YQ, Liu YH. Anti-C1q antibodies are associated with systemic lupus erythematosus disease activity and lupus nephritis in northeast of China. Clin Rheumatol. 2011;30(7):967–73.
Trendelenburg M, Lopez-Trascasa M, Potlukova E, et al. High prevalence of anti-C1q antibodies in biopsy-proven active lupus nephritis. Nephrol Dial Transplant. 2006;21(11):3115–21.
Marto N, Bertolaccini ML, Calabuig E, Hughes GR, Khamashta MA. Anti-C1q antibodies in nephritis: correlation between titres and renal disease activity and positive predictive value in systemic lupus erythematosus. Ann Rheum Dis. 2005;64(3):444–8.
Sinico RA, Radice A, Ikehata M, et al. Anti-C1q autoantibodies in lupus nephritis: prevalence and clinical significance. Ann N Y Acad Sci. 2005;1050:193–200.
Oelzner P, Deliyska B, Funfstuck R, Hein G, Herrmann D, Stein G. Anti-C1q antibodies and antiendothelial cell antibodies in systemic lupus erythematosus - relationship with disease activity and renal involvement. Clin Rheumatol. 2003;22(4–5):271–8.
Miyakis S, Lockshin MD, Atsumi T, et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost. 2006;4(2):295–306.
Crowther MA, Ginsberg JS, Julian J, et al. A comparison of two intensities of warfarin for the prevention of recurrent thrombosis in patients with the antiphospholipid antibody syndrome. N Engl J Med. 2003;349(12):1133–8.
Kaplan AA. The use of apheresis in immune renal disorders. Ther Apher Dial. 2003;7(2):165–72.
Appel G. Antiphospholipid syndrome and the kidney. UpToDate [Webpage]. 2011. Accessed April 24, 2011.
Brunet P, Aillaud MF, San Marco M, et al. Antiphospholipids in hemodialysis patients: relationship between lupus anticoagulant and thrombosis. Kidney Int. 1995;48(3):794–800.
Madaio MP, Harrington JT. Current concepts. The diagnosis of acute glomerulonephritis. N Engl J Med. 1983;309(21):1299–302.
Nast C, Appel G. A Case of Lupus Nephritis. Clin J Am Soc Nephrol. 2008;3(1):296–306.
Saint Marcoux B, De Bandt M. Vasculitides induced by TNFalpha antagonists: a study in 39 patients in France. Joint Bone Spine. 2006;73(6):710–3.
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Liang, K.V., Liang, K.P. (2013). Interpretation of Serologic Testing in Glomerular Diseases. In: Lerma, E., Rosner, M. (eds) Clinical Decisions in Nephrology, Hypertension and Kidney Transplantation. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-4454-1_8
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