Pulmonary-Renal Syndrome with a Focus on Anti-GBM Disease

Jan-Stephan F Sanders; Abraham Rutgers; Coen A Stegeman; Cees G.M. Kallenberg

doi:10.1055/s-0031-1279829

Seminars in Respiratory and Critical Care Medicine, Table of Contents

Semin Respir Crit Care Med 2011; 32(3): 328-334
DOI: 10.1055/s-0031-1279829

Pulmonary-Renal Syndrome with a Focus on Anti-GBM Disease

Jan-Stephan F. Sanders¹ , Abraham Rutgers² , Coen A. Stegeman¹ , Cees G.M. Kallenberg²

¹Department of Nephrology, University Medical Center Groningen (UMCG), University of Groningen, The Netherlands
²Department of Rheumatology and Clinical Immunology, University Medical Center Groningen (UMCG), University of Groningen, The Netherlands

Abstract

ABSTRACT

Pulmonary-renal syndrome is a potentially life-threatening combination of pulmonary hemorrhage and acute renal failure. Several pathological entities can cause this syndrome. This review discusses the diagnostic strategy required to initiate appropriate therapy. Rapid serological testing and appropriate interpretation can be of great additive diagnostic value. Also discussed are the pathogenesis, therapy, and outcome of anti–glomerular basement membrane disease, one of the pathological entities that can cause pulmonary-renal syndrome.

KEYWORDS

Anti-GBM - Goodpasture - ANCA - rapidly progressive glomerulonephritis - vasculitis

Full Text

References

REFERENCES

1 Goodpasture E W. Landmark publication from the American Journal of the Medical Sciences: the significance of certain pulmonary lesions in relation to the etiology of influenza. Am J Med Sci. 2009; 338 (2) 148-151
2 Stanton M C, Tange J D. Goodpasture's syndrome (pulmonary haemorrhage associated with glomerulonephritis). Australas Ann Med. 1958; 7 (2) 132-144
3 Lerner R A, Glassock R J, Dixon F J. The role of anti-glomerular basement membrane antibody in the pathogenesis of human glomerulonephritis. J Exp Med. 1967; 126 (6) 989-1004
4 Gallagher H, Kwan J T, Jayne D R. Pulmonary renal syndrome: a 4-year, single-center experience. Am J Kidney Dis. 2002; 39 (1) 42-47
5 Cortese G, Nicali R, Placido R, Gariazzo G, Anrò P. Radiological aspects of diffuse alveolar haemorrhage. Radiol Med (Torino). 2008; 113 (1) 16-28
6 Bowley N B, Steiner R E, Chin W S. The chest X-ray in antiglomerular basement membrane antibody disease (Goodpasture's syndrome). Clin Radiol. 1979; 30 (4) 419-429
7 Perazella M A, Coca S G, Kanbay M, Brewster U C, Parikh C R. Diagnostic value of urine microscopy for differential diagnosis of acute kidney injury in hospitalized patients. Clin J Am Soc Nephrol. 2008; 3 (6) 1615-1619
8 O'Neill W C. Sonographic evaluation of renal failure. Am J Kidney Dis. 2000; 35 (6) 1021-1038
9 Niles J L, Böttinger E P, Saurina G R et al.. The syndrome of lung hemorrhage and nephritis is usually an ANCA-associated condition. Arch Intern Med. 1996; 156 (4) 440-445
10 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) 143-152
11 Couser W G. Rapidly progressive glomerulonephritis: classification, pathogenetic mechanisms, and therapy. Am J Kidney Dis. 1988; 11 (6) 449-464
12 Jennette J C, Falk R J, Andrassy K et al.. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum. 1994; 37 (2) 187-192
13 Davies D J, Moran J E, Niall J F, Ryan G B. Segmental necrotising glomerulonephritis with antineutrophil antibody: possible arbovirus aetiology?. Br Med J (Clin Res Ed). 1982; 285 (6342) 606
14 Savige J, Davies D, Falk R J, Jennette J C, Wiik A. Antineutrophil cytoplasmic antibodies and associated diseases: a review of the clinical and laboratory features. Kidney Int. 2000; 57 (3) 846-862
15 Savige J, Gillis D, Benson E et al.. International Consensus Statement on Testing and Reporting of Antineutrophil Cytoplasmic Antibodies (ANCA). Am J Clin Pathol. 1999; 111 (4) 507-513
16 Hagen E C, Daha M R, 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) 743-753
17 Chen M, Kallenberg C G, Zhao M H. ANCA-negative pauci-immune crescentic glomerulonephritis. Nat Rev Nephrol. 2009; 5 (6) 313-318
18 Reinhold-Keller E, de Groot K, Holl-Ulrich K et al.. Severe CNS manifestations as the clinical hallmark in generalized Wegener's granulomatosis consistently negative for antineutrophil cytoplasmic antibodies (ANCA): a report of 3 cases and a review of the literature. Clin Exp Rheumatol. 2001; 19 (5) 541-549
19 Jones D A, Jennette J C, Falk R J. Goodpasture's syndrome revisited: a new perspective on glomerulonephritis and alveolar hemorrhage. N C Med J. 1990; 51 (8) 411-415
20 Hudson B G, Kalluri R, Gunwar S, Noelken M E, Mariyama M, Reeders S T. Molecular characteristics of the Goodpasture autoantigen. Kidney Int. 1993; 43 (1) 135-139
21 Salama A D, Dougan T, Levy J B et al.. Goodpasture's disease in the absence of circulating anti-glomerular basement membrane antibodies as detected by standard techniques. Am J Kidney Dis. 2002; 39 (6) 1162-1167
22 Short A K, Esnault V L, Lockwood C M. Anti-neutrophil cytoplasm antibodies and anti-glomerular basement membrane antibodies: two coexisting distinct autoreactivities detectable in patients with rapidly progressive glomerulonephritis. Am J Kidney Dis. 1995; 26 (3) 439-445
23 Rutgers A, Damoiseaux J, Roozendaal C, Limburg P C, Stegeman C A, Tervaert J W. ANCA-GBM dot-blot: evaluation of an assay in the differential diagnosis of patients presenting with rapidly progressive glomerulonephritis. J Clin Immunol. 2004; 24 (4) 435-440
24 Tervaert J W, Damoiseaux J. Fifty years of antineutrophil cytoplasmic antibodies (ANCA) testing: do we need to revise the international consensus statement on testing and reporting on ANCA?. APMIS Suppl. 2009 June; (127) 55-59
25 Kaul R, Johnson K, Scholz H, Marr G. Performance of the BioPlex 2200 Autoimmune Vasculitis kit. Autoimmun Rev. 2009; 8 (3) 224-227
26 Meyers K E, Allen J, Gehret J et al.. Human antiglomerular basement membrane autoantibody disease in XenoMouse II. Kidney Int. 2002; 61 (5) 1666-1673
27 Cui Z, Zhao M H. Avidity of anti-glomerular basement membrane autoantibodies was associated with renal pathological damage [in Chinese]. Zhonghua Yi Xue Za Zhi. 2005; 85 (13) 903-907
28 Hudson B G, Tryggvason K, Sundaramoorthy M, Neilson E G. Alport's syndrome, Goodpasture's syndrome, and type IV collagen. N Engl J Med. 2003; 348 (25) 2543-2556
29 Salama A D, Chaudhry A N, Holthaus K A et al.. Regulation by CD25+ lymphocytes of autoantigen-specific T-cell responses in Goodpasture's (anti-GBM) disease. Kidney Int. 2003; 64 (5) 1685-1694
30 Wu J, Hicks J, Borillo J, Glass II W F, Lou Y H. CD4(+) T cells specific to a glomerular basement membrane antigen mediate glomerulonephritis. J Clin Invest. 2002; 109 (4) 517-524
31 Zou J, Hannier S, Cairns L S et al.. Healthy individuals have Goodpasture autoantigen-reactive T cells. J Am Soc Nephrol. 2008; 19 (2) 396-404
32 Wolf D, Hochegger K, Wolf A M et al.. CD4+ CD25+ regulatory T cells inhibit experimental anti-glomerular basement membrane glomerulonephritis in mice. J Am Soc Nephrol. 2005; 16 (5) 1360-1370
33 Phelps R G, Rees A J. The HLA complex in Goodpasture's disease: a model for analyzing susceptibility to autoimmunity. Kidney Int. 1999; 56 (5) 1638-1653
34 Johnson J P, Moore Jr J, Austin III H A, Balow J E, Antonovych T T, Wilson C B. Therapy of anti-glomerular basement membrane antibody disease: analysis of prognostic significance of clinical, pathologic and treatment factors. Medicine (Baltimore). 1985; 64 (4) 219-227
35 Salama A D, Levy J B, Lightstone L, Pusey C D. Goodpasture's disease. Lancet. 2001; 358 (9285) 917-920
36 Levy J B, Turner A N, Rees A J, Pusey C D. Long-term outcome of anti-glomerular basement membrane antibody disease treated with plasma exchange and immunosuppression. Ann Intern Med. 2001; 134 (11) 1033-1042
37 Hirayama K, Yamagata K, Kobayashi M, Koyama A. Anti-glomerular basement membrane antibody disease in Japan: part of the nationwide rapidly progressive glomerulonephritis survey in Japan. Clin Exp Nephrol. 2008; 12 (5) 339-347
38 Jayne D R, Gaskin G, Rasmussen N European Vasculitis Study Group et al. Randomized trial of plasma exchange or high-dosage methylprednisolone as adjunctive therapy for severe renal vasculitis. J Am Soc Nephrol. 2007; 18 (7) 2180-2188
39 Laczika K, Knapp S, Derfler K, Soleiman A, Hörl W H, Druml W. Immunoadsorption in Goodpasture's syndrome. Am J Kidney Dis. 2000; 36 (2) 392-395
40 Arzoo K, Sadeghi S, Liebman H A. Treatment of refractory antibody mediated autoimmune disorders with an anti-CD20 monoclonal antibody (rituximab). Ann Rheum Dis. 2002; 61 (10) 922-924
41 García-Cantón C, Toledo A, Palomar R et al.. Goodpasture's syndrome treated with mycophenolate mofetil. Nephrol Dial Transplant. 2000; 15 (6) 920-922
42 Deegens J K, Artz M A, Hoitsma A J, Wetzels J F. Outcome of renal transplantation in patients with pauci-immune small vessel vasculitis or anti-GBM disease. Clin Nephrol. 2003; 59 (1) 1-9
43 Cameron J S. Glomerulonephritis in renal transplants. Transplantation. 1982; 34 (5) 237-245
44 Sauter M, Schmid H, Anders H J, Heller F, Weiss M, Sitter T. Loss of a renal graft due to recurrence of anti-GBM disease despite rituximab therapy. Clin Transplant. 2009; 23 (1) 132-136
45 Khandelwal M, McCormick B B, Lajoie G, Sweet J, Cole E, Cattran D C. Recurrence of anti-GBM disease 8 years after renal transplantation. Nephrol Dial Transplant. 2004; 19 (2) 491-494
46 Rutgers A, Slot M, van Paassen P, van Breda Vriesman P, Heeringa P, Tervaert J W. Coexistence of anti-glomerular basement membrane antibodies and myeloperoxidase-ANCAs in crescentic glomerulonephritis. Am J Kidney Dis. 2005; 46 (2) 253-262
47 Bosch X, Mirapeix E, Font J et al.. Prognostic implication of anti-neutrophil cytoplasmic autoantibodies with myeloperoxidase specificity in anti-glomerular basement membrane disease. Clin Nephrol. 1991; 36 (3) 107-113
48 Levy J B, Hammad T, Coulthart A, Dougan T, Pusey C D. Clinical features and outcome of patients with both ANCA and anti-GBM antibodies. Kidney Int. 2004; 66 (4) 1535-1540

Jan-Stephan F SandersM.D. Ph.D.

Department of Nephrology, University Medical Center Groningen (UMCG), University of Groningen

P.O. Box 30.001, 9700 RB Groningen, The Netherlands

Email: j.sanders@int.umcg.nl