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

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
Further Information

Publication History

Publication Date:
14 June 2011 (online)

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.

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

    >