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Das pulmorenale Syndrom auf der Intensivstation

Pulmonary renal syndrome in the ICU

  • Leitthema
  • Published:
Der Pneumologe Aims and scope

Zusammenfassung

Das pulmorenale Syndrom ist ein internistischer Notfall, der unbehandelt mit einer hohen Letalität einhergeht. Die häufigsten Ursachen sind die ANCA-assoziierten Vaskulitiden – unter diesen am häufigsten die Wegener-Granulomatose und die mikroskopische Polyangiitis – und das Goodpasture-Syndrom.

Nach primärer respiratorischer und hämodynamischer Stabilisierung der Patienten ist eine schnelle Diagnose unter Berücksichtigung vieler Differenzialdiagnosen wichtig, um unverzüglich eine adäquate Therapie einleiten zu können. Hilfreich sind hier neben einer gezielten Anamnese und Untersuchung die Bestimmung von Autoantikörpern sowie Bildgebung und Bronchoskopie, außerdem sollte eine Nierenbiopsie erfolgen. Der diagnostische Wert einer Lungenbiopsie ist bisher nicht belegt.

Die Therapie basiert im Wesentlichen auf einer aggressiven Immunsuppression und der Plasmapherese.

Abstract

Pulmonary renal syndrome is an emergency situation in internal medicine, associated with high mortality if not treated adequately. The underlying diseases include mainly the vasculitides associated with antineutrophil cytoplasmatic antibodies – among these mainly Wegener’s granulomatosis and microscopic polyangiitis – and Goodpasture’s syndrome.

After having stabilized the hemodynamic and respiratory status of the patient, it is of great importance to identify the underlying disease and exclude a variety of other diseases that can mimic a pulmonary renal syndrome. Besides taking a history and a complete physical examination, X-ray imaging, scanning for auto-immunologic antibodies and bronchoscopy have proved to be helpful for this purpose.

In addition, a renal biopsy should be performed if possible. Whether a biopsy of the lung is of further diagnostic value is unclear. Therapy is based on intense immunosuppression and plasmapheresis.

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Literatur

  1. Ramaswami A, Kandaswamy T, Rajendran T et al (2008) Goodpasture’s syndrome with positive c-ANCA and normal renal function: a case report. J Med Case Reports 2:223

    Article  PubMed  Google Scholar 

  2. Le Quang C, Delevaux I, Trouillier S et al (2008) Intra-alveolar haemorrhage without renal damage as the initial presenting feature of Goodpasture’s syndrome: case report and review of literature. Rev Med Interne 29:1038–1042

    Article  Google Scholar 

  3. Lauque D, Cadranel J, Lazor R et al (2000) Microscopic polyangiitis with alveolar hemorrhage. A study of 29 cases and review of the literature. Groupe d’Etudes et de Recherche sur les Maladies „Orphelines“ Pulmonaires (GERM“O“P). Medicine (Baltimore) 79:222–233

    Google Scholar 

  4. Tobler A, Schurch E, Altermatt HJ et al (1991) Anti-basement membrane antibody disease with severe pulmonary haemorrhage and normal renal function. Thorax 46:68–70

    Article  CAS  PubMed  Google Scholar 

  5. Schwarz MI, Brown KK (2000) Small vessel vasculitis of the lung. Thorax 55:502–510

    Article  CAS  PubMed  Google Scholar 

  6. Couser WG (1988) Rapidly progressive glomerulonephritis: classification, pathogenetic mechanisms, and therapy. Am J Kidney Dis 11:449–464

    CAS  PubMed  Google Scholar 

  7. Lerner RA, Glassock RJ, Dixon FJ (1967) The role of anti-glomerular basement membrane antibody in the pathogenesis of human glomerulonephritis. J Exp Med 126:989–1004

    Article  CAS  PubMed  Google Scholar 

  8. Falk RJ, Terrell RS, Charles LA et al (1990) Anti-neutrophil cytoplasmic autoantibodies induce neutrophils to degranulate and produce oxygen radicals in vitro. Proc Natl Acad Sci U S A 87:4115–4119

    Article  CAS  PubMed  Google Scholar 

  9. Johnson PA, Alexander HD, McMillan SA et al (1997) Up-regulation of the granulocyte adhesion molecule Mac-1 by autoantibodies in autoimmune vasculitis. Clin Exp Immunol 107:513–519

    Article  CAS  PubMed  Google Scholar 

  10. Xiao H, Heeringa P, Hu P et al (2002) Antineutrophil cytoplasmic autoantibodies specific for myeloperoxidase cause glomerulonephritis and vasculitis in mice. J Clin Invest 110:955–963

    CAS  PubMed  Google Scholar 

  11. Schreiber A, Xiao H, Falk RJ et al (2006) Bone marrow-derived cells are sufficient and necessary targets to mediate glomerulonephritis and vasculitis induced by anti-myeloperoxidase antibodies. J Am Soc Nephrol 17:3355–3364

    Article  PubMed  Google Scholar 

  12. Hoffman GS, Kerr GS, Leavitt RY et al (1992) Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med 116:488–498

    CAS  PubMed  Google Scholar 

  13. Merkel PA, Lo GH, Holbrook JT et al (2005) Brief communication: high incidence of venous thrombotic events among patients with Wegener granulomatosis: the Wegener’s Clinical Occurrence of Thrombosis (WeCLOT) Study. Ann Intern Med 142:620–626

    PubMed  Google Scholar 

  14. Schreiber A, Göbel U, Kettritz R (2009) ANCA-assoziierte Vaskulitis. Nephrologe 4:541–551

    Article  Google Scholar 

  15. Cantasdemir M, Kantarci F, Mihmanli I et al (2002) Emergency endovascular management of pulmonary artery aneurysms in Behçet’s disease: report of two cases and a review of the literature. Cardiovasc Intervent Radiol 25:533–537

    Article  PubMed  Google Scholar 

  16. Moosig F, Csernok E, Reinhold-Keller E et al (1998) Elevated procalcitonin levels in active Wegener’s granulomatosis. J Rheumatol 25:1531–1533

    CAS  PubMed  Google Scholar 

  17. Morath C, Sis J, Haensch GM et al (2007) Procalcitonin as marker of infection in patients with Goodpasture’s syndrome is misleading. Nephrol Dial Transplant 22:2701–2704

    Article  CAS  PubMed  Google Scholar 

  18. Niles JL, Pan GL, Collins AB et al (1991) Antigen-specific radioimmunoassays for anti-neutrophil cytoplasmic antibodies in the diagnosis of rapidly progressive glomerulonephritis. J Am Soc Nephrol 2:27–36

    CAS  PubMed  Google Scholar 

  19. Kitching AR, Hutchinson P, Atkins RC et al (2004) The role of flow cytometric ANCA detection in screening for acute pauci-immune crescentic glomerulonephritis. Nephrol Dial Transplant 19:365–370

    Article  PubMed  Google Scholar 

  20. Sable-Fourtassou R, Cohen P, Mahr A et al (2005) Antineutrophil cytoplasmic antibodies and the Churg-Strauss syndrome. Ann Intern Med 143:632–638

    PubMed  Google Scholar 

  21. Schmitt WH, Woude FJ van der (2004) Clinical applications of antineutrophil cytoplasmic antibody testing. Curr Opin Rheumatol 16:9–17

    Article  PubMed  Google Scholar 

  22. Sinico RA, Radice A, Corace C et al (2006) Anti-glomerular basement membrane antibodies in the diagnosis of Goodpasture syndrome: a comparison of different assays. Nephrol Dial Transplant 21:397–401

    Article  CAS  PubMed  Google Scholar 

  23. McCarty GA, Rice JR, Bembe ML et al (1982) Independent expression of autoantibodies in systemic lupus erythematosus. J Rheumatol 9:691–695

    CAS  PubMed  Google Scholar 

  24. Ross CN, Turner N, Savage P et al (1996) A single-chain Fv reactive with the Goodpasture antigen. Lab Invest 74:1051–1059

    CAS  PubMed  Google Scholar 

  25. Kohler H, Wandel E, Brunck B (1991) Acanthocyturia – a characteristic marker for glomerular bleeding. Kidney Int 40:115–120

    Article  CAS  PubMed  Google Scholar 

  26. Cordier JF, Valeyre D, Guillevin L et al (1990) Pulmonary Wegener’s granulomatosis. A clinical and imaging study of 77 cases. Chest 97:906–912

    Article  CAS  PubMed  Google Scholar 

  27. Betensley AD, Yankaskas JR (2002) Factor viia for alveolar hemorrhage in microscopic polyangiitis. Am J Respir Crit Care Med 166:1291–1292

    PubMed  Google Scholar 

  28. Jindal KK (1999) Management of idiopathic crescentic and diffuse proliferative glomerulonephritis: evidence-based recommendations. Kidney Int Suppl 70:S33–S40

    Article  CAS  PubMed  Google Scholar 

  29. Groot K de, Harper L, Jayne DR et al (2009) Pulse versus daily oral cyclophosphamide for induction of remission in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized trial. Ann Intern Med 150:670–680

    PubMed  Google Scholar 

  30. Jayne D, Rasmussen N, Andrassy K et al (2003) A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N Engl J Med 349:36–44

    Article  CAS  PubMed  Google Scholar 

  31. Jayne DR, Gaskin G, Rasmussen N et al (2007) Randomized trial of plasma exchange or high-dosage methylprednisolone as adjunctive therapy for severe renal vasculitis. J Am Soc Nephrol 18:2180–2188

    Article  CAS  PubMed  Google Scholar 

  32. Klemmer PJ, Chalermskulrat W, Reif MS et al (2003) Plasmapheresis therapy for diffuse alveolar hemorrhage in patients with small-vessel vasculitis. Am J Kidney Dis 42:1149–1153

    Article  PubMed  Google Scholar 

  33. Stone JH, Merkel PA, Spiera R et al (2010) Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med 363:221–232

    Article  CAS  PubMed  Google Scholar 

  34. Jones RB, Cohen Tervaert JW, Hauser T et al (2010) Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis. N Engl J Med 363:211–220

    Article  CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Nierenzentrum Heidelberg, Medizinische Universitätsklinik Heidelberg, Im Neuenheimer Feld 162, 69120, Heidelberg, Deutschland

    D. Stoffler & V. Schwenger

  2. Klinik für Pneumologie und Gastroenterologie, Städtisches Klinikum München-Harlaching, München, Deutschland

    F.J. Meyer

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  1. D. Stoffler
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  3. V. Schwenger
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Correspondence to D. Stoffler.

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Stoffler, D., Meyer, F. & Schwenger, V. Das pulmorenale Syndrom auf der Intensivstation. Pneumologe 7, 434–440 (2010). https://doi.org/10.1007/s10405-010-0412-3

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