Pulmorenales Syndrom aus nephrologischer Sicht - Differentialdiagnose und -therapie

Zusammenfassung

Das „Pulmorenale Syndrom” ist ein klinisch definierter Begriff, unter dem Krankheitsbilder mit einer akuten Verschlechterung der Nierenfunktion, bedingt durch eine akute, rapid-progressive Glomerulonephritis, und pulmonaler, alveolärer Hämorrhagie zusammengefasst werden. Die Wegenersche Granulomatose, andere entzündliche Nierenerkrankungen und das Goodpasture Syndrom sind die häufigsten Ursachen dieser Entität. Diagnostisch steht neben der klinischen Untersuchung die histologische Sicherung der Grundkrankheit, meist mittels Nierenbiopsie, im Vordergrund. Serologische Untersuchungen auf Autoantikörper runden die Diagnostik ab. Die Therapie richtet sich nach der Grunderkrankung. Die Plasmapherese wird immer bei pulmonaler Hämorrhagie, und meist bei Dialysepflichtigkeit des Patienten bei Präsentation empfohlen. Die Immunsuppression richtet sich nach der Grunderkrankung.

Summary

The „pulmorenal syndrome” is a clinically defined syndrome under which one summarises disease entities with acute renal failure due to acute glomerulonephritis and alveolar lung hemorrhage. Wegener’s granulomatosis, other inflammatory renal diseases and Goodpasture’s syndrome are the most frequent causes of this syndrome. The physical examination and histological examination, mostly of a kidney biopsy, represent the mainstay of securing the diagnosis. The diagnosis is completed by serological testing for autoantibodies. The therapy is determined by the underlying disease. Plasmaspheresis is always performed when alveolar hemorrhage is present, and in most cases where the patient presents with the need for immediate dialysis. An immunosuppressive therapy is determined by the underlying disease.

pulmorenales Syndrom - Glomerulonephritis - pulmonale Hämorragie - Vaskulitis

pulmorenal syndrome - glomerulonephritis - pulmonary hemorrhagia - vasculitis

Literatur

• 1 Andrassy K, Erb A, Koderisch J, Waldherr R, Ritz E. Wegener’s granulomatosis with renal involvement: patient survival and correlations between initial renal function, renal histology, therapy and renal outcome.  Clin Nephrol. 1991;  35 139-147
• 2 Arends J, Wu J, Borillo J, Troung L, Zhou C, Vigneswaran N, Lou Y H. T cell epitope mimicry in antiglomerular basement membrane disease.  J Immunol. 2006;  176 1252-1258
• 3 Aries P M, Hellmich B, Gross W L. Diagnosis and therapy of ANCA-associated vasculitis.  Dtsch Med Wochenschr. 2006;  131 443-446
• 4 Brainwood D, Kashtan C, Gubler M C, Turner A N. Targets of alloantibodies in Alport anti-glomerular basement membrane disease after renal transplantation.  Kidney Int. 1998;  53 762-766
• 5 The WGET Research Group. Etanercept plus standard therapy for Wegener’s granulomatosis.  N Engl J Med. 2005;  352 351-361
• 6 de Lind van Wijngaarden R A, Hauer H A, Wolterbeek R, Jayne D R, Gaskin G, Rasmussen N, Noel L H, Ferrario F, Waldherr R, Hagen E C, Bruijn J A, Bajema I M. Clinical and histologic determinants of renal outcome in ANCA-associated vasculitis: A prospective analysis of 100 patients with severe renal involvement.  J Am Soc Nephrol. 2006;  17 2264-2274
• 7 Gayraud M, Guillevin L, le Toumelin P, Cohen P, Lhote F, Casassus P, Jarrousse B. Long-term followup of polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome: analysis of four prospective trials including 278 patients.  Arthritis Rheum. 2001;  44 666-675
• 8 Guillevin L, Durand-Gasselin B, Cevallos R, Gayraud M, Lhote F, Callard P, Amouroux J, Casassus P, Jarrousse B. Microscopic polyangiitis: clinical and laboratory findings in eighty-five patients.  Arthritis Rheum. 1999;  42 421-430
• 9 Hagen E C, Andrassy K, Csernok E, Daha M R, Gaskin G, Gross W L, Hansen B, Heigl Z, Hermans J, Jayne D, Kallenberg C G, Lesavre P, Lockwood C M, Ludemann J, Mascart-Lemone F, Mirapeix E, Pusey C D, Rasmussen N, Sinico R A, Tzioufas A, Wieslander J, Wiik A, Van der Woude F J. Development and standardization of solid phase assays for the detection of anti-neutrophil cytoplasmic antibodies (ANCA). A report on the second phase of an international cooperative study on the standardization of ANCA assays.  J Immunol Methods. 1996;  196 1-15
• 10 Hagen E C, Daha M R, Hermans J, Andrassy K, Csernok E, Gaskin G, Lesavre P, Ludemann J, Rasmussen N, Sinico R A, Wiik A, van der Woude F J. Diagnostic value of standardized assays for anti-neutrophil cytoplasmic antibodies in idiopathic systemic vasculitis. EC/BCR Project for ANCA Assay Standardization.  Kidney Int. 1998;  53 743-753
• 11 Hellmich B, Lamprecht P, Aries P M, Gross W L. Early diagnosis of vasculitides.  Z Rheumatol. 2005;  64 538-546
• 12 Hoffman G S, Kerr G S, Leavitt R Y, Hallahan C W, Lebovics R S, Travis W D, Rottem M, Fauci A S. Wegener granulomatosis: an analysis of 158 patients.  Ann Intern Med. 1992;  116 488-498
• 13 Hoffman G S, Specks U. Antineutrophil cytoplasmic antibodies.  Arthritis Rheum. 1998;  41 1521-1537
• 14 Jayne D R, Marshall P D, Jones S J, Lockwood C M. Autoantibodies to GBM and neutrophil cytoplasm in rapidly progressive glomerulonephritis.  Kidney Int. 1990;  37 965-970
• 15 Jayne D, Rasmussen N, Andrassy K, Bacon P, Tervaert J W, Dadoniene J, Ekstrand A, Gaskin G, Gregorini G, de Groot K, Gross W, Hagen E C, Mirapeix E, Pettersson E, Siegert C, Sinico A, Tesar V, Westman K, Pusey C. A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies.  N Engl J Med. 2003;  349 36-44
• 16 Jennette J C, Falk R J. Pathogenic potential of anti-neutrophil cytoplasmic autoantibodies.  Lab Invest. 1994;  70 135
• 17 Jennette J C, Falk R J, Andrassy K, Bacon P A, Churg J, Gross W L, Hagen E C, Hoffman G S, Hunder G G, Kallenberg C G. et al . Nomenclature of systemic vasculitides. Proposal of an international consensus conference.  Arthritis Rheum. 1994;  37 187-192
• 18 Johnson J P, Moore J, Austin 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;  64 219-227
• 19 Kalluri R, Sun M J, Hudson B G, Neilson E G. The Goodpasture autoantigen. Structural delineation of two immunologically privileged epitopes on alpha3(IV) chain of type IV collagen.  J Biol Chem. 1996;  271 9062-9068
• 20 Kalluri R, van den Heuvel L P, Smeets H J, Schroder C H, Lemmink H H, Boutaud A, Neilson E G, Hudson B G. A COL4A3 gene mutation and post-transplant anti-alpha 3(IV) collagen alloantibodies in Alport syndrome.  Kidney Int. 1995;  47 1199-1204
• 21 Klemmer P J, Chalermskulrat W, Reif M S, Hogan S L, Henke D C, Falk R J. Plasmapheresis therapy for diffuse alveolar hemorrhage in patients with small-vessel vasculitis.  Am J Kidney Dis. 2003;  42 1149-1153
• 22 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 1535-1540
• 23 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 1033-1042
• 24 Maxwell A P, Nelson W E, Hill C M. Reversal of renal failure in nephritis associated with antibody to glomerular basement membrane.  BMJ. 1988;  297 333-334
• 25 Merkel F, Kalluri R, Marx M, Enders U, Stevanovic S, Giegerich G, Neilson E G, Rammensee H G, Hudson B G, Weber M. Autoreactive T-cells in Goodpasture’s syndrome recognize the N-terminal NC1 domain on alpha 3 type IV collagen.  Kidney Int. 1996;  49 1127-1133
• 26 Nachman P H, Hogan S L, Jennette J C, Falk R J. Treatment response and relapse in antineutrophil cytoplasmic autoantibody-associated microscopic polyangiitis and glomerulonephritis.  J Am Soc Nephrol. 1996;  7 33-39
• 27 Niles J L, Bottinger E P, Saurina G R, Kelly K J, Pan G, Collins A B, McCluskey R T. The syndrome of lung hemorrhage and nephritis is usually an ANCA-associated condition.  Arch Intern Med. 1996;  156 440-445
• 28 O’Donoghue D J, Short C D, Brenchley P E, Lawler W, Ballardie F W. Sequential development of systemic vasculitis with anti-neutrophil cytoplasmic antibodies complicating anti-glomerular basement membrane disease.  Clin Nephrol. 1989;  32 251-255
• 29 Schnabel A, Holl-Ulrich K, Dalhoff K, Reuter M, Gross W L. Efficacy of transbronchial biopsy in pulmonary vaculitides.  Eur Respir J. 1997;  10 2738-2743
• 30 Sinico R A, Di Toma L, Maggiore U, Bottero P, Radice A, Tosoni C, Grasselli C, Pavone L, Gregorini G, Monti S, Frassi M, Vecchio F, Corace C, Venegoni E, Buzio C. Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in Churg-Strauss syndrome.  Arthritis Rheum. 2005;  52 2926-2935
• 31 Sinico R A, Di Toma L, Maggiore U, Tosoni C, Bottero P, Sabadini E, Giammarresi G, Tumiati B, Gregorini G, Pesci A, Monti S, Balestrieri G, Garini G, Vecchio F, Buzio C. Renal involvement in Churg-Strauss syndrome.  Am J Kidney Dis. 2006;  47 770-779
• 32 Sinico R A, Radice A, Corace C, Sabadini E, Bollini B. Anti-glomerular basement membrane antibodies in the diagnosis of Goodpasture syndrome: a comparison of different assays.  Nephrol Dial Transplant. 2006;  21 397-401
• 33 Wong C F. Rituximab in refractory antineutrophil cytoplasmic antibody-associated vasculitis: what is the current evidence?.  Nephrol Dial Transplant. 2007;  22 32-36
• 34 Wu J, Borillo J, Glass W F, Hicks J, Ou C N, Lou Y H. T-cell epitope of alpha3 chain of type IV collagen induces severe glomerulonephritis.  Kidney Int. 2003;  64 1292-1301
• 35 Jayne D R, Gaskin G, Rasmussen N. et al. on behalf of the European Vasculitis Stuy Group . Randomized trial of plasma exchange or high-dosage methylprednisolone as adjunctive therapy for severe renal vasculitis.  J Am Soc Nephrol . 2007;  18 2180-2188