Aktuelle Rheumatologie 2010; 35(4): 236-241
DOI: 10.1055/s-0030-1261957
Übersichtsarbeit

© Georg Thieme Verlag KG Stuttgart · New York

Renale Beteiligung beim Sjögren-Syndrom

Renal Involvement in Sjögren SyndromeR. Bergner1
  • 1Klinikum der Stadt Ludwigshafen gGmbH, Medizinische Klinik A, Ludwigshafen
Further Information

Publication History

Publication Date:
27 July 2010 (online)

Zusammenfassung

Das Sjögren-Syndrom kann über die obligate Siccasymptomatik hinaus verschiedene Organe mitbeteiligen. Die Nierenbeteiligung ist dabei eine häufige Organmanifestation. Sie bleibt aber oftmals unerkannt, da die Symptome in den meisten Fällen eher blande sind. Die häufigste Nierenbeteiligung ist die interstitielle Nephritis, die sich klinisch beginnend von einer milden tubulären Proteinurie bis hin zu ausgeprägten tubulären Elektrolytstörungen manifestieren kann. Glomeruläre Beteiligungen sind seltener. Eine typische glomeruläre Nierenbeteiligung ist die membranoproliferative Glomerulonephritis bei Kryoglobulinen. Daneben sind aber auch andere Glomerulonephritiden beschrieben, die sich jedoch histologisch nicht von den primären Formen der entsprechenden Glomerulonephritiden unterscheiden lassen. Akute Verlaufsformen mit sich rasch verschlechternder Nierenfunktion sind nur kasuistisch beschrieben. Die Therapie ist empirisch und besteht hauptsächlich in der Gabe von Kortikosteroiden. Bei Kryoglobulinämie assoziierten Glomerulonephritiden ist auch der erfolgreiche Einsatz der Plasmapherese beschrieben.

Abstract

In addition to the obligatory sicca symptoms, the Sjögren's syndrome can affect different internal organs. The renal involvement is very common, but is undetected in the majority of cases, because of the only mild symptoms. The most frequent renal involvement is an interstitial nephritis. The clinical appearance can range from mild tubular proteinuria to severe electrolyte disorder. Glomerular involvement is more unusual. One rare but typical glomerular involvement is the finding of a membranoproliferative glomerulonephritis, associated to cryoglobulins. In addition, many other types of glomerulonephritis have been published in case series, but histological findings of these types of glomerulonephritis are identical to those of primary glomerulonephritis. Acute renal failure is a very rare finding and was reported only in sporadic cases. The treatment is empirical because of the absence of randomised studies. Case series suggest a positive effect of corticosteroids. In cryoglobulinemic glomerulonephritis also plasmapheresis might be successful.

Literatur

  • 1 Vitali C, Bombardieri S, Jonsson R. et al . Classification criteria for Sjögren's syndrome: A revised version of the European criteria proposed by the American-European Consensus Group.  Ann Rheum Dis. 2002;  61 554-558
  • 2 Vitali C, Tavoni A, Sciuto M. et al . Renal involvement in primary Sjögren's syndrome: a retrospective-prospective study.  Scand J Rheumatol. 1991;  20 132-136
  • 3 Pertovaara M, Korpela M, Kouri T. et al . The occurrence of renal involvement in primary Sjögren's syndrome: a study of 78 patients.  Rheumatology (Oxford). 1999;  38 1113-1120
  • 4 Aasarod K, Haga HJ, Berg KJ. et al . Renal involvement in primary Sjögren's syndrome.  QJM. 2000;  93 297-304
  • 5 Bossini N, Savoldi S, Franceschini F. et al . Clinical and morphological features of kidney involvement in primary Sjögren's syndrome.  Nephrol Dial Transplant. 2001;  16 ((12)) 2328-2336
  • 6 Goules A, Masouridi S, Tzioufas AG. et al . Clinically significant and biopsy-documented renal involvement in primary Sjögren syndrome.  Medicine (Baltimore). 2000;  79 241-249
  • 7 Maripuri S, Grande JP, Osborn TG. et al . Renal involvement in primary Sjögren's syndrome: a clinicopathologic study.  Clin J Am Soc Nephrol. 2009;  4 1423-1431
  • 8 Shiozawa S, Shiozawa K, Shimizu S. et al . Clinical studies of renal disease in Sjögren's syndrome.  Ann Rheum Dis. 1987;  46 ((10)) 768-772
  • 9 Ren H, Chen N, Chen X. et al . Clinical and pathologic analysis of Sjögren's syndrome with renal impairment: a report of 84 cases.  Zhonghua Nei Ke Za Zhi. 2001;  40 367-369
  • 10 Yang J, Li X, Huang Q. Renal involvement in primary Sjögren's syndrome: a clinicopathological study of 26 cases.  Zhonghua Nei Ke Za Zhi. 1997;  36 28-31
  • 11 Iwanaga N, Kamachi M, Fujikawa K. et al . Membraneous glomerulonephritis and non-Hodgkin's lymphoma in a patient with primary Sjögren's syndrome.  Intern Med. 2007;  46 191-194
  • 12 Laraki R, Chauveau D, Noel LH. et al . Membranous glomerulonephritis during primary Gougerot-Sjögren syndrome.  Presse Med. 2005;  34 1069-1072
  • 13 Stefanidis I, Giannopoulou M, Liakopoulos V. et al . A case of membranous nephropathy associated with Sjögren syndrome, polymyositis and autoimmune hepatitis.  Clin Nephrol. 2008;  70 245-250
  • 14 Schlesinger I, Carlson TS, Nelson D. Type III membranoproliferative glomerulonephritis in primary Sjögren's syndrome.  Conn Med. 1989;  53 629-632
  • 15 Adam FU, Torun D, Bolat F. et al . Acute renal failure due to mesangial proliferative glomerulonephritis in a pregnant woman with primary Sjögren's syndrome.  Clin Rheumatol. 2006;  25 75-79
  • 16 Gamron S, Barberis G, Onetti CM. et al . Mesangial nephropathy in Sjögren's syndrome.  Scand J Rheumatol. 2000;  29 ((1)) 65-67
  • 17 Tsai TC, Chen CY, Lin WT. et al . Sjögren's syndrome complicated with IgA nephropathy and leukocytoclastic vasculitis.  Ren Fail. 2008;  30 755-758
  • 18 Guillot X, Solau-Gervais E, Coulon A. et al . Sjögren's syndrome with ANCA-associated crescentic extramembranous glomerulonephritis.  Joint Bone Spine. 2009;  76 188-189
  • 19 Dussol B, Tsimaratos M, Bolla G. et al . Crescentic glomerulonephritis and primary Gougerot-Sjögren syndrome.  Nephrologie. 1994;  15 295-298
  • 20 Ren H, Wang WM, Chen XN. et al . Renal involvement and follow-up of 130 patients with primary Sjögren's syndrome.  J Rheumatol. 2008;  35 278-284
  • 21 Eriksson P, Denneberg T, Larsson L. et al . Biochemical markers of renal disease in primary Sjögren's syndrome.  Scand J Urol Nephrol. 1995 Dec;  29 ((4)) 383-392
  • 22 Poux JM, Peyronnet P, Le Meur Y. et al . Hypokalemic quadriplegia and respiratory arrest revealing primary Sjögren's syndrome.  Clin Nephrol. 1992;  37 189-191
  • 23 Ohtani H, Imai H, Kodama T. et al . Severe hypokalaemia and respiratory arrest due to renal tubular acidosis in a patient with Sjögren syndrome.  Nephrol Dial Transplant. 1999;  14 ((9)) 2201-2203
  • 24 Raynal C, Durupt S, Durieu I. et al . Hypokalemic quadriplegia with distal tubular acidosis revealing a case of primary Gougerot-Sjögren syndrome.  Presse Med. 1999;  28 1935-1937
  • 25 Mukai M, Shibata T, Honda H. et al . A case of Sjögren's syndrome presenting with hypokalemic myopathy due to renal tubular acidosis.  Nippon Jinzo Gakkai Shi. 2001;  43 69-75
  • 26 Harada K, Akai Y, Iwano M. et al . Tubulointerstitial macrophage infiltration in a patient with hypokalemic nephropathy and primary Sjögren's syndrome.  Clin Nephrol. 2005;  64 387-390
  • 27 Bailey RR, Swainson CP. Renal involvement in Sjögren's syndrome.  N Z Med J. 1986;  99 579-580
  • 28 Felip Benach A, Romero Gonzalez R, Lauzurica Valdemoros R. et al . Distal tubular renal acidosis associated with hyperglobulinemic purpura and Sjögren's syndrome.  Med Clin (Barc). 1989;  92 385-387
  • 29 Gorriz L, Molino R, Arjona D. et al . Sjögren syndrome associated with renal tubular acidosis type I.  Rev Med Panama. 2000;  25 30-33
  • 30 Grindulis KA, Roy S. Renal and pancreatic calcification in rheumatoid arthritis with Sjögren's syndrome.  Br J Rheumatol. 1987;  26 212-215
  • 31 Buckalew VM. Nephrolithiasis in renal tubular acidosis.  J Urol. 1989;  141 731-737
  • 32 Moutsopoulos HM, Cledes J, Skopouli FN. et al . Nephrocalcinosis in Sjögren's syndrome: a late sequela of renal tubular acidosis.  J Intern Med. 1991;  230 187-191
  • 33 Diaz Rodriguez C, Gonzalez Rivero C, Trinidad San Jose JC. et al . Osteal complications as first manifestation in a patient with primary Sjögren's Syndrome and with associated distal tubular acidosis (type 1) and chronic renal insufficiency.  Ther Apher Dial. 2004;  8 160-163
  • 34 Kim YK, Song HC, Kim WY. et al . Acquired Gitelman syndrome in a patient with primary Sjögren syndrome.  Am J Kidney Dis. 2008;  52 1163-1167
  • 35 Zheng F, Zhao S, Li X. The clinical and biochemical manifestations of Fanconi syndrome: a report of 42 cases.  Zhonghua Nei Ke Za Zhi. 2000;  39 735-738
  • 36 Yang YS, Peng CH, Sia SK. et al . Acquired hypophosphatemia osteomalacia associated with Fanconi's syndrome in Sjögren's syndrome.  Rheumatol Int. 2007;  27 593-597
  • 37 Enestrom S, Denneberg T, Eriksson P. Histopathology of renal biopsies with correlation to clinical findings in primary Sjögren's syndrome.  Clin Exp Rheumatol. 1995;  13 697-703
  • 38 Matsumura R, Kondo Y, Sugiyama T. et al . Immunohistochemical identification of infiltrating mononuclear cells in tubulointerstitial nephritis associated with Sjögren's syndrome.  Clin Nephrol. 1988;  30 335-340
  • 39 Seror R, Ravaud P, Bowman S. et al . EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI): Development of a consensus systemic disease activity index in primary Sjögren's syndrome.  Ann Rheum Dis. 2009;  published online 6.2009
  • 40 Kubo S, Kasai T, Yuu K. et al . Autopsy findings of primary Sjögren's syndrome with end-stage renal failure.  Nephron. 1993;  65 485
  • 41 Dabadghao S, Aggarwal A, Arora P. et al . Glomerulonephritis leading to end stage renal disease in a patient with primary Sjögren syndrome.  Clin Exp Rheumatol. 1995;  13 509-511
  • 42 Akposso K, Martinant de Preneuf H, Larousserie F. et al . [Rapidly progressive acute renal failure. A rare complication of primary Sjögren syndrome].  Presse Med. 2000;  29 1647-1649
  • 43 el Mallakh RS, Bryan RK, Masi AT. et al . Long-term low-dose glucocorticoid therapy associated with remission of overt renal tubular acidosis in Sjögren's syndrome.  Am J Med. 1985;  79 509-514
  • 44 Saeki Y, Ohshima S, Ishida T. et al . Remission of the renal involvement in a patient with primary Sjögren's syndrome (SS) after pulse high-dose corticosteroid infusion therapy.  Clin Rheumatol. 2001;  20 225-228
  • 45 Vasil’ev VI, Solov’ev SK, Chiklikchi AS. et al . A positive effect of combined therapy (plasmapheresis in combination with pulse therapy) on the course of cryoglobulinemic glomerulonephritis and ulcerative-necrotic vasculitis in patients with Sjögren's disease.  Ter Arkh. 1987;  59 54-59

Korrespondenzadresse

PD. Dr. med. Raoul Bergner

Klinikum der Stadt Ludwigshafen gGmbH

Medizinische Klinik A

Bremserstraße 79

67063 Ludwigshafen

Phone: +49/0621/5033 902

Fax: +49/0621/5033 991

Email: bergnerr@klilu.de

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