Skip to main content
SpringerLink
Log in

Ist eine bioptische Sicherung beim Sjögren-Syndrom sinnvoll?

Vom Focus-Score zur Vaskulitisdiagnostik

Is bioptic assurance reasonable in patients with Sjögren’s syndrome?

From focus score to diagnosing vasculitides

  • Leitthema
  • Published:
Zeitschrift für Rheumatologie Aims and scope Submit manuscript

Zusammenfassung

Das Sjögren-Syndrom ist eine Autoimmunerkrankung, die bevorzugt die Speichel- und Tränendrüse befällt und zu einer Sicca-Symptomatik führt. Extraglanduläre Manifestationen treten häufig auf. Meist wird eine chronische Sialadenitis der Glandula parotis beobachtet, welche ein breites differenzialdiagnostisches Spektrum aufweist. Die histopathologische Diagnostik erfolgt an konventionellen HE- und Giemsa-Präparaten mittels Graduierung und Typisierung (Focus) des lymphozytären Infiltrates: Grad 0: kein, Grad 1: geringes, Grad 2: mäßiges, nichtfokales Infiltrat, Grad 3: 1 Focus (Aggregat ≥50 Lymphozyten) pro 4 mm2, Grad 4: >1 Focus. Eine Infiltration vom Grad 3 entspricht einem Focus-Score von 1, was als eines von 4 krankheitsklassifizierenden Kriterien zur Diagnosestellung anerkannt ist. Die bioptische Untersuchung dient ebenfalls dem Ausschluss anderer (nicht-)immunologischer Sialadenitiden wie der nekrotisierenden oder der epitheloidzelligen Form (bei Sarkoidose) und dem Ausschluss eines extranodalen Marginalzonenlymphoms. Extraglanduläre Manifestationen des Sjögren-Syndroms werden ebenfalls histopathologisch diagnostiziert. Hervorzuheben sind hier die Vaskulitiden und Myositiden. Die bioptische Diagnostik ist somit nicht nur sinnvoll, sondern eine essenzielle Basisdiagnostik des Sjögren-Syndroms.

Abstract

Sjögren’s syndrome is an autoimmune disease which targets the salivary and lacrimal glands in particular, causing sicca syndrome. Extraglandular manifestations are often seen. Chronic sialadenitis of the parotid gland is the most common symptom to be assessed for differential diagnosis. Common HE and Giemsa slices are histopathologically examined and graduated for lymphocyte infiltration (focus): grade 0: absent, grade 1: slight, grade 2: moderate non-focal infiltration, grade 3: 1 focus (≥50 lymphocytes) per 4 mm2, grade 4: >1 focus. Grade 3 infiltrates correspond to a focus score of 1, which is one of four disease-classifying criteria acknowledged for diagnosis. Bioptic examination is also performed to rule out different (non-) immunologic sialadenitises, such as the necrotizing or epithelioid-like form (in sarcoidosis), and the extranodal marginal-zone lymphoma. Extraglandular manifestations of Sjögren’s syndrome can also be safely diagnosed by histopathological examination. Emphases lie on vasculitides and myositides. Bioptic work-up, therefore, is not only reasonable but also an essential tool for diagnostics in Sjögren’s syndrome.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Abb. 1
Abb. 2
Abb. 3
Abb. 4

Literatur

  1. Al-Hashimi I, Wright JM, Cooley CA et al (2001) Reproducibility of biopsy grade in Sjogren’s syndrome. J Oral Pathol Med 30(7):408–412

    Article  CAS  PubMed  Google Scholar 

  2. Berquin K, Mahy P, Weynand B et al (2006) Accessory or sublingual salivary gland biopsy to assess systemic disease: a comparative retrospective study. Eur Arch Otorhinolaryngol 263:233–236

    Article  PubMed  Google Scholar 

  3. Bodeutsch C, Wilde PC de, Kater L et al (1992) Quantitative immunohistologic criteria are superior to the lymphocytic focus score criterion for the diagnosis of Sjogren’s syndrome. Arthritis Rheum 35(9):1075–1087

    Article  CAS  PubMed  Google Scholar 

  4. Brito-Zerón P, Ramos-Casals M, Bove A et al (2007) Predicting adverse outcomes in primary Sjogren’s syndrome: identification of prognostic factors. Rheumatology (Oxford) 46(8):1359–1362

    Google Scholar 

  5. Chisholm DM, Mason DK (1968) Labial salivary gland biopsy in Sjogren’s disease. J Clin Pathol 21(5):656–660

    Article  CAS  PubMed  Google Scholar 

  6. Daniels TE, Whitcher JP (1994) Association of patterns of labial salivary gland inflammation with Keratoconjunctivitis sicca. Analysis of 618 patients with suspected Sjogren’s syndrome. Arthritis Rheum 37(6):869–877

    Article  CAS  PubMed  Google Scholar 

  7. De Wilde PC, Baak JP, Houwelingen JC van et al (1986) Morphometric study of histological changes in sublabial salivary glands due to aging process. J Clin Pathol 39(4):406–417

    Article  Google Scholar 

  8. Egerer T, Martinez-Gamboa L, Dankof A et al (2006) Tissue-specific up-regulation of the proteasome subunit β5i (LMP7) in Sjögren’s syndrome. Arthritis Rheum 54(5):1501–1508

    Article  CAS  PubMed  Google Scholar 

  9. Godwin JT (1952) Benign lymphoepithelial lesion of the parotid gland adenolymphoma, chronic inflammation, lymphoepithelioma, lymphocytic tumor, Mikulicz disease. Cancer 5(6):1089–1103

    Article  CAS  PubMed  Google Scholar 

  10. Holl-Ulrich K, Noack F, Feller AC (2009) Vasculitis: histopathology and differential diagnosis. Z Rheumatol 68(4):320–328

    Article  CAS  PubMed  Google Scholar 

  11. Ianniello A, Ostuni PA, Sfriso P et al (1994) Usefulness of the labial salivary gland biopsy and new diagnostic criteria for Sjogren’s syndrome. Clin Exp Rheumatol 12(4):459–460

    CAS  PubMed  Google Scholar 

  12. Ihrler S, Zietz C, Sendelhofert A et al (1999) Lymphoepithelial duct lesions in Sjogren-type sialadenitis. Virchows Arch 434(4):315–323

    Article  CAS  PubMed  Google Scholar 

  13. Kikuchi M, Inagaki T, Ogawa K et al (2004) Histopathological investigation of salivary glands in the asymptomatic elderly. Arch Gerontol Geriatr 38(2):131–138

    Article  PubMed  Google Scholar 

  14. Krenn V, Morawietz L, Burmester GR et al (2006) Synovitis score: discrimination between chronic low-grade and high-grade synovitis. Histopathology 49(4):358–364

    Article  CAS  PubMed  Google Scholar 

  15. Lindahl G, Hedfors E (1989) Lymphocytic infiltrates and epithelial HLA-DR expression in lip salivary glands in connective tissue disease patients lacking sicca: a prospective study. Br J Rheumatol 28(4):293–298

    Article  CAS  PubMed  Google Scholar 

  16. Manthorpe R (2002) Sjögren’s syndrome criteria. Ann Rheum Dis 61(6):482–484

    Article  CAS  PubMed  Google Scholar 

  17. Manthrope R, Bredberg A, Henrikkson G et al (2006) Progress and regression within primary Sjögren’s syndrome. Scand J Rheumatol 35(1):1–6

    Article  Google Scholar 

  18. Marx RE, Hartman KS, Rethman KV (1988) A prospective study comparing incisional labial to incisional parotid biopsies in the detection and confirmation of sarcoidosis, Sjögren’s disease, sialosis and lymphoma. J Rheumatol 15(4):621

    CAS  PubMed  Google Scholar 

  19. McGuirt WF Jr, Whang C, Moreland W (2002) The role of parotid biopsy in the diagnosis of pediatric Sjogren syndrome. Arch Otolaryngol Head Neck Surg 128(11):1279–1281

    PubMed  Google Scholar 

  20. Morawietz L, Gehrke T, Schröder JH et al (2006) Histopathological diagnostics in endoprosthesis loosening. Pathologe 27(6):439–445

    Article  CAS  PubMed  Google Scholar 

  21. Morbini P, Manzo A, Caporali R, Epis et al (2005) Multilevel examination of minor salivary gland biopsy for Sjogren’s syndrome significantly improves diagnostic performance of AECG classification criteria. Arthritis Res Ther 7(2):343–348

    Article  Google Scholar 

  22. Morgan WS, Castleman B (1953) A clinicopathologic study of Mikulicz’s disease. Am J Pathol 29(3):471–503

    CAS  PubMed  Google Scholar 

  23. Pope JE (2002) Scleroderma overlap syndromes. Curr Opin Rheumatol 14(6):704–710

    Article  PubMed  Google Scholar 

  24. Ramos-Casals M, Brito-Zeron P, Garcia-Carrasco M et al (2004) Sarcoidosis or Sjögren syndrome? Clues to defining mimicry or coexistence in 59 cases. Medicine (Baltimore) 83(2):85–95

    Google Scholar 

  25. Skopouli FN, Dafni U, Ioannidis JP et al (2000) Clinical evolution and morbidity and mortality of primary Sjögren’s syndrome. Semin Arthritis Rheum 29(5):296–304

    Article  CAS  PubMed  Google Scholar 

  26. Stoltenburg-Didinger G, Genth E (2009) Dermatomyositis. Z Rheumatol 68(4):287–294

    Article  CAS  PubMed  Google Scholar 

  27. Stott DI, Hiepe F, Hummel M et al (1998) Antigen-driven clonal proliferation of B cells within the target tissue of an autoimmune disease. The salivary glands of patients with Sjögren’s syndrome. J Clin Invest 102(5):938–946

    Article  CAS  PubMed  Google Scholar 

  28. Tiemann M, Teymoortash A, Herbst H (2004) Lymphome der Kopfspeicheldrüsen. Pathologe 25(1):63–72

    Article  CAS  PubMed  Google Scholar 

  29. Vitali C, Bombardieri S, Moutsopoulos HM et al (1993) Preliminary criteria for the classification of Sjögren’s syndrome. Results of a prospective concerted action supported by the European Community. Arthritis Rheum 36(3):340–347

    Article  CAS  PubMed  Google Scholar 

  30. Vivino FB, Gala I, Hermann GA (2002) Change in final diagnosis on second evaluation of labial minor salivary gland biopsies. J Rheumatol 29(5):938–944

    PubMed  Google Scholar 

  31. Wise CM, Woodruff RD (1993) Minor salivary gland biopsies in patients investigated for primary Sjögren’s syndrome. A review of 187 patients. J Rheumatol 20(9):1515–1518

    CAS  PubMed  Google Scholar 

  32. Zandbelt MM, Wentink JR, Wilde PC de et al (2002) The synergistic value of focus score and IgA% score of sublabial salivary gland biopsy for the accuracy of the diagnosis of Sjögren’s syndrome: a 10-year comparison. Rheumatology (Oxford) 41(7):819–823

    Google Scholar 

Download references

Interessenkonflikt

Der korrespondierende Autor gibt an, dass kein Interessenkonflikt besteht.

Author information

Authors and Affiliations

  1. Zentrum für Histologie, Zytologie und Molekulare Diagnostik, Max-Planck-Str. 18+20, 54296, Trier, Deutschland

    V. Krenn, M. Jakobs & J. Kriegsmann

  2. Abteilung für Radioonkologie, Paracelsus Klinik, Osnabrück, Deutschland

    M.G. Krukemeyer

  3. Institut für Pathologie, Charité--Universitätsmedizin Berlin, Berlin, Deutschland

    A. Rieger

Authors
  1. V. Krenn
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. M. Jakobs
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. J. Kriegsmann
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. M.G. Krukemeyer
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. A. Rieger
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to V. Krenn.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Krenn, V., Jakobs, M., Kriegsmann, J. et al. Ist eine bioptische Sicherung beim Sjögren-Syndrom sinnvoll?. Z. Rheumatol. 69, 11–18 (2010). https://doi.org/10.1007/s00393-009-0514-7

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00393-009-0514-7

Schlüsselwörter

Keywords

Search

Navigation