Skip to main content
PMC home page
Annals of the Rheumatic Diseases logoLink to Annals of the Rheumatic Diseases
. 2001 Nov;60(11):1046–1049. doi: 10.1136/ard.60.11.1046

Rheumatic disorders and primary biliary cirrhosis: an appraisal of 170 Italian patients

B Marasini 1, M Gagetta 1, V Rossi 1, P Ferrari 1
PMCID: PMC1753414  PMID: 11602476

Abstract

OBJECTIVE—To establish the frequency of connective tissue diseases (CTD) in a cohort of Italian patients with primary biliary cirrhosis (PBC) and to evaluate the availability of a marker for the early identification of the more common associated CTD.
METHODS—One hundred and seventy consecutive patients with histologically diagnosed PBC were screened for the presence of a CTD and/or Raynaud's phenomenon (RP). Patients were classified as having a CTD only if they fulfilled standardised criteria.
RESULTS—Forty seven patients had a CTD. The most common CTD was systemic sclerosis (SSc), found in 21 patients. RP was present in 54 patients, most of whom (n=39) had an associated CTD. The most prevalent autoantibodies included antinuclear antibodies (ANA) with anticentromere (ACA) and speckled patterns (34 and 33 patients, respectively) and extractable nuclear antigens (ENA, 27 patients). However, while the frequencies of ACA and ENA were significantly higher in patients with an associated CTD (p<0.0001 and p<0.005, respectively), no relationship was found for speckled ANA. ACA was the best predictor of a CTD in patients with PBC (odds ratio (OR) 24.5, 95% CI 5.5 to 108.8), followed by the presence of ENA (OR 23.9, 95% CI 5.6 to 101.0) and RP (OR 20.2, 95% CI 5.7 to 71.2).
CONCLUSIONS—Using strict standardised classification criteria we have found that SSc is the most common CTD associated with PBC and that ACA and ENA are strong markers for an associated CTD in patients with PBC.



Full Text

The Full Text of this article is available as a PDF (122.8 KB).

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Akimoto S., Ishikawa O., Takagi H., Miyachi Y. Immunological features of patients with primary biliary cirrhosis (PBC) overlapping systemic sclerosis: a comparison with patients with PBC alone. J Gastroenterol Hepatol. 1998 Sep;13(9):897–901. doi: 10.1111/j.1440-1746.1998.tb00758.x. [DOI] [PubMed] [Google Scholar]
  2. Arnett F. C., Edworthy S. M., Bloch D. A., McShane D. J., Fries J. F., Cooper N. S., Healey L. A., Kaplan S. R., Liang M. H., Luthra H. S. The American Rheumatism Association 1987 revised criteria for the classification of rheumatoid arthritis. Arthritis Rheum. 1988 Mar;31(3):315–324. doi: 10.1002/art.1780310302. [DOI] [PubMed] [Google Scholar]
  3. Artlett C. M., Cox L. A., Jimenez S. A. Detection of cellular microchimerism of male or female origin in systemic sclerosis patients by polymerase chain reaction analysis of HLA-Cw antigens. Arthritis Rheum. 2000 May;43(5):1062–1067. doi: 10.1002/1529-0131(200005)43:5<1062::AID-ANR16>3.0.CO;2-P. [DOI] [PubMed] [Google Scholar]
  4. Belch J. J. Raynaud's phenomenon. Curr Opin Rheumatol. 1990 Dec;2(6):937–941. doi: 10.1097/00002281-199002060-00012. [DOI] [PubMed] [Google Scholar]
  5. Beswick D. R., Klatskin G., Boyer J. L. Asymptomatic primary biliary cirrhosis. A progress report on long-term follow-up and natural history. Gastroenterology. 1985 Aug;89(2):267–271. [PubMed] [Google Scholar]
  6. Bohan A., Peter J. B. Polymyositis and dermatomyositis (first of two parts). N Engl J Med. 1975 Feb 13;292(7):344–347. doi: 10.1056/NEJM197502132920706. [DOI] [PubMed] [Google Scholar]
  7. Cimmino M. A., Parisi M., Moggiana G., Mela G. S., Accardo S. Prevalence of rheumatoid arthritis in Italy: the Chiavari Study. Ann Rheum Dis. 1998 May;57(5):315–318. doi: 10.1136/ard.57.5.315. [DOI] [PMC free article] [PubMed] [Google Scholar]
  8. Clarke A. K., Galbraith R. M., Hamilton E. B., Williams R. Rheumatic disorders in primary biliary cirrhosis. Ann Rheum Dis. 1978 Feb;37(1):42–47. doi: 10.1136/ard.37.1.42. [DOI] [PMC free article] [PubMed] [Google Scholar]
  9. Culp K. S., Fleming C. R., Duffy J., Baldus W. P., Dickson E. R. Autoimmune associations in primary biliary cirrhosis. Mayo Clin Proc. 1982 Jun;57(6):365–370. [PubMed] [Google Scholar]
  10. Invernizzi P., Battezzati P. M., Crosignani A., Zermiani P., Bignotto M., Del Papa N., Zuin M., Podda M. Antibody to carbonic anhydrase II is present in primary biliary cirrhosis (PBC) irrespective of antimitochondrial antibody status. Clin Exp Immunol. 1998 Dec;114(3):448–454. doi: 10.1046/j.1365-2249.1998.00735.x. [DOI] [PMC free article] [PubMed] [Google Scholar]
  11. Jacobson D. L., Gange S. J., Rose N. R., Graham N. M. Epidemiology and estimated population burden of selected autoimmune diseases in the United States. Clin Immunol Immunopathol. 1997 Sep;84(3):223–243. doi: 10.1006/clin.1997.4412. [DOI] [PubMed] [Google Scholar]
  12. Kaplan M. M. Primary biliary cirrhosis. N Engl J Med. 1996 Nov 21;335(21):1570–1580. doi: 10.1056/NEJM199611213352107. [DOI] [PubMed] [Google Scholar]
  13. Klippel J. H. Systemic lupus erythematosus: demographics, prognosis, and outcome. J Rheumatol Suppl. 1997 May;48:67–71. [PubMed] [Google Scholar]
  14. Maddison P. J. Mixed connective tissue disease, overlap syndromes, and eosinophilic fasciitis. Ann Rheum Dis. 1991 Nov;50 (Suppl 4):887–893. doi: 10.1136/ard.50.suppl_4.887. [DOI] [PMC free article] [PubMed] [Google Scholar]
  15. Marasini B., Pipia C., DeValle G., Crosignani A., Petroni L., Cugno M., Zeni S., Bruno S., Bassani C. Vascular impairment in patients with primary biliary cirrhosis. Int J Microcirc Clin Exp. 1995 Mar-Apr;15(2):75–79. doi: 10.1159/000178953. [DOI] [PubMed] [Google Scholar]
  16. Mayo M. J., Combes B., Jenkins R. N. T-cell receptor Vbeta gene utilization in primary biliary cirrhosis. Hepatology. 1996 Nov;24(5):1148–1155. doi: 10.1002/hep.510240528. [DOI] [PubMed] [Google Scholar]
  17. Medsger T. A., Jr Epidemiology of systemic sclerosis. Clin Dermatol. 1994 Apr-Jun;12(2):207–216. doi: 10.1016/s0738-081x(94)90323-9. [DOI] [PubMed] [Google Scholar]
  18. Modena V., Marengo C., Amoroso A., Rosina F., Costantini P., Bellando P., Coppo R., Rizzetto M. Primary biliary cirrhosis and rheumatic diseases: a clinical, immunological and immunogenetical study. Clin Exp Rheumatol. 1986 Apr-Jun;4(2):129–134. [PubMed] [Google Scholar]
  19. Myszor M., James O. F. The epidemiology of primary biliary cirrhosis in north-east England: an increasingly common disease? Q J Med. 1990 Apr;75(276):377–385. [PubMed] [Google Scholar]
  20. Nyberg A., Löf L. Primary biliary cirrhosis: clinical features and outcome, with special reference to asymptomatic disease. Scand J Gastroenterol. 1989 Jan;24(1):57–64. doi: 10.3109/00365528909092240. [DOI] [PubMed] [Google Scholar]
  21. Parikh-Patel A., Gold E., Mackay I. R., Gershwin M. E. The geoepidemiology of primary biliary cirrhosis: contrasts and comparisons with the spectrum of autoimmune diseases. Clin Immunol. 1999 May;91(2):206–218. doi: 10.1006/clim.1999.4690. [DOI] [PubMed] [Google Scholar]
  22. Saarinen U. M., Kajosaari M. Does dietary elimination in infancy prevent or only postpone a food allergy? A study of fish and citrus allergy in 375 children. Lancet. 1980 Jan 26;1(8161):166–167. doi: 10.1016/s0140-6736(80)90657-1. [DOI] [PubMed] [Google Scholar]
  23. Sharp G. C., Irvin W. S., May C. M., Holman H. R., McDuffie F. C., Hess E. V., Schmid F. R. Association of antibodies to ribonucleoprotein and Sm antigens with mixed connective-tissue disease, systematic lupus erythematosus and other rheumatic diseases. N Engl J Med. 1976 Nov 18;295(21):1149–1154. doi: 10.1056/NEJM197611182952101. [DOI] [PubMed] [Google Scholar]
  24. Sherer Y., Shoenfeld Y. Autoimmune diseases and autoimmunity post-bone marrow transplantation. Bone Marrow Transplant. 1998 Nov;22(9):873–881. doi: 10.1038/sj.bmt.1701437. [DOI] [PubMed] [Google Scholar]
  25. Sherlock S., Scheuer P. J. The presentation and diagnosis of 100 patients with primary biliary cirrhosis. N Engl J Med. 1973 Sep 27;289(13):674–678. doi: 10.1056/NEJM197309272891306. [DOI] [PubMed] [Google Scholar]
  26. Siimes M. A., Johansson E., Rapola J. Scleroderma-like graft-versus-host disease as late consequence of bone-marrow grafting. Lancet. 1977 Oct 15;2(8042):831–832. doi: 10.1016/s0140-6736(77)90772-3. [DOI] [PubMed] [Google Scholar]
  27. Tan E. M., Cohen A. S., Fries J. F., Masi A. T., McShane D. J., Rothfield N. F., Schaller J. G., Talal N., Winchester R. J. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1982 Nov;25(11):1271–1277. doi: 10.1002/art.1780251101. [DOI] [PubMed] [Google Scholar]
  28. Uddenfeldt P., Danielsson A. Evaluation of rheumatic disorders in patients with primary biliary cirrhosis. Ann Clin Res. 1986;18(3):148–153. [PubMed] [Google Scholar]
  29. Vitali C., Bombardieri S., Moutsopoulos H. M., Coll J., Gerli R., Hatron P. Y., Kater L., Konttinen Y. T., Manthorpe R., Meyer O. Assessment of the European classification criteria for Sjögren's syndrome in a series of clinically defined cases: results of a prospective multicentre study. The European Study Group on Diagnostic Criteria for Sjögren's Syndrome. Ann Rheum Dis. 1996 Feb;55(2):116–121. doi: 10.1136/ard.55.2.116. [DOI] [PMC free article] [PubMed] [Google Scholar]
  30. Walsh S. J., Rau L. M. Autoimmune diseases: a leading cause of death among young and middle-aged women in the United States. Am J Public Health. 2000 Sep;90(9):1463–1466. doi: 10.2105/ajph.90.9.1463. [DOI] [PMC free article] [PubMed] [Google Scholar]

Articles from Annals of the Rheumatic Diseases are provided here courtesy of BMJ Publishing Group

RESOURCES