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Overlap Syndromes

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Scleroderma

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Abstract

Systemic sclerosis (SSc) overlap syndrome is a term used to describe a very heterogeneous group of patients with features of different connective tissue diseases, combined with clinical signs of SSc. Up to now, no firm classification criteria for SSc overlap syndromes are established, but it is generally considered when musculoskeletal involvement or features of other rheumatic diseases are significantly greater than usually found in general SSc patients.

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References

  1. Iaccarino L, Gatto M, Bettio S, et al. Overlap connective tissue disease syndromes. Autoimmun Rev. 2013;12:363–73. Epub 2012/06/30.

    Article  CAS  PubMed  Google Scholar 

  2. Jury EC, D’Cruz D, Morrow WJ. Autoantibodies and overlap syndromes in autoimmune rheumatic disease. J Clin Pathol. 2001;54:340–7. Epub 2001/05/01.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  3. Balbir-Gurman A, Braun-Moscovici Y. Scleroderma overlap syndrome. Isr Med Assoc J. 2011;13:14–20. Epub 2011/03/31.

    PubMed  Google Scholar 

  4. Pakozdi A, Nihtyanova S, Moinzadeh P, et al. Clinical and serological hallmarks of systemic sclerosis overlap syndromes. J Rheumatol. 2011;38:2406–9. Epub 2011/08/17.

    Article  PubMed  Google Scholar 

  5. Preliminary criteria for the classification of systemic sclerosis (scleroderma). Subcommittee for scleroderma criteria of the American Rheumatism Association Diagnostic and Therapeutic Criteria Committee. Arthritis Rheum 1980;23:581–90. Epub 1980/05/01.

    Google Scholar 

  6. van den Hoogen F, Khanna D, Fransen J, et al. 2013 classification criteria for systemic sclerosis: an American College of Rheumatology/European League against rheumatism collaborative initiative. Arthritis Rheum. 2013;65:2737–47. Epub 2013/10/15.

    Article  PubMed  PubMed Central  Google Scholar 

  7. LeRoy EC, Black C, Fleischmajer R, et al. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol. 1988;15:202–5. Epub 1988/02/01.

    CAS  PubMed  Google Scholar 

  8. Galluccio F, Walker UA, Nihtyanova S, et al. Registries in systemic sclerosis: a worldwide experience. Rheumatology (Oxford). 2011;50:60–8. Epub 2010/12/15.

    Article  Google Scholar 

  9. Tan EM, Cohen AS, Fries JF, et al. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1982;25:1271–7. Epub 1982/11/01.

    Article  CAS  PubMed  Google Scholar 

  10. Kay J, Upchurch KS. ACR/EULAR 2010 rheumatoid arthritis classification criteria. Rheumatology (Oxford). 2012;51 Suppl 6:vi5–9. Epub 2012/12/19.

    Article  Google Scholar 

  11. Arnett FC, Edworthy SM, Bloch DA, et al. The American Rheumatism Association 1987 revised criteria for the classification of rheumatoid arthritis. Arthritis Rheum. 1988;31:315–24. Epub 1988/03/01.

    Article  CAS  PubMed  Google Scholar 

  12. Johnson PJ, McFarlane IG. Meeting report: International Autoimmune Hepatitis Group. Hepatology. 1993;18:998–1005. Epub 1993/10/01.

    Article  CAS  PubMed  Google Scholar 

  13. Habets WJ, de Rooij DJ, Salden MH, et al. Antibodies against distinct nuclear matrix proteins are characteristic for mixed connective tissue disease. Clin Exp Immunol. 1983;54:265–76. Epub 1983/10/01.

    CAS  PubMed  PubMed Central  Google Scholar 

  14. Tani C, Carli L, Vagnani S, et al. The diagnosis and classification of mixed connective tissue disease. J Autoimmun. 2014;48–49:46–9. Epub 2014/01/28.

    Article  CAS  PubMed  Google Scholar 

  15. Koschik 2nd RW, Fertig N, Lucas MR, et al. Anti-PM-Scl antibody in patients with systemic sclerosis. Clin Exp Rheumatol. 2012;30:S12–6. Epub 2012/01/21.

    PubMed  Google Scholar 

  16. Kaji K, Fertig N, Medsger Jr TA, et al. Autoantibodies to RuvBL1 and RuvBL2: a novel systemic sclerosis-related antibody associated with diffuse cutaneous and skeletal muscle involvement. Arthritis Care Res. 2014;66:575–84. Epub 2013/09/12.

    Article  CAS  Google Scholar 

  17. Gheita TA, Hussein H. Cartilage Oligomeric Matrix Protein (COMP) in systemic sclerosis (SSc): role in disease severity and subclinical rheumatoid arthritis overlap. Joint Bone Spine: Rev Rhum. 2012;79:51–6. Epub 2011/04/19.

    Article  CAS  Google Scholar 

  18. Moinzadeh P, Aberer E, Ahmadi-Simab K, et al. Disease progression in systemic sclerosis-overlap syndrome is significantly different from limited and diffuse cutaneous systemic sclerosis. Ann Rheum Dis. 2014. Epub 2014/01/07.

    Google Scholar 

  19. Elhai M, Avouac J, Kahan A, et al. Systemic sclerosis at the crossroad of polyautoimmunity. Autoimmun Rev. 2013;12:1052–7. Epub 2013/06/25.

    Article  CAS  PubMed  Google Scholar 

  20. Caramaschi P, Biasi D, Volpe A, et al. Coexistence of systemic sclerosis with other autoimmune diseases. Rheumatol Int. 2007;27:407–10. Epub 2006/10/19.

    Article  PubMed  Google Scholar 

  21. Hudson M, Rojas-Villarraga A, Coral-Alvarado P, et al. Polyautoimmunity and familial autoimmunity in systemic sclerosis. J Autoimmun. 2008;31:156–9. Epub 2008/07/23.

    Article  CAS  PubMed  Google Scholar 

  22. Hunzelmann N, Genth E, Krieg T, et al. The registry of the German Network for Systemic Scleroderma: frequency of disease subsets and patterns of organ involvement. Rheumatology (Oxford). 2008;47:1185–92. Epub 2008/06/03.

    Article  CAS  Google Scholar 

  23. Koumakis E, Dieude P, Avouac J, et al. Familial autoimmunity in systemic sclerosis – results of a French-based case-control family study. J Rheumatol. 2012;39:532–8. Epub 2012/01/17.

    Article  PubMed  Google Scholar 

  24. Bhansing KJ, Lammens M, Knaapen HK, et al. Scleroderma-polymyositis overlap syndrome versus idiopathic polymyositis and systemic sclerosis: a descriptive study on clinical features and myopathology. Arthritis Res Ther. 2014;16:R111. Epub 2014/06/03.

    Article  PubMed  PubMed Central  Google Scholar 

  25. Mahler M, Raijmakers R. Novel aspects of autoantibodies to the PM/Scl complex: clinical, genetic and diagnostic insights. Autoimmun Rev. 2007;6:432–7. Epub 2007/07/24.

    Article  CAS  PubMed  Google Scholar 

  26. Rigolet A, Musset L, Dubourg O, et al. Inflammatory myopathies with anti-Ku antibodies: a prognosis dependent on associated lung disease. Medicine. 2012;91:95–102. Epub 2012/03/07.

    Article  CAS  PubMed  Google Scholar 

  27. Lin HK, Wang JD, Fu LS. Juvenile diffuse systemic sclerosis/systemic lupus erythematosus overlap syndrome – a case report. Rheumatol Int. 2012;32:1809–11. Epub 2011/05/03.

    Article  PubMed  Google Scholar 

  28. Bloch KJ, Buchanan WW, Wohl MJ, et al. Sjoegren’s syndrome. A clinical, pathological, and serological study of sixty-two cases. Medicine. 1965;44:187–231. Epub 1965/05/01.

    Article  CAS  PubMed  Google Scholar 

  29. Ramos-Casals M, Brito-Zeron P, Font J. The overlap of Sjogren’s syndrome with other systemic autoimmune diseases. Semin Arthritis Rheum. 2007;36:246–55. Epub 2006/09/26.

    Article  PubMed  Google Scholar 

  30. Avouac J, Sordet C, Depinay C, et al. Systemic sclerosis-associated Sjogren’s syndrome and relationship to the limited cutaneous subtype: results of a prospective study of sicca syndrome in 133 consecutive patients. Arthritis Rheum. 2006;54:2243–9. Epub 2006/06/28.

    Article  CAS  PubMed  Google Scholar 

  31. Salliot C, Gottenberg JE, Bengoufa D, et al. Anticentromere antibodies identify patients with Sjogren’s syndrome and autoimmune overlap syndrome. J Rheumatol. 2007;34:2253–8. Epub 2007/10/17.

    CAS  PubMed  Google Scholar 

  32. Sharp GC, Irvin WS, Tan EM, et al. Mixed connective tissue disease – an apparently distinct rheumatic disease syndrome associated with a specific antibody to an extractable nuclear antigen (ENA). Am J Med. 1972;52:148–59. Epub 1972/02/01.

    Article  CAS  PubMed  Google Scholar 

  33. Ortega-Hernandez OD, Shoenfeld Y. Mixed connective tissue disease: an overview of clinical manifestations, diagnosis and treatment. Best Pract Res Clin Rheumatol. 2012;26:61–72. Epub 2012/03/20.

    Article  CAS  PubMed  Google Scholar 

  34. Hoffman RW, Greidinger EL. Mixed connective tissue disease. Curr Opin Rheumatol. 2000;12:386–90. Epub 2000/09/16.

    Article  CAS  PubMed  Google Scholar 

  35. Paradowska-Gorycka A, Jurkowska M, Czuszynska Z, et al. IL-10, IL-12B and IL-17 gene polymorphisms in patients with mixed connective tissue disease. Mod Rheumatol/Jpn Rheum Assoc. 2014;25:1–3. Epub 2014/08/28.

    Google Scholar 

  36. Sordet C, Goetz J, Sibilia J. Contribution of autoantibodies to the diagnosis and nosology of inflammatory muscle disease. Joint Bone Spine (revue du rhumatisme). 2006;73:646–54. Epub 2006/11/18.

    Article  CAS  Google Scholar 

  37. Mok CC, Cheung JC, Yee YK, et al. Unusual overlap of systemic lupus erythematosus and diffuse scleroderma. Clin Exp Rheumatol. 2001;19:113–4. Epub 2001/03/15.

    CAS  PubMed  Google Scholar 

  38. Cutolo M, Matucci Cerinic M. Nailfold capillaroscopy and classification criteria for systemic sclerosis. Clin Exp Rheumatol. 2007;25:663–5. Epub 2007/12/15.

    CAS  PubMed  Google Scholar 

  39. Cutolo M, Sulli A, Secchi ME, et al. The contribution of capillaroscopy to the differential diagnosis of connective autoimmune diseases. Best Pract Res Clin Rheumatol. 2007;21:1093–108. Epub 2007/12/11.

    Article  PubMed  Google Scholar 

  40. Valentini G, D’Angelo S, Della Rossa A, et al. European Scleroderma Study Group to define disease activity criteria for systemic sclerosis. IV. Assessment of skin thickening by modified Rodnan skin score. Ann Rheum Dis. 2003;62:904–5. Epub 2003/08/19.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  41. Czirjak L, Nagy Z, Aringer M, et al. The EUSTAR model for teaching and implementing the modified Rodnan skin score in systemic sclerosis. Ann Rheum Dis. 2007;66:966–9. Epub 2007/01/20.

    Article  PubMed  PubMed Central  Google Scholar 

  42. Akesson A, Fiori G, Krieg T, et al. Assessment of skin, joint, tendon and muscle involvement. Clin Exp Rheumatol. 2003;21:S5–8. Epub 2003/08/02.

    CAS  PubMed  Google Scholar 

  43. Hunzelmann N, Genth E, Krieg T, et al. Organ-specific diagnosis in patients with systemic sclerosis: recommendations of the German Network for Systemic Sclerosis (DNSS). Z Rheumatol. 2008;67:334–6. 7–40. Epub 2008/04/18. Organspezifische Diagnostik von Patienten mit systemischer Sklerodermie : Empfehlungen des Deutschen Netzwerkes fur Systemische Sklerodermie (DNSS).

    Article  CAS  PubMed  Google Scholar 

  44. Jaovisidha K, Csuka ME, Almagro UA, et al. Severe gastrointestinal involvement in systemic sclerosis: report of five cases and review of the literature. Semin Arthritis Rheum. 2005;34:689–702. Epub 2005/02/05.

    Article  PubMed  Google Scholar 

  45. Thakkar V, Stevens W, Prior D, et al. The inclusion of N-terminal pro-brain natriuretic peptide in a sensitive screening strategy for systemic sclerosis-related pulmonary arterial hypertension: a cohort study. Arthritis Res Ther. 2013;15:R193. Epub 2013/11/20.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  46. Steen VD, Mayes MD, Merkel PA. Assessment of kidney involvement. Clin Exp Rheumatol. 2003;21:S29–31. Epub 2003/08/02.

    CAS  PubMed  Google Scholar 

  47. Kistler AD. [In Process Citation]. Therapeutische Umschau Revue therapeutique. Lupusnephritis. 2015;72:171–7. Epub 2015/02/28.

    Google Scholar 

  48. Santiago ML, Seisdedos MR, Garcia Salinas RN, et al. Usefulness of antibodies and minor salivary gland biopsy in the study of sicca syndrome in daily clinical practice. Utilidad de los anticuerpos y de la biopsia de glandula salival menor en el estudio del complejo sicca en la practica diaria. Rheumatol Clin. 2015;11(3):156–60. Epub 2015/01/13.

    Google Scholar 

  49. Kowal-Bielecka O, Distler O. Use of methotrexate in patients with scleroderma and mixed connective tissue disease. Clin Exp Rheumatol. 2010;28:S160–3. Epub 2010/11/26.

    CAS  PubMed  Google Scholar 

  50. Fendler C, Braun J. Use of methotrexate in inflammatory myopathies. Clin Exp Rheumatol. 2010;28:S164–7. Epub 2010/11/26.

    CAS  PubMed  Google Scholar 

  51. Walker KM, Pope J. Expert agreement on EULAR/EUSTAR recommendations for the management of systemic sclerosis. J Rheumatol. 2011;38:1326–8. Epub 2011/04/05.

    Article  PubMed  Google Scholar 

  52. Levy Y, Amital H, Langevitz P, et al. Intravenous immunoglobulin modulates cutaneous involvement and reduces skin fibrosis in systemic sclerosis: an open-label study. Arthritis Rheum. 2004;50:1005–7. Epub 2004/03/17.

    Article  CAS  PubMed  Google Scholar 

  53. Levine TD. Rituximab in the treatment of dermatomyositis: an open-label pilot study. Arthritis Rheum. 2005;52:601–7. Epub 2005/02/05.

    Article  CAS  PubMed  Google Scholar 

  54. Mok CC, Ho LY, To CH. Rituximab for refractory polymyositis: an open-label prospective study. J Rheumatol. 2007;34:1864–8. Epub 2007/08/28.

    CAS  PubMed  Google Scholar 

  55. Lundberg IE. The prognosis of mixed connective tissue disease. Rheum Dis Clin N Am. 2005;31:535–47. vii–viii. Epub 2005/08/09.

    Article  Google Scholar 

  56. Feltsan T, Stanko P, Mracna J. Sjogren’s syndrome in present. Bratisl Lek Listy. 2012;113:514–6. Epub 2012/08/18.

    CAS  PubMed  Google Scholar 

  57. van den Hoogen FH, Boerbooms AM, Swaak AJ, et al. Comparison of methotrexate with placebo in the treatment of systemic sclerosis: a 24 week randomized double-blind trial, followed by a 24 week observational trial. Br J Rheumatol. 1996;35:364–72. Epub 1996/04/01.

    Article  PubMed  Google Scholar 

  58. Pope JE, Bellamy N, Seibold JR, et al. A randomized, controlled trial of methotrexate versus placebo in early diffuse scleroderma. Arthritis Rheum. 2001;44:1351–8. Epub 2001/06/16.

    Article  CAS  PubMed  Google Scholar 

  59. Das SN, Alam MR, Islam N, et al. Placebo controlled trial of methotrexate in systemic sclerosis. Mymensingh Med J. 2005;14:71–4. Epub 2005/02/08.

    CAS  PubMed  Google Scholar 

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Author information

Authors and Affiliations

  1. Department of Dermatology and Venerology, University of Cologne, Cologne, RNW, 50937, Germany

    Pia Moinzadeh MD

  2. Division of Medicine, Department of Inflammation, Centre for Rheumatology and Connective Tissue Diseases, UCL Division of Medicine, Royal Free Hospital, London, UK

    Christopher P. Denton PhD, FRCP

Authors
  1. Pia Moinzadeh MD
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  2. Christopher P. Denton PhD, FRCP
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Corresponding author

Correspondence to Christopher P. Denton PhD, FRCP .

Editor information

Editors and Affiliations

  1. Feinberg School of Medicine, Northwestern University, Chicago, Illinois, USA

    John Varga

  2. Division of Medicine, Department of Inflammation, Centre for Rheumatology and Connective Tissue Diseases, UCL Division of Medicine, Royal Free Hospital, London, United Kingdom

    Christopher P. Denton

  3. Division of Rheumatology, Johns Hopkins University, Baltimore, Maryland, USA

    Fredrick M. Wigley

  4. Service de Rhumatologie A, INSERMU1016, Universit. Paris Descartes, Hôpital Cochin, Paris, France

    Yannick Allanore

  5. Department of Allergy and Rheumatology, Nippon Medical School, Graduate School of Medicine, Tokyo, Japan

    Masataka Kuwana

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Moinzadeh, P., Denton, C.P. (2017). Overlap Syndromes. In: Varga, J., Denton, C., Wigley, F., Allanore, Y., Kuwana, M. (eds) Scleroderma. Springer, Cham. https://doi.org/10.1007/978-3-319-31407-5_6

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  • DOI: https://doi.org/10.1007/978-3-319-31407-5_6

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