Advertisement

Oral Signs of Connective Tissue Disease

  • Kenisha R. Heath
  • Nasim FazelEmail author
Chapter

Abstract

In this chapter, we aim to discuss the epidemiology, pathogenesis, differential diagnosis, and treatment of oral manifestations encountered in the autoimmune diseases of Sjögren’s syndrome (SS), systemic lupus erythematosus (SLE), systemic sclerosis (SSc), mixed connective tissue disease (MCTD), and dermatomyositis (DM). Systemic autoimmune conditions are estimated to affect 5%–8% of Americans. Oral manifestations are encountered with high frequency and are often the first clinical signs or symptoms of the disease.

Keywords

Sjögren’s syndrome Systemic lupus erythematosus Systemic sclerosis Mixed connective tissue disease Dermatomyositis 

References

  1. 1.
    Autoimmune Diseases Coordinating Committee. Department of Health and Human Services, National Institutes of Health, National Institute of Allergy and Infectious Disease; 2002. NIH Publication No. 03-5140.Google Scholar
  2. 2.
    Jonsson R, Bowman SJ, Gordon TP. Sjögren’s syndrome. In: Koopman WJ, Moreland LW, editors. Arthritis and allied disorders (a textbook of rheumatology). 15th ed. Philadelphia: Lippincott Williams & Wilkins; 2005. p. 1681–706.Google Scholar
  3. 3.
    Venables PJ. Management of patients presenting with Sjogren’s syndrome. Best Pract Res Clin Rheumatol. 2006;20(4):791–807.PubMedGoogle Scholar
  4. 4.
    Skopouli FN, Drosos AA, Papaioannou T, Moutsopoulos HM. Preliminary diagnostic criteria for Sjögren’s syndrome. Scand J Rheumatol. 1986;61(Suppl):22–5.Google Scholar
  5. 5.
    Manoussakis MN, Georgopoulou C, Zintzaras E, Spyropoulou M, Stavropoulou A, Skopouli FN, et al. Sjögren’s syndrome associated with systemic lupus erythematosus: clinical and laboratory profiles and comparison with primary Sjögren’s syndrome. Arthritis Rheumatol. 2004;50:882–91.Google Scholar
  6. 6.
    Vitali C, Bombardieri S, Moutsopoulos HM. Classification criteria for Sjogren’s syndrome: a revised version of the European criteria proposed by the American European consensus group. Ann Rheum Dis. 2002;61:554–8.PubMedPubMedCentralGoogle Scholar
  7. 7.
    Langegger C, Wenger M, Duftner C, Dejaco C, Baldissera I, Moncayo R, et al. Use of the European preliminary criteria, the Breiman classification tree and the American-European criteria for diagnosis of primary Sjögren’s syndrome in daily practice: a retrospective analysis. Rheumatol Int. 2007;27:699–702.PubMedGoogle Scholar
  8. 8.
    Uhlig T, Kvien TK, Jensen JL, Ax T II. Sicca symptoms, saliva and tear production, and disease variables in 636 patients with rheumatoid arthritis. Ann Rheum Dis. 1999;58:415–22.PubMedPubMedCentralGoogle Scholar
  9. 9.
    Qin B, Wang J, Yang Z, Yang M, Ma N, Huang F, Zhong R. Epidemiology of primary Sjögren’s syndrome: a systematic review and meta-analysis. Ann Rheum Dis. 2015;74(11):1983–9.PubMedGoogle Scholar
  10. 10.
    Fox R. Sjögren’s syndrome. Lancet. 2005;366:321–31.PubMedGoogle Scholar
  11. 11.
    Matthews SA, Kurien BT, Scofield RH. Oral manifestations of Sjögren’s syndrome. J Dent Res. 2008;87:308.Google Scholar
  12. 12.
    Roguedas AM, Misery L, Sassolas B, Le Masson G, Pennec YL, Youinou P. Cutaneous manifestations of primary Sjögren’s syndrome are underestimated. Clin Exp Rheumatol. 2004;22:632–6.PubMedGoogle Scholar
  13. 13.
    Alexander EL, Provost TT. Cutaneous manifestations of primary Sjögren’s syndrome: a reflection of vasculitis and association with anti-Ro (SSA) antibodies. J Investig Dermatol. 1983;80:386–91.PubMedGoogle Scholar
  14. 14.
    Jubert C, Cosnes A, Clerici T, Gaulard P, Andre P, Revuz J, et al. Sjogren’s syndrome and cutaneous B cell lymphoma revealed by anetoderma. Arthritis Rheumatol. 1993;36:133.Google Scholar
  15. 15.
    Stroehmann A, Dorner T, Lukowsky A, Feist E, Hiepe F, Burmester GR. Cutaneous T cell lymphoma in a patient with primary biliary cirrhosis and secondary Sjogren’s syndrome. J Rheumatol. 2002;29:1326.PubMedGoogle Scholar
  16. 16.
    Selva-O’Callaghan A, Perez-Lopez J, Solans-Laque R, Lopez-Peig C, Angel-Bosch GJ, Vilardell-Tarres M. Primary cutaneous large B-cell lymphoma of the legs in a patient with primary Sjogren’s syndrome. Clin Exp Rheumatol. 2003;21:672.PubMedGoogle Scholar
  17. 17.
    Sarzi-Puttini P, Doria A, Girolomoni G, Kuhn A. The skin in systemic autoimmune diseases. In: Bombardieri S, Baldini C, editors. Handbook of systemic autoimmune diseases, Ch 12 Mucocutaneous manifestations of Sjogren’s syndrome: Elsevier B.V.; 2006.  https://doi.org/10.1016/S1571-5078(05)05012-9.
  18. 18.
    Mandl T, Ekberg O, Wollmer P, Manthorpe R, Jacobsson L. Dysphagia and dysmotility of the pharynx and oesophagus in patients with primary Sjögren’s syndrome. Scand J Rheumatol. 2007;36:394–401.PubMedGoogle Scholar
  19. 19.
    Delande S, de Seze J, Fauchais AL, Hachulla E, Stojkovic T, Ferriby D, et al. Neurologic manifestations in primary Sjögren syndrome: a study of 82 patients. Medicine. 2004;83:280–91.Google Scholar
  20. 20.
    Barendregt PJ, Van den Bent MJ, Van Raaijvan den Aarssen VJ, Van den Meiracker AH, Vecht CJ, Markusse HM. Involvement of the peripheral nervous system in primary Sjögren’s syndrome. Ann Rheum Dis. 2001;60:876–81.PubMedPubMedCentralGoogle Scholar
  21. 21.
    Napeñas JJ, Rouleau TS. Oral complications of Sjögren’s syndrome. Oral Maxillofac Surg Clin N Am. 2014;26:55–62.Google Scholar
  22. 22.
    Astor FC, Hanft KL, Ciocon JO. Xerostomia: a prevalent condition in the elderly. Ear Nose Throat J. 1999;78:476–9.PubMedGoogle Scholar
  23. 23.
    Mavragani CP, Moutsopoulos NM, Moutsopoulos HM. The management of Sjögren’s syndrome. Nat Clin Pract Rheumatol. 2006;2:252–61.PubMedGoogle Scholar
  24. 24.
    Perdson AM, Reibel J, Nordgarden H, Bergrem HO, Jensen JL, Nauntofte B. Primary Sjögren’s syndrome: salivary gland function and clinical oral findings. Oral Dis. 1999;5:128–38.Google Scholar
  25. 25.
    Gonzalez S, Sung H, Sepulveda D, Gonzalez MJ, Molina C. Oral manifestations and their treatment in Sjögren’s syndrome. Oral Dis. 2014;20(2):153–61.PubMedGoogle Scholar
  26. 26.
    Soto-Rojas AE, Kraus A. The oral side of Sjögren syndrome. Diagnosis and treatment. A review. Arch Med Res. 2002;33:96–106.Google Scholar
  27. 27.
    Pertovaara M, Korpela M, Uusitalo H, Pukander J, Miettinen A, Helin H, et al. Clinical follow up study of 87 patients with sicca symptoms (dryness of eyes or mouth, or both). Ann Rheum Dis. 1999;58:423–7.PubMedPubMedCentralGoogle Scholar
  28. 28.
    Harris NL. Lymphoid proliferations of the salivary glands. Am J Clin Pathol. 1999;111:S94–103.PubMedGoogle Scholar
  29. 29.
    Russell SL, Reisine S. Investigation of xerostomia in patients with rheumatoid arthritis. J Am Dent Assoc. 1998;129:733–9.PubMedGoogle Scholar
  30. 30.
    Percival RS, Challacombe SJ, Marsh PD. Flow rates of resting whole and stimulated parotid saliva in relation to age and gender. J Dent Res. 1994;73(8):1416–20.PubMedGoogle Scholar
  31. 31.
    Scott J. Qualitative and quantitative observations on the histology of human labial salivary glands obtained post mortem. J Biol Buccale. 1980;8(3):187–200.PubMedGoogle Scholar
  32. 32.
    Greenburg G, Anderson R, Sharpstone P, James DG. Enlargement of parotid gland due to sarcoidosis. Br Med J. 1964;2(5413):861–2.Google Scholar
  33. 33.
    Kazi S, Cohen PR, Williams F, Schempp R, Reveille JD. The diffuse infiltrative lymphocytosis syndrome. Clinical and immunogenetic features in 35 patients. AIDS. 1996;10(4):385.PubMedGoogle Scholar
  34. 34.
    Cheuk W, Chan JK. IgG4-related sclerosing disease: a critical appraisal of an evolving clinicopathologic entity. Adv Anat Pathol. 2010;17(5):303.PubMedGoogle Scholar
  35. 35.
    Liu SY, Vlantis AC, Lee WC. Bilateral parotid and submandibular gland enlargement: rare features of Wegener’s granulomatosis. J Laryngol Otol. 2003;117(2):148–50.PubMedGoogle Scholar
  36. 36.
    Feinstein AJ, Ciarleglio MM, Cong X, Otremba MD, Judson BL. Parotid gland lymphoma: prognostic analysis of 2140 patients. Laryngoscope. 2013;123(5):1199.PubMedGoogle Scholar
  37. 37.
    Amarasena R, Bowman S. Sjögren’s syndrome. Clin Med. 2007;7:53–6.Google Scholar
  38. 38.
    Van der Reijden WA, Vissink A, Veerman E, Nieuw-Amerongen AV. Treatment of oral dryness related complaints (xerostomia) in Sjögren’s syndrome. Ann Rheum Dis. 1999;58:465–73.PubMedPubMedCentralGoogle Scholar
  39. 39.
    Kruize AA, Hene RJ, Kallenberg CG, van Bijsterveld OP, van der Heide A, Kater L, et al. Hydroxychloroquine treatment for primary Sjogren’s syndrome; a two year, double blind cross over trial. Ann Rheum Dis. 1993;52:60–4.Google Scholar
  40. 40.
    Petrone D, Condemi JJ, Fife R, Gluck O, Cohen S, Dalgin P. A double-blind, randomized, placebo-controlled study of cevimeline in Sjogren’s syndrome patients with xerostomia and keratoconjunctivitis sicca. Arthritis Rheum. 2002;46:748–54.PubMedGoogle Scholar
  41. 41.
    Fife RS, Chase WF, Dore RK, Wiesenhutter CW, Lockhart PB, Tindal E, et al. Cevimeline for the treatment of xerostomia in patients with Sjögren syndrome: a randomized trial. Arch Intern Med. 2002;162:1293–300.PubMedGoogle Scholar
  42. 42.
    Thanou-Stavraki A, James JA. Primary Sjögren’s syndrome: current and prospective therapies. Semin Arthritis Rheum. 2008;37(5):273–92.PubMedGoogle Scholar
  43. 43.
    Steinfeld SD, Demols P, Salmon I, Kiss R, Appelboom T. Infliximab in patients with primary Sjögren’s syndrome: a pilot study. Arthritis Rheumatol. 2001;44:2371–5.Google Scholar
  44. 44.
    Sankar V, Brennan MT, Kok MR, Leakan RA, Smith JA, Manny J, et al. Etanercept in Sjögren’s syndrome: a twelve-week randomized, double-blind, placebo-controlled pilot clinical trial. Arthritis Rheumatol. 2004;50:2240–5.Google Scholar
  45. 45.
    Goldenberg DM. Epratuzumab in the therapy of oncological and immunological diseases. Expert Rev Anticancer Ther. 2006;6:1341–53.PubMedGoogle Scholar
  46. 46.
    Steinfeld SD, Tant L, Burmester GR, Teoh NK, Wegener WA, Goldenberg DM, et al. Epratuzumab (humanized anti-CD22 antibody) in primary Sjögren’s syndrome: an open-label phase I/II study. Arthritis Res Ther. 2006;8:129.Google Scholar
  47. 47.
    Ramos-Casals M, Brito-Zerón P. Emerging biological therapies in primary Sjögren’s syndrome. Rheumatology. 2007;46:1389–96.PubMedGoogle Scholar
  48. 48.
    Steller M, Chou L, Daniels TE. Electrical stimulation of salivary flow in patients with Sjögren’s syndrome. J Dent Res. 1988;67:1334–7.PubMedGoogle Scholar
  49. 49.
    Fortuna G, Brennan M. Systemic lupus erythematosus epidemiology, pathophysiology, manifestations, and management. Dent Clin N Am. 2013;57:631–55.PubMedGoogle Scholar
  50. 50.
    Schur PH, Hahn BH. Epidemiology and pathogenesis of systemic lupus erythematosus. In: Basow DS, editor. UpToDate. Netherlands: Wolters Kluwer; 2013. Retrieved 17 Sept 2014.Google Scholar
  51. 51.
    Pons-Estel GJ, Alacron GS, Scofield L, Reinlib L, Cooper GS. Understanding the epidemiology and progression of systemic lupus erythematous. Semin Arthritis Rheum. 2010;39:257–68.PubMedGoogle Scholar
  52. 52.
    Pisetsky DS. Systemic lupus erythematosus. A. Epidemiology, pathology, and pathogenesis. In: Klippel JH, Crofford LJ, Stone JH, Weyand CM, editors. Primer on the rheumatic diseases. Atlanta: The Arthritis Foundation; 2001. p. 329–52.Google Scholar
  53. 53.
    Tan EM, Cohen AS, Fries JF, Masi AT, McShane DJ, Rothfield NF, et al. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheumatol. 1982;25(11):1271–7.Google Scholar
  54. 54.
    Boumpas DT, Fessler BJ, Austin HA, Balow JE, Klippel JH, Lockshin MD. Systemic lupus erythematosus: emerging concepts. Part 2: dermatologic and joint disease, the antiphospholipid antibody syndrome, pregnancy and hormonal therapy, morbidity and mortality, and pathogenesis. Ann Intern Med. 1995;123(1):42–53.PubMedGoogle Scholar
  55. 55.
    Tench CM, McCurdie I, White PD, D’Cruz DP. The prevalence and associations of fatigue in systemic lupus erythematosus. Rheumatology. 2000;39:1249–54.PubMedGoogle Scholar
  56. 56.
    Stahl NI, Klippel JH, Decker JL. Fever in systemic lupus erythematosus. Am J Med. 1979;67:935–40.PubMedGoogle Scholar
  57. 57.
    Brennan M, Valerin M, Napenas J, Lockhart PB. Oral manifestations of patients with lupus erythematosus. Dent Clin N Am. 2005;49:127–41.PubMedGoogle Scholar
  58. 58.
    Grossman JM. Lupus arthritis. Best Pract Res Clin Rheumatol. 2009;23:495–506.PubMedGoogle Scholar
  59. 59.
    Cronin ME. Musculoskeletal manifestations of systemic lupus erythematosus. Rheum Dis Clin N Am. 1988;14:99–116.Google Scholar
  60. 60.
    Borchers AT, Leibushor N, Naguwa SM, Cheema GS, Shoenfeld Y, Gershwin ME. Lupus nephritis: a critical review. Autoimmun Rev. 2012;12:174–94.PubMedGoogle Scholar
  61. 61.
    Tian XP, Zhang X. Gastrointestinal involvement in systemic lupus erythematosus: insight into pathogenesis, diagnosis and treatment. World J Gastroenterol. 2010;16:2971–7.PubMedPubMedCentralGoogle Scholar
  62. 62.
    Quadrelli SA, Alvarez C, Arce SC, Paz L, Sarano J, Sobrino EM, et al. Pulmonary involvement of systemic lupus erythematosus: analysis of 90 necropsies. Lupus. 2009;18:1053–60.PubMedGoogle Scholar
  63. 63.
    Kamen DL, Strange C. Pulmonary manifestations of systemic lupus erythematosus. Clin Chest Med. 2010;31:479–88.PubMedGoogle Scholar
  64. 64.
    Frostegard J. SLE, atherosclerosis and cardiovascular disease. J Intern Med. 2005;257:485–95.PubMedGoogle Scholar
  65. 65.
    Hanly JG, Fisk JD, McCurdy G, Fougere L, Douglas J. Neuropsychiatric syndromes in patients with systemic lupus erythematosus and rheumatoid arthritis. J Rheumatol. 2005;32:1459–66.PubMedGoogle Scholar
  66. 66.
    Mok CC, Lau CS, Wong RW. Neuropsychiatric manifestations and their clinical associations in southern Chinese patients with systemic lupus erythematosus. J Rheumatol. 2001;28:766–71.PubMedGoogle Scholar
  67. 67.
    Hepburn AL, Narat S, Mason JC. The management of peripheral blood cytopenias in systemic lupus erythematosus. Rheumatology (Oxford). 2010;49:2243–54.Google Scholar
  68. 68.
    Palejwala NV, Walia HS, Yeh S. Ocular manifestations of systemic lupus erythematosus: a review of the literature. Autoimmune Dis. 2012;2012:290898.  https://doi.org/10.1155/2012/290898.CrossRefPubMedPubMedCentralGoogle Scholar
  69. 69.
    Uva L, Miguel D, Pinheiro C, Freitas JP, Gomes MM, Filipe P. Cutaneous manifestations of systemic lupus erythematosus. Autoimmune Dis. 2012;2012:834291.PubMedPubMedCentralGoogle Scholar
  70. 70.
    Tebbe B, Orfanos CE. Epidemiology and socioeconomic impact of skin disease in lupus erythematosus. Lupus. 1997;6(2):96–104.PubMedGoogle Scholar
  71. 71.
    Ghosh A. Cutaneous manifestations of systemic lupus erythematosus. Indian J Rheumatol. 2007;2(4):156–64.Google Scholar
  72. 72.
    Louis PJ, Fernandes R. Review of systemic lupus erythematosus. Oral Surg Oral Med Oral Pathol. 2001;91:512–6.Google Scholar
  73. 73.
    Menta M, Nico S, Apparecida M, Vilela C, Ararigoia E, Rivitti SV. Oral lesions in lupus erythematosus: correlation with cutaneous lesions. Eur J Dermatol. 2008;18(4):376–81.Google Scholar
  74. 74.
    Lourenco SV, De Carvalho FR, Boggio P, Sotto M, Vilela MA, Rivitti E, et al. Lupus erythematosus: clinical and histopathological study of oral manifestations and immunohistochemical profile of the inflammatory infiltrate. J Cutan Pathol. 2007;34(7):558–64.PubMedGoogle Scholar
  75. 75.
    Lopez-Labady J, Villarroel-Dorrego M, Gonzalez N, Perez R, Mata de Henning M. Oral manifestations of systemic and cutaneous lupus erythematosus in a Venezuelan population. J Oral Pathol Med. 2007;36(9):524–7.PubMedGoogle Scholar
  76. 76.
    Cappelli S, Bellando Randone S, Martinović D, Tamas MM, Pasalić K, Allanore Y, et al. To be or not to be, ten years after: evidence for mixed connective tissue disease as a distinct entity. Semin Arthritis Rheum. 2012;41(4):589–98.PubMedGoogle Scholar
  77. 77.
    Mosca M, Tani C, Neri C, Baldini C, Bombardieri S. Undifferentiated connective tissue diseases (UCTD). Autoimmun Rev. 2006;6(1):1.PubMedGoogle Scholar
  78. 78.
    Schur PH, Wallace DJ. Diagnosis and differential diagnosis of systemic lupus erythematosus in adults. In: UpToDate; 2014. Retrieved 12 Sept 2014 from http://www.uptodate.com/contents/diagnosis-and-differential-diagnosis-of-systemic-lupus-erythematosus-in-adults?source=search_result&search=Differential+diagnosis+of+systemic+lupus+erythematosus&selectedTitle=1%7E150.
  79. 79.
    Brown RS, Flaitz CM, Hays GL, Trejo PM. The diagnosis and treatment of discoid lupus erythematosus with oral manifestations only: a case report. Compendium. 1994;15(6):724–32.PubMedGoogle Scholar
  80. 80.
    Boehm IB, Boehm GA, Bauer R. Management of cutaneous lupus erythematosus with low dose methotrexate: indication for modulation of inflammatory mechanisms. Rheumatol Int. 1998;18(2):59–62.PubMedGoogle Scholar
  81. 81.
    Ferri C, Valentini G, Cozzi F, Sebastiani M, Michelassi C, La Montagna G, et al. Systemic sclerosis: demographic, clinical, and serologic features and survival in 1,012 Italian patients. Medicine. 2002;81(2):139–53.PubMedGoogle Scholar
  82. 82.
    Chung L, Lin J, Furst DE, Fiorentino D. Systemic and localized scleroderma. Clin Dermatol. 2006;24(5):374–92.PubMedGoogle Scholar
  83. 83.
    Denton CP. Systemic sclerosis. Medicine. 2002:36–40.Google Scholar
  84. 84.
    Cleveland DB, Rinaggio J. Oral and maxillofacial manifestations of systemic and generalized disease. Endod Top. 2003;4:69–90.Google Scholar
  85. 85.
    Black CM. The aetiopathogenesis of systemic sclerosis: thick skin – thin hypotheses. In: The Parkes Weber Lecture 1994. J R Coll Phys Edinb. 1995;29(2):119.Google Scholar
  86. 86.
    Reveille JD, Solomon DH. Evidence-based guidelines for the use of immunologic tests: anticentromere, Scl-70, and nucleolar antibodies. American College of Rheumatology Ad Hoc Committee of Immunologic Testing Guidelines. Arthritis Rheumatol. 2003;49(3):399.Google Scholar
  87. 87.
    Tan FK, Arnett FC, Antohi S, Saito S, Mirarchi A, Spiera H, et al. Autoantibodies to the extracellular matrix microfibrillar protein, fibrillin-1, in patients with scleroderma and other connective tissue diseases. J Immunol. 1999;163(2):1066.PubMedGoogle Scholar
  88. 88.
    Bunn CC, Black CM. Systemic sclerosis: an autoantibody mosaic. Clin Exp Immunol. 1999;117(2):207.PubMedPubMedCentralGoogle Scholar
  89. 89.
    Islam NM, Bhattacharyya I, Cohen DM. Common oral manifestations of systemic disease. Otolaryngol Clin N Am. 2011;44:161–82.Google Scholar
  90. 90.
    Chan LS, Ahmed AR, Anhalt GJ, Bernauer W, Cooper KD, Elder MJ, et al. The first international consensus on mucous membrane pemphigoid. Arch Dermatol. 2002;138:370–9.PubMedGoogle Scholar
  91. 91.
    Rout PG, Hamburger J, Potts AJ. Orofacial radiological manifestations of systemic sclerosis. Dentomaxillofac Radiol. 1996;25:193–6.PubMedGoogle Scholar
  92. 92.
    Varga J. Diagnosis and differential diagnosis of systemic sclerosis (scleroderma) in adults. In: UpToDate; 2014. Retrieved 09 Oct 2014 from http://www.uptodate.com/contents/diagnosis-and-differential-diagnosis-of-systemic-sclerosis-scleroderma-inadults?source=search_result&search=systemic+sclerosis&selectedTitle=4%7E150.
  93. 93.
    Minkin W, Rabhan N. Mixed connective tissue disease. Arch Dermatol. 1976;112:1535–8.PubMedGoogle Scholar
  94. 94.
    Bennett RM. Anti-U1 RNP antibodies in mixed connective tissue disease. In: UpToDate; 2014. Retrieved 12 Oct 2014 from http://www.uptodate.com/contents/anti-u1-rnp-antibodies-in-mixed-connective-tissue-disease?source=search_result&search=mixed+connective+tissue+disease&selectedTitle=4%7E73.
  95. 95.
    Sharp GC. Diagnostic criteria for classification of MCTD. In: Kasukawa R, Sharp GC, editors. Mixed connective tissue. Diseases and anti-nuclear antibodies. Amsterdam: Elsevier; 1987. p. 23–32.Google Scholar
  96. 96.
    Greidinger EL, Hoffman RW. Autoantibodies in the pathogenesis of mixed connective tissue disease. Rheum Dis Clin N Am. 2005;31:437–50.Google Scholar
  97. 97.
    Datta SK, Zhang L, Xu L. T-helper cell intrinsic defect in lupus that break peripheral tolerance to nuclear autoantigens. J Mol Med. 2005;83:267–78.PubMedGoogle Scholar
  98. 98.
    Hoffman RW. T cells in the pathogenesis of systemic lupus erythematosus. Clin Immunol. 2004;113:4–13.PubMedGoogle Scholar
  99. 99.
    Bennett RM, O’Connell DJ. The arthritis of mixed connective tissue disease. Ann Rheum Dis. 1987;37(5):397–403.Google Scholar
  100. 100.
    Pope JE. Other manifestations of mixed connective tissue disease. Rheum Dis Clin N Am. 2005;31(3):519.Google Scholar
  101. 101.
    Hassoun PM. Pulmonary arterial hypertension complicating connective tissue diseases. Sem Respir Crit Care Med. 2009;30(4):429.Google Scholar
  102. 102.
    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.PubMedGoogle Scholar
  103. 103.
    Ramos-Niembro F, Alarcon-Segovia D, Hernandez-Ortiz J. Articular manifestations of mixed connective tissue disease. Arthritis Rheum. 1979;22:43–51.PubMedGoogle Scholar
  104. 104.
    Mimura Y, Ihn H, Jinnin M, Asano Y, Yamane K, Tamaki K. Rheumatoid factor isotypes in mixed connective tissue disease. Clin Rheumatol. 2006;25(4):572.PubMedGoogle Scholar
  105. 105.
    Sullivan WD, Hurst DJ, Harmon CE, Esther JH, Agia GA, Maltby JD, et al. A prospective evaluation emphasizing pulmonary involvement in patients with mixed connective tissue disease. Medicine. 1984;63(2):92.PubMedGoogle Scholar
  106. 106.
    Alpert MA, Goldberg SH, Singsen BH, Durham JB, Sharp GC, Ahmad M, et al. Cardiovascular manifestations of mixed connective tissue disease in adults. Circulation. 1983;68:1182–93.PubMedGoogle Scholar
  107. 107.
    Ungprasert P, Wannarong T, Panichsillapakit T, Cheungpasitporn W, Thongprayoon C, Ahmed S, et al. Cardiac involvement in mixed connective tissue disease: a systematic review. Intern J Cardiol. 2014;171(3):326–30.. Epub 2013 Dec 29Google Scholar
  108. 108.
    Marshall JB, Kretschmar JM, Gerhardt DC, Winship DH, Winn D, Treadwell EL, et al. Gastrointestinal manifestations of mixed connective tissue disease. Gastroenterology. 1990;98:1232–8.PubMedGoogle Scholar
  109. 109.
    Kitridou RC, Akmal M, Turkel SB, Ehresmann GR, Quismorio FP Jr, Massry SG. Renal involvement in mixed connective tissue disease: a longitudinal clinicopathologic study. Semin Arthritis Rheum. 1986;16:135–45.PubMedGoogle Scholar
  110. 110.
    Bennett RM, Spargo BH. Immune complex nephropathy in mixed connective tissue disease. Am J Med. 1977;63:534–41.PubMedGoogle Scholar
  111. 111.
    Klasser GD, Balasubramaniam R, Epstein J. Topical review-connective tissue diseases: orofacial manifestations including pain. J Orofac Pain. 2007;21:171–84.PubMedGoogle Scholar
  112. 112.
    Fujita Y, Fujii T, Nakashima R, Tanaka M, Mimori T. Aseptic meningitis in mixed connective tissue disease: cytokine and anti-U1RNP antibodies in cerebrospinal fluids from two different cases. Mod Rheumatol. 2008;18:184–8.PubMedGoogle Scholar
  113. 113.
    Gonzales TS, Coleman GC. Periodontal manifestations of collagen vascular disorders. Periodontology. 2000;1999(21):94–105.Google Scholar
  114. 114.
    Prystowsky SD. Mixed connective tissue disease. West J Med. 1980;132:288–93.PubMedPubMedCentralGoogle Scholar
  115. 115.
    Hoffman RW, Sharp GC, Deutscher SL. Analysis of anti-U1 RNA antibodies in patients with connective tissue disease. Association with HLA and clinical manifestations of disease. Arthritis Rheum. 1995;38:1837–44.PubMedGoogle Scholar
  116. 116.
    Ha Hassan AB, Nikitina-Zake L, Padyukov L, Karlsson G, Gupta M, Lundberg IE, et al. MICA4/HLA-DRB1*04/TNF1 haplotype is associated with mixed connective tissue disease in Swedish patients. Hum Immunol. 2003;64:290–6.Google Scholar
  117. 117.
    Kim P, Grossman JM. Treatment of mixed connective tissue disease. Rheum Dis Clin N Am. 2005;31:549–65.Google Scholar
  118. 118.
    Lundberg I. The prognosis of mixed connective tissue disease. Rheum Dis Clin N Am. 2005;31:535–47.Google Scholar
  119. 119.
    Bohan A, Peter JB. Polymyositis and dermatomyositis (first of two parts). N Engl J Med. 1975;292:344–7.PubMedGoogle Scholar
  120. 120.
    Na SJ, Kim SM, Sunwoo IN, Choi YC. Clinical characteristics and outcomes of juvenile and adult dermatomyositis. J Kor Med Sci. 2009;24:715–21.Google Scholar
  121. 121.
    Medsger TA, Dawson WN, Masi AT. The epidemiology of polymyositis. Am J Med. 1970;48:715–23.PubMedGoogle Scholar
  122. 122.
    Kovacs SO, Kovacs SC. Dermatomyositis. J Am Acad Dermatol. 1998;39(6):899–920.PubMedGoogle Scholar
  123. 123.
    Callen JP, Wortmann RL. Dermatomyositis. Clin Dermatol. 2006;24:363–73.PubMedGoogle Scholar
  124. 124.
    Appleyard ST, Dunn MJ, Dubowitz V, et al. Increased expression of HLA ABC class I antigens by muscle fibers in Duchenne muscular dystrophy, inflammatory myopathy, and other neuromuscular disorders. Lancet. 1985;1:361–3.PubMedGoogle Scholar
  125. 125.
    Tanaka TI, Geist SM. Dermatomyositis: a contemporary review for oral health care providers. Med Manag Pharmacol Updat. 2012;114(5):e1–8.Google Scholar
  126. 126.
    Callen JP. Dermatomyositis. Lancet. 2000;355:53–7.PubMedGoogle Scholar
  127. 127.
    Dalakas MC. Polymyositis, dermatomyositis, and inclusion-body myositis. N Engl J Med. 1991;325:1487–98.PubMedGoogle Scholar
  128. 128.
    Marie I, Hatron P-Y, Hachulla E, Wallaert B, Michon-Pasturel U, Devulder B. Pulmonary involvement in polymyositis and in dermatomyositis. J Rheumatol. 1998;25:1336–43.PubMedGoogle Scholar
  129. 129.
    Gonzalez-Lopez L, Gamez-Nava JI, Sanchez L, Rosas S, Suarez-Almazor M, Cardona-Munoz C, et al. Cardiac manifestations in dermato-polymyositis. Clin Exp Rheumatol. 1996;14:373–9.PubMedGoogle Scholar
  130. 130.
    Hill CL, Zhang Y, Sigurgeirsson B, Pukkala E, Mellemkajaer L, Airio A, et al. Frequency of specific cancer types in dermatomyositis and polymyositis: a population-based study. Lancet. 2001;357:96–100.PubMedGoogle Scholar
  131. 131.
    Keil H. The manifestations in the skin and mucous membranes in dermatomyositis, with special reference to the differential diagnosis from systemic lupus erythematosus. Ann Intern Med. 1942;16:828–31.Google Scholar
  132. 132.
    Sanger RG, Kirby JW. The oral and facial manifestations of dermatomyositis with calcinosis. Oral Surg Oral Med Oral Pathol Oral Radiol. 1973;35:476–88.Google Scholar
  133. 133.
    Mammen AL. Dermatomyositis and polymyositis: clinical presentation, autoantibodies, and pathogenesis. Ann N Y Acad Sci. 2010;1184:134–53.PubMedGoogle Scholar
  134. 134.
    Curiel RV, Jones R, Brindle K. Magnetic resonance imaging of the idiopathic inflammatory myopathies: structural and clinical aspects. Ann N Y Acad Sci. 2009;1154:101–14.PubMedGoogle Scholar
  135. 135.
    Woo TY, Callen JP, Voorhees JJ, Bickers DR, Hanno R, Hawkins C. Cutaneous lesions of dermatomyositis are improved by hydroxychloroquine. J Am Acad Dermatol. 1984;10:590–600.Google Scholar
  136. 136.
    Zieglschmid-Adams ME, Pandya AG, Cohen SB, Sontheimer RD. Treatment of dermatomyositis with methotrexate. J Am Acad Dermatol. 1995;32:754–7.PubMedGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Air Force Institute of TechnologyWright-Patterson AFBUSA
  2. 2.Department of DermatologyUniversity of California, DavisSacramentoUSA

Personalised recommendations