Summary
Eighteen patients diagnosed as suffering from MCTD were reexamined during follow-up (mean duration 4.6 years). The clinical features of these patients were compared with those of 19 patients with systemic lupus erythematosus (SLE), 11 with progressive systemic sclerosis (PSS) and 22 with rheumatoid arthritis (RA). Considerable overlapping of abnormal features was found between MCTD and the other syndromes. At the end of the follow- up period, 70 per cent of the cases initially diagnosed as MCTD evolved to a more classical connective tissue disease, i.e., either PSS or SLE. Generally, however, the clinical evolution of the individual MCTD patient was not predictable. Abnormal aortic valve calcifications were found in the MCTD group. Four of the 18 MCTD patients were anti-RNP negative at reexamination. There was a tendency for HLA antigens B7 and B8 to be increased in the MCTD group, but this difference was not statistically significant. Three MCTD patients died before they could be reexamined (two of them from pulmonary hypertension with proliferative endarteritis of the lung vessels and one from septicaemia and multiple cerebral infarctions).
Article PDF
Similar content being viewed by others
Avoid common mistakes on your manuscript.
References
Sharp, G.C., Irvin, W.S., Tan, E.M., Gould, R.G., Holman, H.R. Mixed connective tissue disease: an apparently distinct rheumatic disease syndrome, associated with a specific antibody to extractable nuclear antigen (ENA). Am J Med 1972, 52, 148–159.
Farber, S.J., Bole, G.G. Antibodies to components of extractable nuclear antigen. Clinical characteristics of patients. Arch Intern Med 1976, 136, 425–431.
Bennett, R.M., O'Connel, D.J. Mixed connective tissue disease: a clinicopathologic study of 20 cases. Semin Arthritis Rheum 1980, 10, 25–51.
Grant, K.D., Adams, L.E., Hess, E.V. MCTD — A subset with sequential clinical and laboratory features. J Rheumatol 1981, 8, 587–598.
Matter, L., Schapper, K., Wilhelm, J.A., Nyffenegger, T., Parisot, R.F., DeRoberts, E.M. Molecular characterisation of ribonucleoprotein antigens bound by antinuclear antibodies: a diagnostic evaluation. Arthritis Rheum 1982, 25, 1297–1304.
Nimelstein, S.H., Brody, S., McShane, D., Holman, H.R. Mixed connective tissue disease: a subsequent evaluation of the original 25 patients, Medicine 1980, 59, 239–248.
Eulderinck, F., Cats, A. Fatal primary pulmonary hypertension in mixed connective tissue disease. Z Rheumatol 1981, 40, 25–29.
Esther, J.H., Sharp, G.C., Agia, G., Hurst, D.J. Pulmonary hypertension in patients with mixed connective tissue disease and antibody to nuclear ribonucleoprotein. Arthritis Rheum 1981, 24S, 105.
Piirainen, H., Gripenberg, M., Laitinen, O. Antiribonucleoprotein antibodies in inflammatory rheumatic diseases. Scand J Rheumatol 1984, 13, 177–180.
Gaudreau, A., Amar, B., Kahn, M. Clinical significance of antibodies to soluble extractable nuclear antigens (anti-ENA). Ann Rheum Dis 1978, 37, 321–327.
Maddison, P.J., Mogovero, H., Reichlin, M. Patterns of clinical disease associated with antibodies to nuclear ribonucleoprotein. J Rheumatol 1978, 5, 407–411.
LeRoy, E.C., Maricq, H.R., Kahaleh, M.B. Undifferentiated connective tissue syndromes. Arthritis Rheum 1980, 23, 341–343.
Hochberg, M.C., Dorsch, CA., Feinglass, E.J., Stevens, M.B. Survivorship in SLE: effect of antibody to extractable nuclear antigen. Arthritis Rheum 1981, 24, 54–59.
Ginsburg, W.W., Conn, D.G., Bunch, T.W., McDuffie, F.C. Comparison of clinical and serologic markers in systemic lupus erythematosus and overlap syndrome: a review of 247 patients. J Rheumatol 1983, 10, 235–241.
Lemmer, J.P., Curry, N.H., Mallory, J.H., Waller, M.V. Clinical characteristics and course in patients with high titre anti-RNP antibodies. J Rheumatol 1982, 9, 536–542.
Bennet, R.M., O'Connel, D.J. The arthritis of mixed connective tissue disease. Ann Rheum Dis 1978, 37, 397–403.
Hijmans, W., Schuit, H.R.E., de Leeuw, B., Möller, H.F. De diagnostische betekenis van anticuleaire serumfactoren, aangetoond met behulp van fluorescerende antistoffen. Ned Tijdschr Geneesk 1962, 106, 2097–2101.
Valkenburg, H.A. Human erythrocyte agglutination test (HEAT). In: The epidemiology of chronic rheumatism, Editors: Kellgren, J.H., Jeffrey, M.R., Bal, J., Oxford, Blackwell Science Publishers, 1963, 1, 330–333.
Aarden, L.A., de Groot, E.R., Feltkamp, T.E.W. Immunology of DNA III. Crithdia luciliae, a simple substrate for the determination of anti-ds DNA with the immunofluorescence technique. Ann NY Acad Sci 1975, 254, 505–515.
Kievits, J.H., Goslings, J., Schuit, H.R.E., Hijmans, W. Rheumatoid arthritis and the positive L.E.-cell phenomenon. Ann Rheum Dis 1956, 15, 211–216.
Kurata, N., Tan, E.M. Identification of antibodies to nuclear acidic antigens by counterimmunoelectrophoresis. Arthritis Rheum 1976, 19, 574–580.
van Rood, J.J. Microlymphocytotoxicity Method. In: NIAID Manual of Tissue Typing Techniques, Editor: John, G., Ray, Maryland, NIH 80-545, 1979, 104–105.
van Rood, J.J., van Leeuwen, A., Ploem, J.S. Simultaneous detection of two cell populations by two-colour fluorescence and application to the recognition of B-cell determinants. Nature, 1976, 262, 795–797.
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. 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum, 1982, 25, 1271–1277.
Bresnihan, B., Bunn, C., Snaith, M.L., Hughes, G.R.V. Antiribonucleoprotein antibodies in connective tissue diseases: estimation by counterimmunoelectrophoresis. Br Med J 1977, 1, 610–611.
Habets, W.J., De Rooy, D.J., Hoet, M.H., Van De Putte, L.B., Van Venrooy, W.J. Quantitation of anti-RNP and anti-Sm antibodies in MCTD and SLE patients by immunoblotting. Clin Exp Immunol 1985, 59, 457–466.
Becker, A.E., Anderson, R.H. The aging heart. In: Cardiac pathology, New York, Raven press, 1982, 7, 3.
Oetgen, W.J., Mutter, M.L., Lawless, O.J., Davia, J.E. Cardiac abnormalities in mixed connective tissue disease. Chest 1983, 83, 185–188.
Lash, A.D., Wittman, A.L., Quismorio, F.P. Myocarditis in mixed connective tissue disease: clinical and pathologic study of three cases and review of the literature. Semin Arthritis Rheum 1986, 15, 288–296.
Horn, J.R., Kaper, J.J., Walker, S.E. Mixed connective tissue disease in siblings. Arthritis Rheum 1978, 21, 709–714.
Ramos-Niembro, F., Alarcon-Segovia, D. Familial aspects of MCTD: 1. Occurrence of SLE in another member of two families and aggregation of MCTD in another family. J Rheumatol 1978, 5, 433–438.
Stahl, N.I., Johnson, A.H., Decker, J.L., Sharp, G.C., Mann, D.L. B lymphocyte antigens in mixed connective tissue disease. Arthritis Rheum 1980, 23S, 751.
Wiener-Kronish, J.P., Solinger, A.M., Warnoch, M.L., Churg, A., Ordonez, N., Golden, J.A. Severe pulmonary involvement in mixed connective tissue disease. Am Rev Resp Dis, 1981, 124, 499–503.
Jones, M.B., Osterholm, R.K., Wilson, R.B., Martin, F.H., Commers, J.R., Bachmayer, J.D. Fatal pulmonary hypertension and resolving immune complex glomerulonephritis in mixed connective tissue disease. Am J Med 1978, 65, 855–863.
Manthorpe, R., Elling, H., Van Der Meulen, J.T., Freiesleben Sorensen, S. Two fatal cases of mixed connective tissue disease. Scand J Rheumatol 1980, 9, 7–10.
Graziano, F.M., Friedman, L.C., Grossman, J. Pulmonary hypertension in a patient with MCTD: Clinical and pathology findings, and review of the literature. Clin Exp Rheum 1983, 1, 251–255.
Kitridou, R.C., Akmal, M., Turkel, S.B., Ehresmann, G.R., Quismorio, F.P., Massry, S.G. Renal involvement in mixed connective tissue disease: a longitudinal clinicopathologic study. Semin Arthritis Rheum 1986, 16, 135–145.
Author information
Authors and Affiliations
Additional information
Presently Department of Immunology-Rheumatology, University Hospital, Antwerp, Belgium.
Rights and permissions
About this article
Cite this article
De Clerck, L.S., Meijers, K.A.E. & Cats, A. Is MCTD a distinct entity ? Comparison of clinical and laboratory findings in MCTD, SLE, PSS, and RA patients. Clin Rheumatol 8, 29–36 (1989). https://doi.org/10.1007/BF02031065
Received:
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF02031065