DOI: http://dx.doi.org/10.18203/2320-6012.ijrms20162967

Mixed connective tissue disease presenting with isolated pulmonary hypertension, and limited cutaneous sclerosis

Vaishal Kamlesh Shah, Dhaval Dave, Rushabh Parikh, Divya Lala, S.T. Nabar, Archana Bhate

Abstract


Mixed connective tissue disease (MCTD) was first recognized by Sharp and Colleagues in 1972 among a group of patients with overlapping clinical features of systemic lupus erythematosis (SLE), scleroderma and myositis, with the presence of distinctive antibodies against, what now is known to be U1ribonucleoprotein (RNP). We report an unusual case of a 29-year old female with MCTD characterized by the coexistence of signs, symptoms and immunological features of 3 defined autoimmune diseases isolated PHT, cutaneous sclerosis (SSc), hypothyroidism.


Keywords


Mixed connective tissue disease, Hypothyroidism, Pulmonary hypertension, Limited cutaneous sclerosis

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References


Bennett RM, O'Connell DJ. Mixed connective tissuedisease: a clinicopathologic study of 20 cases. Semin Arthritis Rheum. 1980;10:25.

Dubowitz V. Inflammatory myopathies. In: Muscledisorders in childhood. In the series of Major

problems in clinical practice. Editors: A. J. Schaeffer, M. Markowitz, W. B. Saunders Co. Ltd.,Philadelphia, London. 1978;16:202.

Lorber M, Gershwin ME, Shoenfeld Y. The coexistence of systemic lupus erythematosus with other autoimmune diseases: the kaleidoscope of autoimmunity. Semin Arthritis Rheum. 1994;24:105-13.

Jury EC, D'Cruz D, Morrow WJ. Autoantibodies and overlap syndromes in autoimmune rheumatic disease. J Clin Pathol. 2001;54:340-7.

Sharp GC, Irvin WS, Tan EM, Gould RG, Holman HR. 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.

Mercado U. Mixed connective tissue disease, or should it be Sharp's syndrome? Comment on the article by Farhey and Hess. Arthritis Care Res. 1999;12:227.

Venables PJ. Mixed connective tissue disease. Lupus. 2006;15:132-7.

Maddison PJ. Mixed connective tissue disease: overlap syndromes. Baillieres Best Pract Res ClinRheu- matol. 2000;14:111-24.