Title: A case of Mixed Connective Tissue Disease with Right Heart Failure

Authors: Dr Venkatesh R, Dr Nagarajan K, Dr Anand P

 DOI: https://dx.doi.org/10.18535/jmscr/v7i7.09

Abstract

Background: Sharp and colleagues in 1972 was first described the Mixed connectivetissue disease (MCTD).They described MCTD in a group of people havingwith overlapping clinical features of scleroderma, myositis and systemic lupus erythematosus (SLE) with the presence of a specific antibody against U1-ribonucleoprotein (RNP).1 Sharp's syndrome is the other name for MCTD.2 It is more common in females than males. Estimations of the female to male ratio varies from approximately 3:1 to 16:1.3,4It is typically occuring between 15-25 years oldbut it can occur at any age but in our country has rarely been reported but here by reported rare presentation of MCTD occurs in 65 years old female.

Keywords: Mixed connective tissue disease; Pulmonary hypertension; Extractable nuclear antigen.

Background: Sharp and colleagues in 1972 was first described the Mixed connectivetissue disease (MCTD).They described MCTD in a group of people havingwith overlapping clinical features of scleroderma, myositis and systemic lupus erythematosus (SLE) with the presence of a specific antibody against U1-ribonucleoprotein (RNP).1 Sharp's syndrome is the other name for MCTD.2 It is more common in females than males. Estimations of the female to male ratio varies from approximately 3:1 to 16:1.3,4It is typically occuring between 15-25 years oldbut it can occur at any age but in our country has rarely been reported but here by reported rare presentation of MCTD occurs in 65 years old female.

Keywords: Mixed connective tissue disease; Pulmonary hypertension; Extractable nuclear antigen.

References

  1. Sharp GC, Irvin WS, Tan EM, Gould RG, Holman HR (February 1972). "Mixed connective tissue disease--an apparently distinct rheumatic disease syndrome associated with a specific antibody to an extractable nuclear antigen (ENA)". The American Journal of Medicine. 52(2):148–59. doi:1016/0002-9343(72)90064-2. PMID 4621694.
  2. Rapini RP, Bolognia JL, Jorizzo JL (2007). Dermatology: 2-Volume Set. St. Louis: Mosby. ISBN1-4160-2999-0.
  3. Nakae K, Furusawa F, Kasukawa R, et al.A nationwide epidemiological survey on diffuse collagen diseases: Estimation of prevalence rate in Japan. Kasukawa R, Sharp G, eds. Mixed Connective Tissue Disease and Anti-nuclear Antibodies. Amsterdam: Excerpta Medica; 1987. 9.
  4. Gunnarsson R, Molberg O, Gilboe IM, Gran JT, PAHNOR1 Study Group. The prevalence and incidence of mixed connective tissue disease: a national multicentre survey of Norwegian patients. Ann Rheum Dis. 2011 Jun. 70 (6):1047-51.
  5. Martinez-Barrio J, Valor L, Lopez-Longo FJ. Facts and controversies in mixed connective tissue disease. Med Clin (Barc). 2017 Aug 29.
  6. Hoffman RW (1 June 2009). "Mixed Connective Disease". In Stone J. Pearls & Myths in Rheumatology. Springer. pp. 169–172. ISBN978-1-84800-933-2. Retrieved 26 June 2010.
  7. Eric L Greidinger, MD Associate Professor, Department of Medicine, Division of Rheumatology and Immunology, University of Miami Miller School of Medicine, Miami Veterans Affairs Medical Center.

Corresponding Author

Dr Venkatesh R

Final year post graduate, Department of General medicine

Room no 12, SMVMCH medical boys PG hostel, Sri Manakula Vinayagar Medical College and Hospital (SMVMCH), Kalitheerthalkuppam, Puducherry 605107, India