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A case of undifferentiated connective tissue disease: is it a distinct clinical entity?

Nature Clinical Practice Rheumatology volume 4, pages 328–332 (2008)Cite this article

Abstract

Background In November 2001, a 24-year-old woman with thrombocytopenia and Raynaud's phenomenon presented to our clinic. Her physical examination was unremarkable except for bruising on her legs and arms.

Investigations Laboratory assays detected the presence of antinuclear and anti-ribonucleoprotein antibodies. Tests for antibodies to double-stranded DNA and for antiphospholipid (lupus anticoagulant and anticardiolipin), anticentromere, anti-Scl-70, and antiplatelet antibodies were negative, as was a Coombs test. An echocardiogram, chest X-ray, and abdominal scan showed no abnormalities. Nailfold digital capillaroscopy revealed minor capillary changes not specific for scleroderma.

Diagnosis Undifferentiated connective tissue disease.

Management The patient was successfully treated initially with high doses of corticosteroids and azathioprine, followed by rapid dose tapering. Therapy was continued for 2 years and then stopped. Over the next 4 years the patient's disease history was unremarkable, apart from mild Raynaud's phenomenon of the hands and the presence antinuclear and anti-ribonucleoprotein antibodies. The diagnosis of undifferentiated connective tissue disease was confirmed at her most-recent assessment, in October 2007.

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Acknowledgements

Désirée Lie, University of California, Irvine, CA, is the author of and is solely responsible for the content of the learning objectives, questions and answers of the Medscape-accredited continuing medical education activity associated with this article.

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Authors and Affiliations

  1. Department of Internal Medicine, M Mosca is Assistant Professor of Rheumatology, C Tani is a PhD Fellow, and S Bombardieri is Professor of Rheumatology, at the Rheumatology Unit, University of Pisa, Pisa, Italy.,

    Marta Mosca, Chiara Tani & Stefano Bombardieri

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  1. Marta Mosca
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  2. Chiara Tani
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Correspondence to Marta Mosca.

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Mosca, M., Tani, C. & Bombardieri, S. A case of undifferentiated connective tissue disease: is it a distinct clinical entity?. Nat Rev Rheumatol 4, 328–332 (2008). https://doi.org/10.1038/ncprheum0799

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