Literature DB >> 29507032

Following leads: connecting dysphagia to mixed connective tissue disease.

Rita de Sousa Gameiro1, Ana Isabel Alves Reis1, Ana Cristina Grilo1, Carla Noronha1.   

Abstract

Mixed connective tissue disease (MCDT) is a rare condition characterised by the presence of high titres of anti-U1 ribonucleoprotein antibodies and selected clinical features of systemic lupus erythematosus, systemic sclerosis and polymyositis/dermatomyositis. Early symptoms are non-specific, including easy fatigability, myalgia, arthralgia and Raynaud's phenomenon. Some reports emphasised the favourable outcome and excellent response to glucocorticoids, but there are contradictory studies reporting worse prognosis. Also, a subset of patients evolve into a clinical picture more consistent with a major diffuse connective tissue disease. We present the case of a 50-year-old black woman whose inaugural presentation of MCDT was oropharyngeal dysphagia, symmetrical proximal muscle weakness, tongue atrophy and skin sclerosis. High-dose corticosteroids and methotrexate were given with little improvement, maintaining disabling dysphagia leading to a percutaneous endoscopic gastrostomy tube placement. She was then started on intravenous immunoglobulin with progressive remission of symptoms. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

Entities:  

Keywords:  connective tissue disease; medical management; oesophagus

Mesh:

Substances:

Year:  2018        PMID: 29507032      PMCID: PMC5847916          DOI: 10.1136/bcr-2017-223699

Source DB:  PubMed          Journal:  BMJ Case Rep        ISSN: 1757-790X


  15 in total

Review 1.  The role of high-dose intravenous immunoglobulin in rheumatology.

Authors:  Prashantha M Vaitla; Elizabeth M McDermott
Journal:  Rheumatology (Oxford)       Date:  2010-02-23       Impact factor: 7.580

Review 2.  Treatment of mixed connective tissue disease.

Authors:  Paul Kim; Jennifer M Grossman
Journal:  Rheum Dis Clin North Am       Date:  2005-08       Impact factor: 2.670

Review 3.  Other manifestations of mixed connective tissue disease.

Authors:  Janet E Pope
Journal:  Rheum Dis Clin North Am       Date:  2005-08       Impact factor: 2.670

Review 4.  Mixed connective tissue disease: an overview of clinical manifestations, diagnosis and treatment.

Authors:  Oscar-Danilo Ortega-Hernandez; Yehuda Shoenfeld
Journal:  Best Pract Res Clin Rheumatol       Date:  2012-02       Impact factor: 4.098

5.  Clinical course, prognosis, and causes of death in mixed connective tissue disease.

Authors:  Agota Hajas; Peter Szodoray; Britt Nakken; Janos Gaal; Eva Zöld; Renata Laczik; Nora Demeter; Gabor Nagy; Zoltan Szekanecz; Margit Zeher; Gyula Szegedi; Edit Bodolay
Journal:  J Rheumatol       Date:  2013-05-01       Impact factor: 4.666

Review 6.  Does mixed connective tissue disease exist? Yes.

Authors:  Martin Aringer; Günter Steiner; Josef S Smolen
Journal:  Rheum Dis Clin North Am       Date:  2005-08       Impact factor: 2.670

Review 7.  The prognosis of mixed connective tissue disease.

Authors:  Ingrid E Lundberg
Journal:  Rheum Dis Clin North Am       Date:  2005-08       Impact factor: 2.670

Review 8.  Mixed connective tissue disease.

Authors:  Ragnar Gunnarsson; Siri Opsahl Hetlevik; Vibke Lilleby; Øyvind Molberg
Journal:  Best Pract Res Clin Rheumatol       Date:  2016-04-12       Impact factor: 4.098

9.  The prevalence and incidence of mixed connective tissue disease: a national multicentre survey of Norwegian patients.

Authors:  Ragnar Gunnarsson; Oyvind Molberg; Inge-Margrethe Gilboe; Jan Tore Gran
Journal:  Ann Rheum Dis       Date:  2011-03-11       Impact factor: 19.103

10.  Long-term outcome in juvenile-onset mixed connective tissue disease: a nationwide Norwegian study.

Authors:  Siri Opsahl Hetlevik; Berit Flatø; Marite Rygg; Ellen Berit Nordal; Cathrine Brunborg; Helene Hetland; Vibke Lilleby
Journal:  Ann Rheum Dis       Date:  2016-06-09       Impact factor: 19.103

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