Mixed connective tissue disease, or should it be Sharp's syndrome? Comment on the article by Farhey and Hess

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

“…6 Over the past 30 years there has been a continuing debate as to whether MCTD constitutes a "distinct clinical entity" and it still remains a controversial diagnosis. 7 Most authors agree that MCTD is a distinctive entity rather than a haphazard association of clinical and serological features and that the presence of high titres of autoantibodies to U1RNP influences the expression of connective tissue disease in ways that are relevant to prognosis and treatment. 8 In present patient, the clinical examination and the data obtained by investigations did not permit us to include this case in one of the typical, well established CTDs.…”

A rare case of mixed connective tissue disease presenting with central nervous system glioma, vasculitis and polymyositis

“…5 The relevance of defining MCTD as a separate disease entity has been challenged, some authors considering it just a subset of SLE. 6 Over the past 30 years there has been a continuing debate as to whether MCTD constitutes a "distinct clinical entity" and it still remains a controversial diagnosis. 7 Most authors agree that MCTD is a distinctive entity rather than a haphazard association of clinical and serological features and that the presence of high titres of autoantibodies to U1RNP influences the expression of connective tissue disease in ways that are relevant to prognosis and treatment.…”