Title:Mixed Connective Tissue Disease (MCTD) – A Coming of Age
Volume: 8
Issue: 1
Author(s): Yolanda Farhey
Affiliation:
Keywords:
MCTD, anti-u1rnp, raynaud’s, pulmonary hypertension, interstitial lung disease
Abstract: In Mixed Connective Tissue Disease (MCTD), features of various connective tissue disorders such as systemic
lupus erythematosus (SLE), progressive systemic sclerosis (PSSc), dermatomyositis/polymyositis (DM/PM), and
occasionally Sjogren’s syndrome and rheumatoid arthritis (RA) can coexist and overlap. The picture is marked by the
presence of high titer anti-U1 ribonucleoprotein (RNP) antibodies. Over the last 30 years since first described a lot of
controversial studies have been published regarding the nature, the severity or the very existence of the condition. MCTD
is not a benign condition easily responsive to treatment or without major complication as previously believed and every
effort should be made from the start to identify the type and extent of organ involvement. Overall the mortality is not as
high as in SLE but pulmonary hypertension and its cardiac complications are the major cause of death in MCTD and the
patients should be monitored closely for its development and progression. This is a review of the clinical aspects and an
update of the management of the main morbidities of MCTD.