Undifferentiated connective tissue syndromes

LeRoy, E. Carwile; Maricq, Hildegard R.; Kahaleh, M. Bashar

doi:10.1002/art.1780230312

Cited by 186 publications

(78 citation statements)

References 9 publications

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“…Historically, many such patients were described as having Bcollagen^or Bconnective tissue^diseases since they shared similar clinical and pathologic features of widespread inflammation [6][7][8][9]. Now, these patients are generally considered to have Bdiffuseô r Bundifferentiated^(or sometimes Bearly undifferentiated^) systemic rheumatic diseases (a term we prefer to Bconnective tissue diseases^or Bcollagen vascular diseases^, as these are not really disorders of connective tissue or collagen as originally conceived) [6,[10][11][12][13][14][15]. Other patients with rheumatic diseases have systemic features that overlap two or more specific recognized entities and also cannot be diagnosed definitively [5,6,[16][17][18].…”

mentioning

confidence: 99%

Diagnostic uncertainty and epistemologic humility

Kelly

Panush

2017

Clin Rheumatol

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mentioning

confidence: 99%

Diagnostic uncertainty and epistemologic humility

Kelly

Panush

2017

Clin Rheumatol

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“…The serological abnormalities can include the presence of the following autoantibodies: anti-nuclear (ANA), anti-dsDNA, -Sm, -RNP, -SSA, -SSB, -Scl-70, -centromere, -Jo1, or -PM-Scl, amongst others [1]. However, this clinical-serologic constellation does not fulfill the diagnostic criteria of any well-defined rheumatic, systemic autoimmune diseases, amongst others rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), Sjögren's syndrome, idiopathic inflammatory myopathies (IIMs), mixed connective tissue disease (MCTD), or systemic sclerosis (SSc) [2][3][4][5][6]. The most important clinical feature of UCTD is that over one-third of these patients consequently transform into a full-blown systemic autoimmune/rheumatic disease [1].…”

Section: Undifferentiated Connective Tissue Disease (Uctd)mentioning

confidence: 99%

Cytokine Milieu in Undifferentiated Connective Tissue Disease: a Comprehensive Review

Nakken

Bodolay

Szodoray

2014

Clinic Rev Allerg Immunol

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Undifferentiated connective tissue disease (UCTD) is a unique clinical entity, a potential forerunner of well-established systemic autoimmune/rheumatic diseases. UCTD is characterized by the presence of various clinical symptoms, as well as a diverse repertoire of autoantibodies, resembling systemic autoimmune diseases. Since approximately one-third of these patients consequently transform into a full-blown systemic autoimmune/rheumatic disease, it is of major importance to assess pathogenic factors leading to this progression. In view of the fact that the serological and clinical picture of UCTD and systemic autoimmune diseases are very similar, it is assumed that analogous pathogenic factors perpetuate both disease entities. In systemic autoimmune conditions a quantitative and qualitative impairment of regulatory T cells have been shown previously and in parallel a relative dominance of proinflammatory Th17 cells has been introduced. Moreover the imbalance between regulatory and Th17 cells plays a pivotal role in the initiation and propagation of UCTD. Additionally, we depict a cytokine imbalance, which give raise to a biased T-cell homeostasis from the UCTD phase throughout the fully developed systemic autoimmune disease stage. The levels of IL-6, IL-12, IL-17, IL-23 and IFN- were pathologically increased with a parallel reduction of IL-10. We believe that the assessment of Th17/Treg cell ratio, as well as the simultaneous quantitation of cytokines may give a useful diagnostic tool at the early UCTD stage to identify patients with a higher chance of consecutive disease progression towards serious systemic autoimmune diseases. Moreover, the early-targeted immunomodulating therapy in these patients may decelerate, or even stop this progression, before the development of serious autoimmune conditions with organ damage.

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“…In 1972, Sharp et al [1] described mixed connective tissue disease (MCTD) as a distinct overlap syndrome which was characterized by a benign course, and an excellent responsiveness to cortieosteroids, and which was defined by the presence of high antibody titres against the nuclear ribonucleoprotein (RNP), Since then the concept of MCTD has required considerable modification [2], With follow-up studies, many patients with MCTD subsequently developed a classical rheumatic disease [3], most commonly progressive systemic sclerosis (PSS), Since in those patients MCTD represented an early, transient phase of a better defined disease LeRoy, Maricq & Kahaleh [4] proposed to designate this condition as 'undifferentiated'.…”

Section: Introductionmentioning

confidence: 99%

Imbalance of CD4+ lymphocyte subsets in patients with mixed connective tissue disease

Becker¹,

Langrock²,

Federlin³

1992

Clinical and Experimental Immunology

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SUMMARY CD4+ (helper/inducer) T lymphocyte subsets were studied in the peripheral blood from patients with mixed connective tissue disease (MCTD) by double‐labelling immunofluorescence. The proportion of CD4+CD45RA+ cells was higher (P < 0·01) when compared with controls, whereas CD4+CD29+ cells were markedly diminished (P < 0·001). CD4+CD29+ cells were lower than in patients with progressive systemic sclerosis who were studied in parallel. Upon stimulation with phytohaemagglutinin, CD4+ cells from MCTD patients showed a strong reactivity to acquire the CD29+ phenotype. Expression of high levels of CD29 and other adhesion molecules might lead to facilitated localization of CD4+ cells to inflamed tissue. It is suggested that an increased responsiveness of CD4+ cells to activation signals in vivo and accumulation of CD4+CD29+ cells at tissue sites could result in depletion of this cell subset in the peripheral blood of patients with MCTD.

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Undifferentiated connective tissue syndromes

Cited by 186 publications

References 9 publications

Diagnostic uncertainty and epistemologic humility

Diagnostic uncertainty and epistemologic humility

Cytokine Milieu in Undifferentiated Connective Tissue Disease: a Comprehensive Review

Imbalance of CD4+ lymphocyte subsets in patients with mixed connective tissue disease

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