Mixed Connective Tissue Disease, a Roundabout to Rheumatic Diseases?

Bellando-Randone, Silvia; Cutolo, Maurizio; Czirják, László; Matucci‐Cerinic, Marco

doi:10.2174/157339709788298437

Cited by 8 publications

(5 citation statements)

References 39 publications

(60 reference statements)

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“…The overlap of SSc, SLE and PM symptoms in mixed connective tissue disease seems to be ‘sequential’ rather than ‘concurrent’. 2,3 The prevalence of MCTD is unknown, but it is presumed to be four times less frequent than SLE. 4 Four different classification criteria for MCTD have been proposed by Sharp, 5 Alarcon-Segovia and Villareal, 6 Kahn, 7 and Kasukawa et al 8 There has been continuing debate as to whether it can be considered a distinct clinical syndrome, but recently it has been concluded that there is sufficient evidence to designate MCTD as a distinct disease.…”

Section: Introductionmentioning

confidence: 99%

“…4 Four different classification criteria for MCTD have been proposed by Sharp, 5 Alarcon-Segovia and Villareal, 6 Kahn, 7 and Kasukawa et al 8 There has been continuing debate as to whether it can be considered a distinct clinical syndrome, but recently it has been concluded that there is sufficient evidence to designate MCTD as a distinct disease. 3,9 A longitudinal study by Burdt et al 10 showed that clinical symptoms of MCTD tended to develop over time. Although some of them diminished, other manifestations, such as pulmonary hypertension and central nervous system (CNS) involvement, persisted over time in spite of treatment.…”

Section: Introductionmentioning

confidence: 99%

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Neuropsychological assessment in mixed connective tissue disease: comparison with systemic lupus erythematosus

Nowicka-Sauer

Czuszyńska

Majkowicz

et al. 2012

Lupus

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Neuropsychological assessment in mixed connective tissue disease: comparison with systemic lupus erythematosus

Nowicka-Sauer

Czuszyńska

Majkowicz

et al. 2012

Lupus

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“…Furthermore, Cappelli et al showed that out of 161 patients, initially diagnosed as MCTD, after a mean follow-up of 7.9 years, 93 (57.9%) still had the diagnosis of MCTD while 17.3% were diagnosed with SSc [8]. For the abovementioned reasons, a part of patients classified as MCTD and UCTD [8,10] are already cases that may belong to the very early phase of SSc, thus having a different pattern of progression to SSc.…”

Section: Introductionmentioning

confidence: 99%

Insights into molecular and clinical characteristics of very early systemic sclerosis

Bellando-Randone

Galdo

Matucci‐Cerinic

2022

Current Opinion in Rheumatology

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Purpose of reviewThe early heterogenous presentation of systemic sclerosis (SSc), in particular without skin involvement, has been a confounding factor delaying early diagnosis. In fact, early signs of SSc as Raynaud's phenomenon and puffy fingers, are also typical of other connective tissue diseases (CTDs) such as mixed CTD and undifferentiated CTD. In the last decade, a significant effort has been dedicated in defining molecular characteristics that could be used as early SSc biomarkers. In this narrative review, we address the present situation where several clinical scenarios are in search of a correct positioning into the prescleroderma (pre-SSc) phase as well as in the very early phase of SSc. Recent findingsLiterature data showed that a part of patients classified as sine scleroderma SSc (ssSSc), mixed CTD and undifferentiated CTD may already belong to the very early phase of SSc, thus having a different pattern of progression to SSc. Recently, the very early diagnosis of systemic sclerosis (VEDOSS) criteria has been validated. Summarywhile the area of pre-SSc still remains fuzzy, the VEDOSS study has shown that a 'window of opportunity' does exist also for SSc. In the very next future, this may allow to start the treatment to prevent the disease progression to a more advanced fibrotic stage.

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“…At disease onset, patients usually manifest RP, polyarthritis and swollen hands and are often initially diagnosed as having another CTD rather than MCTD. Whatever the complexity of this clinical and immunological entity, the disease shares similarities with SSc as with other CTDs 26. The initial presence of antinuclear antibodies, thickening of fingers and higher age at onset are important predictors for a possible transition from RP to a CTD 192728.…”

mentioning

confidence: 99%

“…The question of whether MCTD is a distinct disease entity is still debated 23242526. At disease onset, patients usually manifest RP, polyarthritis and swollen hands and are often initially diagnosed as having another CTD rather than MCTD.…”

mentioning

confidence: 99%

The challenge of early systemic sclerosis for the EULAR Scleroderma Trial and Research group (EUSTAR) community. It is time to cut the Gordian knot and develop a prevention or rescue strategy

et al. 2009

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Early diagnosis of systemic sclerosis (SSc) may allow the start of treatment that could slow disease progression. For this reason early diagnosis of the disease is of pivotal importance. However, the lack of diagnostic criteria and valid predictors significantly limit patient evaluation and the use of potentially effective drugs in the earliest phase of SSc. Early SSc may be suspected on the basis of Raynaud's phenomenon, puffy fingers, autoantibodies and SSc capillaroscopic pattern. In practice, the aim is to have criteria for the diagnosis of very early SSc. The criteria that are proposed are obviously provisional and need to be validated: (a) initially through a Delphi technique; (b) thereafter perhaps using already available datasets; but (c) of critical importance, through prospective studies. Only after prospective studies can these potential criteria be considered validated. The consensus on criteria for the classification of very early SSc might be part of the evolving EULAR/ACR project of reclassification of SSc.

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Mixed Connective Tissue Disease, a Roundabout to Rheumatic Diseases?

Cited by 8 publications

References 39 publications

Neuropsychological assessment in mixed connective tissue disease: comparison with systemic lupus erythematosus

Neuropsychological assessment in mixed connective tissue disease: comparison with systemic lupus erythematosus

Insights into molecular and clinical characteristics of very early systemic sclerosis

The challenge of early systemic sclerosis for the EULAR Scleroderma Trial and Research group (EUSTAR) community. It is time to cut the Gordian knot and develop a prevention or rescue strategy

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