Mixed connective tissue disease: state of the art on clinical practice guidelines

Chaigne, Benjamin; Scirè, Carlo Alberto; Talarico, Rosaria; Amoura, Zahir; Avčin, Tadej; Beretta, Lorenzo; Doria, Andrea; Guffroy, Aurélien; Guimarães, Vera; Hachulla, É.; Krieg, Thomas; Launay, David; Lepri, Gemma; Moinzadeh, Pia; Müller‐Ladner, Ulf; Rednic, Simona; Rodrigues, Ana; Tas, Sander W.; Vollenhoven, Ronald F. van; Vieira, Ana; Bombardieri, Stefano; Fonseca, João Eurico; Galetti, Ilaria; Schneider, Matthias; Smith, Vanessa; Cutolo, Maurizio; Mosca, Marta; Fischer‐Betz, Rebecca

doi:10.1136/rmdopen-2018-000783

Cited by 44 publications

(39 citation statements)

References 15 publications

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“…This effect was the most pronounced for MCTD, both for anti-PC and anti-MDA. www.nature.com/scientificreports www.nature.com/scientificreports/ MCTD is a relatively rare autoimmune disease and it's sheer existence as a distinct disorder and diagnostic criteria are still debated 30,31 . Our findings could favour the notion that it indeed is a disease of its own.…”

Section: Discussionmentioning

confidence: 99%

IgM antibodies against malondialdehyde and phosphorylcholine in different systemic rheumatic diseases

Thiagarajan

Oparina

Lundström

et al. 2020

Sci Rep

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the pReciSeSADS clinical consortium*, Marta Alarcon-Riquelme 1,3 & Johan frostegård 1 ✉ igM antibodies against phosphorylcholine (anti-pc) and malondialdehyde (anti-MDA) may have protective properties in cardiovascular and rheumatic diseases. We here compare these antibodies in systemic rheumatic conditions and study their properties. Anti-pc and anti-MDA was measured using ELISA in patients with SLE (374), RA (354), Mixed connective tissue disease (MCTD, 77), Systemic sclerosis (SSc, 331), Sjögren's syndrome (SjS, 324), primary antiphospholipid syndrome (PAPs, 65), undifferentiated connective tissue disease (UCTD, 118) and 515 matched healthy controls (HC). Cardiovascular score (CV) was broadly defined based on clinical disease symptoms. Anti-PC and anti-MDA peptide/protein characterization were compared using a proteomics de novo sequencing approach. anti-MDA and anti-PC were extracted from total IgM. The proportion of Treg cells was determined by flow cytometry. The maximal difference between cases and controls was shown for MCTD: significantly lower IgM Anti-PC but not anti-MDA among patients (median 49.3RU/ml vs 70.4 in healthy controls, p(t-test) = 0.0037). IgM low levels were more prevalent in MCTD, SLE, SjS, SSc and UCTD. IgM anti-PC variable region profiles were different from and more homologous than anti-MDA. Anti-PC but not anti-MDA were significantly negatively correlated with CV in the whole patient group. In contrast to IgM anti-PC, anti-MDA did not promote polarization of Tregs. Taken together, Anti-pc is decreased in MctD and also in SLe, SjS and SSc but not in other studied diseases. Anti-pc may thus differentiate between these. In contrast, anti-MDA did not show these differences between diseases studied. Anti-pc level is negatively correlated with cV in the patient group cohort. in contrast to anti-PC, anti-MDA did not promote Treg polarization. These findings could have both diagnostic and therapeutic implications, one possibility being active or passive immunization with pc in some rheumatic conditions.

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Section: Discussionmentioning

confidence: 99%

IgM antibodies against malondialdehyde and phosphorylcholine in different systemic rheumatic diseases

Thiagarajan

Oparina

Lundström

et al. 2020

Sci Rep

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“…The management of our patient relied on interpolation of treatment guidelines for other connective tissue diseases. 28 Musculoskeletal manifestations were the main complaints of our patient, and she had severe erosive arthritis.…”

Section: Discussionmentioning

confidence: 71%

“…The management of our patient relied on interpolation of treatment guidelines for other connective tissue diseases 28 …”

Section: Discussionmentioning

confidence: 99%

Mixed connective tissue disease: Not always an obvious diagnosis

Rahmouni

Maatallah

Ferjani

et al. 2020

Clinical Case Reports

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“…Since ERN ReCONNET aims at improving the level of adherence to CPGs across European HCPs, by providing practical tools and training to healthcare professionals, many of the initiatives emerging from this survey are being realised under the scope of the ERN ReCONNET, following the suggestions, expectations and points of view of the member HCPs and ePAGs. The ERN ReCONNET has already reviewed the landscape of the existing CPGs in rCTDs [10][11][12][13][14][15][16][17][18][19] and the existence of partially sufficient CPGs for some rCTDs was highlighted, while demonstrating the absence of evidence-based CPGs for other rCTDs. [10][11][12][13][14][15][16][17][18][19] Among the different unmet needs on CPGs, the results raised the issue that more attention should be devoted to the assessment of CPGs adherence in routine clinical practice.…”

Section: Discussionmentioning

confidence: 99%

Clinical practice guidelines adherence, knowledge and awareness in rare and complex connective tissue diseases across Europe: results from the first ERN ReCONNET survey

et al. 2020

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IntroductionThe European Reference Network (ERN) ReCONNET is the ERN aimed at improving the management of rare and complex connective tissue and musculoskeletal diseases (rCTDs) across the European Union (EU). In the mission of ERN ReCONNET, clinical practice guidelines (CPGs) play a crucial role, representing a valid tool towards the harmonisation of the management of rCTDs while improving effectiveness and quality of care delivered to patients.MethodsERN ReCONNET developed two surveys to map the adherence to rCTDs CPGs among healthcare providers and to assess the knowledge and awareness of CPGs for their diseases among patients, family members and caregivers.ResultsThe results of the surveys highlighted that healthcare professionals find it useful to apply CPGs in clinical practice (93%), while 62% of them experience difficulties and barriers in the application in their centres. Healthcare professionals also highlighted the need to develop CPGs for all rCTDs and to implement the use of the existing CPGs in clinical practice. On the other hand, patients, families and caregivers are relatively aware of the purpose of CPGs (51%) and 62% of them were aware of the existence of CPGs for their disease. Patient-friendly versions of CPGs and patients’ lifestyle guidelines should be systematically developed contributing to the empowerment of patients in the disease management.ConclusionERN ReCONNET is addressing the main issues identified in the results of the survey, promoting practical actions for the local adaptation of CPGs across Europe, improving their routine clinical use and increasing the awareness on CPGs among rCTDs patients, family members and caregivers.

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Mixed connective tissue disease: state of the art on clinical practice guidelines

Cited by 44 publications

References 15 publications

IgM antibodies against malondialdehyde and phosphorylcholine in different systemic rheumatic diseases

IgM antibodies against malondialdehyde and phosphorylcholine in different systemic rheumatic diseases

Mixed connective tissue disease: Not always an obvious diagnosis

Clinical practice guidelines adherence, knowledge and awareness in rare and complex connective tissue diseases across Europe: results from the first ERN ReCONNET survey

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