Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2018 Oct 18;4(Suppl 1):e000783.
doi: 10.1136/rmdopen-2018-000783. eCollection 2018.

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

Affiliations

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

Benjamin Chaigne et al. RMD Open. .

Abstract

Mixed connective tissue disease (MCTD) is a complex overlap disease with features of different autoimmune connective tissue diseases (CTDs) namely systemic sclerosis, poly/dermatomyositis and systemic lupus erythematous in patients with antibodies targeting the U1 small nuclear ribonucleoprotein particle. In this narrative review, we summarise the results of a systematic literature research which was performed as part of the European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases project, aimed at evaluating existing clinical practice guidelines (CPGs) or recommendations. Since no specific CPGs on MCTD were found, other CPGs developed for other CTDs were taken into consideration in order to discuss what can be applied to MCTD even if designed for other diseases. Three major objectives were proposed for the future development of CPGs: MCTD diagnosis (diagnostic criteria), MCTD initial and follow-up evaluations, MCTD treatment. Early diagnosis, epidemiological data, assessment of burden of disease and QOL aspects are among the unmet needs identified by patients.

Keywords: ERN reconnet; clinical practice guidelines; european reference networks; mixed connective tissue disease; unmet needs.

PubMed Disclaimer

Conflict of interest statement

Competing interests: None declared.

Figures

Figure 1
Figure 1
Flow diagram of the systematic literature search on mixed connective tissue disease (MCTD) clinical practice guidelines.

Similar articles

  • Undifferentiated connective tissue disease: state of the art on clinical practice guidelines.
    Antunes M, Scirè CA, Talarico R, Alexander T, Avcin T, Belocchi C, Doria A, Franceschini F, Galetti I, Govoni M, Hachulla E, Launay D, Lepri G, Macieira C, Matucci-Cerinic M, Montecucco CM, Moraes-Fontes MF, Mouthon L, Paolino S, Ramoni V, Tani C, Tas SW, Tincani A, Van Vollenhoven R, Zen M, Fonseca J, Bombardieri S, Fonseca JE, Schneider M, Smith V, Cutolo M, Mosca M, Beretta L. Antunes M, et al. RMD Open. 2019 Feb 26;4(Suppl 1):e000786. doi: 10.1136/rmdopen-2018-000786. eCollection 2018. RMD Open. 2019. PMID: 30886731 Free PMC article. Review.
  • Mixed connective tissue disease.
    Gunnarsson R, Hetlevik SO, Lilleby V, Molberg Ø. Gunnarsson R, et al. Best Pract Res Clin Rheumatol. 2016 Feb;30(1):95-111. doi: 10.1016/j.berh.2016.03.002. Epub 2016 Apr 12. Best Pract Res Clin Rheumatol. 2016. PMID: 27421219 Review.
  • IgG4-related diseases: state of the art on clinical practice guidelines.
    Iaccarino L, Talarico R, Scirè CA, Amoura Z, Burmester G, Doria A, Faiz K, Frank C, Hachulla E, Hie M, Launay D, Montecucco C, Monti S, Mouthon L, Tincani A, Toniati P, Van Hagen PM, Van Vollenhoven RF, Bombardieri S, Mueller-Ladner U, Schneider M, Smith V, Cutolo M, Mosca M, Alexander T. Iaccarino L, et al. RMD Open. 2019 Jan 19;4(Suppl 1):e000787. doi: 10.1136/rmdopen-2018-000787. eCollection 2018. RMD Open. 2019. PMID: 30729031 Free PMC article. Review.
  • Sjögren's syndrome: state of the art on clinical practice guidelines.
    Romão VC, Talarico R, Scirè CA, Vieira A, Alexander T, Baldini C, Gottenberg JE, Gruner H, Hachulla E, Mouthon L, Orlandi M, Pamfil C, Pineton de Chambrun M, Taglietti M, Toplak N, van Daele P, van Laar JM, Bombardieri S, Schneider M, Smith V, Cutolo M, Mosca M, Mariette X. Romão VC, et al. RMD Open. 2018 Oct 18;4(Suppl 1):e000789. doi: 10.1136/rmdopen-2018-000789. eCollection 2018. RMD Open. 2018. PMID: 30402274 Free PMC article.
  • Clinical practice guidelines adherence, knowledge and awareness in rare and complex connective tissue diseases across Europe: results from the first ERN ReCONNET survey.
    Talarico R, Marinello D, Bombardieri S, Burmester G, Fonseca J, Frank C, Galetti I, Hachulla E, Houssiau F, Mueller-Ladner U, Schneider M, Smith V, Turchetti G, van Laar JM, Vieira A, Cutolo M, Mosca M. Talarico R, et al. RMD Open. 2020 Aug;6(2):0. doi: 10.1136/rmdopen-2020-001344. RMD Open. 2020. PMID: 32868449 Free PMC article.

Cited by

References

    1. Sharp GC, Irvin WS, Tan EM, et al. . Mixed connective tissue disease--an apparently distinct rheumatic disease syndrome associated with a specific antibody to an Extractable Nuclear Antigen (ENA). Am J Med 1972;52:148–59. 10.1016/0002-9343(72)90064-2 - DOI - PubMed
    1. Institute of Medicine (US) Committee to Advise the Public Health Service on Clinical Practice Guidelines In: Field MJ, Lohr KN, Clinical practice guidelines: directions for a new program. Washington (DC): National Academies Press (US), 1990. - PubMed
    1. Institute of Medicine (US) Committee on Standards for Developing Trustworthy Clinical Practice Guidelines In: Graham R, Mancher M, Miller Wolman D, Clinical practice guidelines we can trust. Washington (DC): National Academies Press (US), 2011. - PubMed
    1. Brouwers MC, Kho ME, Browman GP, et al. . AGREE II: advancing guideline development, reporting and evaluation in health care. Can Med Assoc J 2010;182:E839–42. 10.1503/cmaj.090449 - DOI - PMC - PubMed
    1. Brouwers MC, Kho ME, Browman GP, et al. . Development of the AGREE II, part 1: performance, usefulness and areas for improvement. Can Med Assoc J 2010;182:1045–52. 10.1503/cmaj.091714 - DOI - PMC - PubMed