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. 2023 Aug 1;62(8):2845-2849.
doi: 10.1093/rheumatology/keac703.

Population-based prevalence and incidence estimates of mixed connective tissue disease from the Manhattan Lupus Surveillance Program

Ghadeer Hasan  1 , Elizabeth D Ferucci  2 , Jill P Buyon  1 , H Michael Belmont  1 , Jane E Salmon  3 , Anca Askanase  4 , Joan M Bathon  4 , Laura Geraldino-Pardilla  4 , Yousaf Ali  5 , Ellen M Ginzler  6 , Chaim Putterman  7 , Caroline Gordon  8 , Charles G Helmick  9 , Hilary Parton  10 , Peter M Izmirly  1
Affiliations

Population-based prevalence and incidence estimates of mixed connective tissue disease from the Manhattan Lupus Surveillance Program

Ghadeer Hasan et al. Rheumatology (Oxford). .

Abstract

Objective: Epidemiological data for MCTD are limited. Leveraging data from the Manhattan Lupus Surveillance Program (MLSP), a racially/ethnically diverse population-based registry of cases with SLE and related diseases including MCTD, we provide estimates of the prevalence and incidence of MCTD.

Methods: MLSP cases were identified from rheumatologists, hospitals and population databases using a variety of International Classification of Diseases, Ninth Revision codes. MCTD was defined as one of the following: fulfilment of our modified Alarcon-Segovia and Kahn criteria, which required a positive RNP antibody and the presence of synovitis, myositis and RP; a diagnosis of MCTD and no other diagnosis of another CTD; and a diagnosis of MCTD regardless of another CTD diagnosis.

Results: Overall, 258 (7.7%) cases met a definition of MCTD. Using our modified Alarcon-Segovia and Kahn criteria for MCTD, the age-adjusted prevalence was 1.28 (95% CI 0.72, 2.09) per 100 000. Using our definition of a diagnosis of MCTD and no other diagnosis of another CTD yielded an age-adjusted prevalence and incidence of MCTD of 2.98 (95% CI 2.10, 4.11) per 100 000 and 0.39 (95% CI 0.22, 0.64) per 100 000, respectively. The age-adjusted prevalence and incidence were highest using a diagnosis of MCTD regardless of other CTD diagnoses and were 16.22 (95% CI 14.00, 18.43) per 100 000 and 1.90 (95% CI 1.49, 2.39) per 100 000, respectively.

Conclusions: The MLSP provided estimates for the prevalence and incidence of MCTD in a diverse population. The variation in estimates using different case definitions is reflective of the challenge of defining MCTD in epidemiologic studies.

Keywords: MCTD; epidemiology; incidence; prevalence.

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Conflict of interest statement

Conflicts of Interest:

Hasan: None, Ferucci: None, Buyon: None, Belmont: None, Salmon: None, Askanase: None, Bathon: None, Geraldino-Pardilla: None, Ali: None, Ginzler: None, Putterman: None, Gordon: Centers for Disease Control and Prevention, AbbVie, AstraZeneca, MGP, Sanofi, UCB, grants from UCB and Sandwell and West Birmingham Hospitals NHS Trust. Helmick: None, Parton: None, Izmirly: GlaxoSmithKline, Momenta/Janssen

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