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

Ghadeer Hasan; Elizabeth D Ferucci; Jill P Buyon; H. Michael Belmont; Jane E Salmon; Anca Askanase; Joan M. Bathon; Laura Geraldino-pardilla; Yousaf Ali; Ellen Ginzler; Chaim Putterman; Caroline Gordon; Charles G Helmick; Hilary Parton; Peter M. Izmirly

doi:10.1093/rheumatology/keac703

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

Ghadeer Hasan, Elizabeth D Ferucci, Jill P Buyon, H. Michael Belmont, Jane E Salmon, Anca Askanase, Joan M. Bathon, Laura Geraldino-pardilla, Yousaf Ali, Ellen Ginzler, Chaim Putterman, Caroline Gordon, Charles G Helmick, Hilary Parton, Peter M. Izmirly^*

^*Corresponding author for this work

Inflammation and Ageing

Research output: Contribution to journal › Article › peer-review

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Abstract

Objective: Epidemiologic data for mixed connective tissue disease (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 ICD-9 codes. MCTD was defined as one of the following: 1) fulfillment of our modified Alarcon-Segovia and Kahn criteria which required a positive RNP antibody and the presence of synovitis, myositis, and Raynaud’s phenomenon, 2) a diagnosis of MCTD and no other diagnosis of another connective tissue disease (CTD), and 3) a diagnosis of MCTD regardless of another CTD diagnosis.

Results: Overall, 258 (7.7%) of 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 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.

Original language	English
Article number	keac703
Number of pages	5
Journal	Rheumatology
Early online date	20 Dec 2022
DOIs	https://doi.org/10.1093/rheumatology/keac703
Publication status	E-pub ahead of print - 20 Dec 2022

Keywords

mixed connective tissue disease
Epidemiology
Prevalence
incidence

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HassanG2022Population
This is a pre-copyedited, author-produced version of an article accepted for publication in Rheumatology following peer review. The version of record Ghadeer Hasan, Elizabeth D Ferucci, Jill P Buyon, H Michael Belmont, Jane E Salmon, Anca Askanase, Joan M Bathon, Laura Geraldino-Pardilla, Yousaf Ali, Ellen M Ginzler, Chaim Putterman, Caroline Gordon, Charles G Helmick, Hilary Parton, Peter M Izmirly, Population-based prevalence and incidence estimates of mixed connective tissue disease from the Manhattan Lupus Surveillance Program, Rheumatology, 2022;, keac703, is available online at: https://academic.oup.com/rheumatology/advance-article/doi/10.1093/rheumatology/keac703/6947990, https://doi.org/10.1093/rheumatology/keac703
Accepted author manuscript, 502 KBLicence: Other (please specify with Rights Statement)

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Hasan, G., Ferucci, E. D., Buyon, J. P., Belmont, H. M., Salmon, J. E., Askanase, A., Bathon, J. M., Geraldino-pardilla, L., Ali, Y., Ginzler, E., Putterman, C., Gordon, C., Helmick, C. G., Parton, H., & Izmirly, P. M. (2022). Population-based prevalence and incidence estimates of mixed connective tissue disease from the Manhattan Lupus Surveillance Program. Rheumatology, Article keac703. Advance online publication. https://doi.org/10.1093/rheumatology/keac703

@article{74e4b721ab1b4c089de5c4b27c52d390,

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

abstract = "Objective: Epidemiologic data for mixed connective tissue disease (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 ICD-9 codes. MCTD was defined as one of the following: 1) fulfillment of our modified Alarcon-Segovia and Kahn criteria which required a positive RNP antibody and the presence of synovitis, myositis, and Raynaud{\textquoteright}s phenomenon, 2) a diagnosis of MCTD and no other diagnosis of another connective tissue disease (CTD), and 3) a diagnosis of MCTD regardless of another CTD diagnosis.Results: Overall, 258 (7.7%) of 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 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 = "mixed connective tissue disease, Epidemiology, Prevalence, incidence",

author = "Ghadeer Hasan and Ferucci, {Elizabeth D} and Buyon, {Jill P} and Belmont, {H. Michael} and Salmon, {Jane E} and Anca Askanase and Bathon, {Joan M.} and Laura Geraldino-pardilla and Yousaf Ali and Ellen Ginzler and Chaim Putterman and Caroline Gordon and Helmick, {Charles G} and Hilary Parton and Izmirly, {Peter M.}",

year = "2022",

month = dec,

day = "20",

doi = "10.1093/rheumatology/keac703",

language = "English",

journal = "Rheumatology",

issn = "1462-0324",

publisher = "Oxford University Press",

}

Hasan, G, Ferucci, ED, Buyon, JP, Belmont, HM, Salmon, JE, Askanase, A, Bathon, JM, Geraldino-pardilla, L, Ali, Y, Ginzler, E, Putterman, C, Gordon, C, Helmick, CG, Parton, H & Izmirly, PM 2022, 'Population-based prevalence and incidence estimates of mixed connective tissue disease from the Manhattan Lupus Surveillance Program', Rheumatology. https://doi.org/10.1093/rheumatology/keac703

TY - JOUR

T1 - Population-based prevalence and incidence estimates of mixed connective tissue disease from the Manhattan Lupus Surveillance Program

AU - Hasan, Ghadeer

AU - Ferucci, Elizabeth D

AU - Buyon, Jill P

AU - Belmont, H. Michael

AU - Salmon, Jane E

AU - Askanase, Anca

AU - Bathon, Joan M.

AU - Geraldino-pardilla, Laura

AU - Ali, Yousaf

AU - Ginzler, Ellen

AU - Putterman, Chaim

AU - Gordon, Caroline

AU - Helmick, Charles G

AU - Parton, Hilary

AU - Izmirly, Peter M.

PY - 2022/12/20

Y1 - 2022/12/20

N2 - Objective: Epidemiologic data for mixed connective tissue disease (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 ICD-9 codes. MCTD was defined as one of the following: 1) fulfillment of our modified Alarcon-Segovia and Kahn criteria which required a positive RNP antibody and the presence of synovitis, myositis, and Raynaud’s phenomenon, 2) a diagnosis of MCTD and no other diagnosis of another connective tissue disease (CTD), and 3) a diagnosis of MCTD regardless of another CTD diagnosis.Results: Overall, 258 (7.7%) of 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 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.

AB - Objective: Epidemiologic data for mixed connective tissue disease (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 ICD-9 codes. MCTD was defined as one of the following: 1) fulfillment of our modified Alarcon-Segovia and Kahn criteria which required a positive RNP antibody and the presence of synovitis, myositis, and Raynaud’s phenomenon, 2) a diagnosis of MCTD and no other diagnosis of another connective tissue disease (CTD), and 3) a diagnosis of MCTD regardless of another CTD diagnosis.Results: Overall, 258 (7.7%) of 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 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.

KW - mixed connective tissue disease

KW - Epidemiology

KW - Prevalence

KW - incidence

UR - https://academic.oup.com/rheumatology

U2 - 10.1093/rheumatology/keac703

DO - 10.1093/rheumatology/keac703

M3 - Article

SN - 1462-0324

JO - Rheumatology

JF - Rheumatology

M1 - keac703

ER -

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

Abstract

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