Epidemiology of Mixed Connective Tissue Disease, 1985-2014: A Population-Based Study

Patompong Ungprasert, Cynthia Crowson, Vaidehi R. Chowdhary, Floranne C. Ernste, Kevin G. Moder, Eric Lawrence Matteson

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22 Citations (Scopus)

Abstract

Objective: To characterize the epidemiology of mixed connective tissue disease (MCTD) from 1983 to 2014. Methods: An inception cohort of patients with incident MCTD in 1985-2014 in Olmsted County, Minnesota was identified based on comprehensive individual medical record review. Diagnosis of MCTD required fulfillment of at least 1 of the 4 widely accepted diagnostic criteria without fulfillment of classification criteria for other connective tissue diseases. Data were collected on demographic characteristics, clinical presentation, laboratory investigations, and mortality. Results: A total of 50 incident cases of MCTD were identified (mean age 48.1 years and 84% were female). The annual incidence of MCTD was 1.9 per 100,000 population. Raynaud's phenomenon was the most common initial symptoms (50%), followed by arthralgia (30%) and swollen hands (16%). The diagnosis was frequently delayed with the median time from first symptom to fulfillment of criteria of 3.6 years. At fulfillment of criteria, arthralgia was the most prevalent manifestation (86%), followed by Raynaud's phenomenon (80%), swollen hands (64%), leukopenia/lymphopenia (44%), and heartburn (38%). Evolution to other connective tissue occurred infrequently with a 10-year rate of evolution of 8.5% and 6.3% for systemic lupus erythematosus and systemic sclerosis, respectively. The overall mortality was not different from the general population with a standardized mortality ratio of 1.1 (95% confidence interval 0.4-2.6). Conclusion: This study was the first population-based study of MCTD to provide a complete picture of epidemiology and clinical characteristics of MCTD. MCTD occurred in about 2 persons per 100,000 per year. Evolution to other connective diseases occurred infrequently and the mortality was not affected.

Original languageEnglish (US)
JournalArthritis Care and Research
DOIs
StateAccepted/In press - 2016

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Mixed Connective Tissue Disease
Epidemiology
Population
Raynaud Disease
Mortality
Arthralgia
Hand
Heartburn
Lymphopenia
Connective Tissue Diseases
Systemic Scleroderma
Leukopenia
Systemic Lupus Erythematosus
Connective Tissue
Medical Records
Demography
Confidence Intervals
Incidence

ASJC Scopus subject areas

  • Rheumatology

Cite this

Epidemiology of Mixed Connective Tissue Disease, 1985-2014 : A Population-Based Study. / Ungprasert, Patompong; Crowson, Cynthia; Chowdhary, Vaidehi R.; Ernste, Floranne C.; Moder, Kevin G.; Matteson, Eric Lawrence.

In: Arthritis Care and Research, 2016.

Research output: Contribution to journalArticle

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