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Arthritis Care Res (Hoboken). 2016 Dec;68(12):1843-1848. doi: 10.1002/acr.22872. Epub 2016 Oct 1.

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

Author information

1
Mayo Clinic, Rochester, Minnesota.

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.

PMID:
26946215
PMCID:
PMC5426802
DOI:
10.1002/acr.22872
[Indexed for MEDLINE]
Free PMC Article

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