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Original Investigation |

Morphea in Adults and Children Cohort III:  Nested Case-Control Study—The Clinical Significance of Autoantibodies in Morphea

Jennifer Warner Dharamsi, MD1; Sandra Victor, BS1; Nancy Aguwa, BS2; Chul Ahn, PhD3; Frank Arnett, MD4; Maureen D. Mayes, MD, MPH4; Heidi Jacobe, MD, MSCS1
[+] Author Affiliations
1Department of Dermatology, University of Texas Southwestern Medical Center, Dallas
2University of Texas Health Science Center at San Antonio, San Antonio
3Department of Clinical Sciences, University of Texas Southwestern Medical Center, Dallas
4Division of Rheumatology and Clinical Immunogenetics, Department of Internal Medicine, University of Texas Health Science Center at Houston, Houston
JAMA Dermatol. 2013;149(10):1159-1165. doi:10.1001/jamadermatol.2013.4207.
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Importance  Small studies have implicated the association of specific autoantibodies with morphea subtype or severity, but no large-scale studies have been conducted. This prospective case-control study confirmed the presence of antinuclear antibodies (ANAs) and other autoantibodies in morphea but found they are of limited significance.

Objective  To determine the prevalence of ANAs, extractable nuclear antigens such as antihistone antibodies (AHAs), and anti–single-stranded DNA antibodies (ssDNA abs) in patients with morphea vs a healthy control population and their association with clinical measures of morphea severity.

Design, Setting, and Participants  Nested case-control study, conducted at the University of Texas Southwestern Medical Center, Dallas, and University of Texas Health Science Center, Houston. Study participants included individuals enrolled in the Morphea in Adults and Children (MAC) cohort and Scleroderma Family Registry and DNA Repository.

Main Outcomes and Measures  Prevalence of ANAs, AHAs, ssDNA abs in patients with morphea vs matched controls and association of the presence of autoantibodies with clinical indicators of morphea severity.

Results  The prevalence of ANAs, AHAs, and ssDNA abs in patients with morphea was 34%, 12%, and 8%, respectively. Antinuclear antibodies and AHAs, but not ssDNA abs, were present more frequently in cases than in controls. There was no difference in ANA prevalence among morphea subtypes. Among patients with linear morphea, the presence of autoantibodies was associated with clinical indicators of severe morphea including functional limitation (ssDNA ab, P = .005; and AHA, P = .006), extensive body surface area involvement (ssDNA ab, P = .01; and ANA, P = .005), and higher skin scores (ANA, P = .004). The presence of autoantibodies was not associated with clinical measures of morphea activity.

Conclusions and Relevance  Our results demonstrate that ANAs and AHAs are more prevalent among patients with morphea but are of limited clinical utility except in linear morphea, where their presence, although infrequent, is associated with greater lesion burden and functional impairment.

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Figure 1.
Algorithm Summarizing Patients Included in the Present Study

MAC indicates Morphea in Adults and Children

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Figure 2.
Serum Samples That Had Antinuclear Antibodies (ANAs) Present at a Cutoff Titer of 1:80 Were Serially Diluted up to a Titer of 1:1280

The frequency (number of patients) of a positive ANA at each dilution and pattern of immunofluorescence on Hep-2 cells are presented.aData are given as number of patients

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