School of Medicine and Health Sciences Poster Presentations

Title

ANA negative scleroderma

Poster Number

168

Document Type

Poster

Status

Medical Resident

Abstract Category

Clinical Specialties

Keywords

systemic scleroderma, anti-nuclear autoantibodies

Publication Date

Spring 2018

Abstract

Background

Systemic Sclerosis (SSc) is an autoimmune disease characterized by inflammation, fibrosis, and vasculopathy. More than 90% of patients with SSc test positive for anti-nuclear antibodies using immunofluorescence (IF-ANA) as well as for scleroderma specific extractable nuclear antibodies such as topoisomerase (Scl-70), centromere, THTO, U3RNP, U1RNP and RNA Polymerase III. However, a small subset of scleroderma patients test negative for anti-nuclear antibodies. The purpose of this study was to investigate whether scleroderma patients with negative IF-ANA had different characteristics when compared to scleroderma patients with positive IF-ANA.

Methods

This study was IRB approved and all patients consented to be included in the study. At the time of data lock, 65 patients fulfilled diagnostic criteria for systemic sclerosis. Data was collected on patient demographics, autoantibody profile, and disease characteristics.

Results

Of the 65 patients, 49 had positive IF-ANA and 16 had negative IF-ANA. There was no significant difference in age, sex, race or scleroderma subtype (sine, limited, diffuse, or localized) between the IF-ANA positive and negative groups. The IF-ANA negative group were more likely to have positive U3RNP (p=0.009).

Conclusion

The U3RNP is one of the nucleolar antibodies which is known to be missed using direct ANA testing; however, this study shows that this antibody subtype may also be missed using the IF-ANA test. This is an important finding since scleroderma patients with U3RNP are at higher risk for life threatening complications of scleroderma including pulmonary hypertension and pulmonary fibrosis.

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Creative Commons License
This work is licensed under a Creative Commons Attribution-Noncommercial-No Derivative Works 4.0 License.

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ANA negative scleroderma

Background

Systemic Sclerosis (SSc) is an autoimmune disease characterized by inflammation, fibrosis, and vasculopathy. More than 90% of patients with SSc test positive for anti-nuclear antibodies using immunofluorescence (IF-ANA) as well as for scleroderma specific extractable nuclear antibodies such as topoisomerase (Scl-70), centromere, THTO, U3RNP, U1RNP and RNA Polymerase III. However, a small subset of scleroderma patients test negative for anti-nuclear antibodies. The purpose of this study was to investigate whether scleroderma patients with negative IF-ANA had different characteristics when compared to scleroderma patients with positive IF-ANA.

Methods

This study was IRB approved and all patients consented to be included in the study. At the time of data lock, 65 patients fulfilled diagnostic criteria for systemic sclerosis. Data was collected on patient demographics, autoantibody profile, and disease characteristics.

Results

Of the 65 patients, 49 had positive IF-ANA and 16 had negative IF-ANA. There was no significant difference in age, sex, race or scleroderma subtype (sine, limited, diffuse, or localized) between the IF-ANA positive and negative groups. The IF-ANA negative group were more likely to have positive U3RNP (p=0.009).

Conclusion

The U3RNP is one of the nucleolar antibodies which is known to be missed using direct ANA testing; however, this study shows that this antibody subtype may also be missed using the IF-ANA test. This is an important finding since scleroderma patients with U3RNP are at higher risk for life threatening complications of scleroderma including pulmonary hypertension and pulmonary fibrosis.