Antinuclear antibody-negative systemic sclerosis

Gloria A Salazar¹, Shervin Assassi², Fredrick Wigley³, Laura Hummers³, John Varga⁴, Monique Hinchcliff⁴, Dinesh Khanna⁵, Elena Schiopu⁵, Kristine Phillips⁵, Daniel E Furst⁶, Virginia Steen⁷, Murray Baron⁸, Marie Hudson⁸, Suzanne S Taillefer⁸, Janet Pope⁹, Niall Jones¹⁰, Peter Docherty¹¹, Nader A Khalidi¹², David Robinson¹³, Robert W Simms¹⁴, Richard M Silver¹⁵, Tracy M Frech¹⁶, Barri J Fessler¹⁷, Jerry A Molitor¹⁸, Marvin J Fritzler¹⁹, Barbara M Segal¹⁸, Firas Al-Kassab²⁰, Marilyn Perry², Jeremy Yang², Sara Zamanian²¹, John D Reveille², Frank C Arnett², Claudia Pedroza², Maureen D Mayes²

Affiliations

¹ Division of Rheumatology, University of Texas Medical School at Houston, 6431 Fannin St. MSB 5.270, Houston, TX 77030. Electronic address: gloria.salazarcintora@uth.tmc.edu.
² Division of Rheumatology, University of Texas Medical School at Houston, 6431 Fannin St. MSB 5.270, Houston, TX 77030.
³ Asthma and Allergy Center, Johns Hopkins University, Baltimore, MD.
⁴ Department of Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL.
⁵ Division of Rheumatology, University of Michigan, Ann Arbor, MI.
⁶ Division of Rheumatology, University of California Los Angeles, Los Angeles, CA.
⁷ Division of Rheumatology, Georgetown University Medical Center, Washington, DC.
⁸ Division of Rheumatology, McGill University, Jewish General Hospital, Montreal, Québec, Canada.
⁹ Division of Rheumatology, University of Western Ontario, London, Ontario, Canada.
¹⁰ Division of Rheumatology, University of Alberta, Edmonton, Alberta, Canada.
¹¹ The Moncton Hospital, South East Regional Health Authority, Moncton, New Brunswick, Canada.
¹² Division of Rheumatology, McMaster University, Hamilton, Ontario, Canada.
¹³ Arthritis Center, University of Manitoba, Winnipeg, Manitoba, Canada.
¹⁴ Arthritis Center/Rheumatology, Boston University School of Medicine, Boston, MA.
¹⁵ Division of Rheumatology, Medical University of South Carolina, Charleston, SC.
¹⁶ Division of Rheumatology, University of Utah School of Medicine, Salt Lake City, UT.
¹⁷ Division of Rheumatology, University of Alabama Birmingham, Birmingham, AL.
¹⁸ Division of Rheumatic and Autoimmune Diseases, University of Minnesota, Minneapolis, MN.
¹⁹ Department of Medicine, University of Calgary Cumming School of Medicine, Calgary, Alberta, Canada.
²⁰ Division of Rheumatology, University of Massachusetts Medical School, Worcester, MA.
²¹ Department of Internal Medicin, Case Western Reserve University, Cleveland, OH.

PMID: 25578738
PMCID: PMC4447614
DOI: 10.1016/j.semarthrit.2014.11.006

Antinuclear antibody-negative systemic sclerosis

Gloria A Salazar et al. Semin Arthritis Rheum. 2015 Jun.

. 2015 Jun;44(6):680-6.

doi: 10.1016/j.semarthrit.2014.11.006. Epub 2014 Nov 28.

Authors

Affiliations

¹ Division of Rheumatology, University of Texas Medical School at Houston, 6431 Fannin St. MSB 5.270, Houston, TX 77030. Electronic address: gloria.salazarcintora@uth.tmc.edu.
² Division of Rheumatology, University of Texas Medical School at Houston, 6431 Fannin St. MSB 5.270, Houston, TX 77030.
³ Asthma and Allergy Center, Johns Hopkins University, Baltimore, MD.
⁴ Department of Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL.
⁵ Division of Rheumatology, University of Michigan, Ann Arbor, MI.
⁶ Division of Rheumatology, University of California Los Angeles, Los Angeles, CA.
⁷ Division of Rheumatology, Georgetown University Medical Center, Washington, DC.
⁸ Division of Rheumatology, McGill University, Jewish General Hospital, Montreal, Québec, Canada.
⁹ Division of Rheumatology, University of Western Ontario, London, Ontario, Canada.
¹⁰ Division of Rheumatology, University of Alberta, Edmonton, Alberta, Canada.
¹¹ The Moncton Hospital, South East Regional Health Authority, Moncton, New Brunswick, Canada.
¹² Division of Rheumatology, McMaster University, Hamilton, Ontario, Canada.
¹³ Arthritis Center, University of Manitoba, Winnipeg, Manitoba, Canada.
¹⁴ Arthritis Center/Rheumatology, Boston University School of Medicine, Boston, MA.
¹⁵ Division of Rheumatology, Medical University of South Carolina, Charleston, SC.
¹⁶ Division of Rheumatology, University of Utah School of Medicine, Salt Lake City, UT.
¹⁷ Division of Rheumatology, University of Alabama Birmingham, Birmingham, AL.
¹⁸ Division of Rheumatic and Autoimmune Diseases, University of Minnesota, Minneapolis, MN.
¹⁹ Department of Medicine, University of Calgary Cumming School of Medicine, Calgary, Alberta, Canada.
²⁰ Division of Rheumatology, University of Massachusetts Medical School, Worcester, MA.
²¹ Department of Internal Medicin, Case Western Reserve University, Cleveland, OH.

PMID: 25578738
PMCID: PMC4447614
DOI: 10.1016/j.semarthrit.2014.11.006

Abstract

Objective: To examine the demographic and clinical characteristics of systemic sclerosis (SSc) patients without antinuclear antibodies (ANA) compared to ANA-positive patients.

Methods: SSc patients enrolled in the Scleroderma Family Registry and DNA Repository were included. Relevant demographic and clinical data were entered by participating sites or obtained by chart review. ANA and SSc-related antibodies were determined in all investigated patients using commercially available kits at our laboratories.

Results: This study included 3249 patients, of whom 208 (6.4%) were ANA negative. The proportion of male patients was higher in the ANA-negative group (OR = 1.65; p = 0.008). ANA-negative patients experienced less vasculopathic manifestations of SSc. The percent predicted diffusing capacity of carbon monoxide (DLCO) was higher in ANA-negative patients (p = 0.03). Pulmonary arterial hypertension (PAH) per right heart catheterization was less common in the ANA-negative group (OR = 0.28; p = 0.03). Furthermore, patients with negative ANA had a lower prevalence of telangiectasias and digital ulcers/pits (OR = 0.59, p = 0.03 and OR = 0.38, p = 0.01, respectively). Although diffuse cutaneous involvement was more common, the modified Rodnan Skin Score (mRSS) was lower in the ANA-negative group (2.4 points lower, p = 0.05). Furthermore, they experienced more malabsorption (p = 0.05). There was no difference in the frequency of pulmonary fibrosis or scleroderma renal crisis. All-cause mortality was not different between the 2 groups (p = 0.28).

Conclusions: In conclusion, the results of this study suggest that SSc patients who are ANA negative constitute a distinct subset of SSc with less vasculopathy (less PAH, digital ulcers, and fewer telangiectasias), a greater proportion of males, and possibly, more frequent lower gastrointestinal involvement.

Keywords: ANA; Antinuclear antibody; Negative; Scleroderma; Systemic sclerosis; Vasculopathy.

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Conflict of interest statement

DISCLOSURES: The authors report no conflicts of interest.

Figures

**Figure 1**
Kaplan-Meier survivor function of ANA negative SSc patients compared to ANA positive patients

See this image and copyright information in PMC

References

1. Ho KT, Reveille JD. The clinical relevance of autoantibodies in scleroderma. Arthritis Res Ther. 2003;5:80–93. - PMC - PubMed
1. Hamaguchi Y. Autoantibody profiles in systemic sclerosis: predictive value for clinical evaluation and prognosis. J Dermatol. 2010;37:42–53. - PubMed
1. Steen VD. Autoantibodies in systemic sclerosis. Semin Arthritis Rheum. 2005;35:35–42. - PubMed
1. Arnett FC. Is scleroderma an autoantibody mediated disease? Curr Opin Rheumatol. 2006;18:579–81. - PubMed
1. Mayes MD. The establishment and utility of a population-based registry to understand the epidemiology of systemic sclerosis. Curr Rheumatol Rep. 2000;2:512–6. - PubMed

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Antinuclear antibody-negative systemic sclerosis

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Antinuclear antibody-negative systemic sclerosis

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Conflict of interest statement

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