ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 2586

Antinuclear Antibody Negative Systemic Sclerosis Patients Have Less Vasculopathic Disease Manifestations

Gloria Salazar1, Shervin Assassi1, Fredrick M. Wigley2, Daniel Furst3,4,5, Laura K. Hummers2, Dinesh Khanna6, John Varga7, Elena Schiopu8, Virginia D. Steen9, Murray Baron10, Marie Hudson11, Janet E. Pope12,13, Monique E. Hinchcliff14, Marvin J. Fritzler15, David B. Robinson16, Robert W. Simms17, Richard M. Silver18, Tracy M. Frech19, Barri J. Fessler20, Jerry A. Molitor21, Sara Zamanian22, Niall Jones23, John D. Reveille1, Frank C. Arnett1 and Maureen D. Mayes24, 1Rheumatology, University of Texas Health Science Center at Houston, Houston, TX, 2Division of Rheumatology, Johns Hopkins University School of Medicine, Baltimore, MD, 3David Geffen School of Medicine, Div of Rheumatology, University of California at Los Angeles, Los Angeles, CA, 4Div of Rheumatology, UCLA Medical School, Los Angeles, CA, 5Rheumatology, UCLA, Los Angeles, CA, 6University of Michigan Health System, Ann Arbor, MI, 7Rheumatology, Northwestern University Feinberg School of Medicine, Chicago, IL, 8Rheumatology/Internal Medicine, University of Michigan Medical School, Ann Arbor, MI, 9Department of Rheumatology, Georgetown University Medical Center, Washington, DC, 10McGill University, Montreal, QC, Canada, 11Rheumatology, Lady David Institute for Medical Research and Jewish General Hospital, Montreal, QC, Canada, 12Univ of Western Ontario, London, ON, Canada, 13St Joseph Health Care, London, ON, Canada, 14Division of Rheumatology, Northwestern University Medical School, Chicago, IL, 15Medicine, University of Calgary, Calgary, AB, Canada, 16Arthritis Centre, University of Manitoba, Winnipeg, MB, Canada, 17Rheumatology, Boston University School of Medicine, Boston, MA, 18Div Rheumatology & Immunology, Medical University of South Carolina,Charleston,USA, Charleston, SC, 19Internal Medicine-Division of Rheumatology, University of Utah School of Medicine, SLC, UT, 20Department of Medicine, University of Alabama at Birmingham, Birmingham, AL, 21Rheumatic/Autoimmune Diseases, Univ of MN MMC108, Minneapolis, MN, 22Internal Medicine/Rheumatology, University of Texas Health Science Center at Houston, Houston, TX, 23Rheumatology Clinic at 124th Street Medical Clinic, Edmonton, AB, Canada, 24University of Texas Health Science Center at Houston, Houston, TX

Meeting: 2013 ACR/ARHP Annual Meeting

Keywords: ,

Session Information

Title: Systemic Sclerosis, Fibrosing Syndromes, and Raynaud’s - Clinical Aspects and Therapeutics II

Session Type: Abstract Submissions (ACR)

Antinuclear Antibody Negative Systemic Sclerosis Patients Have Less Vasculopathic Disease Manifestations

Background/Purpose:

Systemic Sclerosis (SSc) is an autoimmune disease characterized by fibrosis of skin and internal organs, as well as vasculopathy and immune dysregulation.

Autoantibody formation is one of the hallmarks of SSc. Autoantibodies in patients with SSc carry considerable value in diagnosis and in predicting various clinical outcomes; however their role in the pathogenesis of SSc is unclear.

While the great majority of patients with SSc have circulating antinuclear antibodies (ANA) (90-95%), a small percentage is ANA negative (5-10%). The detailed demographic and clinical characteristics of patients without ANA have not been clearly explored.

The objective of this study was 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 directly by participating sites or by chart review. Autoantibodies were determined at one site utilizing commercially available kits.

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 significantly less vasculopathic manifestations of SSc. The percent predicted diffusion capacity of carbon monoxide (DLco) was higher in ANA negative patients (p=0.03). Seven ANA negative patients had pulmonary arterial hypertension (PAH) per right heart catheterization (RHC) versus 213 ANA positive (OR= 0.23 p<0.001) indicating that PAH was significantly less common in the ANA negative group. They also had less often telangiectasias and digital ulcers/ pits (p=0.01 and p<0.001, 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=0.018). Furthermore, they experienced more malabsorption (p=0.003). There was no difference in the frequency of pulmonary fibrosis and scleroderma renal crisis. All-cause mortality was also not different between the two groups (p=0.28).

The above observations remained significant after adjusting for potential confounders (age, disease duration, gender, disease type) (Table 1).

Conclusion:

ANA negative patients constitute a distinct subset of SSc characterized by fewer vasculopathic features of the disease, a higher proportion of men, more frequent lower gastrointestinal involvement as well as higher proportion of diffuse cutaneous disease but less severity of skin fibrosis overall.

Table 1. Multivariable analysis of clinical parameters in systemic sclerosis (SSc) patients who are ANA negative compared with ANA positive patients.

 

OR/ coef

95% CI

p

Telangiectasias

0.59

0.38, 0.91

0.01

Digital ulcers and pits

0.38

0.24, 0.59

<0.001

MPAP per RHC

-5.58

-10.85, -0.32

0.04

PAH per RHC

0.28

0.11, 0.70

0.006

 

 

Disclosure:

G. Salazar,
None;

S. Assassi,
None;

F. M. Wigley,
None;

D. Furst,

Abbott, Actelion, Amgen, BMS, Gilead, GSK, NIH, Novartis, Pfizer, Roche/Genentech, UCB,

2,

Abbott, Actelion, Amgen, BMS, Janssen, Gilead, GSK, NIH, Novartis, Pfizer, Roche/Genentech, UCB,

5,

Abbott, Actelion, UCB,

8;

L. K. Hummers,

Actelion Pharmaceuticals US,

2,

medimmune,

2;

D. Khanna,

NIH,

2,

Scleroderma Foundation,

2;

J. Varga,
None;

E. Schiopu,
None;

V. D. Steen,

Gilead Science, ,

2,

Gilead Science,

5,

Actelion Pharmaceuticals US,

2,

Actelion Pharmaceuticals US,

8,

Roche Pharmaceuticals,

2,

Celgene,

2,

Sanofi-Aventis Pharmaceutical,

2;

M. Baron,
None;

M. Hudson,
None;

J. E. Pope,
None;

M. E. Hinchcliff,
None;

M. J. Fritzler,

Inova Diagnostics, Inc.,

5;

D. B. Robinson,
None;

R. W. Simms,
None;

R. M. Silver,

Genentech and Biogen IDEC Inc.,

8,

Actelion Pharmaceuticals US,

9;

T. M. Frech,
None;

B. J. Fessler,
None;

J. A. Molitor,
None;

S. Zamanian,
None;

N. Jones,
None;

J. D. Reveille,
None;

F. C. Arnett,
None;

M. D. Mayes,
None.

« Back to 2013 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/antinuclear-antibody-negative-systemic-sclerosis-patients-have-less-vasculopathic-disease-manifestations/