Antinuclear antibody-negative systemic sclerosis

Gloria A. Salazar, Shervin Assassi, Fredrick Wigley, Laura Hummers, John Varga, Monique Hinchcliff, Dinesh Khanna, Elena Schiopu, Kristine Phillips, Daniel E. Furst, Virginia Steen, Murray Baron, Marie Hudson, Suzanne S. Taillefer, Janet Pope, Niall Jones, Peter Docherty, Nader A. Khalidi, David Robinson, Robert W. SimmsRichard M. Silver, Tracy M. Frech, Barri J. Fessler, Jerry A. Molitor, Marvin J. Fritzler, Barbara M. Segal, Firas Al-Kassab, Marilyn Perry, Jeremy Yang, Sara Zamanian, John D. Reveille, Frank C. Arnett, Claudia Pedroza, Maureen D. Mayes

Research output: Contribution to journalArticlepeer-review

36 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)680-686
Number of pages7
JournalSeminars in Arthritis and Rheumatism
Issue number6
StatePublished - Jun 1 2015


  • ANA
  • Antinuclear antibody
  • Negative
  • Scleroderma
  • Systemic sclerosis
  • Vasculopathy

ASJC Scopus subject areas

  • Rheumatology
  • Anesthesiology and Pain Medicine


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