title: Antinuclear antibody-negative systemic sclerosis.
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.
authors.affiliation: 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.
authors.forename: Gloria A
authors.initials: GA
authors.lastname: Salazar
authors.affiliation: Division of Rheumatology, University of Texas Medical School at Houston, 6431 Fannin St. MSB 5.270, Houston, TX 77030.
authors.forename: Shervin
authors.initials: S
authors.lastname: Assassi
authors.affiliation: Asthma and Allergy Center, Johns Hopkins University, Baltimore, MD.
authors.forename: Fredrick
authors.initials: F
authors.lastname: Wigley
authors.affiliation: Asthma and Allergy Center, Johns Hopkins University, Baltimore, MD.
authors.forename: Laura
authors.initials: L
authors.lastname: Hummers
authors.affiliation: Department of Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL.
authors.forename: John
authors.initials: J
authors.lastname: Varga
authors.affiliation: Department of Medicine, Northwestern University Feinberg School of Medicine, Chicago, IL.
authors.forename: Monique
authors.initials: M
authors.lastname: Hinchcliff
authors.affiliation: Division of Rheumatology, University of Michigan, Ann Arbor, MI.
authors.forename: Dinesh
authors.initials: D
authors.lastname: Khanna
authors.affiliation: Division of Rheumatology, University of Michigan, Ann Arbor, MI.
authors.forename: Elena
authors.initials: E
authors.lastname: Schiopu
authors.affiliation: Division of Rheumatology, University of Michigan, Ann Arbor, MI.
authors.forename: Kristine
authors.initials: K
authors.lastname: Phillips
authors.affiliation: Division of Rheumatology, University of California Los Angeles, Los Angeles, CA.
authors.forename: Daniel E
authors.initials: DE
authors.lastname: Furst
authors.affiliation: Division of Rheumatology, Georgetown University Medical Center, Washington, DC.
authors.forename: Virginia
authors.initials: V
authors.lastname: Steen
authors.affiliation: Division of Rheumatology, McGill University, Jewish General Hospital, Montreal, Québec, Canada.
authors.forename: Murray
authors.initials: M
authors.lastname: Baron
authors.affiliation: Division of Rheumatology, McGill University, Jewish General Hospital, Montreal, Québec, Canada.
authors.forename: Marie
authors.initials: M
authors.lastname: Hudson
authors.affiliation: Division of Rheumatology, McGill University, Jewish General Hospital, Montreal, Québec, Canada.
authors.forename: Suzanne S
authors.initials: SS
authors.lastname: Taillefer
authors.affiliation: Division of Rheumatology, University of Western Ontario, London, Ontario, Canada.
authors.forename: Janet
authors.initials: J
authors.lastname: Pope
authors.affiliation: Division of Rheumatology, University of Alberta, Edmonton, Alberta, Canada.
authors.forename: Niall
authors.initials: N
authors.lastname: Jones
authors.affiliation: The Moncton Hospital, South East Regional Health Authority, Moncton, New Brunswick, Canada.
authors.forename: Peter
authors.initials: P
authors.lastname: Docherty
authors.affiliation: Division of Rheumatology, McMaster University, Hamilton, Ontario, Canada.
authors.forename: Nader A
authors.initials: NA
authors.lastname: Khalidi
authors.affiliation: Arthritis Center, University of Manitoba, Winnipeg, Manitoba, Canada.
authors.forename: David
authors.initials: D
authors.lastname: Robinson
authors.affiliation: Arthritis Center/Rheumatology, Boston University School of Medicine, Boston, MA.
authors.forename: Robert W
authors.initials: RW
authors.lastname: Simms
authors.affiliation: Division of Rheumatology, Medical University of South Carolina, Charleston, SC.
authors.forename: Richard M
authors.initials: RM
authors.lastname: Silver
authors.affiliation: Division of Rheumatology, University of Utah School of Medicine, Salt Lake City, UT.
authors.forename: Tracy M
authors.initials: TM
authors.lastname: Frech
authors.affiliation: Division of Rheumatology, University of Alabama Birmingham, Birmingham, AL.
authors.forename: Barri J
authors.initials: BJ
authors.lastname: Fessler
authors.affiliation: Division of Rheumatic and Autoimmune Diseases, University of Minnesota, Minneapolis, MN.
authors.forename: Jerry A
authors.initials: JA
authors.lastname: Molitor
authors.affiliation: Department of Medicine, University of Calgary Cumming School of Medicine, Calgary, Alberta, Canada.
authors.forename: Marvin J
authors.initials: MJ
authors.lastname: Fritzler
authors.affiliation: Division of Rheumatic and Autoimmune Diseases, University of Minnesota, Minneapolis, MN.
authors.forename: Barbara M
authors.initials: BM
authors.lastname: Segal
authors.affiliation: Division of Rheumatology, University of Massachusetts Medical School, Worcester, MA.
authors.forename: Firas
authors.initials: F
authors.lastname: Al-Kassab
authors.affiliation: Division of Rheumatology, University of Texas Medical School at Houston, 6431 Fannin St. MSB 5.270, Houston, TX 77030.
authors.forename: Marilyn
authors.initials: M
authors.lastname: Perry
authors.affiliation: Division of Rheumatology, University of Texas Medical School at Houston, 6431 Fannin St. MSB 5.270, Houston, TX 77030.
authors.forename: Jeremy
authors.initials: J
authors.lastname: Yang
authors.affiliation: Department of Internal Medicin, Case Western Reserve University, Cleveland, OH.
authors.forename: Sara
authors.initials: S
authors.lastname: Zamanian
authors.affiliation: Division of Rheumatology, University of Texas Medical School at Houston, 6431 Fannin St. MSB 5.270, Houston, TX 77030.
authors.forename: John D
authors.initials: JD
authors.lastname: Reveille
authors.affiliation: Division of Rheumatology, University of Texas Medical School at Houston, 6431 Fannin St. MSB 5.270, Houston, TX 77030.
authors.forename: Frank C
authors.initials: FC
authors.lastname: Arnett
authors.affiliation: Division of Rheumatology, University of Texas Medical School at Houston, 6431 Fannin St. MSB 5.270, Houston, TX 77030.
authors.forename: Claudia
authors.initials: C
authors.lastname: Pedroza
authors.affiliation: Division of Rheumatology, University of Texas Medical School at Houston, 6431 Fannin St. MSB 5.270, Houston, TX 77030.
authors.forename: Maureen D
authors.initials: MD
authors.lastname: Mayes
mesh_terms: D000328:Adult; D000974:Antibodies, Antinuclear / Q000276:immunology*; D016022:Case-Control Studies; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology; D007674:Kidney Diseases / Q000209:etiology; D008168:Lung / Q000503:physiopathology; D008286:Malabsorption Syndromes / Q000209:etiology; D008297:Male; D008875:Middle Aged; D011653:Pulmonary Diffusing Capacity; D011658:Pulmonary Fibrosis / Q000209:etiology; D012189:Retrospective Studies; D012595:Scleroderma, Systemic / Q000150:complications / Q000276:immunology* / Q000503:physiopathology; D012737:Sex Factors; D012883:Skin Ulcer / Q000209:etiology; D013684:Telangiectasis / Q000209:etiology
chemical_list: D000974:Antibodies, Antinuclear
keywords: ANA; Antinuclear antibody; Negative; Scleroderma; Systemic sclerosis; Vasculopathy