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Research ArticleArticle

Autoantibodies to Dense Fine Speckles in Pediatric Diseases and Controls

Heinrike Schmeling, Michael Mahler, Deborah M. Levy, Katharine Moore, Anne M. Stevens, James Wick, Jacob D. McMillan, Gerd Horneff, Shervin Assassi, Julio Charles, Gloria Salazar, Maureen D. Mayes, Earl D. Silverman, Marissa Klien-Gitelman, Tzelan Lee, Hermine I. Brunner, Ann M. Reed and Marvin J. Fritzler
The Journal of Rheumatology December 2015, 42 (12) 2419-2426; DOI: https://doi.org/10.3899/jrheum.150567
Heinrike Schmeling
From the Department of Paediatrics, Alberta Children’s Hospital, and McCaig Institute for Bone and Joint Health, and Faculty of Medicine, University of Calgary; Alberta Children’s Hospital Research Institute, Calgary, Alberta; Hospital for Sick Children; University of Toronto, Toronto, Ontario, Canada; Inova Diagnostics Inc., San Diego, California; Stanford University/Lucile Packard Children’s Hospital, Stanford, California; Seattle Children’s Research Institute, Department of Pediatrics, University of Washington, Seattle, Washington; Division of Rheumatology, University of Texas Houston Medical School, Houston, Texas; Northwestern University/Lurie Children’s Hospital, Chicago, Illinois; University of Cincinnati; Cincinnati Children’s Hospital and Medical Center, Cincinnati, Ohio; Department of Pediatrics, Duke University, Durham, North Carolina, USA; Department of General Paediatrics, Centre of Paediatrics and Neonatology, Asklepios Clinics, Sankt Augustin, Germany.
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Michael Mahler
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Deborah M. Levy
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Katharine Moore
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Anne M. Stevens
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James Wick
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Jacob D. McMillan
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Gerd Horneff
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Shervin Assassi
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Julio Charles
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Gloria Salazar
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Maureen D. Mayes
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Earl D. Silverman
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Marissa Klien-Gitelman
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Tzelan Lee
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Hermine I. Brunner
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Ann M. Reed
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Marvin J. Fritzler
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  • For correspondence: fritzler@ucalgary.ca
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    Figure 1.

    Prevalence of anti-DFS70 autoantibodies as detected by CIA in samples grouped according to the ANA IIF patterns on HEp-2 cell substrates. DFS70: dense fine speckled 70 kDa antigen; CIA: chemiluminescence immunoassay; ANA: antinuclear antibody; IIF: indirect immunofluorescence.

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    Table 1.

    Frequency of anti-DFS70 antibodies in pediatric cohorts.

    CohortnDFS70-positive, nDFS70-positive, %p†
    Reference cohorts, n = 345
      ANA referrals, qCTD*20094.5NS
      Healthy14532.1NA
    AARD, n = 383
      cSLE331195.7NS
      JDM11218.2NS
      Diffuse cSSc2214.5NS
      Limited cSSc1900.0NS
    Other inflammatory conditions, n = 360
      JIA without uveitis18331.6NS
      JIA with uveitis19210.5NS
      Arthralgia3200.0NS
      Celiac disease4924.1NS
      Localized scleroderma29413.80.0310
      Idiopathic uveitis7114.3NS
      Reactive arthritis2100.0NS
    Other**2000.0NS
    • ↵* Sera referred for an ANA/ENA test as part of the investigations for qCTD. Sera were from children ≤ 18 years of age.

    • ↵** Cases of Crohn disease, Reiter syndrome, panniculitis, sarcoidosis, primary antiphospholipid syndrome, viral myopathy, eosinophilic fasciitis.

    • ↵† P value is calculated against healthy individuals by Student t test. DFS70: dense fine speckled 70 kDa antigen; ANA: antinuclear antibody; AARD: ANA-associated rheumatic disease; qCTD: query connective tissue disease (children being investigated for an AARD); cSLE: childhood systemic lupus erythematosus; cSSc: childhood systemic sclerosis; JIA: juvenile idiopathic arthritis; NA: not applicable; NS: not statistically significant; JDM: juvenile dermatomyositis.

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    Table 2.

    Overview of patients with anti-DFS70 antibodies.

    Patient No.DiagnosisAge, yrsSexRacial GroupDFS70 Reactivity by CIA, CUANA Pattern/titer by IIFENA/LIA/other Autoantibodies*
    1JIA10MWhite36.3NTNegative
    2JIA13MWhite49.9NTNegative
    3JIA13FWhite27.9DFS 1/320Negative
    4JIA uveitis6FWhite131.5DFS 1/5120Negative
    5JIA uveitis9MAsian38.2DFS 1/1280Negative
    6Uveitis16MWhite78.2DFS 1/2560Negative
    7cSLE15FWhite212.0DFS 1/1280Sm, U1RNP weak positive
    8cSLE16FWhite138.4DFS 1/1280dsDNA, U1RNP
    9cSLE18FAsian88.6DFS, MNDdsDNA, U1RNP
    10cSLE10FMixed140.1DFS 1/2560Sm, U1RNP, Ro52
    11cSLE*15FWhite59.1DFS 1/2560U1RNP, weak dsDNA
    12cSLE14FBlack45.6DFS, NLR, cytoplasmic 1/1280U1RNP, Ro60, weak Rib P, Ro52
    13cSLE13FAsian39.1DFS 1/320Negative
    14cSLE*15FWhite81.5DFS, NLR 1/1280U1RNP
    15cSLE13FAsian30.7DFS 1/2560Sm, U1RNP, Ro60
    16cSLE7FHispanic71.1DFS 1/2560Sm, U1RNP, Ro60, Ro52, weak SS-B
    17cSLE12FWhite194.0DFS 1/2560Negative
    18cSLE14MWhite312.3DFS 1/2560Negative
    19cSLE5FAsian140.7DFS 1/1280Negative
    20cSLE18FAsian232.1DFS 1/320U1RNP
    21cSLE17FUnknown> 450.8DFS 1/5120Low dsDNA
    22cSLE16MUnknown209.6DFS 1/1280Negative
    23cSLE16FWhite22.1Cytoplasmic 1/160Negative
    24cSLE16FAsian26.7Homo, NLR 1/1280Low U1RNP
    25cSLE15FAsian35.6DFS 1/640Low U1RNP
    26LS8MWhite120.5DFS 1/640Negative
    27LS14FWhite60.1MND, NLR, CS 1/1280Negative
    28LS15MMixed35.9DFS 1/320Negative
    29LS12FMixed57.2DFS, NLR 1/1280Negative
    30cdSSc18FWhite67.8DFS, CENP 1/1280CENP-B
    31JDM6FAsian408.3NegativeNegative
    32CD12FWhite51.4DFS 1/1280Anti-tTG–positive
    33CD14MWhite67.1DFS 1/1280Anti-tTG–negative
    34qCTD6MWhite115.8DFS 1/2560Negative
    35qCTD7FWhite72.8DFS 1/5120Negative
    36qCTD13FWhite286.3DFS 1/5120Anti-dsDNA
    37qCTD7FWhite79.4DFS 1/5120Negative
    38qCTD14FWhite326.9DFS 1/5120Negative
    39qCTD5MWhite87.6DFS 1/640Negative
    40qCTD10MWhite23.5DFS 1/160Negative
    41qCTD7FWhite80.3DFS 1/640Negative
    42qCTD9FWhite32.3DFS 1/1280NOR90
    43Healthy9MWhite21.7DFSNT
    44Healthy7FAsian59.1MNDNT
    45Healthy14FWhite26.1DFSNegative
    • ↵* Patients 11 and 14 are identical twins. DFS70: dense fine speckled 70 kDa antigen; CIA: chemiluminescence immunoassay; CU: chemiluminescence units; ANA: antinuclear antibody; IIF: indirect immunofluorescence; ENA: extractable nuclear antigens; LIA: line immunoassay; JIA: juvenile idiopathic arthritis; cSLE: childhood systemic lupus erythematosus; LS: localized scleroderma (morphea, linear scleroderma); cdSSc: childhood diffuse cutaneous systemic sclerosis; JDM: juvenile dermatomyositis; CD: celiac disease; qCTD: query connective tissue disease, children being investigated for an AARD; AARD: ANA-associated rheumatic disease; NT: not tested; MND: multiple nuclear dots; NLR: nucleolar; CS: centrosome; CENP: centromere protein; tTG: tissue transglutaminase.

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The Journal of Rheumatology
Vol. 42, Issue 12
1 Dec 2015
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Autoantibodies to Dense Fine Speckles in Pediatric Diseases and Controls
Heinrike Schmeling, Michael Mahler, Deborah M. Levy, Katharine Moore, Anne M. Stevens, James Wick, Jacob D. McMillan, Gerd Horneff, Shervin Assassi, Julio Charles, Gloria Salazar, Maureen D. Mayes, Earl D. Silverman, Marissa Klien-Gitelman, Tzelan Lee, Hermine I. Brunner, Ann M. Reed, Marvin J. Fritzler
The Journal of Rheumatology Dec 2015, 42 (12) 2419-2426; DOI: 10.3899/jrheum.150567

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Autoantibodies to Dense Fine Speckles in Pediatric Diseases and Controls
Heinrike Schmeling, Michael Mahler, Deborah M. Levy, Katharine Moore, Anne M. Stevens, James Wick, Jacob D. McMillan, Gerd Horneff, Shervin Assassi, Julio Charles, Gloria Salazar, Maureen D. Mayes, Earl D. Silverman, Marissa Klien-Gitelman, Tzelan Lee, Hermine I. Brunner, Ann M. Reed, Marvin J. Fritzler
The Journal of Rheumatology Dec 2015, 42 (12) 2419-2426; DOI: 10.3899/jrheum.150567
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Keywords

AUTOANTIBODIES
ANTINUCLEAR ANTIBODIES
DENSE FINE SPECKLES
AUTOIMMUNE DISEASES
PEDIATRICS

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Keywords

  • autoantibodies
  • ANTINUCLEAR ANTIBODIES
  • DENSE FINE SPECKLES
  • autoimmune diseases
  • pediatrics

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