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Abstract Number: 791

Closing the Seronegative Gap in Pediatric Localized Scleroderma and Systemic Sclerosis

May Choi1, Emily Mirizio 2, Fernanda Quinteros 3, Katherine Buhler 4, Marvin Fritzler 1 and Kathryn Torok 5, 1Cumming School of Medicine, University of Calgary, Calgary, AB, Canada, 2University of Pittsburgh, Pittsburgh, 3University of Calgary, Calgary, 4University of Calgary, Calgary, Canada, 5UPMC Children's Hospital of Pittsburgh, Pittsburgh

Meeting: 2019 ACR/ARP Annual Meeting

Keywords: ANA, autoantibodies, Juvenile scleroderma, systemic sclerosis and localized scleroderma

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Session Information

Date: Sunday, November 10, 2019

Session Title: Pediatric Rheumatology – ePoster I: Basic Science, Biomarkers, & Sclerodermic Fever

Session Type: Poster Session (Sunday)

Session Time: 9:00AM-11:00AM

Background/Purpose: It has become increasingly recognized that extra-cutaneous manifestations, such as musculoskeletal and neurologic involvement, are common in pediatric patients with localized scleroderma (LS). We previously demonstrated that the presence of certain autoantibodies (Aab) is associated with deep tissue and internal organ involvement compared to pediatric healthy controls. In this study, a wider screening Aab array was used in a study to compare pediatric LS to pediatric systemic sclerosis (SSc).

Methods: A total of 133 pediatric patients (n=109 LS and 24 SSc) were included. Sera was tested for antinuclear antibodies (ANA) using indirect immunofluorescence (IIF) on HEp-2 cells (Inova Diagnostics, San Diego, CA) where a titre of equal to or greater than 1/80 was considered to be positive. Circular heat maps were generated to compare the frequency of ANA patterns in each group. The international consensus on ANA patterns (ICAP, www.anapatterns.org) nomenclature was used to denote ANA IIF pattern nomenclature.

Results: A total of 59/109 (54%) LS patients and 16/24 (67%) SSc patients were ANA positive. LS had the greatest number of distinct ANA patterns (n=11) compared to SSc (n=7) (Figure 1). AC-4 (nuclear speckled) was the most common pattern (23/109, 21%) while AC-8/9/10 (nucleolar) was the least common in LS (2/109, 2%). The opposite was true for SSc where AC-8/9/10 (9/24, 38%) was the most common and AC-4 was uncommon (3/24, 13%). Other ANA IIF patterns such as AC-2 (dense fine speckles) (4/109, 4%) and AC-24 (centrosome) (8/109, 7%) were seen in LS, but was absent or rare in SSc.

Conclusion: Pediatric SSc had higher frequency of ANA positivity compared to pediatric LS, as clinically expected, but not by a high margin. Interestingly, patients with LS had more heterogeneous expression of ANA patterns, including patterns that are rarely seen in SSc such as AC-24 (centrosome). Furthermore, since the centrosome is in the cytoplasm, the centrosome pattern would not be reported by laboratories that only report nuclear IIF patterns as a positive ANA. Therefore, an ANA test that includes nuclear, cytoplasmic, and mitotic patterns should be considered as the screening test in patients with scleroderma, especially LS.

Figure 1. Circular heat maps for anti-nuclear antibody indirect immunofluorescence patterns in localized scleroderma -A- and systemic sclerosis -B- pediatric patients using the international consensus on ANA patterns -ICAP- nomenclature -www.anapatterns.org-.


Disclosure: M. Choi, None; E. Mirizio, None; F. Quinteros, None; K. Buhler, None; M. Fritzler, Alexion Canada, 7, BioRad, 5, Dr. Fooke Laboratorien GmbH, 5, Euroimmun GmbH, 5, 7, ImmunoConcepts, 7, Inova Diagnostics, 5, 7, 8, Inova Diagnostics Inc. San diego, CA, 5, Inova Dx, Mikrogen GmbH, 5, Werfen International, 5; K. Torok, None.

To cite this abstract in AMA style:

Choi M, Mirizio E, Quinteros F, Buhler K, Fritzler M, Torok K. Closing the Seronegative Gap in Pediatric Localized Scleroderma and Systemic Sclerosis [abstract]. Arthritis Rheumatol. 2019; 71 (suppl 10). https://acrabstracts.org/abstract/closing-the-seronegative-gap-in-pediatric-localized-scleroderma-and-systemic-sclerosis/. Accessed May 27, 2023.
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