Patients with antinuclear antibody–positive juvenile idiopathic arthritis constitute a homogeneous subgroup irrespective of the course of joint disease

Abstract

Objective. We recently hypothesized that in the International League of Associations for Rheumatology (ILAR) classification of juvenile idiopathic arthritis (JIA), the presumably homogeneous patient group characterized by early onset of disease, a female predilection, the presence of antinuclear antibodies (ANA), asymmetric arthritis, and the risk for iridocyclitis is classified into different categories. We sought to investigate whether ANA-positive patients belonging to the ILAR categories of oligoarthritis and rheumatoid factor (RF)-negative polyarthritis share homogeneous features and to compare these features with those of ANA-negative patients with JIA in the same categories.Methods. We identified patients who were followed up during a 15-year period. All patients had JIA according to the ILAR criteria, with oligoarticular or polyarticular onset. ANA positivity was defined as 2 or more positive results at a titer of >1:160. Demographic and clinical features, including the number of joints involved over time and measures of JIA severity at the last followup visit, were recorded retrospectively.Results. A total of 256 patients were included: 190 were ANA positive (109 had persistent oligoarthritis, 48 had extended oligoarthritis, and 33 had RF-negative polyarthritis), and 66 were ANA negative (35 had RFnegative polyarthritis, and 31 had oligoarthritis). All patients who were positive for ANA were similar in terms of age at disease presentation, female-to-male ratio, and frequency of symmetric arthritis and iridocyclitis. Compared with ANA-positive patients with polyarticular disease, ANA-negative patients with polyarticular arthritis were older at disease presentation and had a lower frequency of iridocyclitis, a higher frequency of symmetric arthritis, a greater cumulative number of joints affected over time, and a different pattern of joint disease, with a greater frequency of shoulder and hip involvement. The strong relationship between the presence of ANA and younger age at disease presentation, asymmetric arthritis, and development of iridocyclitis was confirmed by multivariate regression analysis.Conclusion. Our results support the hypothesis that patients with similar characteristics are currently classified into different JIA categories. The value of ANA positivity as a possible modifier of the current classification system deserves consideration.Juvenile idiopathic arthritis (JIA) is a heterogeneous condition that comprises all forms of arthritis of unknown etiology that begin before age 16 years and persist for Ͼ6 weeks. Over the years, several classification systems based on the clinical characteristics during the first 6 months of disease have been developed (1,2). The recent classification system proposed by the International League of Associations for Rheumatology (ILAR) (3) is intended to be a "work in progress" and is primarily aimed at identifying homogeneous disease groups for research purposes (4).We recently hypothesized that although some of the JIA categories in the ILAR classification ...

Epidemiology of juvenile idiopathic arthritis in a multiethnic cohort: Ethnicity as a risk factor

“…It has been suggested that patients with onset of ANA-positive arthritis at a younger age constitute a homogeneous disease subset. We therefore examined whether this was true across different ethnicities (27). We found that the RR for developing young-onset ANA-positive arthritis in children of European origin as compared with children of non-European origin was 4.21 (P Ͻ 0.0001) ( Table 4), and that patients in the young-onset ANApositive disease subset made up 31.6% of the patients of European origin but only 23.6% of the patients of non-European origin (P ϭ 0.03 by chi-square test).…”

“…Ravelli et al (27), in a study from Italy, suggested that patients with early-onset (diagnosis before age 6 years) ANA-positive, RF-negative JIA constitute a distinct disease subset, irrespective of the ILAR classification. When we examined patients with young-onset ANA-positive arthritis without regard to the number of involved joints or the presence of psoriasis, we found 1982 SAURENMANN ET AL that this group contained a significantly higher than expected proportion of patients of European ancestry compared with those of non-European ancestry, and that the TMA pediatric population of patients of European ancestry had a higher RR of developing this particular disease entity than did patients of nonEuropean ancestry.…”

A syndrome of immunodeficiency, autoimmunity, and spondylometaphyseal dysplasia

“…The positive ANA immunofluorescence, the presence of anti-ds-DNA with the presence of lupus nephritis suggested a diagnosis of SLE [Bizzaro and Villalta, 2001]. History of joint pain, clinical findings suggestive of arthritis, and presence of multiple anti-nuclear antibodies pointed towards JRA [Ravelli et al, 2005]. Presence of anti-platelet antibodies with mild thrombocytopenia was suggestive of autoimmune thrombocytopenia [Hamidpour et al, 2006].…”

Proteomic Profile of Tears for the Diagnosis of Uveitis Associated with Juvenile Idiopathic Arthritis: Setting Targets to Achieve Results

“…Данные о распространенности увеита, ассоции-рованного с ЮИА, весьма вариабельны: от 11,6 [5] до 30 % [66] всех детей с ЮИА, или у 30 % всех детей с ЮИА, имеющих антинуклеарные антитела [75].…”