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Systemic onset juvenile idiopathic arthritis: a retrospective study of 80 consecutive patients followed for 10 years
C Lomater1, V Gerloni, M Gattinara
1Institute of Rheumatology, Centre for Rheumatic Children, Department of Rheumatology, Milan, Italy.
Insights
Systemic onset juvenile idiopathic arthritis has varied clinical courses. Longer active disease periods correlate with poorer functional outcomes, emphasizing the need for early intervention to improve long-term disability in children.
Area of Science:
- Rheumatology
- Pediatric Rheumatology
- Immunology
Background:
- Systemic onset juvenile idiopathic arthritis (SOJIA) is a chronic autoimmune condition affecting children.
- Understanding disease course and functional outcomes is crucial for managing SOJIA.
- Steinbrocker functional class provides a standardized measure of disability in arthritis.
Purpose of the Study:
- To examine the relationship between disease activity, disease course, and functional class in SOJIA.
- To identify predictors of functional outcomes in children with systemic onset juvenile idiopathic arthritis.
Main Methods:
- Retrospective review of 80 pediatric patients with SOJIA and a minimum 3-year follow-up.
- Calculation of cumulative duration of active periods (CDAP) in months for each patient.
- Evaluation of functional class (Steinbrocker) and disease activity at the last follow-up.
Main Results:
- Three disease course patterns identified: monocyclic (I), intermittent (II), and persistent (III).
- Monocyclic cases showed remission with good functional class; persistent cases had higher disability.
- A significant linear regression demonstrated that functional class (y) depends on CDAP (x): y = 0.0083x + 1.266 (r=0.586, p<0.0001).
Conclusions:
- SOJIA exhibits diverse clinical trajectories, impacting long-term functional status.
- Monocyclic disease courses generally lead to favorable functional outcomes.
- The cumulative duration of active disease significantly predicts the severity of disability in SOJIA.
Objective:
To investigate the relationships between systemic onset juvenile idiopathic arthritis disease activity, course of the disease, and functional class according to Steinbrocker.
Methods:
The records of all children with systemic onset juvenile arthritis (JA) according to the American College of Rheumatology criteria attending our center since 1971 with a minimum followup period of 3 years were reviewed. A cohort of 80 consecutive patients entered the study: 42 males, 38 females, mean age at onset 6.3 years (range 0.7-16), mean followup period 10.7 years (range 3-33). The cumulative duration of the active periods (CDAP) in months was calculated for every patient.
Results:
Three patterns of disease course were apparent: monocyclic (subtype I), intermittent (subtype II), and persistent (subtype III). At the last control the functional class and disease activity status were evaluated. In all subtype I patients (9 cases) the disease was in remission and no patient was in class II, III, or IV. In subtype II patients (27 cases), 16 were inactive or in remission and 6 in class III. In subtype III (44 cases) 21 were inactive or in remission and 17 were in class III or IV. The equation relating the Steinbrocker class to the CDAP was calculated considering the functional outcome as the dependent variable. The linear regression equation y = 0.0083 x + 1.266 was found with a correlation coefficient r = 0.586 (p < 0.0001). The majority of our patients were treated with disease modifying antirheumatic drugs, which in many cases were effective in reducing the duration of the active phases of disease.
Conclusion:
Systemic onset JA may present with different clinical courses; the functional outcome is always good in subtype I (monocyclic), but can be poor in subtypes II and III. The severity of disability evaluated according to Steinbrocker classes is dependent on the cumulative duration of the active periods of the disease.