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Sacroiliitis at diagnosis as a protective predictor against disease flare after stopping medication: outcomes of a Southeast Asian enthesitis-related arthritis (ERA) longitudinal cohort

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Abstract

Objectives

To assess short- and long-term outcomes of ERA in a large monocentric cohort in Singapore.

Methods

Children diagnosed with ERA according to ILAR criteria from 2002 to 2021 were recruited. Nonparametric statistics were used to describe the data. Outcomes were defined according to modified Wallace criteria, and probabilities and predictors were determined using Kaplan–Meier survival and logistic regression analyses.

Results

One hundred fifty-one ERA patients (male 86%; Chinese 81%) were included. The median age at onset was 11.9 years (IQR: 9.4–13.9), and disease duration was 5.3 years (IQR: 2.9–8.4). At diagnosis, 39% of the patients had sacroiliitis. HLA-B27 was positive in 83%, and biologics were used in 72% of the patients. Clinical inactive disease (CID) was achieved in 92% of the patients, of which 27% achieved within 6 months. Sacroiliitis at diagnosis is an unfavorable predictor of early CID at 6 months. Medication was discontinued in one-third of the patients. Favorable predictor of medication withdrawal includes male gender, while unfavorable predictors include positive HLA-B27 and ANA. Two-thirds of the patients with CID had at least one disease flare. Sacroiliitis at diagnosis is a protective predictor of flare after stopping medication.

Conclusion

Despite a high proportion of ERA patients achieving CID, only one-third could stop medication with high rates of disease flare. Unfavorable predictors include older age at onset, HLA-B27, and ANA positivity. While sacroiliitis at diagnosis is a negative predictor of CID at 6 months, it is associated with less disease flare after discontinuing medication.

Key Points

• Majority of the ERA patients achieved clinical inactive disease with treatment.

• Only one-third of the patients could discontinue all medications with high rates of disease flare after.

• Favorable outcome predictors include male gender and sacroiliitis at diagnosis, while unfavorable predictors include positive HLA-B27 and ANA.

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Data availability

The data presented in this study are available on request from the corresponding author. The data are not publicly available due to ethical reasons.

Code availability

Not applicable.

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Acknowledgements

We thank our ERA children and families for participating in the inception cohort and registry. Their physical and mental support to the rheumatology team and ERA families in need of help throughout the past decade is much appreciated.

Funding

This work was supported by grants from the National Arthritis Foundation of Singapore and the International League of Associations for Rheumatology (ILAR 2011).

Author information

Authors and Affiliations

  1. Rheumatology and Immunology Service, Department of Paediatric Subspecialties, KK Women’s and Children’s Hospital, Singapore, Singapore

    Kai Liang Teh, Lena Das, Yun Xin Book & Thaschawee Arkachaisri

  2. Division of Nursing, KK Women’s and Children’s Hospital, Singapore, Singapore

    Sook Fun Hoh & Xiaocong Gao

  3. Duke-NUS Medical School, Singapore, Singapore

    Thaschawee Arkachaisri

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  1. Kai Liang Teh
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Contributions

All authors contributed to the study’s conception and design. KLT, LD, YXB, SFH, XG, and TA performed data collection and interpretation. Data analysis was done by KLT, YXB, and TA. KLT wrote the first draft of the manuscript, and all authors commented on previous versions of the manuscript. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Thaschawee Arkachaisri.

Ethics declarations

Ethics approval

The SingHealth Centralised Institutional Review Board (CIRB) approved this study and waived the need for informed consent for this database study (CIRB 2019/2274).

Conflict of interest

All authors declare no conflicts of interest. The funders had no role in the study’s design, in the collection, analyses, or interpretation of data, in the writing of the manuscript, or in the decision to publish the results.

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Teh, K.L., Das, L., Book, Y.X. et al. Sacroiliitis at diagnosis as a protective predictor against disease flare after stopping medication: outcomes of a Southeast Asian enthesitis-related arthritis (ERA) longitudinal cohort. Clin Rheumatol 41, 3027–3034 (2022). https://doi.org/10.1007/s10067-022-06275-z

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