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Phenotypic characteristics and outcome of juvenile dermatomyositis in Arab children

  • Epidemiology of RMD
  • Published:
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Abstract

This study describes the disease characteristics and outcome of Arab children with juvenile dermatomyositis (JDM) and compares the findings with other ethnicities. We retrospectively reviewed the hospital registries of the participating hospitals for children with JDM seen between 1990 and 2016 in three Arab countries. All patients fulfilled Bohan and Peter criteria for JDM, diagnosed before 14 years of age and were of Arab ethnicity. Clinical and laboratory features as well as the long-term outcomes including accrual disease damage were collected at the last follow-up visit. A total of 92 JDM patients (58 girls) were included. Mean age at the onset was 6 ± 3 years, with a mean follow-up duration of 5 ± 4.4 years. Forty-three patients (46.7%) had polycyclic disease course, 34 (36.9%) had a monocyclic course, while 15 (16.3%) had a continuous progressive course. Forty-five patients (48.9%) had arthritis, 14 (15.2%) patients had an upper airway and dysphagia, and 10 patients (10.9%) had lung involvement. Eight patients (8.7%) were admitted to the intensive care unit (ICU), 4 of them required mechanical ventilation. Methotrexate had been the most frequently used immunosuppressive drug (86%) and rituximab was used in eight patients. Additionally, 31 patients received IVIG. Most of the patients achieved a complete clinical response, but 16 ended up with permanent skin changes and 12 had a residual muscle weakness. Twenty-seven patients developed calcinosis. There were two deaths due to infection during the follow-up period. We report the largest phenotypic data on Arab children with JDM. Our patients have similar characteristics to previously described cohorts. Majority of the patients remained with inactive disease.

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Authors and Affiliations

  1. Consultant and Section Head, Rheumatology, Department of Pediatrics, King Faisal Specialist Hospital and Research Center, Po Box 3354, Riyadh, 11211, Saudi Arabia

    Sulaiman M. Al-Mayouf, Nora AlMutiari & Abdullah Alsonbul

  2. King Abdulaziz University Hospital, Jeddah, Saudi Arabia

    Mohammed Muzaffer

  3. Maternity-Children Hospital, Jeddah, Saudi Arabia

    Rawiah shehata

  4. Queen Rania Children Hospital, Amman, Jordan

    Adel Al-Wahadneh, Mohammed Abu-shukair & Zeyad El-Habahbeh

  5. Sultan Qaboos University Hospital, Muscat, Oman

    Reem Abdwani

  6. Royal Hospital, Muscat, Oman

    Safia Al-Abrawi

  7. Pediatrics, Alfaisal University, Riyadh, Saudi Arabia

    Sulaiman M. Al-Mayouf

Authors
  1. Sulaiman M. Al-Mayouf
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  2. Nora AlMutiari
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  3. Mohammed Muzaffer
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  4. Rawiah shehata
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  5. Adel Al-Wahadneh
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  6. Reem Abdwani
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  7. Safia Al-Abrawi
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  8. Mohammed Abu-shukair
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  9. Zeyad El-Habahbeh
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  10. Abdullah Alsonbul
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Corresponding author

Correspondence to Sulaiman M. Al-Mayouf.

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Funding

This study was not funded by any agencies in the public, commercial, or not-for-profit sectors.

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed consent

Taking into consideration the nature of this retrospective work, informed consent was not required from patient or parent.

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Al-Mayouf, S.M., AlMutiari, N., Muzaffer, M. et al. Phenotypic characteristics and outcome of juvenile dermatomyositis in Arab children. Rheumatol Int 37, 1513–1517 (2017). https://doi.org/10.1007/s00296-017-3770-x

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  • DOI: https://doi.org/10.1007/s00296-017-3770-x

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