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Remission rates and risk factors for relapse in pediatric morphea: a multicenter retrospective study of Pediatric Rheumatology Academy (PeRA)-Research Group (RG)

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Abstract

Aim

Morphea, also known as localized scleroderma, is an immune-mediated disease and the most common form of scleroderma in children. It is a localized sclerosing disease of the skin, but can also involve such adjacent tissues as the fascia, muscle, bone, and underlying tissues. This multicenter study aimed to evaluate Turkish pediatric morphea patients, regarding demographics, treatments, and response to treatment.

Materials and methods

The study was performed by the Pediatric Rheumatology Academy and included pediatric morphea patients from 6 Turkish pediatric rheumatology centers who were followed up for ≥6 months. Demographic, clinical, and laboratory findings and treatment modalities were analyzed. The patients were divided into 3 groups according to treatment response, as follows: group 1: topical treatment response, group 2: methotrexate response, and group 3: methotrexate resistance. Clinical findings were compared between the 3 groups.

Results

The study included 76 patients, of which 53 (69.7%) were female. Mean age at diagnosis of morphea was 9.7 ± 4.3 years and mean duration of follow-up was 3.2 ± 2.9 years. Linear morphea was the most common form, accounting for 43.4% (n = 33) of the patients. Extracutaneous features were noted in 17 patients (22.4%) and anti-nuclear antibody positivity was noted in 32 (42.1%). In all, 14.4% of the patients received topical treatment only, whereas 86.6% received both topical and systemic treatment. The methotrexate response rate was 76.9% in the patients that received systemic immunosuppressive therapy. The overall relapse rate while under treatment was 19.7%.

Conclusion

In this study, most of the pediatric morphea patients responded well to methotrexate. Bilateral lesions were more common in the methotrexate-resistant group. Multiple involvement, and bilateral lesions, were more common in relapsed patients than in non-relapsed patients.

Key points

• Most of the pediatric morphea patients respond well to MTX.

• Multiple involvement, and bilateral involvement, were more common in relapsed patients than in non-relapsed patients.

• Presence of extracutaneous findings in patients increased relapse rate 5.7 times.

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Authors and Affiliations

  1. Department of Pediatric Rheumatology, Dr. Sami Ulus Gynecology Obstetrics and Child Health and Diseases Training and Research Hospital, 06080, Ankara, Turkey

    Esra Bağlan, Semanur Özdel & Mehmet Bülbül

  2. Department of Pediatric Rheumatology, Dokuz Eylül University Medical School, Izmir, Turkey

    Zehra Kızıldağ, Balahan Makay & Şevket Erbil Ünsal

  3. Department of Pediatric Rheumatology, Umraniye Research and Training Hospital, University of Health Science, Istanbul, Turkey

    Şengül Çağlayan, Mustafa Çakan & Betül Sözeri

  4. Department of Pediatric Rheumatology, Istanbul University Medical School, Istanbul, Turkey

    Figen Çakmak & Nuray Aktay Ayaz

  5. Medical Park Hospital, Gaziantep, Turkey

    Gülçin Otar Yener

  6. Department of Pediatric Rheumatology, Göztepe Research and Training Hospital, Istanbul, Turkey

    Kübra Öztürk

Authors
  1. Esra Bağlan
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  2. Zehra Kızıldağ
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  3. Şengül Çağlayan
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  9. Mustafa Çakan
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  11. Betül Sözeri
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  12. Şevket Erbil Ünsal
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  13. Mehmet Bülbül
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Contributions

E. B. and S. O. designed the study. E. B. drafted the initial manuscript and had full access to all the data in the study, conducted the data analyses, drafted the initial manuscript, and had full access to all the data in the study. B. M., S. C., F. C., G. O. Y., K. O., Z. K., and M. C. performed the data acquisition. M. B. contributed to writing the manuscript. S. O., N. A. A., S. E. U., and B. S. reviewed the manuscript.

Corresponding author

Correspondence to Esra Bağlan.

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Ethics approval

The study was approved by the responsible ethics review board (ethics approval number: 2022-05/07).

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Bağlan, E., Kızıldağ, Z., Çağlayan, Ş. et al. Remission rates and risk factors for relapse in pediatric morphea: a multicenter retrospective study of Pediatric Rheumatology Academy (PeRA)-Research Group (RG). Clin Rheumatol 42, 2855–2860 (2023). https://doi.org/10.1007/s10067-023-06677-7

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  • DOI: https://doi.org/10.1007/s10067-023-06677-7

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