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<p>Fibrous dysplasia/McCune–Albright (FD/MAS) syndrome is a rare bone disorder with a broad clinical manifestation. Pain is the most frequently reported complaint and can significantly impair quality of life. While most existing data are cross-sectional, little is known about symptom progression over time. This study aimed to assess changes in pain and QoL over 2&#xa0;years follow-up. Patients in the PROFID study completed yearly questionnaires aligned with check-ups or independently if check-ups were less frequent. At baseline, 143 (85.6%) reported pain, of which 38 (26.6%) mild, 105 (73.4%) moderate/severe pain, and 24 (14.4%) had no pain. Emotional wellbeing and energy/fatigue were the most affected in the SF-36 domains. Patients viewed their disease as chronic, with moderate daily fluctuation and impact showed that active and palliative coping were the most frequently used coping mechanism. After 2&#xa0;years, 27.4% of those with no or mild pain at baseline reported a significant increase in pain (1.3–5.0, <i>p</i> &lt; 0.001), while scores in the moderate/severe group remained stable (6.6–6.3, <i>p</i> = 0.5). Emotional wellbeing improved significantly (37.6–55.5, <i>p</i> &lt; 0.001). Patients with moderate/severe pain reported increased disease control, whereas those with no/mild pain felt less control (<i>p</i> = 0.01). Higher pain scores correlated with poorer physical (r = − 0.627), social (r = − 0.541), and general health (r = − 0.452), worse illness perceptions (e.g., illness identity r = 0.3), and greater palliative coping (r = 0.4), all <i>p</i> &lt; 0.05. These findings emphasize the need to address both physical and psychological aspects of FD/MAS.</p>

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The Natural Course of Pain in Fibrous Dysplasia/McCune Albright Syndrome: A Prospective Follow Up Study

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年12月1日

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  • O. O. Bulaicon,
  • M. Hagelstein-Rotman,
  • M. E. Meier,
  • I. van der Geest,
  • F. M. van Haalen,
  • J. Hogewoning-Rewijk,
  • S. W. van der Meeren,
  • S. E. C. Pichardo,
  • A. C. van de Ven,
  • P. B. de Witte,
  • Natasha M. Appelman-Dijkstra

摘要

Fibrous dysplasia/McCune–Albright (FD/MAS) syndrome is a rare bone disorder with a broad clinical manifestation. Pain is the most frequently reported complaint and can significantly impair quality of life. While most existing data are cross-sectional, little is known about symptom progression over time. This study aimed to assess changes in pain and QoL over 2 years follow-up. Patients in the PROFID study completed yearly questionnaires aligned with check-ups or independently if check-ups were less frequent. At baseline, 143 (85.6%) reported pain, of which 38 (26.6%) mild, 105 (73.4%) moderate/severe pain, and 24 (14.4%) had no pain. Emotional wellbeing and energy/fatigue were the most affected in the SF-36 domains. Patients viewed their disease as chronic, with moderate daily fluctuation and impact showed that active and palliative coping were the most frequently used coping mechanism. After 2 years, 27.4% of those with no or mild pain at baseline reported a significant increase in pain (1.3–5.0, p < 0.001), while scores in the moderate/severe group remained stable (6.6–6.3, p = 0.5). Emotional wellbeing improved significantly (37.6–55.5, p < 0.001). Patients with moderate/severe pain reported increased disease control, whereas those with no/mild pain felt less control (p = 0.01). Higher pain scores correlated with poorer physical (r = − 0.627), social (r = − 0.541), and general health (r = − 0.452), worse illness perceptions (e.g., illness identity r = 0.3), and greater palliative coping (r = 0.4), all p < 0.05. These findings emphasize the need to address both physical and psychological aspects of FD/MAS.