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Background <p>Anti-MDA5 positive dermatomyositis is a&#xa0;rare subtype of idiopathic inflammatory myopathies often accompanied by rapidly progressive interstitial lung disease and severe cutaneous ulcerations. It is associated with a&#xa0;high mortality. The simultaneous occurrence of sarcoidosis with multisystemic involvement of myositis has only been described very rarely in the literature to date.</p> Case report <p>We report the case of a&#xa0;39-year-old man with a&#xa0;3-year history of anti-MDA‑5 positive dermatomyositis who developed a&#xa0;worsening of dyspnea and hypercalcemia with acute kidney injury and a&#xa0;marked deterioration in his general condition after successive reductions in immunosuppressive therapy. A&#xa0;bronchoscopy and lymph node biopsy ultimately confirmed the diagnosis of sarcoidosis.</p> Conclusion <p>This case report illustrates the importance of continuously re-evaluating the diagnosis when new symptoms occur during the course of a&#xa0;rare disease. In the presence of pronounced lymphadenopathy, new hypercalcemia and lung parenchymal changes atypical for dermatomyositis, the differential diagnosis of sarcoidosis should also be considered.</p>

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Sequenzielle Autoimmunität? Der seltene Fall einer MDA5-Dermatomyositis und Sarkoidose

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年2月27日

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  • Phillip Kremer,
  • Nikolas Ruffer,
  • Tim Oqueka,
  • Stefan Steurer,
  • Daniel Köhler,
  • Ina Kötter,
  • Martin Krusche

摘要

Background

Anti-MDA5 positive dermatomyositis is a rare subtype of idiopathic inflammatory myopathies often accompanied by rapidly progressive interstitial lung disease and severe cutaneous ulcerations. It is associated with a high mortality. The simultaneous occurrence of sarcoidosis with multisystemic involvement of myositis has only been described very rarely in the literature to date.

Case report

We report the case of a 39-year-old man with a 3-year history of anti-MDA‑5 positive dermatomyositis who developed a worsening of dyspnea and hypercalcemia with acute kidney injury and a marked deterioration in his general condition after successive reductions in immunosuppressive therapy. A bronchoscopy and lymph node biopsy ultimately confirmed the diagnosis of sarcoidosis.

Conclusion

This case report illustrates the importance of continuously re-evaluating the diagnosis when new symptoms occur during the course of a rare disease. In the presence of pronounced lymphadenopathy, new hypercalcemia and lung parenchymal changes atypical for dermatomyositis, the differential diagnosis of sarcoidosis should also be considered.