Polymyositis and the Spectrum of Scleroderma Disorders

  • Joana Cochicho Coimbra Hospital and Universitary Centre
  • João Madaleno Coimbra Hospital and Universitary Centre
  • Emília Louro Coimbra Hospital and Universitary Centre
  • Adélia Simão Coimbra Hospital and Universitary Centre
  • Armando Carvalho Coimbra Hospital and Universitary Centre

Keywords

Polymyositis, Systemic sclerosis sine scleroderma, Overlap syndrome

Abstract

Polymyositis (PM) is usually associated to other autoimmune or connective tissue diseases. The authors report the case of a 59-year-old man with pulmonary fibrosis, who presented with constitutional symptoms and gradually developed proximal muscle weakness, Raynaud phenomenon, and dysphagia. Besides creatine kinase (CK) elevation, he had positive anti-Polymyositis-Scleromyositis (PM-Scl) and anti-Sjögren's-syndrome A (SSA) antibodies. Nailfold capillaroscopy showed a scleroderma pattern and muscle biopsy revealed necrosis, regeneration of muscle fibers, and inflammatory infiltrate. Prednisolone was started, with great improvement. Taking into account the overlap features between PM and systemic sclerosis sine scleroderma, it is important to closely monitor the patient for signs of pulmonary and cardiac decompensation.

References

  • Dalakas MC, Hohlfeld R. Polymyositis and dermatomyositis. Lancet 2003;362:972–982.

  • Jury E C, ’Cruz D D, Morrow W J W. Autoantibodies and overlap syndromes in autoimmune rheumatic disease. J Clin Pathol 2001;54:340–347.

  • do Rosário e Souza EJ, Kayser C. Nailfold capillaroscopy: relevance to the practice of rheumatology. Rev Bras Reumatol 2015;55:264–271.

  • Bhansing KJ, Lammens M, Knaapen HKA, van Riel PLCM, van Engelen BGM, Vonk MC. Scleroderma-polymyositis overlap syndrome versus idiopathic polymyositis and systemic sclerosis: a descriptive study on clinical features and myopathology. Arthritis Res Ther. 2014;16:R111 doi:10.1186/ar4562.
  • Published: 2015-12-22

    Issue: Vol 3 No 1 (view)

    Section: Articles

    How to cite:
    Cochicho, J., Madaleno, J., Louro, E., Simão, A., & Carvalho, A. (2015). Polymyositis and the Spectrum of Scleroderma Disorders. European Journal of Case Reports in Internal Medicine, 3(1). https://doi.org/https://doi.org/10.12890/2015_000346