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Distal muscle involvement in granulomatous myositis can mimic inclusion body myositis
  1. Sandrine Larue1,
  2. Thierry Maisonobe2,3,
  3. Olivier Benveniste1,4,
  4. Catherine Chapelon-Abric5,
  5. Olivier Lidove6,
  6. Thomas Papo6,
  7. Bruno Eymard1,
  8. Odile Dubourg1,2
  1. 1Centre de Référence des Maladies Neuromusculaires ‘Paris-Est,’ APHP, CHU Pitié-Salpêtrière, Bd de l'hôpital, Paris, France
  2. 2Laboratoire de Neuropathologie, CHU Pitié-Salpêtrière, Bd de l'hôpital, Paris, France
  3. 3Fédération de Neurophysiologie clinique, CHU Pitié-Salpêtrière, Bd de l'hôpital, Paris, France
  4. 4Service de Médecine Interne 1, CHU Pitié-Salpêtrière, Bd de l'hôpital, Paris, France
  5. 5Service de Médecine Interne 2, CHU Pitié-Salpêtrière, Bd de l'hôpital, Paris, France
  6. 6Service de Médecine Interne, Groupe Hospitalier Bichat–Claude-Bernard, 46, rue Henri-Huchard, Paris, France
  1. Correspondence to Dr O Dubourg, Laboratoire de neuropathologie, Hôpital Pitié-Salpêtrière, 47 Bd de l'hôpital, Cedex 13, 75651 Paris; odile.dubourg{at}


The authors report on four patients aged over 50 with chronic myopathy suggestive of sporadic inclusion body myositis. They present progressive and selective weakness of the quadriceps femoris muscles. Asymmetrical and selective atrophy of the forearm muscles were noted in all, with more severe involvement of the flexors than the extensors. Biopsy revealed granulomatous myositis. Histological features of sporadic inclusion body myositis were lacking. Evidence for systemic sarcoidosis was found in one patient. Corticosteroid treatment was associated with a partial but significant improvement in two patients. Granulomatous myositis may mimic inclusion body myositis and may be steroid-responsive.

  • Inflammatory myopathy
  • inclusion body myositis
  • granulomatous myositis
  • sarcoidosis
  • incl body myositis
  • muscle disease
  • neuropathology
  • muscle

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  • Funding This work was supported by the Association Française contre les Myopathies (AFM), the Assistance Publique des Hôpitaux de Paris (APHP) and the Université Pierre et Marie Curie.

  • Competing interests None.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.