Macroglossia in rapidly progressive inclusion body myositis

Inclusion body myositis (IBM) is a refractory muscle disease characterized by inflammatory and degenerative features in myofibers. Macroglossia is common in systemic amyloid light chain amyloidosis; however, no reports have been published on patients with IBM. We encountered a female patient with cl...

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Bibliographic Details
Published in:Neuropathology 2023-06, Vol.43 (3), p.252-256
Main Authors: Yamasaki, Yoshimune, Mukaino, Akihiro, Yamashita, Satoshi, Takeuchi, Yousuke, Tawara, Nozomu, Yoshida, Ryoji, Honda, Yumi, Yamashita, Taro, Kakimoto, Asako, Ueyama, Hidetsugu, Ando, Yukio
Format: Article
Language:English
Subjects:
Amyloid
Amyloidosis
Biopsy
Dysphagia
Female
Fibrosis
Gait
Humans
Immunotherapy
Inclusion bodies
inclusion body myositis
Inflammatory diseases
Leukocytes (mononuclear)
Macroglossia
Movement disorders
Musculoskeletal diseases
Myocardium - pathology
Myositis
Myositis, Inclusion Body - complications
Myositis, Inclusion Body - diagnosis
Myositis, Inclusion Body - pathology
Oral administration
Parotid gland
Prednisolone
rapid progression
Sjogren's syndrome
Sjögren's syndrome
Vacuoles
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Description
Summary:Inclusion body myositis (IBM) is a refractory muscle disease characterized by inflammatory and degenerative features in myofibers. Macroglossia is common in systemic amyloid light chain amyloidosis; however, no reports have been published on patients with IBM. We encountered a female patient with clinicopathologically defined IBM who exhibited relatively rapid progression of dysphagia, gait disturbance, and macroglossia. Muscle biopsy demonstrated endomysial mononuclear inflammatory infiltrates, fiber necrosis and regeneration with rimmed vacuoles, and sarcoplasmic inclusions of p62. Tongue biopsy demonstrated fiber degeneration with fatty replacement and fibrosis, nonnecrotic fibers surrounded and invaded by mononuclear cells, and sarcoplasmic dotlike inclusions of p62. Based on the parotid gland, lip, and muscle biopsy, she was diagnosed as having IBM with Sjögren's syndrome. She was treated with steroid pulse and intravenous immunoglobulin therapy followed by oral administration of prednisolone, which resulted in temporary clinical improvement. Macroglossia might be an indicator of immunotherapy effectiveness.
ISSN:0919-6544
1440-1789
DOI:10.1111/neup.12879