Macroglossia in rapidly progressive inclusion body myositis.
Sjögren's syndrome
inclusion body myositis
macroglossia
rapid progression
Journal
Neuropathology : official journal of the Japanese Society of Neuropathology
ISSN: 1440-1789
Titre abrégé: Neuropathology
Pays: Australia
ID NLM: 9606526
Informations de publication
Date de publication:
Jun 2023
Jun 2023
Historique:
revised:
13
10
2022
received:
20
06
2022
accepted:
18
10
2022
medline:
5
6
2023
pubmed:
10
11
2022
entrez:
9
11
2022
Statut:
ppublish
Résumé
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.
Types de publication
Case Reports
Langues
eng
Sous-ensembles de citation
IM
Pagination
252-256Subventions
Organisme : Japan Society for the Promotion of Science
ID : Grant-in-Aid for Scientific Research (16K09674)
Organisme : Ministry of Health, Labour and Welfare
ID : Grant-in-Aid for Research on Intractable Diseases
Organisme : Kumamoto University
Informations de copyright
© 2022 Japanese Society of Neuropathology.
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