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Table of Contents    
COMMENTARY
Year : 2021  |  Volume : 69  |  Issue : 3  |  Page : 642

Sporadic Inclusion Body Myositis (Sibm): Challenges in Diagnosis and Treatment


Department of Neurology, AIIMS, Delhi, India

Date of Submission20-Mar-2021
Date of Decision28-Apr-2021
Date of Acceptance04-May-2021
Date of Web Publication24-Jun-2021

Correspondence Address:
Dr. Deepti Vibha
Department of Neurology, AIIMS, Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.319211

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How to cite this article:
Vibha D. Sporadic Inclusion Body Myositis (Sibm): Challenges in Diagnosis and Treatment. Neurol India 2021;69:642

How to cite this URL:
Vibha D. Sporadic Inclusion Body Myositis (Sibm): Challenges in Diagnosis and Treatment. Neurol India [serial online] 2021 [cited 2021 Jul 25];69:642. Available from: https://www.neurologyindia.com/text.asp?2021/69/3/642/319211




The case series by Murthy et al. on sIBM collected over five years, is, therefore, an important step towards defining the clinic-pathological characteristics.[1] Sporadic Inclusion Body Myositis (sIBM) is characterized by the slow and progressive asymmetric weakness and atrophy of proximal and distal muscles, preferentially involving finger flexor and quadriceps muscle groups. A combination of unique clinical, pathological and lack of treatment responsiveness makes it an enigmatic disease. First described in 1978 as case series of six cases[2], it has undergone several revisions in its classification and diagnostic criteria since then. The most recent classification is the European Neuromuscular Center (ENMC) IBM research diagnostic criteria 2011, which divides it into clinic-pathological IBM, clinical IBM and probable IBM.[3] Duration of more than 12 months, age of more than 45 years, weakness of knee extensors and finger flexors in combination with a creatine kinase of >15 times the upper limit of normal constitutes the clinical criteria. Pathologically, the presence of endomysial inflammatory infiltrates, up-regulation of MHC class I T-cells, rimmed vacuoles, protein accumulation (amyloid or other protein accumulation) or 15–18 nm filaments characterize sIBM. Hence, the presence of a clinical neurology and pathology team with expertise as well as infrastructure is required to diagnose and manage this challenging diagnosis.

To add to the confusion, the term, hereditary inclusion body myopathies (hIBMs) (disorders due to mutations in GNE, VCP or MYH2) are also sometimes abbreviated as IBM, despite not sharing any clinical or pathological features.[4] They neither resemble in a pattern of muscle weakness and do not have muscle immune cell infiltration on histology.

The prevalence studies of sIBM are very few and in limited parts of the world with an estimate of 45.6/1,000,000 (95% CI: 35.9–55.2).[5] The prevalence is not known in the Indian population. The reasons are challenges in the ascertainment of the diagnosis in resource poor settings as well as the evolving diagnostic criteria. The treatments which are FDA-approved so far are intravenous immunoglobulin, with and without prednisone, methotrexate, interferon beta and oxandrolone, bimagrumab and arimoclomol. All these treatments have shown minor or no clinically meaningful benefit in clinical trials.

Although most of the cases were classified as probable, this was mainly due to the inability to demonstrate amyloid in the vacuoles. All patients were unresponsive to steroids, and a systematic follow up of disability progression and outcome over time may have added to the knowledge of the behaviour of the rare disease. The study highlights the advantages of a well-defined classification system for this rare inflammatory myositis. It provides a repository of clinic-pathological information, which is lacking from this part of the world.



 
  References Top

1.
Challa S, Jakati S, Narla S, Uppin MS, Kannan MA, Jagarlapudi MM. Sporadic Inclusion Body Myositis: A Clinicopathological Study. Neurol India 2021;69:638-41.  Back to cited text no. 1
  [Full text]  
2.
Carpenter S, Karpati G, Heller I, Eisen A. Inclusion body myositis: a distinct variety of idiopathic inflammatory myopathy. Neurology 1978;28:8-17.  Back to cited text no. 2
    
3.
Rose MR. 188th ENMC International Workshop: Inclusion Body Myositis, 2–4 December 2011, Naarden, The Netherlands. Neuromuscul Disord 2013;12.  Back to cited text no. 3
    
4.
Greenberg SA. Inclusion body myositis: clinical features and pathogenesis. Nat Rev Rheumatol 2019;15:257-72.  Back to cited text no. 4
    
5.
Callan A, Capkun G, Vasanthaprasad V, Freitas R, Needham M. A Systematic Review and Meta-Analysis of Prevalence Studies of Sporadic Inclusion Body Myositis. J Neuromuscul Dis 2017;4:127-37.  Back to cited text no. 5
    




 

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