Sporadic inclusion body myositis—diagnosis, pathogenesis and therapeutic strategies
@article{Dalakas2006SporadicIB, title={Sporadic inclusion body myositis—diagnosis, pathogenesis and therapeutic strategies}, author={Marinos C. Dalakas}, journal={Nature Clinical Practice Neurology}, year={2006}, volume={2}, pages={437-447} }
Sporadic inclusion body myositis (sIBM) presents with a characteristic clinical phenotype of slow-onset weakness and atrophy, affecting proximal and distal limb muscles and facial and pharyngeal muscles. Histologically, sIBM is characterized by chronic myopathic features, lymphocytic infiltrates invading non-vacuolated fibers, vacuolar degeneration, and accumulation of amyloid-related proteins. The cause of sIBM is unclear, but two processes—one autoimmune and the other degenerative—appear to…
238 Citations
Clinical, Histological, and Immunohistochemical Findings in Inclusion Body Myositis
- MedicineBioMed research international
- 2018
Clinical and muscle histopathological findings, including immunostaining for LC3B, p62, α-synuclein, and TDP-43, in 18 patients withporadic inclusion body myositis were presented, and the use of degeneration and autophagic markers can be useful for the diagnosis.
Review Sporadic inclusion body myositis: a continuing puzzle
- Biology
- 2008
It is proposed that the disproportionate involvement of certain muscles in sIBM may be due to the existence of muscle group-specific transcriptomes which are differentially affected by the disease process and that the male predominance of the disease may indicate the influence of genes preferentially expressed in males.
Myouclear Breakdown in Sporadic Inclusion Body Myositis
- Biology
- 2011
Figures that show abnormal localization of nuclear proteins associated with MAP kinase in s-IBM muscle fibers suggest that inhibition of nuclear transport during myogenesis is suggested and the theory that nuclear damage is closely associated with its etiology is supported.
Idiopathic Inflammatory Myopathies: A Review of Immunopathological Features and Current Models of Pathogenesis
- Medicine, Biology
- 2011
The idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of chronic systemic disorders characterized by muscle inflammation and progressive muscle weakness, and a growing body of evidence suggests that genetically susceptible individuals probably develop an idiopathy in response to particular environmental stimuli.
Mitochondrial Biology in Sporadic Inclusion Body Myositis
- Biology, Medicine
- 2014
The role of the mitochondria in the development of sIBM and the role of amyloid beta on mitochondrial function in skeletal muscle are evaluated to help to identify novel prevention and/ or treatment strategies.
Inclusion body myositis: new insights into pathogenesis
- Biology, MedicineCurrent opinion in rheumatology
- 2008
Understanding the paradoxes in sporadic inclusion body myositis is important in determining rational therapy for the disease.
Pathogenesis and therapies of immune-mediated myopathies.
- Biology, MedicineAutoimmunity reviews
- 2012
Sporadic inclusion body myositis: an unsolved mystery.
- Medicine, BiologyActa reumatologica portuguesa
- 2009
It remains uncertain whether sIBM is primarily an immune-mediated inflammatory myopathy or a degenerative myopathy with an associated inflammatory component.
References
SHOWING 1-10 OF 69 REFERENCES
Understanding the immunopathogenesis of inclusion-body myositis: present and future prospects.
- Biology, MedicineRevue neurologique
- 2002
The disease is mysteriously resistant to conventional immunotherapies in spite of the immunopathologic similarities with PM, and the cause of the vacuolar formation in IBM is also unknown and the role, that the tiny amyloid deposits play in the disease remain unclear.
Inflammatory, immune, and viral aspects of inclusion-body myositis
- Biology, MedicineNeurology
- 2006
Muscle biopsies from patients with sporadic inclusion-body myositis (sIBM) consistently demonstrate that the inflammatory T cells almost invariably invade intact (not vacuolated) fibers, whereas the…
Inclusion body myositis in HIV-1 and HTLV-1 infected patients.
- Medicine, BiologyBrain : a journal of neurology
- 1996
It is concluded that IBM occurs in HIV-1 and HTLV-1 infected individuals and has a clinical, histological and immunological pattern identical to sporadic IBM in the non-retrovirally infected patients.
Amyloid filaments in inclusion body myositis. Novel findings provide insight into nature of filaments.
- BiologyArchives of neurology
- 1991
It is demonstrated that the filaments in IBM share properties with amyloid proteins, which raises the likely possibility that the Filaments represent a modification of a normal protein within an acidic degradative vacuolar compartment.
An inflammatory, familial, inclusion body myositis with autoimmune features and a phenotype identical to sporadic inclusion body myositis. Studies in three families.
- Biology, MedicineBrain : a journal of neurology
- 1997
The view that s-IBM behaves like other autoimmune diseases and has disease susceptibility linked to the DR3 allele is strengthened, as the combination of the clinical, histological, immunopathological and immunogenetic features indicate that these patients have a disease identical to sporadic inclusion body myositis.
Inclusion body myositis. Observations in 40 patients.
- MedicineBrain : a journal of neurology
- 1989
The findings support the notion that IBM is a distinct entity in which a set of pathological features is associated with a constellation of clinical findings, and Prednisone treatment at dose levels frequently effective in polymyositis failed to prevent disease progression in those patients observed for 2 or more years.
Molecular immunology and genetics of inflammatory muscle diseases.
- Biology, MedicineArchives of neurology
- 1998
In myositis, several of the identified molecules and adhesion receptors play a role in the process of inflammation, fibrosis, and muscle fiber loss, and could be targets for the design of semispecific therapeutic interventions.
Proposed pathogenetic cascade of inclusion-body myositis: importance of amyloid-&bgr;, misfolded proteins, predisposing genes, and aging
- BiologyCurrent opinion in rheumatology
- 2003
This article summarizes the most recent findings leading to better understanding of the players in the pathogenetic cascade and suggests that lymphocytic inflammatory component is probably secondary, and it may contribute only slightly to muscle fiber damage in sporadic inclusion-body myositis.
Myofibrillar myopathy: clinical, morphological and genetic studies in 63 patients.
- Medicine, BiologyBrain : a journal of neurology
- 2004
MFM is morphologically distinct but genetically heterogeneous, and advances in defining the molecular causes of MFM will probably come from linkage studies of informative kinships or from systematic search for mutations in proteins participating in the intricate network supporting the Z-disk.
Inclusion Body Myositis: Clonal Expansions of Muscle‐Infiltrating T Cells Persist Over Time
- Biology, MedicineScandinavian journal of immunology
- 2003
Results show that in IBM there is clonal restriction of TCR expression in muscle‐infiltrating lymphocytes, and this indicates an important, continuous, antigen‐driven inflammatory reaction in IBM.