Correlation of muscle biopsy, clinical course, and outcome in PM and sporadic IBM

Objective: To correlate muscle biopsy findings with prebiopsy and postbiopsy clinical course and response to therapy in polymyositis (PM) and sporadic inclusion body myositis (IBM). Background: Existence of pure PM has recently been questioned; subsequently, the definition and criteria for diagnosing PM were debated. Methods: Patient records, follow-up information, and muscle biopsies were analyzed in 107 patients whose biopsies were initially read as PM and IBM. Results: The patients fell into three groups by combined biopsy and clinical criteria: PM, 27 patients; IBM, 64 patients; PM/IBM, 16 patients with biopsy diagnosis of PM but clinical features of IBM. For the three groups, the respective mean periods from disease onset to end of follow-up were 5.9, 8.5, and 9.6 years. Another autoimmune disease was present in 4 of 27 PM, 8 of 64 IBM, and 1 of 16 PM/IBM cases. An autoimmune serologic marker occurred in one-third of each group. Nineteen PM patients had no associated autoimmune disease or marker. Nonnecrotic fiber invasion by mononuclear cells appeared in all IBM, 17 of 27 PM, and 13 of 16 PM/IBM patients. The density of both invaded fibers and cytochrome-c oxidase–negative fibers was higher in IBM and PM/IBM than in PM. Immunotherapy improved 22 of 27 PM patients but had only transient beneficial effects in 2 of 32 IBM and 1 of 14 PM/IBM patients. Conclusions: 1) Sixteen of 43 patients (37%) with biopsy features of polymyositis (PM) had clinical features of inclusion body myositis (IBM). 2) Absence of canonical biopsy features of IBM from clinically affected muscles of IBM patients challenges biopsy criteria for IBM, or the IBM markers appear late in some patients, or their distribution in muscle is patchy and restricted compared with that of the inflammatory exudate. 3) The muscle biopsy is a reliable instrument in the diagnosis of PM and IBM in close to 85% of the patients. Errors of diagnosis in the remaining 15% can be avoided or reduced by combined evaluation of the clinical and pathologic findings.

[1]  R. Griggs,et al.  A prospective natural history study of inclusion body myositis , 2001, Neurology.

[2]  H Vogel.  Inclusion Body Myositis—A Review , 1998, Advances in anatomic pathology.

[3]  A. Eisen,et al.  Inclusion body myositis , 1978, Neurology.

[4]  W. Mair,et al.  Ultrastructural changes in polymyositis. , 1979, Brain : a journal of neurology.

[5]  T. Bieber,et al.  Association of inclusion body myositis with subacute cutaneous lupus erythematosus , 2001, Rheumatology International.

[6]  K. Moons,et al.  Rimmed vacuoles and the added value of SMI-31 staining in diagnosing sporadic inclusion body myositis , 2001, Neuromuscular Disorders.

[7]  Kucharz Ej.  [Inclusion body myositis]. , 1998, Polskie Archiwum Medycyny Wewnetrznej.

[8]  J. Fulthorpe,et al.  Inclusion body myositis: a case with associated collagen vascular disease responding to treatment. , 1985, Journal of neurology, neurosurgery, and psychiatry.

[9]  L. Rowland,et al.  Muscular dystrophies, polymyositis, and other myopathies. , 1958, Journal of chronic diseases.

[10]  F. Authier,et al.  Cytochrome c oxidase deficiencies in the muscle of patients with inflammatory myopathies , 1996, Acta Neuropathologica.

[11]  Khraishi Mm,et al.  Inclusion body myositis in association with vitamin B12 deficiency and Sjögren's syndrome. , 1992, The Journal of rheumatology.

[12]  A. Engel,et al.  Monoclonal antibody analysis of mononuclear cells in myopathies. II: Phenotypes of autoinvasive cells in polymyositis and inclusion body myositis , 1984, Annals of neurology.

[13]  P. Chambon,et al.  Targeted Somatic Mutagenesis in Mouse Epidermis , 2001, Hormone Research in Paediatrics.

[14]  G. Jansen,et al.  Absence of characteristic features in two patients with inclusion body myositis , 1998, Journal of neurology, neurosurgery, and psychiatry.

[15]  A. Engel,et al.  Monoclonal antibody analysis of mononuclear cells in myopathies. I: Quantitation of subsets according to diagnosis and sites of accumulation and demonstration and counts of muscle fibers invaded by T cells , 1984, Annals of neurology.

[16]  A. Oldfors,et al.  Inclusion body myositis: morphological clues to correct diagnosis , 2002, Neuromuscular Disorders.

[17]  J. Mendell,et al.  Amyloid filaments in inclusion body myositis. Novel findings provide insight into nature of filaments. , 1991, Archives of neurology.

[18]  Robert G. Cooper,et al.  Polymyositis and Dermatomyositis , 2022 .

[19]  G. Gronseth,et al.  Inclusion body myositis: Clinical and pathological boundaries , 1996, Annals of neurology.

[20]  A. Engel,et al.  Monoclonal antibody analysis of mononuclear cells in myopathies. III: Immunoelectron microscopy aspects of cell‐mediated muscle fiber injury , 1986, Annals of neurology.

[21]  W. V. van Venrooij,et al.  Polymyositis , 2003, Neurology.

[22]  H. Mitsumoto,et al.  Inclusion body myositis presenting as treatment-resistant polymyositis. , 1987, Arthritis and rheumatism.

[23]  P. Blumbergs,et al.  Inclusion body myositis in association with rheumatoid arthritis. , 1994, The Journal of rheumatology.

[24]  W J Litchy,et al.  Inclusion body myositis. Observations in 40 patients. , 1989, Brain : a journal of neurology.

[25]  J. Malley,et al.  Disease progression in sporadic inclusion body myositis: Observations in 78 patients , 2000, Neurology.

[26]  L. Gutmann,et al.  Inclusion body myositis and Sjogren's syndrome. , 1985, Archives of neurology.

[27]  M. Dalakas,et al.  Immune‐mediated conditions and antibodies associated with sporadic inclusion body myositis , 1998, Muscle & nerve.

[28]  L. M. Eaton.  The Perspective of Neurology in Regard to Polymyositis , 1954, Neurology.

[29]  R. Ike,et al.  Muscle biopsy , 1995, Current opinion in rheumatology.

[30]  B. V. van Engelen,et al.  Anti-Jo-1 positive inclusion body myositis with a marked and sustained clinical improvement after oral prednisone , 2001, Journal of neurology, neurosurgery, and psychiatry.

[31]  M. Dalakas,et al.  Inclusion body myositis and paraproteinemia: Incidence and immunopathologic correlations , 1997, Annals of neurology.

[32]  Richard J. Barohn,et al.  Inclusion body myositis , 2000, Current treatment options in neurology.

[33]  W. Engel,et al.  Use of anti‐neurofilament antibody to identify paired‐helical filaments in inclusion‐body myositis , 1996, Annals of neurology.

[34]  M. Dalakas,et al.  Magnetic resonance imaging of the forearm as a diagnostic aid in patients with sporadic inclusion body myositis , 1997, Neurology.

[35]  A. Lagueny,et al.  Association myosite à inclusions et sarcoïdose : à propos de trois cas , 2001 .

[36]  J. Verschuuren,et al.  Associations with autoimmune disorders and HLA class I and II antigens in inclusion body myositis , 2004, Neurology.

[37]  F L Mastaglia,et al.  Patterns of muscle involvement in inclusion body myositis: Clinical and magnetic resonance imaging study , 2001, Muscle & nerve.

[38]  Baziel G M van Engelen,et al.  Inclusion body myositis. Clinical features and clinical course of the disease in 64 patients. , 2005, Journal of neurology.

[39]  A. Pestronk,et al.  Polymyositis: An overdiagnosed entity , 2004, Neurology.

[40]  A. Bohan,et al.  Polymyositis and dermatomyositis (second of two parts). , 1975 .

[41]  J. Marler,et al.  Clinical Trials Recruiting Section , 2004, Neurology.

[42]  L. Gutmann,et al.  Inclusion body myositis and chronic immune thrombocytopenia. , 1984, Archives of neurology.

[43]  E. Holme,et al.  Mitochondrial DNA deletions in inclusion body myositis. , 1993, Brain : a journal of neurology.