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PubMed2005

[Inflammatory muscle diseases: dermatomyositis, polymyositis, and inclusion body myositis].

Dermatomyositis, polymyositis, inclusion body myositis and myositis overlap syndromes are systemic immune disorders of unknown origin with muscle weakness and elevated values of creatinkinase in the serum. Muscle biopsy is pivotal for a proper clinical diagnosis. Extramuscular findings at the skin, the joints or internal organs (lung, heart) are characteristic for the different clinical presentations of dermato- or polymyositis and are usually absent in inclusion body myositis. With the exception of inclusion body myositis myositis-associated autoantibodies are frequently present and associated with distinct clinical manifestations (e. g. antisynthetase syndrome). The rate of malignancy is elevated for several years after onset of myositis. Especially in polymyositis an appropriate differential diagnosis of infectious, endocrine, metabolic or neuromuscular causes of muscle disease is necessary. Glucocorticosteroids are the first choice of treatment in dermato- or polymyositis. Methotraxate, azathioprine, cyclophosphamamide, i.v. immunoglobulins and other drugs are used in diseases courses with continuous high dose requirement of corticosteroids.

Adrenal Cortex Hormones↗
PubMed1994

Abnormal accumulation of prion protein mRNA in muscle fibers of patients with sporadic inclusion-body myositis and hereditary inclusion-body myopathy.

Sporadic inclusion-body myositis is the most common progressive muscle disease of older patients. The muscle biopsy demonstrates mononuclear cell inflammation and vacuolated muscle fibers containing paired helical filaments and 6 to 10-nm fibrils, both resembling those of Alzheimer brain, and Congo-red positivity. Hereditary inclusion-body myopathy designates patients cytopathologically similar but without inflammation. In both muscle diseases, prion, and several proteins characteristic of Alzheimer brain--eg, beta-amyloid protein and hyperphosphorylated tau (which normally are expressed mainly in neurons), and apolipoprotein E--are abnormally accumulated in vacuolated muscle fibers, by unknown mechanisms. We now demonstrate in both muscle diseases that prion mRNA is strongly expressed in the vacuolated muscle fibers, which suggests that their accumulated prion protein results, at least partly, from increased gene expression. This, to our knowledge, is the first demonstration of abnormally increased prion mRNA in human disease. Another novel finding is the increased prion mRNA in human muscle macrophages, and both increased prion protein and prion mRNA in regenerating muscle fibers. The latter indicates that prion may play a role in human muscle development.

Adult↗
PubMed1997

An inflammatory, familial, inclusion body myositis with autoimmune features and a phenotype identical to sporadic inclusion body myositis. Studies in three families.

We describe the occurrence of an inflammatory inclusion body myositis in siblings of a single generation in three separate families. The disease in this total of seven patients was characterized by selective and early involvement of forearm and finger flexors, confirmed by MRI, and weakness of the quadriceps, triceps and foot extensors. Muscle biopsies in at least two members from each family showed endomysial inflammation, red-rimmed vacuoles, intracellular amyloid deposition and 15-18-nm tubulo-filaments within the vacuolated muscle fibres. Immunocytochemistry on serial muscle biopsy sections demonstrated an abundance of CD8+ cells invading non-necrotic, MHC-1-expressing muscle fibres. Immunogenetic studies showed the presence of the DR3 allele (DRB1*0301/0302) in all seven patients. The combination of the clinical, histological, immunopathological and immunogenetic features indicate that these patients have a disease identical to sporadic inclusion body myositis (s-IBM). We conclude that the classic, inflammatory, s-IBM can also occur in families (familial inclusion body myositis), in a pattern analogous to the familial occurrence of other autoimmune neurological diseases such as myasthenia gravis and multiple sclerosis. These observations strengthen the view that s-IBM behaves like other autoimmune diseases and has disease susceptibility linked to the DR3 allele.

Aged↗
PubMed1991

Inclusion body myositis and paramyxoviruses.

Inclusion body myositis (IBM) is a distinct type of muscle disease. The characteristic electron microscopic findings, intranuclear or intracytoplasmic inclusions composed of microtubular filaments, morphologically resemble paramyxovirus nucleocapsids. These findings and the reported immunoreactivity of the inclusions with mumps virus antibodies have suggested that inclusion body myositis is a chronic virus infection. We analyzed skeletal muscle specimens from three patients with characteristic light microscopic features and electron microscopically verified inclusions of IBM by immunocytochemistry using antibodies raised against members of the paramyxovirus group, and by in situ hybridization with a cRNA probe representing the mumps virus nucleocapsid gene. The specificity of the reactions was demonstrated with infected and uninfected cultured cells. No immunocytochemical staining or hybridization signal was observed in biopsy specimens from IBM patients. These findings speak against a paramyxovirus etiology of IBM.

Aged↗
PubMed2001

Presence of BACE1 and BACE2 in muscle fibres of patients with sporadic inclusion-body myositis.

Sporadic inclusion-body myositis (IBM) is the most common, progressive muscle disease of older individuals. We investigated the presence of BACE1 and BACE2-two beta secretases that cleave amyloid-beta-precursor protein-in muscle-biopsy samples from patients with IBM and from controls. On immunofluorescence, BACE1 and BACE2 co-localised with amyloid beta in IBM vacuolated muscle fibres, but were not found in controls. Immunoblotting showed increased BACE2 but not BACE1 in patients with IBM compared with controls. Our study suggests that both of these proteases might participate in processing of amyloid-beta-precursor protein in IBM muscle fibres.

Amyloid Precursor Protein Secretases↗
PubMed1998

Inclusion body myositis--a review.

Inclusion body myositis (IBM), a sporadic inflammatory myopathy, is the most frequently occurring progressive myopathy in adults older than 55 years. It more commonly affects men and usually is clinically and pathologically distinguishable from dermatomyositis or polymyositis. Muscle biopsy specimens will display inflammation in virtually all cases of sporadic IBM, along with rimmed vacuoles accompanied by the accumulation of beta-amyloid and other substances similar to those found in the degenerating neurons of Alzheimer's disease. The similarities between IBM and other inflammatory myopathies may contribute to its low level of diagnosis by pathologists. The proper recognition of IBM is important because, unlike other inflammatory myopathies, IBM is unresponsive to anti-inflammatory drugs.

Actin Cytoskeleton↗
PubMed1996

Inclusion body myositis--a case report.

Inclusion body myositis is a rare myopathy that clinically resembles a chronic polymyositis and histopathologically is characterized by the presence of rimmed vacuoles containing ultrastructural cytoplasmic degradation products with filamentous intranuclear and cytoplasmic inclusions. Since clinical features are not uniform, histopathologic and ultrastructural studies are necessary to confirm the diagnosis. We report a typical case of inclusion body myositis with histopathologic and ultrastructural study. The patient was a 31 year old male who presented with progressive weakness of both forearms, hands and lower extremities for 10 years.

Adult↗
PubMed1996

Inclusion body myositis: clinical and pathological boundaries.

Inclusion body myositis, polymyositis, and dermatomyositis are three distinct categories of inflammatory myopathy. Some authorities commented on the selective early weakness of the volar forearm muscles, quadriceps, and ankle dorsiflexors in inclusion body myositis. The most important feature distinguishing inclusion body myositis from the other two inflammatory myopathies is the lack of responsiveness to immunosuppressive treatment. Although most patients with inclusion body myositis have characteristic muscle biopsy findings, some cannot be distinguished histologically early from polymyositis. Predicting responsiveness to immunosuppressive medications, independent of muscle histology, would be valuable to clinicians. We retrospectively reviewed the pattern of weakness and other clinical features of 46 patients newly diagnosed with either inclusion body myositis, polymyositis, or dermatomyositis. Asymmetrical muscle weakness with prominent wrist flexor, finger flexor, and knee extensor involvement was specific for inclusion body myositis and unresponsive polymyositis. Male sex, lower creatine kinase levels, slower rate of progression, and peripheral neuropathy were also more common in inclusion body myositis and unresponsive polymyositis than in responsive polymyositis and dermatomyositis patients. Repeat muscle biopsy in 2 patients in the unresponsive polymyositis group demonstrated histological features of inclusion body myositis. We suspect that patients with clinical features of inclusion body myositis but lacking histological confirmation may nonetheless have inclusion body myositis. Our study supports the recently proposed criteria for definite and possible inclusion body myositis.

Aged↗
PubMed1989

[Clinical forms of inclusion body myositis: 12 cases].

Inclusion body myositis is now a well-known disease but its incidence is underestimated. We report 12 cases with clinical heterogeneity. Three groups of patients could be described. The first one corresponded to asymmetrical muscle involvement and distribution with a slow clinical course (4 cases). The second was characterized by a polymyositis-like syndrome (3 cas), but steroid therapy was ineffective. The last group mimicked a chronic spinal muscular atrophy (4 cases). One patient showed a scapuloperoneal syndrome. Both myopathic and neurogenic EMG patterns were present in 6 patients; a neurogenic pattern was found in 4 cases and a myopathic pattern in 2 cases. In all patients, muscle biopsies showed rimmed vacuoles with eosinophilic inclusions. In 9 cases ultrastructural studies displayed abnormal filaments of 15-18 nm in diameter in the vacuoles. Intranuclear filaments were rarely observed. The significance of the filaments is unknown and their specificity is doubtful because they are present in other myopathies with rimmed vacuoles (some distal myopathies and oculopharyngeal muscular dystrophies). Finally a rich inflammatory exudate was present in 8 patients only.

Adult↗
PubMed1996

Inclusion body myositis: atypical clinical presentations.

Inclusion body myositis affects primarily the proximal muscles but distal limb muscles are involved too in this chronic myopathy. Characteristic histopathologic findings include "rimmed vacuoles', inflammation and typical cytoplasmic and nuclear filamentous inclusions. The patients are usually unresponsive to steroids. We present four inclusion body myositis patients with atypical clinical presentations: one with scapuloperoneal syndrome, one with post-polio-like syndrome and two with associated immune-mediated diseases (one with undefined autoimmune disorder and the second with scleroderma). Two patients responded to high-dose steroid therapy. We suggest that the clinical spectrum of inclusion body myositis is wider than previously appreciated.

Adult↗
PubMed2006

Therapeutic options in autoimmune inflammatory myopathies (dermatomyositis, polymyositis, inclusion body myositis).

Polymyositis, dermatomyositis, and inclusion body myositis are idiopathic inflammatory myopathies of unknown etiology with autoimmune pathogenesis. For choosing an individual and efficient therapy, diagnostic assignment is an important factor. Therapeutic options in dermatomyositis and polymyositis include corticosteroids and immunosuppressives. Intravenous immunoglobulins are only needed in special cases. In inclusion body myositis, corticosteroids and immunosuppressives are not successful. At the moment intravenous immunoglobulins are the only therapeutic possibility.

Adrenal Cortex Hormones↗
PubMed1993

Inclusion body myositis: an underdiagnosed condition?

Inclusion body myositis is an increasingly recognised form of inflammatory myopathy with characteristic clinical and histopathological features which has seldom been reported in the United Kingdom. This paper presents the clinicopathological features of a series of patients diagnosed in Nottingham from 1986 to 1990. During this period, 1319 muscle biopsy samples were processed by this laboratory and rimmed vacuoles were seen in 17 patients. Eleven patients had definite or probable inclusion body myositis according to published criteria. The mean age of the group was 69.4 years with a male to female ratio of 8:3. Typical clinical features were a slowly progressive painless, proximal lower limb weakness, with muscle wasting and early loss of reflexes. The median duration of illness from first symptom to presentation was five years (range 2-18 years). Falls were a prominent symptom in six patients and distal weakness occurred in nine patients. Creatine kinase was increased in 10 patients but only one had a level > 1000 IU/l; the erythrocyte sedimentation rate was normal in five patients. Treatment with steroids or cytotoxic drugs, or both, did not prevent disease progression. It is confirmed that inclusion body myositis is a distinct cause of inflammatory myopathy which is probably underdiagnosed in the United Kingdom. Clinically, it should be suspected in older patients presenting with muscle weakness of insidious onset. Pathologically, a careful search should be made for rimmed vacuoles and inflammation; ultrastructurally, the presence of inclusions will confirm the diagnosis.

Aged↗
PubMed1998

Light and electron microscopic immunolocalization of presenilin 1 in abnormal muscle fibers of patients with sporadic inclusion-body myositis and autosomal-recessive inclusion-body myopathy.

Sporadic inclusion-body myositis (s-IBM) is the most common progressive muscle disease of older persons. The muscle biopsy demonstrates mononuclear cell inflammation and vacuolated muscle fibers containing paired helical filaments and 6- to 10-nm fibrils, both resembling those of Alzheimer disease brain and Congo red positivity. The term hereditary inclusion-body myopathies (h-IBMs) designates autosomal-recessive or autosomal-dominant disorders with muscle biopsies cytopathologically similar to s-IBM but without inflammation. Vacuolated muscle fibers of both s-IBM and the h-IBMs contain accumulations of several "Alzheimer-characteristic proteins" including beta-amyloid protein and beta-amyloid precursor protein, and their paired helical filaments are composed of phosphorylated tau. We used six well characterized antibodies against several residues of presenilin 1 (PS1) to immunostain muscle biopsies of 12 patients with s-IBM, 5 patients with autosomal-recessive inclusion-body myopathy, and 16 normal and disease controls. Seventy to eighty percent of the vacuolated muscle fibers of both s-IBM and autosomal-recessive inclusion-body myopathy had inclusions that were strongly PS1-immunoreactive, which by immunoelectron microscopy localized mainly to paired helical filaments and 6- to 10-nm filaments. None of the control biopsies had PS1-positive inclusions characteristic of the s- and h-IBM abnormal muscle fibers. Mutations of the newly discovered PS1 gene are responsible for early-onset familial Alzheimer disease (AD), and PS1 is abnormally accumulated in sporadic and familial AD brain. Our study provides the first demonstration of PS1 abnormality in non-neural tissue and in diseases other than AD and suggests that the cytopathogenesis in AD brain and IBM muscle may share similarities.

Adult↗
PubMed1986

Total body irradiation not effective in inclusion body myositis.

Four patients with inclusion body myositis were treated with 150 rad of total body irradiation given in 5 weeks. One patient responded subjectively and transiently, but no patient showed clear benefit. This treatment is not recommended for inclusion body myositis.

Adult↗
PubMed1992

Inclusion body myositis: analysis of 32 cases.

Inclusion body myositis is characterized by an insidious onset, progressive indolent course, and is generally felt to be refractory to standard therapy for myositis. We reviewed the charts of 32 patients with muscle biopsy findings suggestive of inclusion body myositis. The average time from symptom onset to diagnosis was 37 months, but initially 40% were incorrectly diagnosed. Twenty-eight patients (88%) were classified as definite or probable inclusion body myositis and were treated with various combinations of prednisone and immunosuppressive agents. Sixty-eight percent of those treated experienced a decrement in function and muscle strength. Three patients exhibited longterm improvement while 12 patients experienced delayed progression, defined by short term improvement in strength or a stable functional class, All of these patients received therapy, 5 in the form of methotrexate and prednisone. All untreated patients deteriorated clinically. In summary, (1) inclusion body myositis is a clinically distinct entity which is frequently misdiagnosed initially. (2) While clinical improvement with therapy is rare, our observations support recent reports that therapy may be associated with a slower rate of clinical progression. (3) Optimal therapy remains uncertain, but the use of low dose methotrexate and prednisone may warrant further study.

Adult↗
PubMed2003

[Inclusion body myositis (IBM) -- a review].

Inclusion body myositis (IBM) is characterized by the insidious onset of slowly progressive proximal and distal weakness. The clinical hallmark of IBM are atrophy and weakness of the quadriceps and the wrist and finger flexors. Although frequently misdiagnosed, IBM is the most common inflammatory myopathy in patients over the age of 50 years. The diagnosis of IBM can be made, even in absence of a typical clinical history, exclusive on the basis of muscle biopsy when all of the characteristic histopathological findings are present (inflammation, rimmed vacuoles, protein deposits, and 15-18 nm tubofilaments). Apart from sporadic IBM there is a group of heterogeneous inherited myopathies with histopathologic similarities to sporadic IBM. Although some immunomodulating therapies may exert transient and mild benefits, there is no effective treatment for IBM.

Adjuvants, Immunologic↗