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At least 19 recordsLinked to original sources
PubMed1997

Clinical features of lupus myositis versus idiopathic myositis: a review of 30 cases.

Myositis is a rare but well-recognized complication of systemic lupus erythematosus (SLE). It is reputed to be milder than primary myositis in terms of morbidity and treatment response. This study compares clinical and laboratory features of idiopathic inflammatory myositis in patients with and without evidence of SLE overlap. We performed a case note review of 30 patients with probable or definite polymyositis/dermatomyositis of whom 11 also had definite or probable SLE. Lupus patients were slightly younger at diagnosis than those with primary disease, and more likely to be female. At presentation, quadriceps strength (expressed as a percentage of expected) was significantly reduced in both the lupus (48.9%; 95% CI 29.0-70.4%) and primary (52.0%; 95% CI 43.6-59.4%) myositis groups, and serum creatine phosphokinase (expressed as a multiple of the upper limit of normal) was significantly elevated (11.2; 95% CI 5.3-29.1 vs 10.7; 95% CI 6.1-17.6). During a mean (S.D.) follow-up period of 7.4 (4.1) yr, both groups tended to follow either a relapsing and remitting, or a chronic persistent course, and when last seen quadriceps muscle strength remained significantly depressed. One of the lupus patients and two of the primary myositis patients died due to direct complications of the disease, and one further death was attributable to a complication of therapy. Our results suggest that lupus myositis is often as severe as primary disease and should be treated with equal vigour.

Adrenal Cortex Hormones↗
PubMed1995

Acute neutrophilic myositis in Sweet's syndrome: late phase transformation into fibrosing myositis and panniculitis.

Early in the course of myeloblastic leukemia a patient concurrently developed febrile neutrophilic dermatosis and sterile acute myositis. The dermatitis and myositis were unresponsive to antibiotic therapy but remitted within a few days of institution of steroid treatment. The patient died of myocardial infarction. At autopsy the dermis was normal. Previously effected muscles were scarred. The overlying fascia and subcutaneous septa were fibrotically thickened. In addition, segmental acute aortitis was detected. Acute myositis and aortitis may reflect further organ manifestations of the Sweet's reactivity pattern. It is proposed that Sweet's myositis and dermatitis may evolve into a fibrosing myositis and panniculitis.

Acute Disease↗
PubMed2002

Inflammatory myositis associated with anti-U1-small nuclear ribonucleoprotein antibodies: a subset of myositis associated with a favourable outcome.

OBJECTIVES: Inflammatory myositides are rare chronic disorders which may be either isolated or associated with other conditions such as connective tissue diseases or neoplasia. A large variety of autoantibodies can be detected in patients with myositis, some of which have a diagnostic and/or a prognostic value. Myositis associated with anti-U1-small nuclear ribonucleoprotein antibodies (anti-U1-snRNP Abs) are usually considered as overlapping syndromes, mainly mixed connective tissue diseases (MCTD) in which muscle symptoms occur insidiously during the disease course and are characterized by a favourable outcome. METHODS: The clinical, biological, immunological and pathological findings as well as the outcome of five patients with anti-U1-snRNP-associated myositis were retrospectively analysed. RESULTS: Patients were mainly black females. In all five patients, myositis was the predominant manifestation at presentation. Associated conditions consisted of interstitial lung disease (ILD) (three), arthritis (three) and neurological symptoms (two). No patient presented Raynaud's phenomenon nor met criteria for MCTD. Biological inflammatory features, rheumatoid factor and polyclonal hypergammaglobulinaemia were present in all cases. Besides anti-U1-snRNP Abs, one patient had anti-Ro/SSA and anti-La/SSB Abs at presentation and one additional patient developed anti-double-stranded-DNA and anti-Sm Abs after a follow-up of more than 4 yr. No patient had anti-PM/sclerosis (Scl) nor anti-aminoacyl-tRNA synthetase Abs. All patients dramatically improved with steroids, and reached complete remission (CR) within 3 weeks. Two patients relapsed 18 months after CR. They both reached rapidly second CR using steroids associated or not with oral methotrexate. CONCLUSION: Our data suggest that anti-U1-snRNP Abs may define a subset of myositis characterized by a favourable outcome, though often associated with ILD and/or neurological manifestations.

Adult↗
PubMed1975

The subcutaneous fascial analogue of myositis proliferans: electron microscopic examination of two cases and comparison with myositis ossificans localisata.

Two cases of the so-called fascial analogue of myositis proliferans were investigated by histological and electron microscopic methods. It was found that the structure of the fascial variant corresponds almost completely to the true myositis proliferans localized within the musculature. The electron microscopic observations show a preponderantly histiocytic differentiation of the cells and strongly activated proliferating capillaries, and exclude a myogenic origin of the characteristic ganglion-like giant cells. Ultrastructurally a traumatic genesis appears possibly, the cells of the lesion could derive from multipotent cells of the microvasculature. The relations to myositis ossificans and fascitis nodularis are discussed.

Adult↗
PubMed1994

A broadened spectrum of juvenile myositis. Myositis-specific autoantibodies in children.

OBJECTIVE: Myositis-specific autoantibodies (MSA) define relatively homogeneous clinical and immunogenetic patient groups in adults with idiopathic inflammatory myopathies (IIM). This study explores the usefulness of MSA in defining groups of children with myositis. METHODS: Sera from 77 children with myositis and other connective tissue diseases were tested for MSA by immunoprecipitation and immunodiffusion. Clinical data were collected and analyzed. RESULTS: The MSA anti-PL-12 (alanyl-transfer RNA synthetase), anti-Jo-1 (histidyl-tRNA synthetase), anti-signal recognition particle, and anti-Mi-2 were each identified in the sera of 12 children with IIM. In these patients, the clinical manifestations, disease courses, and responses to therapy closely resembled those in adults with the same autoantibodies. CONCLUSION: These observations suggest that the clinical syndromes defined by particular MSA are similar in children and adults with IIM. By defining similar clinical syndromes in children who have MSA, this study provides a basis for future studies of MSA in the idiopathic inflammatory myopathies of childhood, which may be useful in predicting the clinical courses of a subset of these patients and improving their therapy.

Adolescent↗
PubMed1997

Ubiquitin immunostaining and inclusion body myositis: study of 30 patients with inclusion body myositis.

Distinction of inclusion body myositis (IBM) from other forms of inflammatory myopathy is significant from prognostic and therapeutic standpoints. This study retrospectively examines ubiquitin expression by paraffin immunohistochemistry in muscle biopsy material from 30 patients with IBM. Patients included 19 men and 11 women (ages 29 to 80 years; mean, 64 years). All biopsies were characterized by endomysial chronic inflammation, muscle fiber degeneration and regeneration, rimmed vacuoles, and angular atrophic esterase-positive muscle fibers. Ragged red fibers were identified in biopsies of five patients and a partial cytochrome C-oxidase deficiency by enzyme histochemistry in biopsies of 10 patients. Evidence of intranuclear or cytoplasmic tubulofilamentous structures confirming a diagnosis of IBM was observed in all 30 cases. Paracrystalline mitochondrial inclusions were noted in five patients. Discrete myocyte intranuclear ubiquitin-positive inclusions were noted in 14 patients (47%). Discrete intracytoplasmic ubiquitin-positive inclusions were noted in 24 (80%) patients. Positive staining of rimmed vacuoles by ubiquitin was observed in 25 (83%) patients. Diffuse staining of scattered muscle fibers was observed in 21 (70%) patients. In a control group including patients with polymyositis (n = 3), dermatomyositis (n = 3), necrotizing vasculitis (n = 1), and granulomatous myositis (n = 1), discrete intranuclear or cytoplasmic ubiquitin-positive inclusions were not observed. Rimmed vacuoles were not seen either by light microscopy or ubiquitin immunostaining in any of the eight cases. Occasional myofibers from all eight cases showed diffuse, positive muscle fiber staining. Although not present in all cases, evidence of ubiquitin-positive myocytic intranuclear or cytoplasmic inclusions or positive-staining rimmed vacuoles in the setting of an inflammatory myopathy may be suggestive of a diagnosis of inclusion body myositis. Use of ubiquitin immunohistochemistry may be useful in cases in which frozen tissue or tissue processed for electron microscopy is not available, and IBM is suspected. Light or electron microscopic evidence of mitochondrial abnormalities were noted in a significant subset of patients (13 of 30; 43%) of patients with IBM.

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↗
PubMed1999

[Facial myositis: a localized form of generalized myositis?].

We report a case of myositis presenting as an apparently unique progressive facial weakness. The only biological abnormality after an 8-year follow-up was an immunocytoma. Molecular analyses excluded facioscapulohumeral muscular dystrophy. Based on this single case and a review of the literature, we suggest a continuum between focal myositis and generalized myositis.

Diagnosis, Differential↗
PubMed2002

Clinical and serological characteristics of 125 Dutch myositis patients. Myositis specific autoantibodies aid in the differential diagnosis of the idiopathic inflammatory myopathies.

The idiopathic inflammatory myopathies (IIM) are a heterogeneous group of systemic diseases that include the familiar disease entities of dermatomyositis (DM), polymyositis (PM), and inclusion body myositis (IBM). A subset of patients has unique autoantibodies which are specific for IIM (myositis specific autoantibodies; MSAs). We studied the clinical and serological characteristics of IIM in 125 Dutch patients. Sera were analysed by immunoblotting, enzyme-linked immunosorbent assay, and immunoprecipitation. The most frequently encountered MSA was the anti-Jo-1 autoantibody (20%), followed by anti-tRNAHis (6%), anti-Mi-2 (6%), and anti-SRP (4%). The presence of certain MSAs was clearly associated with specific clinical characteristics. Anti-Jo-1 and anti-tRNAHis were associated with the anti-synthetase syndrome, anti-SRP with PM with severe myalgia and arthralgia and a moderate response to immunosuppressive treatment. A novel finding was the presence of anti-Mi-2, not only in DM, but also in PM. MSAs were frequently present in DM/PM sera, but were hardly ever detected in the sera of IBM patients. The few IBM patients with MSAs demonstrated a significant response to immunosuppressive treatment. It can be concluded that MSAs define specific clinical syndromes within the spectrum of IIM and that they can assist in the differential diagnosis and treatment plan of these enigmatic disorders by virtually excluding IBM by their presence, and by potentially identifying a subgroup of steroid-responsive IBM patients.

Adenosine Triphosphatases↗
PubMed2000

Conditional up-regulation of MHC class I in skeletal muscle leads to self-sustaining autoimmune myositis and myositis-specific autoantibodies.

In the human inflammatory myopathies (polymyositis and dermatomyositis), the early, widespread appearance of MHC class I on the surface of muscle cells and the occurrence of certain myositis-specific autoantibodies are striking features. We have used a controllable muscle-specific promoter system to up-regulate MHC class I in the skeletal muscles of young mice. These mice develop clinical, biochemical, histological, and immunological features very similar to human myositis. The disease is inflammatory, limited to skeletal muscles, self-sustaining, more severe in females, and often accompanied by autoantibodies, including, in some mice, autoantibodies to histidyl-tRNA synthetase, the most common specificity found in the spontaneous human disease, anti-Jo-1. This model suggests that an autoimmune disease may unfold in a highly specific pattern as the consequence of an apparently nonspecific event-the sustained up-regulation of MHC class I in a tissue-and that the specificity of the autoantibodies derives not from the specificity of the stimulus, but from the context, location, and probably the duration of the stimulus. This model further suggests that the presumed order of events as an autoimmune disease develops needs to be reconsidered.

Animals↗
PubMed2000

Update on myositis-specific and myositis-associated autoantibodies.

Myositis-specific autoantibodies or myositis-associated autoantibodies can often be found in serum of patients with polymyositis and dermatomyositis. The presence of these autoantibodies can be significant in patient diagnosis and classification. Recent studies have provided new information about many of these specific autoantibodies. Among the more important developments were identification of a new antisynthetase, reacting with asparaginyl-tRNA synthetase; the detection of antibodies to the tRNA(his) in a over a third of anti-Jo-1 sera; and the description of distinctive features of the histopathology of patients with anti-Jo-1. New information about the cellular role of the antigens was discovered, including a role for Mi-2 antigen in chromosomally-mediated regulation of transcription as part of a nucleosome remodeling complex, and a potential role for PM-Scl antigen in ribosomal RNA processing as part of an exosome. The reason for the production of the autoantibodies, and the reason particular antigens are targeted, are key questions. Recent studies have suggested that antigen cleavage during apoptosis, particularly by granzyme B, may be an important factor. Whether the antibodies play a role in tissue injury remains unknown.

Amino Acyl-tRNA Synthetases↗
PubMed2000

Association of active extracellular signal-regulated protein kinase with paired helical filaments of inclusion-body myositis muscle suggests its role in inclusion-body myositis tau phosphorylation.

The possible role of extracellular signal-regulated kinase (ERK) in the pathogenesis of inclusion-body myositis (IBM) was investigated by immunostaining the active phosphorylated form of ERK in muscle biopsies of six IBM and 14 control patients. Between 80% and 90% of IBM vacuolated muscle fibers contained well-defined ERK-immunoreactive inclusions, which were co-localized by light microscopy, with phosphorylated tau in 70 to 80% of those fibers. Immunoelectronmicroscopy colocalized ERK to small amorphous tufts adjacent to the muscle fiber paired-helical filaments. Strong ERK immunoreactivity was also present at the postsynaptic domain of all human neuromuscular junctions. Our study suggests 1) that ERK, a signal transducer, might play a role in IBM pathogenesis, including participation in the pathological phosphorylation of IBM tau; and 2) that signal transduction abnormalities may be a component of the IBM pathogenic cascade. Our novel immunolocalization of ERK at the postsynaptic domain of human neuromuscular junctions supports a role in transcription of junctional-protein genes. The ERK localized in nonjunctional regions of IBM fibers may underlie the known pathological up-regulation of junctional proteins there.

Humans↗
PubMed1986

Inclusion body myositis: a chronic persistent mumps myositis?

Among the generalized chronic idiopathic inflammatory myopathies, inclusion body myositis (IBM) has emerged as a clinico-pathologic variant during the past two decades. It occurs primarily in elderly persons (in approximately the sixth decade of life), but young adults (in approximately the second decade of life) may also be affected. Slowly progressive weakness of distal as well as proximal muscle groups in IBM is usually not associated with skin rash, malignancy or collagen-vascular disease, and is refractory to treatment with steroids or other immunosuppressants. Exceptions to each of these general rules have been found. Muscle biopsy and electromyography may suggest a neurogenic process mixed with myopathic features. Rimmed vacuoles with basophilic granules in cryostat sections stained with hematoxylin-eosin are strongly suggestive of IBM if accompanied by the histopathologic triad of polymyositis. The presence of eosinophilic intranuclear or cytoplasmic inclusions in affected myofibers is further suggestive of IBM. The ultimate diagnosis, however, depends on ultrastructural demonstration of characteristic microtubular filaments resembling the nucleocapsids of the paramyxovirus group. Recent reports of immunostaining of the inclusions for mumps virus antigen strongly suggest a chronic persistent mumps virus infection as the cause of IBM. IBM is considered to be pathologically related to both distal myopathy (DM) and oculopharyngeal muscular dystrophy (OPMD).

Adult↗
PubMed2002

Focal myositis of the neck with idiopathic orbital myositis.

Focal myositis (of Heffner) in the right trapezius and paraspinal muscles accompanied by a pseudotumour of the left orbit has not been reported previously. The clinical, pathological and radiological features of these unusual benign pseudotumours of the head and neck are discussed. Computerized tomography (CT) scan of the neck and orbital ultrasound were suggestive of an inflammatory process without an abscess formation. This was followed by fine needle aspiration cytology (FNAC), which confirmed the diagnosis. The patient was treated with intravenous steroids and antibiotics, that led to complete resolution of symptoms, and there was no recurrence at six months follow-up. This report highlights the importance of imaging in inflammatory neck swellings.

Anti-Bacterial Agents↗