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Fasciitis, perimyositis, myositis, polymyositis, and eosinophilia.

Several groups of cases of fasciitis and myositis with eosinophilia are reported. The common features are inflammation into fascia and/or perimysium, and/or muscle fibers; eosinophilia in blood and/or in muscle biopsy. The following classification of 24 cases is suggested: at one end of the spectrum are fasciitis with eosinophilia: diffuse fasciitis (Shulman syndrome): 10 cases (3 with hematological complications); 2 cases of diffuse fasciitis with muscle atrophy; 3 cases of restricted fasciitis. Relapsing perimyositis with eosinophilia belong to the same spectrum, either diffuse (5 cases) with myalgias, or localized (2 cases). Other cases are focal myositis or multiple myositis, polymyositis with eosinophilia. The relationship among these cases is discussed. There is a continuum among the different groups. The pathophysiology remains unknown.

Adult↗

The association of anti-Ro52 autoantibodies with myositis and scleroderma autoantibodies.

The presence of autoantibodies to the Ro52 protein in sera from patients with idiopathic inflammatory myopathies has recently been reported. These antibodies were found predominately in sera with the myositis-specific autoantibody anti-histidyl-tRNA synthetase (anti-Jo-1). In this report, we analysed sera from 216 patients to determine whether anti-Ro52 antibodies are associated with myositis autoantibodies other than anti-Jo-1. These included sera containing antibodies that recognize threonyl- or alanyl-tRNA synthetases, Mi-2, PM-Scl, signal recognition particle (SRP), as well as the systemic sclerosis-related antibodies anti-topoisomerase I (Scl-70) and anti-centromere. A high proportion of sera that contain anti-aminoacyl-tRNA synthetase antibodies, anti-SRP, or anti-PM-Scl antibodies were found to contain antibodies to the Ro52 protein. In contrast, in sera containing anti-Mi-2, anti-Scl-70 or anti-centromere antibodies, anti-Ro52 antibodies were absent or occurred infrequently. In addition, only one serum from 41 rheumatoid arthritis patients was positive for anti-Ro52 autoantibodies. These data indicate that anti-Ro52 antibodies are produced in particular subsets of myositis patients, and are not limited to sera with anti-Jo-1 antibodies.

Amino Acyl-tRNA Synthetases↗

Myositis ossificans with atypical clinical, radiographic, or pathologic findings: a review of 23 cases.

Myositis ossificans is a relatively rare, well-defined entity. The 23 cases sent for consultation to two of us (L.V.A. and A.N.) were reviewed. Clinical, radiologic, and microscopic information was reexamined, and special attention was given to features infrequently seen in typical myositis ossificans. Average age at presentation was 23 years, but 1 patient was younger than 2 years, and 2 were younger than 10 years. Due to the uncommon location of 15 lesions and an unusual presentation in 5, the correct diagnosis was not obvious in these cases. A history of trauma was elicited in 8 patients, denied by 7, and was not queried in the remainder. Radiologic studies raised the possibility of a malignant bone-forming tumor in at least three instances; myositis ossificans was originally diagnosed in 6 cases radiologically. In 8 cases, histologic evidence suggested malignancy, including osteosarcoma, either parosteal or extraosseous, in 6. Other diagnoses included epithelioid sarcoma and callus formation. Presentation of these variations from the norm highlights the importance of recognizing the evolution of a nonneoplastic fibro-osseous and cartilaginous entity in which conservative treatment is curative.

Adolescent↗

Changes in NADPH-diaphorase activity in the rat dorsal horn following an acute experimental myositis.

Previous neurophysiological experiments have shown that in rats with an acute myositis of the gastrocnemius-soleus muscle, dorsal horn neurones exhibit an increase in responsiveness to peripheral stimulation and in background activity. The present study investigated the possible correlation between changes in NADPH-diaphorase activity and neurophysiological alterations. In the animals used for the electrophysiological experiments the diaphorase activity in sections of the lumbar spinal cord was determined with the NADPH-nitroblue tetrazolium reaction. The main findings was a massive reduction in the number of diaphorase-positive cells in the superficial dorsal horn in animals with a myositis. The staining intensity in the remaining neurones was unchanged. The results are interpreted as indicating that the myositis in addition to the surgical operations represents a supramaximal input to the dorsal horn causing neurotoxic effects in diaphorase- positive neurones.

Acute Disease↗

Myositis of small foot muscles.

Idiopathic focal myositis is a rare clinical entity and is mostly localized in the neck and thigh muscles presenting as a pseudotumor. We describe a 49-year-old patient with inflammation restricted to the small muscles of one forefoot. Systemic disease was not present and progression to polymyositis did not occur within 2 years of follow-up. The myositis improved promptly after initiation of immunosuppressive therapy with corticosteroids and azathioprine. This unusual location of focal myositis has not been previously reported.

Adrenal Cortex Hormones↗

Association of inclusion body myositis with subacute cutaneous lupus erythematosus.

We present the case of a 71-year-old man with inclusion body myositis combined with subacute cutaneous lupus erythematosus and dysphagia. Although inclusion body myositis is usually resistant to immunosuppressive therapy, this patient improved under treatment with corticosteroids. The presented case is discussed in the context of earlier reports of inclusion body myositis and lupus erythematosus.

Aged↗

Skeletal muscle disorders associated with inflammatory bowel diseases: occurrence of myositis in a patient with ulcerative colitis and Hashimoto's thyroiditis--case report and review of the literature.

Ulcerative colitis (UC) and Crohn's disease are inflammatory bowel diseases often associated with extra-intestinal manifestations. Of these, neutrophilic dermatoses and arthropathies are the more frequently observed, while the occurrence of striated muscle disorders, namely, myositis, has been very rarely diagnosed in these kinds of patients. The coexistence of immuno-mediated diseases in patients with inflammatory bowel diseases and myositis suggests a common aetiopathogenetic mechanism underlying these conditions. The present report refers to a rare case of a 51-year-old female with UC and Hashimoto's thyroiditis who developed myositis.

Colitis, Ulcerative↗

Myositis ossificans in the neck.

Myositis ossificans is a non-neoplastic heterotopic bone formation within muscle or soft tissues. The most commonly involved muscles are the muscles of the upper arm and thigh. Occurrence in the head and neck is rarely encountered clinically. A 53-year-old Turkish man (farmer) was operated on for traumatic myositis ossificans circumscripta in his neck. During the operation the mass was found to originate from the scalenus medius muscle and was readily and completely dissected from surrounding tissues. The histologic examination of the specimen revealed focal cartilage and mature bone tissue, which was compatible with the late stage of myositis ossificans. In 5 years of follow-up, the patient has remained asymptomatic and no signs of recurrence have been noted.

Humans↗

Evidence of a polyclonal nature of myositis ossificans.

Myositis ossificans is a localized, self-limiting, reparative lesion that is composed of reactive hypercellular fibrous tissue and bone. Although it is clearly a benign lesion, its clinical, radiological, and histological appearance may sometimes mimic a malignant tumor. Whether myositis ossificans represents a monoclonal or polyclonal hyperplastic proliferation is not yet known. To address this question, we therefore extracted DNA from the respective paraffin-embedded tumor tissues of nine women with a median age of 50 years at diagnosis (range: 20-84 years) and studied the X inactivation pattern by means of methylation-sensitive polymerase chain reaction and primers that target the polymorphic CGG trinucleotide repeat of the FMR1 gene. The fact that we did not detect any skewing of the X inactivation pattern in the five successfully analyzed cases corroborates the notion that myositis ossificans results from a polyclonal proliferation and confirms that it is a reactive, reparative process. Analysis of the X inactivation pattern may, thus, supplement the differential diagnostic work-up of cases with an uncertain histology, at least in the informative proportion of female patients.

Adult↗

Paediatric Behçet disease manifested as recurrent myositis: from an incomplete to a full-blown form.

UNLABELLED: A 12-year-old boy presented with a limp and findings suggesting localised myositis of his right calf and a working diagnosis of Behçet disease was made. During 3 years of follow-up, he had another three episodes of calf myositis, all responsive to corticosteroids within days. CONCLUSION: A case of recurrent localised myositis as a main manifestation of Behçet disease is reported. The evolution of incomplete Behçet disease, which is common in children, to the full blown form, with the emphasis on muscle involvement and the importance of early diagnosis of Behçet disease, is discussed.

Adolescent↗

Myositis ossificans of the abdominal rectus muscle: report of a case.

We herein report a rare case of myositis ossificans originating from the abdominal rectus muscle, found in a 38-year-old woman who presented with a left upper abdominal mass. The mass was initially suspected to be a malignant neoplasm because no history of either operation or trauma existed for this patient. Moreover, the location of the mass was unusual and the enhancement patterns of the dynamic magnetic resonance images were similar to that of a malignant tumor. Based on the radiologic findings, a surgical exploration was performed. A well-circumscribed mass, which measured 3.2 x 2 cm, was found in the rectus muscle. Microscopic findings showed a typical zonal pattern with a fibroblastic central zone and a zone of ossification at the periphery. Although abdominal myositis ossificans is extremely rare, it is one of the causes of abdominal mass lesions and can be mistaken for a malignant tumor. Therefore, a thorough knowledge of the evolution of myositis ossificans is necessary and in cases with malignant suggestion on magnetic resonance imaging, like that seen in our case, we suggest that a surgical excision may be necessary for both the diagnosis and treatment.

Abdominal Neoplasms↗

MR imaging findings of an unusual case of myositis ossificans presenting as a progressive mass with features of fluid-fluid level.

We present a case of myositis ossificans involving the upper arm in which features of fluid-fluid level became apparent on magnetic resonance imaging (MRI). Serial MR images obtained over a 6-month period exhibited progressive appearances in the absence of dense mineralization in the early and mid phases, which simulate neoplastic conditions. Twenty-four weeks following the biopsy, MR images revealed that the mass had decreased in size with the disappearance of most of the features of fluid-fluid level. To the best of our knowledge, no example of myositis ossificans accompanied by features of fluid-fluid level, which was closely monitored by MRI, exists in the literature. These features of image examination should be evaluated cautiously to avoid unnecessary surgical intervention, especially in instances where lesions exhibit expansive appearance. This case provided beneficial information regarding the sequence of changes in terms of MR appearance of myositis ossificans.

Adult↗

Usefulness of ultrasonography for early recurrent myositis ossificans.

Myositis ossificans is a benign heterotopic ossifying condition of soft tissues. The lesion may cause persistent functional impairment of adjacent joints. Surgical intervention is often needed to regain normal function, even though a high recurrence rate is reported. In the present case, we found that recurrent myositis ossificans could not be demonstrated by radiography during the early stage. However, ultrasonography proved to be an excellent tool for detecting early recurrence of myositis ossificans and for distinguishing the condition from extraosseous sarcomas.

Alkaline Phosphatase↗

Interstitial lung disease in a patient with antisynthetase syndrome and no myositis.

Interstitial lung disease in patients with antisynthetase syndrome and no evidence of myositis is rare and may precede other disease manifestations. We report a patient who initially presented with symptoms primarily related to lung involvement. The diagnosis of the antisynthetase syndrome without myositis was made many months later when he developed a characteristic hand rash (mechanic's hands), which was confirmed by positive antibodies to Jo-1. With treatment, both the hand rash and the interstitial lung disease improved. Antisynthetase syndrome should be considered in patients presenting with interstitial lung disease with no evidence of myositis. Appropriate laboratory testing with measurement of specific autoantibodies may help in the early diagnosis and treatment of the syndrome.

Adult↗

Improved detection of anti-Jo-1 antibody, a marker for myositis, using purified histidyl-tRNA synthetase.

The increased detection of anti-Jo-1 antibody afforded by the use of the purified antigen, histidyl-tRNA synthetase, in counterimmunoelectrophoresis is demonstrated. Using purified antigen, anti-Jo-1 antibody was detected in the sera of 16/33 (48.5%) patients with confirmed myositis and in 20/45 (44.5%) patients with confirmed or possible myositis. This rate is approximately double that obtained with commercial thymus extracts both in this study and seven others reported in the literature. The presence of antibody shows marked correlation with the activity of myositis at the time of serum sampling and with the presence of interstitial lung disease. Detection rates are similar in patients with polymyositis and dermatomyositis both with and without additional connective tissue diseases.

Adult↗

Myositis ossificans of medial pterygoid muscle. A cause for temporomandibular joint ankylosis.

An unusual case of myositis ossificans of the medial pterygoid muscle has been discussed. It is important to know the exact cause of the temporomandibular joint ankylosis, for successful treatment. Computed tomographic scan and panoramic radiographs are essential diagnostic aids for evaluating conditions such as myositis ossificans. Myositis ossificans can be one of the causes of extra-articular temporomandibular joint ankylosis.

Adult↗

Cutaneous changes of dermatomyositis in patients with normal muscle enzymes: dermatomyositis sine myositis?

BACKGROUND: Dermatomyositis sine myositis may have various connotations. Controversy exists as to nomenclature, degree of evaluation required, therapy, and course (e.g., does true dermatomyositis of the skin only exist?). OBJECTIVE: The purpose of this study was to assess prospectively patients with the clinicopathologic features of dermatomyositis and normal muscle enzyme serum levels to determine their course in terms of the onset of muscle disease. METHODS: Thirteen patients were studied by complete history and clinical examination, laboratory studies, electromyography, and skin and muscle biopsy. They were observed for 1 to 6 years. RESULTS: Patients were classifiable into three groups: (1) cutaneous changes only, (2) cutaneous changes only at baseline with subsequent development of myositis, and (3) cutaneous changes with normal muscle enzyme serum levels at baseline but with myositis demonstrated by electromyography and/or muscle biopsy specimens. CONCLUSION: Significantly different prognostic and therapeutic implications are present in patients with dermatomyositis with normal muscle enzyme serum levels depending on the results of electromyography, muscle biopsy, and clinical observation.

Adolescent↗

Apolipoprotein E type epsilon 4 allele frequency is not increased in patients with sporadic inclusion-body myositis.

The apolipoprotein E genotype was determined for 11 patients with sporadic inclusion-body myositis. Seven cases had the genotype epsilon 3/epsilon 3, the other four cases, epsilon 3/epsilon 4. The frequency of the epsilon 4 allele in this group of patients (0.182) was not significantly increased compared with elderly controls (0.147; n = 58), in contrast to Alzheimer's disease in which there was a significant increase (0.328; n = 67). The epsilon 2 allele was not found in any of the 11 sporadic inclusion-body myositis patients and its frequency was decreased in Alzheimer's disease. Despite certain pathological similarities that exist between inclusion body myositis and Alzheimer's disease, their association with particular apolipoprotein E genotypes is distinct.

Aged↗