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Keeping an eye on Crohn's disease: orbital myositis as the presenting symptom.

Episodic periorbital swelling due to presumed orbital inflammation and myositis caused intermittent apparent proptosis and was the presenting symptom of ileocecal Crohn's disease (CD) in a teenage female with a family history of autoimmune disorders and CD. Orbital myositis, a very rare extraintestinal manifestation of inflammatory bowel disease (IBD), likely represents a process of impaired immunoregulation related to the underlying intestinal inflammation. This rare manifestation of IBD simulates the more commonly encountered thyroid orbitopathy (ophthalmopathy), but IBD should be considered if all thyroid tests are negative. It is important to recognize that orbital myositis may be an extraintestinal manifestation of Crohn's disease so that the diagnosis can be made and appropriate therapy commenced.

Autoimmune Diseases↗

Benign acute childhood myositis.

OBJECTIVE: To evaluate the clinical and laboratory findings of benign acute childhood myositis. SUBJECTS AND METHODS: Six children, 1 girl and 5 boys, aged 3-9 years, were admitted to Karadeniz Technical University Paediatric Neurology Department in Trabzon, Turkey, with the diagnosis of benign acute childhood myositis in January and February 2002. The clinical and laboratory findings on the patients were analysed. RESULTS: Serum creatine kinase and aspartate aminotransferase levels were increased in all cases. Two patients had leucopenia. Viral studies were negative. All the patients had a good outcome, and full recovery was achieved within 12 h to 3 days. CONCLUSION: Benign acute childhood myositis is a self-limiting disease with a good prognosis. Pediatricians and pediatric neurologists must be aware of this condition to avoid unnecessary investigations and to differentiate this condition from other causes of acute onset of inability to walk. It may occur in epidemics mainly in the wintertime, suggesting a viral aetiology.

Acute Disease↗

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↗

Necrotizing myositis secondary to Serratia marcescens in a renal allograft recipient.

We describe a fatal case of spontaneous necrotizing myositis due to a highly resistant strain of Serratia marcescens in a renal transplant recipient. Though Staphylococcus aureus and Clostridium are the usual agents which cause either pyomyositis or necrotizing myositis, gram-negative bacteria are a dangerous and rarely suspected possibility. Such an aggressive disease should be promptly recognized because immunosuppression in susceptible hosts makes conservative management unsuccessful. The prognosis for myositis in immunodepressed hosts is poor and wide excision of all the necrotic muscles, leaving the wound open, and intensive antibiotic therapy are required.

Adult↗

Fine needle aspiration cytology of focal myositis: a case report.

BACKGROUND: Focal myositis is an unusual inflammatwy lesion of the skeletal muscle first described by Heffizer. It is a benign condition and usually involves the muscles of the limbs. CASE: A man presented with a palpable mass in the left leg of 6 months' duration. Nuclear magnetic resonance of the leg showed a mass in the tibial muscle; the presumptive diagnosis was sarcoma of the muscle. Smears showed inflammatory cells, skeletal muscle fibers with degenerative and regenerative changes, and fibrous tissue, suggesting a diagnosis of focal myositis. An incisional muscle biopsy was performed, confirming the diagnosis. CONCLUSION: Focal myositis should always he considered when aspirating muscle masses because it is a clinical mimic of a neoplasm. The prognosis is good, and all cases reported in the literature were self-limiting and gradually resolved.

Biopsy, Fine-Needle↗

Voluntary activation failure is detectable in some myositis patients with persisting quadriceps femoris weakness: an observational study.

This cross-sectional, observational study was undertaken to examine whether voluntary activation failure could contribute to the persisting weakness observed in some patients with treated idiopathic inflammatory myositis. In 20 patients with myositis of more than six months' duration (5 males, 15 females; mean [+/- 1 SD] age 53 11 years) and 102 normal subjects (44 males, 58 females; mean age 32 8 years), isometric maximum voluntary contractions (MVCs) of the dominant quadriceps femoris (QF) were quantified. Absolute MVC results of normal subjects and patients were then normalised with respect to lean body mass (force per units of lean body mass), giving a result in Newtons per kilogram. Based on mass-normalised force data of normal subjects, patients were arbitrarily stratified into "weak" and "not weak" subgroups. During further MVC attempts, the "twitch interpolation" technique was used to assess whether the QF voluntary activation of patients was complete. This technique relies on the fact that, because muscle activation is incomplete during submaximal voluntary contractions, electrical stimulation of the muscle can induce force increments superimposed on the submaximal voluntary force being generated. No between-gender differences were seen in the mass-normalised MVC results of healthy subjects, so the gender-combined results of 6.6 (1.5) N/kg were used for patient stratification. No between-gender difference was found for mass-normalised MVCs in patients: males 5.4 (3.2) and females 3.0 (1.7) N/kg (p > 0.05). Mass-normalised MVCs of male patients were as great as those of normal subjects (p > 0.05), but mass-normalised MVCs of female patients were significantly smaller than those of the normal subjects (p < 0.001). Only one of the six "not weak" patients exhibited interpolated twitches during electrical stimulation, but six of the 14 "weak" patients did, the biggest twitches being seen in the weakest patient. That interpolated twitches can be induced in some myositis patients with ongoing QF weakness during supposed MVCs clearly suggests that voluntary activation failure does contribute to QF weakness in those patients.

Adult↗

Cardiac involvement in autoimmune myositis and mixed connective tissue disease.

The clinical cardiac manifestations most frequently reported in idiopathic inflammatory myopathies, myositis, are congestive heart failure, conduction abnormalities, that may lead to complete heart block and coronary artery disease. Although clinically overt cardiac involvement is rarely reported in myositis patients, subclinical manifestations are frequently observed and are predominated by conduction abnormalities and arrhythmias detected by ECG. Furthermore, cardiovascular manifestations constitute a major cause of death in myositis, thus cardiac involvement maybe overlooked in these patients. Also children with juvenile dermatomyositis may develop cardiac involvement although the frequency seems to be low. The underlying pathophysiologic mechanisms that may cause cardiac manifestations could involve myocarditis and coronary artery disease as well as involvement of the small vessels of the myocardium. In patients with mixed connective tissue disease (MCTD) clinically significant cardiac involvement is also rare, the most frequently reported manifestations being pericarditis and pulmonary hypertension, the latter often attributable to small vessel disease, and often a prognostic unfavourable manifestation.

Autoantibodies↗

Plasma cells in muscle in inclusion body myositis and polymyositis.

BACKGROUND: Previous immunohistochemical studies of muscle from patients with inclusion body myositis and polymyositis found many more T cells than B cells, suggesting a role for intramuscular cell-mediated immune mechanisms rather than humoral mechanisms. METHODS: Microarray studies were performed on muscle biopsy specimens from 40 patients with inclusion body myositis (IBM; n = 23), polymyositis (PM; n = 6), and without neuromuscular disease (n = 11). Reverse transcription PCR of selected immunoglobulin gene transcripts was performed on two patient samples. Qualitative immunohistochemical studies for B-cell lineage cell surface markers were performed on 28 muscle specimens and quantitative studies performed on a subset of 19 untreated patients with IBM or PM. CD138+ cells were isolated from muscle using laser capture microdissection, and immunoglobulin transcripts were PCR amplified to determine the presence or absence of immunoglobulin gene rearrangements unique to the B-cell lineage. RESULTS: Immunoglobulin gene transcripts accounted for 59% in IBM and 33% in PM of the most stringently defined highest differentially expressed muscle transcripts compared with normal. Plasma cells, terminally differentiated B cells expressing CD138 but not CD19 or CD20, are present in IBM and PM muscle in numbers several times higher than B cells. CONCLUSIONS: There are differentiated B cells in the form of CD138+ plasma cells within the muscle of patients with inclusion body myositis and polymyositis. The principle of linked recognition of B-cell activation predicts several strategies for autoantigen discovery that could not otherwise be pursued through the study of the infiltrating T-cell population alone.

Antigens, Surface↗

Proliferative myositis of the masseter muscle.

The authors present a case of a 33-year-old white man with a 4-month history of a rapidly enlarging, tender, painful, circumscribed facial mass located in the masseter muscle. CT scan and fine needle aspiration cytology, coupled with clinical assessment, suggested the diagnosis of proliferative myositis. Curative intraoral excisional biopsy was done, thereby avoiding a parotidectomy incision and approach to the lesion. The pathology showed proliferative myositis. A review of the literature revealed 36 cases which indicated that proliferative myositis is a relatively rare etiology of a head and neck mass. Careful clinical, radiologic, and pathologic evaluations were required to make this unusual diagnosis and avoid any unnecessary facial mutilation in its treatment.

Adult↗

Recent advances in the management of adult myositis.

Standard drug therapy of adult polymyositis, dermatomyositis and inclusion body myositis includes high-dose corticosteroids and cytotoxic drugs (methotrexate, azathioprine (AZA) and cyclophosphamide). Recent data are in favour of the early introduction of a cytotoxic or immunomodulating drug in addition to corticosteroid therapy. In patients with corticosteroid- and cytotoxic-resistant myositis, promising novel approaches to management include: iv. megadose pulse methylprednisolone combined with cytotoxic drugs, combination therapy with both methotrexate and AZA, cyclosporin, tacrolimus, fludarabine and iv. immunoglobulin (IVIG). Recent advances in the understanding of the role of cytokines and complement, in the pathogenesis of myositis, have led to preliminary therapeutic trials of three biological agents: etanercept, infliximab and anti-C5 monoclonal antibody.

Adult↗

Myositis ossificans in the tip of the thumb: a case report.

A case of a myositis ossificans occurring in the distal portion of the finger is reported. An 18-year-old female noticed a small nodule in the tip of the left thumb for 11 months. Plain radiographs showed calcified shadow and there was a radiolucent zone between the mass and the distal phalanx. The excised mass was diagnosed as myositis ossificans. To our best knowledge, there has been no report of a myositis ossificans in the tip of the finger in literature.

Adolescent↗

High incidence of acute myositis with type A influenza virus infection in the elderly.

We describe six elderly cases of acute myositis with type A influenza virus infection (Sydney) during the 1998-1999 outbreak. All six cases suffered from myalgia or muscle weakness especially in the lower extremities and the serum creatine kinase (CK) values were elevated above 1,000 IU/l without MB isoenzymes or electrocardiogram abnormalities. There have been a few case reports of acute myositis associated with influenza virus infection in the elderly. However, we noticed a high incidence of acute myositis among elderly patients with type A influenza virus infection. This complication may occur more commonly in elderly patients than has previously been thought.

Acute Disease↗

Myositis ossificans of the temporal muscle as a primary scalp tumor. Case report.

A 38-year-old woman presented with a rare case of myositis ossificans in the temporal muscle manifesting as left temporal scalp mass with mild pain. The mass was elastic-hard and seemed to be located in the temporal muscle. Magnetic resonance imaging revealed a heterogeneously enhanced mass in the muscle. The tumor was resected. The histological diagnosis was myositis ossificans. The clinicopathological features of scalp myositis ossificans may mimic other soft tissue tumors, requiring care for the differential diagnosis.

Adult↗

Myositis ossificans: diagnostic value of arteriography.

Myositis ossificans circumscripta is the heterotopic formation of nonneoplastic bone and cartilage in soft tissue. These benign lesions can mimic more serious lesions, both radiographically and histopathologically. Recognition of the benign character of myositis ossificans is imperative in order to avoid mutilating surgical procedures. Five cases are presented with emphasis on angiographic signs which differentiate this lesion from histopathologically similar lesions. Three of these are illustrated, along with an example of an osteosarcoma for comparison. The angiographic manifestations of myositis ossificans differ in the various phases of the disease. In the active stage, the lesions have numerous fine vessels causing a diffus stain. Malignant tumors such as osteosarcoma, extraosseous osteosarcoma, and fibrosarcoma, which are included in the differential diagnosis, often present clearly defined pathologic vessels as well as arteriovenous shunts, venous lakes, amputated vessels, invasion of large arteries and veins, and large abnormal draining veins. In the healing stage, the lesions are usually avascular, and there is no difficulty in differentiating this condition from malignant bone lesions with the sole exception of well differentiated parosteal osteosarcomas. In these cases, the plain radiographic features are most important in arriving at the correct diagnosis.

Adolescent↗

Computed tomography of orbital myositis.

The computed tomographic (CT) scans of 11 consecutive patients with orbital myositis were reviewed to better characterize the CT appearance of this condition. The findings in this series differed from those of previous reports in several ways. Multiple muscle involvement predominated. Bilateral involvement was more frequent than previously reported. Enlargement of the tendon as well as the muscle was a frequent finding, but a normal tendinous insertion did not preclude the diagnosis of orbital myositis. Although the CT appearance of orbital myositis is often helpful, the findings are not pathognomonic; correlation with history, clinical findings, and therapeutic response must be considered in making the diagnosis.

Adult↗

[Myositis ossificans in recurrent synovial hemangioma of the knee].

A case of myositis ossificans in 15-year old boy that occurred as a complication of the recurrent synovia hemangioma of the left knee was presented. The alteration was diagnosed as: osteosarcoma, chondrosarcoma and chondromyxoid fibroma by the pathologists from other institutions. The diagnosis of myositis ossificans was established using numerous histochemical and immunochemical methods (PAS, PAS diastasis, Alcian blue, Masson trichrom, von Kossa, Azur A, Toluidin blue, Goldner, method by Peris, Citokeratin, S-100, NSE and Vimentin). The presence of zonal phenomenon, as the one observed in the presented case, is one of the most significant criteria for differentiation of myositis ossificans from osteosarcoma.

Adolescent↗

[Myositis specific autoantibodies as a new diagnostic criterion for idiopathic inflammatory myopathies].

Myositis specific autoantibodies (MSA) are the most specific diagnostic criteria for idiopathic inflammatory myopathies (IIM). There is no evidence of MSA presence in patients with other neuromuscular or connective tissue diseases. MSA are associated with homogeneous clinical syndromes: antisynthetases with antisynthetase syndrome, anti-SRP with severe, resistant to treatment myositis, anti-Mi-2 with classic, benign dermatomyositis. Therefore it is important to include the myositis specific antibodies into routine diagnostic scheme of IIM.

Autoantibodies↗

Unusual presentations in myositis ossificans progressiva. A case report.

Myositis ossificans progressiva is a rare connective tissue disorder. We present here a case of myositis ossificans progressiva with some unusual presentations and associated congenital skeletal anomalies that are reported very infrequently in the literature. The case report highlights the importance of early diagnosis in a case of rapidly progressive myositis ossificans progressiva.

Abnormalities, Multiple↗