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Clinical features of influenza A and B in children and association with myositis.

Influenza virus is among the most common causes of respiratory illness, which may manifest as a range of conditions, from mild upper respiratory tract infection to bronchiolitis and pneumonia. Acute childhood myositis associated with influenza occurs mostly in influenza B infection. In this retrospective study, we analyzed the characteristics of 197 children with influenza virus treated from January 2000 to December 2001. Among them, 73 children had influenza A infection and 124 had influenza B infection. Influenza A virus outbreaks occurred in January 2000, July 2001, and December 2001, while influenza B virus outbreaks occurred from March 2000 to May 2000 and from December 2000 to February 2001. The most common clinical manifestations of influenza A and influenza B virus infection included fever, cough, and rhinorrhea. These infections also frequently manifested as laryngo-tracheobronchitis, pneumonia, and unexplained fever, which led to hospitalization. The most common clinical diagnosis was upper respiratory tract infection. The rates of benign acute childhood myositis in influenza A and influenza B were 5.5% and 33.9%, respectively. Creatine kinase levels were elevated in most myositis cases and boys were more commonly affected. Acute childhood myositis was more commonly seen in influenza B infection.

Child↗

Ultrastructural and transmission evidence of Sarcocystis cruzi associated with eosinophilic myositis in cattle.

Skeletal muscle, diaphragm, tongue, esophagus and heart of beef carcasses that were condemned for eosinophilic myositis and those that were unaffected were collected at an abattoir in Colorado and studied to determine the involvement of Sarcocystis spp. All affected carcasses contained similar granulomatous lesions with adjacent infiltrations of leukocytes. Intact or fragments of sarcocysts were found within 32 of 363 granulomas, and whole sarcocysts were present in nearby unaffected muscle cells. Light and electron microscopic examinations revealed that sarcocysts, affected or unaffected by cellular response in condemned carcasses, as well as those found in unaffected carcasses, were consistent with those of S. cruzi. Transmission experiments confirmed that S. cruzi were present in all carcasses, and that dogs, but not cats, were the definitive hosts. The results of pepsin-HCl digestion assays showed that unaffected carcasses that were approved for human consumption generally contained more infective parasites than carcasses that were condemned for eosinophilic myositis. This study provides evidence to support the suggestion that dogs, rather than cats, and unaffected rather than eosinophilic myositis-affected carcasses, have greater potential for contributing to the perpetuation of eosinophilic myositis in the cattle industry.

Animals↗

C4 null genes in American whites and blacks with myositis.

The frequencies of C4A and C4B alleles were determined in 66 adults with myositis in relation to HLA class I and II. In whites with myositis, the C4A*Q0 allele occurred in 13/31 (47%) as compared to 25/101 (25%) normal controls (p = 0.08, relative risk = 2.7). Only 11/35 (31%) of black patients with myositis had a C4A*Q0 allele compared to 11/55 (20%) of controls (p = NS, RR = 1.8). Thus, the MHC class III genes do not appear to be the primary genetic risk factors for myositis in adults.

Alleles↗

[Acute severe myositis due to bezafibrate treatment].

The second generation fibric acid derivative, bezafibrate (Bezalip, Norlip) is widely used as a hypolipemic agent throughout Europe and Israel. Its side-effects are well documented, and include myositis, which is considered very rare. We report a 55-year-old diabetic woman with hypertension who had mild renal dysfunction (creatinine 2.0 mg/dl) who received 400 mg/d bezafibrate because of combined (Type IIb) hyperlipoproteinemia. She developed acute myositis, with extreme muscle weakness, pain and CPK levels of up to 3500 units. On discontinuation of the drug all clinical and biochemical features ceased and complete cure followed. No other symptoms have appeared during 2 years of followup. The few reports of such cases in the German literature point to a greater prevalence of myositis in those with renal dysfunction. Early diagnosis of bezafibrate-induced myositis is crucial, a discontinuation of the drug results in cure.

Acute Disease↗

Autoantibodies against a specific nuclear RNP protein in sera of patients with autoimmune rheumatic diseases associated with myositis.

Polymyositis is an autoimmune, inflammatory disease affecting human skeletal muscle. In the presence of concomitant vasculitis in the skin, the term dermatomyositis is used. In contrast, systemic lupus erythematosus (SLE) is a multisystem disease in which involvement of the skin, kidneys, joints, brain, and other organs may be found. The clinical manifestations vary according to the organ/system involved. It is clinical and therapeutic importance to define which organ/system is involved during the course of the disease. We approached this problem by studying the specificity of autoantibodies that are generated in patients with SLE and polymyositis/dermatomyositis. Among such antibodies are those directed against nuclear components including a variety of ribonucleoprotein (RNP) complexes. We have utilized mammalian nuclear preparations enriched with RNP particles as the antigenic source for immunoblotting studies to identify specific antigenic polypeptides. In the study reported here, sera from five groups of patients were examined: 10 patients with dermatomyositis/polymyositis; six patients with SLE and myositis; 12 lupus patients with cerebral and/or renal disease; eight patients with SLE but no myositis, renal, or cerebral disease; and 5) 11 patients with muscle weakness or muscle disease not due to myositis. In the first two groups of patients with myositis, antibodies against a nuclear RNP protein of 56 KD was identified in 12 of 16 sera. In contrast, such antibodies were found in the serum of only two of 20 patients with SLE but without muscle involvement (groups 3 and 4), and were not found at all in patients with other muscle diseases. This study has identified a new marker, antibodies against a nuclear RNP protein of 56 KD for detecting muscle involvement among the autoimmune rheumatic diseases.

Animals↗

Palatal myositis in horses with dorsal displacement of the soft palate.

The histologic and histochemical features of palatine muscles from 53 horses were studied; 25 of the horses were racehorses that had upper airway obstruction associated with dorsal displacement of the soft palate and 28 of the horses did not have any respiratory disorders and served as controls. Pathologic features observed included myonecrosis, phagocytosis, mononuclear cell infiltration of perimysial connective tissue, alkaline phosphatase-positive myofibers, and myofibers with cytoarchitectural changes that included irregular staining of the intermyofibrillar sarcoplasm and sarcoplasmic masses. These histopathologic features were judged to be indicative of myositis. Those features were found in both control horses and horses with dorsal displacement of the soft palate; however, their prevalence was higher in horses with dorsal displacement of the soft palate. For the control horses as well as the horses with dorsal displacement of the soft palate, the inflammatory changes were more prevalent in older horses. The finding of myositis suggests that palatal muscle weakness may contribute to the development of dorsal displacement of the soft palate; however, the myositis might have been secondary to trauma associated with displacement of the soft palate. We could not distinguish between these 2 possibilities. A large number of horses with dorsal displacement of the soft palate also had concurrent pharyngeal lymphoid hyperplasia. Extension of the mucosal inflammatory changes associated with pharyngeal lymphoid hyperplasia into the underlying palatine muscle might have accounted for the myositis.

Airway Obstruction↗

Vertical pendular nystagmus in chronic myositis of medial and lateral rectus.

A 38-year-old white woman developed left medical rectus myositis possibly due to an immune response to mepivacaine (Carbocaine) injected around her upper left first molar. Ten months after resolution, a recurrence of left medial restus myositis and also left lateral rectus myositis occured after the injection of chemically related lidocaine (Xylocaine) in the same area. At this time, with gaze to the left, there appeared vertical pendular nystagmus of greater amplitude in the involved eye, in the absence of systemic neurologic or posterior fossa disease. This association of vertical pendular nystagmus with chronic myositis of the medial and lateral rectus, to my knowledge, has not been previously reported.

Adult↗

Evaluation of laboratory tests as a guide to diagnosis and therapy of myositis.

Plasma CK concentrations have been widely used as the primary muscle enzyme marker for diagnosis and progression of myositis. Recently, total CK and CK-MB serum concentrations have been compared to, and used in conjunction with, serum concentrations of aspartate aminotransferase in diagnosis of myositis. The algorithmic use of CK, AST, and aldolase plasma concentrations to diagnose and categorize patients with myopathy may be a useful method of diagnosing specific muscle disease without invasive procedures. CAIII, as a specific marker for skeletal muscle damage, may replace CK as the enzyme of choice in diagnosis and progression of myositis and other muscle disease. Additional studies are required to determine the usefulness of carbonic anhydrase for the diagnosis and assessment of myositis.

Biomarkers↗

Ubiquitin expression in inclusion body myositis. An immunohistochemical study.

Ubiquitin has been shown by immunohistochemical studies to be a component of many of the filamentous inclusion bodies that are known in neuropathology. In the current study, we examined the expression of ubiquitin in 14 cases of typical inclusion body myositis, in skeletal muscle specimens from four cases of typical amyotrophic lateral sclerosis, and in muscle specimens from three normal controls. In the cases of inclusion body myositis, rimmed vacuoles were ubiquitin immunoreactive in all cases. Intrasarcoplasmic inclusions were positive in the nine cases that had them. In four cases, there were positive intranuclear inclusions, and in seven, there was homogeneous staining of nuclei. Atrophic fibers and necrotic fibers were positive in 11 and nine cases, respectively. In the cases of amyotrophic lateral sclerosis, atrophic fibers were positive in three cases, and focal nuclear staining was seen in two. In one of the three control cases, a few atrophic fibers had faint sarcoplasmic positivity; no other staining was seen. We conclude that ubiquitin is a component of the inclusions that characterize inclusion body myositis. However, ubiquitin expression in skeletal muscle disease is not pathognomonic of inclusion body myositis.

Adult↗

Symptomatic macroglossia and tongue myositis in polymyositis: treatment with corticosteroids and intravenous immunoglobulin.

Symptomatic macroglossia occurs in some rare congenital muscle diseases, such as Becker's and Duchenne's dystrophies or Pompe's disease. Herein we describe a case of symptomatic macroglossia with myositis of the tongue occurring in a patient with polymyositis. Tongue myositis was evidenced by dysarthria, frequent biting during mastication, swallowing difficulties without aspiration, and noisy breathing. Magnetic resonance imaging showed homogeneous hypertrophy of the tongue, especially the mouth's floor muscles. The diagnosis of tongue myositis was established by electromyography and biopsy. No other cause for the macroglossia was found. Symptoms resolved quickly with corticosteroid and intravenous immunoglobulin treatment. To our knowledge, this is the first reported case of symptomatic tongue myositis occurring in the course of polymyositis.

Humans↗

Acute rectus muscle palsy in children as a result of orbital myositis.

OBJECTIVE: Acute rectus muscle palsy caused by orbital myositis is often confused with infectious orbital cellulitis. The purpose of this article is to clarify the former condition by explaining how it is manifested and treated. METHODS: The seven children less than age 11 years in this study were examined after having an acute onset of ocular pain. Physical examination indicated a loss of motility in the field of action of the involved extraocular rectus muscle. Computed tomographic examination confirmed the existence of orbital myositis. Treatment consisted of intravenous or oral administration of corticosteroids. RESULTS: In all the initial evaluations, computed tomography showed the isolated rectus muscle to be enlarged. In four of the patients in whom the lateral rectus was involved, esotropia was present in the primary position. In the fifth patient with lateral rectus involvement, esotropia was seen only in the field of gaze of the involved lateral rectus muscle. The patient with medial rectus involvement had exotropia, and the patient with superior rectus involvement had hypotropia on the involved side. Each had injection and chemosis of the conjunctiva and Tenon fascia only in the quadrant overlying the involved rectus muscle. CONCLUSIONS: Children with orbital myositis can have an acute rectus muscle palsy. The inflammation and strabismus are exquisitely sensitive to systemic corticosteroid therapy, which produces a rapid resolution of symptoms. If the steroid therapy is discontinued abruptly, the myositis can recur.

Acute Disease↗

[Idiopathic orbital myositis].

INTRODUCTION: Orbital myositis is a subtype of nonspecific orbital inflammation confined to one or more of the extraocular muscles. EXEGESIS: Three female patients presented presumed orbital myositis, in each of whom the diagnosis was suspected clinically and confirmed by the computed tomography (CT) scan appearances. All presented with orbital pain, diplopia and periorbital edema. All patients responded to systemic corticosteroid with prompt resolution of symptoms and signs, but one patient had recurrent episodes of myositis, leading to the prescription of azathioprine. The clinical and radiological constellation of findings allowing a definitive diagnosis of the nonspecific orbital inflammatory syndrome in most cases is discussed. CONCLUSION: The nonspecific orbital inflammatory syndromes are a heterogenous group of conditions and the CT scan is a valuable tool in differentiating these conditions. Most cases of idiopathic orbital myositis require corticosteroids, but therapeutic alternatives to corticosteroids in refractory diseases are warranted.

Adult↗

Orbital granulomatous giant cell myositis: a case report and review.

To present a case of orbital granulomatous giant cell myositis and review the literature. We describe the case of a 51-year-old woman, with a past history of melanoma, who presented with an acutely painful orbital myositis. This evolved into a chronic relapsing process involving multiple muscles bilaterally; which demonstrated partial steroid responsiveness. Biopsy revealed a granulomatous giant cell myositis. Orbital granulomatous giant cell myositis is a rare histological entity which has an association with giant cell myocarditis and underlying malignancy.

Anti-Inflammatory Agents↗

Functional outcome and quality of life in adult patients with idiopathic inflammatory myositis.

OBJECTIVES: To present the outcome of patients with idiopathic inflammatory myositis, focusing on functional ability and quality of life. METHODS: Analysis was performed using data from 105 adult patients with definitive polymyositis, dermatomyositis or overlap myositis, who were followed up at a single centre. The diagnosis was made between 1979 and 2000 based on Bohan and Peter's criteria. Functional ability was assessed after a minimum follow-up of 3 yr with the Health Assessment Questionnaire Disability Index (HAQDI) and quality of life was measured with the Short Form 36-item questionnaire (SF-36). RESULTS: Fifteen patients in our cohort died and 87 participated in the evaluation of functional outcome. Functional ability after a median follow-up of 107.1 months (range 36.4-273.3) was heterogeneous. The median HAQDI score was 0.875 (range 0-2.875). Polyphasic or chronic-progressive disease course, osteoporosis and long-term follow-up were predictive of higher HAQDI scores. In terms of quality of life, significant differences from population norms were shown in all domains of the SF-36. There were no significant differences in the SF-36 scores among the patients according to clinicopathological subset or disease course. CONCLUSIONS: Although the mortality of our cohort was favourable, myositis continues to have a great impact on life in the medium and long term. The present work indicates that myositis patients have a significantly poorer quality of life than the normal population, but there was no difference among the patients according to clinicopathological subsets.

Adult↗

[A perspective on the control of myositis ossificans progressiva]

OBJECTIVE: To report four cases of myositis ossificans progressiva, its form of presentation, in addition to new control and treatment options. DESCRIPTION: The authors describe four cases of myositis ossificans progressiva, presenting its clinical manifestations, evolution, and available forms of control and treatment. The main congenital anomalies associated with myosistis ossificans progressiva are described, namely: hallux valgus, clinodactyly, short thumbs, exostosis, and syndactyly. The evolution and therapeutic responses of clinical improvement to oral ascorbic acid and biphosphonate in patients 1 and 4 are also described; patient 1 had a better response after receiving intravenous biphosphonate, while patients 2 and 3 showed clinical improvement after the administration of oral ascorbic acid. COMMENTS: Ascorbic acid relieves the pain and improves psychism in patients with myositis ossificans progressiva. Intravenous biphosphonate is an alternative to patients with significant worsening of the disease, especially those with ambulatory restrictions. The orientation towards avoiding recurrent trauma in myositis ossificans progressiva patients is important.

Journal Article↗

The arthritis of inflammatory childhood myositis syndromes.

OBJECTIVE: Arthritis has been an associated finding in juvenile dermatomyositis (JDM), but its prevalence, course, and response to therapy has not been well described. We investigated the frequency, course, and clinical and radiographic features in a large cohort of patients with JDM. METHODS: The charts of 94 patients with idiopathic myositis (1984-99) were reviewed: 80 JDM, 3 juvenile polymyositis (JPM), 5 amyopathic JDM, and 6 overlap myositis syndromes. Compiled data included demographics, clinical features, a detailed description of the arthritis, investigations (radiographs, autoantibodies), course, and response to therapy. All radiographs were independently reviewed by a single radiologist. RESULTS: Sixty-one percent (95% CI 50-72%) of patients with JDM had arthritis. The arthritis was reported a median 4.5 mo (range -73.6 to 76.6 mo) after the JDM onset. When compared to patients with no arthritis, the occurrence of arthritis was not significantly related to sex, race, positive antinuclear antibody or rheumatoid factor, calcinosis, nodules, vasculitis, or Raynaud's phenomenon. The initial involvement was pauciarticular in 67% and polyarticular in 33%. In the pauci group, asymptomatic knee effusions were the predominant finding (n = 19, 58%), and in 18 patients may have been the result of steroid therapy. Two patients evolved from a pauci onset to a polyarticular course. All responded to therapy (corticosteroids; 47 were taking other medications) with remission of the arthritis within a median of 2.0 mo (range 0.1-64.5 mo). However, the arthritis recurred in 39% as the corticosteroids were tapered. Four patients with JDM eventually required corticosteroid wrist injections, with resolution of the arthritis. The arthritis was nonerosive in all cases. No patient with JPM had arthritis. Three of 5 patients with amyopathic JDM and 4 of 6 with overlap myositis syndrome had a nonerosive polyarthritis. CONCLUSION: Nonerosive arthritis involving the knees, wrists, elbows, and fingers is a frequent manifestation of JDM and other idiopathic childhood myositis. The arthritis is seen early in the course of JDM and often responds to treatment. However, the arthritis may recur with tapering of corticosteroids despite remission of the JDM. In a significant proportion of JDM cases, arthritis is the major sequela and may warrant further medical therapy or intraarticular corticosteroid injections.

Adolescent↗

[Echography findings in orbital myositis].

In a retrospective study comprising 12 patients with the diagnosis or orbital myositis the authors present an account on the importance of standardized echography for the differential diagnosis and for the follow-up of the disease in relation to treatment. They describe the sonographic finding in affected oculomotor muscles and in myositis of the upper oblique muscle incl. the echographic finding of trochleitis. The authors discuss echographic signs of myositis (thickness and shape of muscle, reflectivity and internal structure) in image A and image B and their correlation with the histopathological finding. The authors discuss differential diagnostically important differences of the echographic picture of myositis, endocrine myopathy and others.

Adult↗

[Arrector pili myositis in a patient with dermatomyositis: an unusual histological feature].

Dermatomyositis is a rare inflammatory disease with a difficult diagnosis on cutaneous biopsies, since alterations are often little marked and non specific. A 52-year-old patient had severe paraneoplastic dermatomyositis. He had an erythematous rash on the trunk, and erythematous papules on the back of the hands. The first histological cutaneous lesion was a striking arrector pili myositis. Arrector pili myositis has rarely been described, and is thought to be linked with Wong type eruption occurring in dermatomyositis. Arrector pili myositis might constitute an helpful sign in the diagnosis of dermatomyositis, but its specificity and sensitivity are unknown. We compared this case to four previously reported cases. In these five cases, arrector pili myositis was not associated with a particular sub-type of dermatomyositis or with poor prognosis.

Biopsy↗