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Inclusion body myositis associated with progressive dysphagia: treatment with cricopharyngeal myotomy.

A 68-year-old man known to have inclusion body myositis underwent a cricopharyngeal myotomy in an attempt to improve his progressive dysphagia. Morphological studies from tissues obtained during this procedure showed the diagnostic features typical of this chronic inflammatory myopathy. To our knowledge this is the first pathological demonstration of inclusion body myositis involving the pharyngeal skeletal musculature.

Aged↗

Management of strabismus due to orbital myositis.

We report on 5 consecutive patients seen at the botulinum toxin clinic at Moorfields Eye Hospital with an ocular motility disorder secondary to orbital myositis. CT scans demonstrated involvement of one or both of the medial recti in the inflammatory process in all 5 patients. In addition 1 patient had involvement of both the lateral recti and the right superior rectus. Two patients had been treated with oral steroids, 3 with non-steroidal anti-inflammatory agents, and 1 with orbital radiotherapy. Prior to toxin injection 3 patients had an esotropia (ranging from 4 delta to 30 delta) and two an exotropia (52 delta and 85 delta). A vertical imbalance was present in 3, and all 5 patients had symptomatic diplopia. A total of six injections were given to 5 patients, 2 of whom later went on to have surgery. Toxin injection reduced the angle of the deviation to less than 10 delta in 4 patients, all of whom are now asymptomatic. The fifth patient has persistent diplopia despite two operations to correct a large exotropia. We discuss the role of botulinum toxin and surgery in the management of strabismus due to orbital myositis.

Adolescent↗

Acute orbital myositis.

We examined 9 consecutive cases of unilateral orbital myositis (7 women and 2 men; age range 15-46 years) presenting to Casualty. Only 3 were correctly diagnosed on the first visit. Eight patients exhibited globe retraction in the acute stages and, after treatment with systemic steroids, all made a full recovery. None of the patients had associated systemic disease and all remain well over a 6-12 month follow-up period. Orbital myositis may be a more common condition than previously thought; it can present with a variety of clinical signs, and may be difficult to diagnose in the early stages. The presence of globe retraction on movement of a painful, injected eye is a useful diagnostic sign which indicates inflammation of extraocular muscles and is present in the acute stages of the disease; we explain how to elicit globe retraction and suggest a management protocol for these patients.

Adolescent↗

Psoriasis, psoriatic arthropathy and relapsing orbital myositis.

Orbital myositis is an inflammatory disorder of the orbital muscles causing orbital pain and restriction of eye movements. Although rare in children, it is most frequently seen after orbital trauma or as a post-infectious process. We describe a child with chronic relapsing psoriasis, juvenile psoriatic arthritis and relapsing bilateral orbital myositis.

Arthritis, Psoriatic↗

Extraocular muscle myositis and restrictive strabismus in 10 dogs.

Ten cases of uni- or bilateral restrictive ventromedial strabismus in young dogs of different breeds are reported. Clinically, abnormalities were restricted to the extraocular muscles with sparing of the masticatory muscles and limb muscles. This was supported in some cases by imaging studies, electrophysiology, and immunocytochemical assay for antibodies against type 2M fibers. Histologically, there was variable lymphocytic plasmacytic mononuclear cell infiltration and fibrosis. This disorder is similar in many aspects to chronic masticatory myositis with focal myositis and subsequent fibrosis. Surgical correction may restore eye position and vision.

Journal Article↗

A case of atypical idiopathic orbital myositis mimicking neurological disease.

A 24 year old patient with acute double vision and headache was admitted to our clinic. A diagnosis of orbital myositis was confirmed after magnetic resonance imaging and orbital echogram. No other clinical manifestations were observed on general examination. Since orbital myositis cases with only neurological symptoms and without typical visual impairment are not frequent, we decided to present this case.

Adult↗

Dermatomyositis associated with Lyme disease: case report and review of Lyme myositis.

Myositis due to the etiologic agent of Lyme disease, Borrelia burgdorferi, has been reported nine times in the English-language literature; there has been but a single report of exacerbation of dermatomyositis due to B. burgdorferi in a patient with known dermatomyositis. Multiple infectious agents, but not B. burgdorferi, have been hypothesized to trigger dermatomyositis. We report the first case of dermatomyositis that appears to have been triggered by B. burgdorferi. This case involved an individual from Westchester County, NY, who presented with skin lesions suggestive of erythema migrans and who was seropositive for Lyme disease. He soon developed a clinical syndrome suggestive of dermatomyositis: periorbital edema, dysphagia, proximal muscle weakness, and a markedly elevated level of creatine phosphokinase. We also review the clinical presentation and response to treatment of patients with Lyme myositis.

Anti-Bacterial Agents↗

A case of atorvastatin combined toxic myopathy and inflammatory myositis.

Muscle toxicity is one adverse reaction reported with the use of 3-hydroxy-3-methylglutaryl-coenzyme A (HMG-CoA) reductase inhibitors (statins). This toxicity may include asymptomatic elevations of muscle enzymes, weakness, myalgia, and myositis. High doses of statins, or the combination of statins with other anti-cholesterol medications, increase the risk of toxicity. In addition, case reports of systemic and autoimmune reactions such as lupus, nephritis, vasculitis, and myositis, suspected to be associated with statins, have been reported. Our 76-year-old patient demonstrates a case of serologically and biopsy-proven inflammatory polymyositis, combined with a statin toxic myopathy. His symptoms and enzyme abnormalities resolved with both the removal of the statin medication and the institution of immunosuppressive therapy. Investigation of muscle enzyme elevation and weakness that do not resolve with statin removal is warranted. Certain muscle biopsy findings, including mononuclear cell infiltrate, distinguish the etiology as inflammatory/possibly autoimmune and do not suggest statin myopathy.

Journal Article↗

Laminin-induced autoimmune myositis in rats.

The present study aimed to examine if immunization with laminin causes myositis in rats and whether the pathologic findings mirror human polymyositis and dermatomyositis. Rats were immunized with an emulsion of laminin and complete Freund's adjuvant. As a result, muscle fiber necrosis with infiltrating macrophages was frequently observed and mononuclear cells were observed in the endomysium. These mononuclear cells were composed of CD4+ cells, CD8+ T cells, and macrophages. CD4+ cells and CD8+ T cells were mainly located in the endomysium, whereas a large number of macrophages were located in the endomysium and infiltrating muscle fibers. A small number of B cells, detected by immunohistochemical staining, were mainly located in the perimysium. The nonnecrotic muscle fiber to which CD4+ T cells, CD8+ T cells, and perforin+ cells adhered was negative for antimerosin and antidystrophin antibodies. Muscle fiber necrosis in rats immunized with laminin may occur after denaturation of basement membrane proteins. In conclusion, the immunization with laminin induces moderate to severe myositis. We suggest that laminin may be an important antigen for connective tissue diseases such as polymyositis and dermatomyositis.

Animals↗

Acute orbital myositis. Case report.

The case of 22-year old, white woman with bilateral orbital myositis following an acute upper respiratory tract infection is reported. The most important clinical findings were ocular pain, proptosis, restricted eye motility and swelling of the eyelids. The enlarged eye muscles were seen on orbital computerized tomography scan. The clinical findings of inflammatory orbital myositis and clinical response to corticotherapy are emphasized.

Acute Disease↗

Paralytic autoimmune myositis develops in nonobese diabetic mice made Th1 cytokine-deficient by expression of an IFN-gamma receptor beta-chain transgene.

Nonobese diabetic (NOD) mice and some human type 1 diabetes (T1D) patients manifest low to high levels of other autoimmune pathologies. Skewing their cytokine production from a Th1 (primarily IFN-gamma) to a Th2 (primarily IL-4 and IL-10) pattern is a widely proposed approach to dampen the pathogenicity of autoreactive diabetogenic T cells. However, it is important that altered cytokine balances not enhance any other autoimmune proclivities to dangerous levels. Murine CD4 T cells are characterized by a reciprocal relationship between the production of IFN-gamma and expression of the beta-chain component of its receptor (IFN-gamma RB). Thus, NOD mice constitutively expressing a CD2 promoter-driven IFN-gamma RB transgene in all T cells are Th1-deficient. Unexpectedly, NOD.IFN-gamma RB Tg mice were found to develop a lethal early paralytic syndrome induced by a CD8 T cell-dependent autoimmune-mediated myositis. Furthermore, pancreatic insulitis levels were not diminished in 9-wk-old NOD.IFN-gamma RB Tg females, and overt T1D developed in the few that survived to an older age. Autoimmune-mediated myositis is only occasionally detected in standard NOD mice. Hence, some manipulations diminishing Th1 responses can bring to the forefront what are normally secondary autoimmune pathologies in NOD mice, while also failing to dependably abrogate pancreatic beta cell destruction. This should raise a cautionary note when considering the use of protocols that induce alterations in cytokine balances as a means of blocking progression to overt T1D in at-risk humans.

Adoptive Transfer↗

Inhibition of CX3CL1 (fractalkine) improves experimental autoimmune myositis in SJL/J mice.

Idiopathic inflammatory myopathy is a chronic inflammatory muscle disease characterized by mononuclear cell infiltration in the skeletal muscle. The infiltrated inflammatory cells express various cytokines and cytotoxic molecules. Chemokines are thought to contribute to the inflammatory cell migration into the muscle. We induced experimental autoimmune myositis (EAM) in SJL/J mice by immunization with rabbit myosin and CFA. In the affected muscles of EAM mice, CX3CL1 (fractalkine) was expressed on the infiltrated mononuclear cells and endothelial cells, and its corresponding receptor, CX3CR1, was expressed on the infiltrated CD4 and CD8 T cells and macrophages. Treatment of EAM mice with anti-CX3CL1 mAb significantly reduced the histopathological myositis score, the number of necrotic muscle fibers, and infiltration of CD4 and CD8 T cells and macrophages. Furthermore, treatment with anti-CX3CL1 mAb down-regulated the mRNA expression of TNF-alpha, IFN-gamma, and perforin in the muscles. Our results suggest that CX3CL1-CX3CR1 interaction plays an important role in inflammatory cell migration into the muscle tissue of EAM mice. The results also point to the potential therapeutic usefulness of CX3CL1 inhibition and/or blockade of CX3CL1-CX3CR1 interaction in idiopathic inflammatory myopathy.

Animals↗

Myositis overlap syndromes.

Myositis-overlap syndromes are characterized by a heterogeneous group of clinical syndromes of which many are closely linked with specific autoantibodies. Clinical and laboratory-based research into this fascinating group of syndromes have given us clues in elucidating the role of immunogenetic, environmental influences on the etiopathogenesis of autoimmune disease. Patients positive for anti-snU1 RNP antibody often present with a constellation of "autoimmune-related" clinical features, often not fulfilling the diagnostic criteria for a single disease entity. Controversy remains as to whether this represents a single disease entity, namely mixed connective tissue disease, or an undifferentiated autoimmune rheumatic/overlap syndrome. Anti-PM-Scl antibodies are linked with myositis-scleroderma overlap and generally tend to follow a benign course, often responding well to minimal immunosuppression. More recently the number of anti-tRNA synthetase antibody-associated overlap syndromes has expanded; antibodies to the autoantigen histidyl-tRNA synthetase (Jo1) being the commonest and best characterized. Patients with these antibodies often have severe interstitial lung disease and the poorest prognosis, often necessitating aggressive immunosuppressive therapy.

Autoimmune Diseases↗

[A case of pseudophlebitis of the great saphenous vein: focal nodular myositis of the gracilis muscle].

A case of focal myositis in a healthy 68-year-old woman is described. The patient was admitted for evaluation of a painful soft-tissue mass localised on the medial side of the left thigh, initially misdiagnosed as thrombophlebitis of the v. saphena magna. Laboratory data were normal, in particular sedimentation rate and muscle enzyme levels. After exclusion of venous thrombosis, the mass localised in the left m. gracilis was surgically removed. Histologic examination of the biopsy specimen showed muscle cell necrosis and severe inflammation, with lymphocytic infiltration leading to the diagnosis of focal myositis. This is a rare benign inflammatory pseudotumour of skeletal muscle. The aetiology and pathogenesis of the disease remain unclear. It is most commonly seen in the lower extremities and may mimic thrombophlebitis or soft-tissue neoplasm. Ultrasound and magnetic-resonance scans are helpful, but definitive diagnosis is obtained only by histology. Because recurrent lesions in other skeletal muscles are possible, and a third of patients develop polymyositis, a follow-up of several years is recommended.

Aged↗

[A case of orbital myositis complicated with optic neuropathy--analysis of the pathological mechanism of optic neuropathy from magnetic resonance imaging findings].

BACKGROUND: We observed a rare case of orbital myositis involving the optic nerve. CASE: A 52-year-old woman complained of visual disturbance, lid swelling, ocular pain, and conjunctival injection in her right eye. Her corrected vision was 0.15 in the right eye and 1.2 in the left eye. Relative afferent pupillary defect(RAPD) and central scotoma in visual field test were noted in the right eye. Ocular movement of her right eye was moderately disturbed in all directions. Although laboratory data showed elevation of erythrocyte sedimentation rate, other hematological data such as thyroid function, autoimmune antibodies, and viral infection antibodies were normal. Magnetic resonance imaging(MRI) findings showed compression of the optic nerve at the orbital apex by marked thickening of the right lateral rectus muscle and superior rectus muscle, and inflammation directly invading the optic nerve. Based on the above findings, we diagnosed the case as orbital myositis complicated with optic neuropathy, and started corticosteroid therapy. Her right corrected vision improved dramatically, and the RAPD, central scotoma, and lid swelling disappeared shortly after administration. Enlargement of the extraocular muscles was still present one month after corticosteroid therapy. CONCLUSION: MRI findings suggested that optic neuropathy in this case was induced not only by mechanical compression by the enlarged extraocular muscles at the orbital apex but also by direct inflammatory infiltration from the extraocular muscles.

Female↗

Development of the myositis activities profile--validity and reliability of a self-administered questionnaire to assess activity limitations in patients with polymyositis/dermatomyositis.

OBJECTIVE: To develop a disease-specific questionnaire for assessing limitations in activities of daily life, the Myositis Activities Profile (MAP), and to investigate its validity and reliability. METHODS: Groups of 10, 27, 31, and 17 patients with polymyositis (PM) or dermatomyositis (DM) participated in different parts of the study. In the first draft of the MAP, patients rated their difficulty and experienced importance of selected activities from the International Classification of Impairments, Disabilities, and Handicaps (ICIDH)-2 Beta-2 draft. The 37 highest rated activities formed a second draft of the MAP, which was analyzed for internal redundancy and consistency. For construct validity a third draft was correlated with CPK levels, the Functional Index in myositis (FI), the Arthritis Impact Measurement Scales-2 (AIMS2), the Health Assessment Questionnaire (HAQ), and subjective global disease impact. Test-retest reliability over one week was investigated. RESULTS: There were several expected correlations (rs > 0.50) between subscales of the MAP and corresponding subscales of the AIMS2, and a 31 item MAP correlated moderately with the HAQ (rs = 0.70) and less with the FI (rs = 0.55), subjective global disease impact (rs = 0.43), and CPK levels (rs = 0.17). No systematic differences were found between test and retest, and weighted kappa coefficients ranged from Kw = 0.56 to 0.77. CONCLUSION: The MAP seems to be a valid and reliable method for assessing activity limitations in patients with PM and DM.

Activities of Daily Living↗

[Clinical and radiological features and clinical course of orbital myositis].

Orbital myositis is idiopathic inflammation of the extraocular muscles in the absence of thyroid orbitopathy and often is included under broad description pseudotumor. We report here a series of three cases. Data from literature, combined with our own results yield a distinguishing pattern of orbital myositis suggesting that the term "orbital pseudotumor" is no longer a useful concept. The diagnostic criteria purposed here are: acute orbital pain exacerbated on eye movement; enlargement of one or more extraocular muscles with the respect of other orbital structures on muscle CT scan; absence of clinical and biological thyroid dysfunction: absence of signs of anterior uveitis or scleritis or visual decrease; rapid response to immunomodulator treatment.

Adult↗

[Orbital myositis, recurrence of Whipple's disease].

Whipple's disease, a chronic systemic and multi-organ disease can be associated with ophthalmological manifestations with or without CNS involvement. Myositis rarely occurs during Whipple's disease. Antibiotic treatment prevents CNS recurrence. We report here the association of an orbital pseudotumor with Whipple's disease. This myositis was discovered on a follow-up CT examination. It appeared only one year after the end of a long course of antibiotic treatment, but it resolved within a few weeks when treatment was reintroduced. Relations between these two diseases are discussed.

Eye Diseases↗