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Query: UMLS:C0027121 (myositis)
 4,538 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Dermatomyositis and polymyositis are the two major idiopathic inflammatory myopathies. The Bohan and Peter's criteria are still useful despite the probably different pathogenesis of the two myopathies. Cutaneous manifestations of dermatomyositis include heliotrope rash and Gottron's papules. The heliotrope rash, with or without edema, in a distribution involving periorbital skin is very suggestive of the diagnosis. Papules may be found overlying the "kneedle" of the hand or the elbows, knees, feet. Periungueal erythema with telangiectasis were characteristic but not pathognomonic. Scalp involvement is common. Skin lesions of dermatomyositis may precede the development of the myopathy and may persist after the control of the myositis. Some patients have an amyopathic dermatomyositis with normal muscle-enzyme, magnetic resonance scan and muscle biopsy. Muscle disease affects the proximal muscles, is generally symmetrical and symptoms are fatigue, weakness and sometimes myalgia. Proximal dysphagia reflects an involvement of striated muscle of the pharynx or proximal esophagus. Camptocormia reflects a severe involvement of paravertebral muscle. Other systemic features may be seen: pulmonary involvement (mostly interstitial pneumonitis and hypoventilation), arthralgias or arthritis, cardiac involvement, vasculatis and calcinosis particularly in children or adolescents with dermatomyositis. Malignant disease is associated with idiopathic inflammatory myopathies with a frequency of approximatively 10 to 15% in dermatomyositis and 5 to 10% in polymyositis and is strongly correlated with age, more than 50% of the patient over 65 years old were found to have a cancer. In the absence of malignant disease, the mainstay therapy for dermatomyositis and polymyositis is systemic corticosteroids (mostly 1mg/kg). In the lake of response or high dose dependance, intravenous immunoglobulins or immunosuppressive drugs like methotrexate or azathioprine may be discuss. Cyclophosphamide show some effectiveness in interstitial pneumonitis. Cyclosporin might be effective in children, less in adults. The efficacy of tacrolimus, mycophenolate mofetil, leflunomide and anti-TNF therapy need some prospective studies to determine if there are of value in idiopathic inflammatory myositis.
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PMID:[Dermatomyositis and polymyositis: clinical aspects and treatment]. 1196 87

The group of idiopathic inflammatory myopathies encompasses polymyositis, dermatomyositis and inclusion body myositis. These diseases share the following features: progressive muscle weakness, an increase in serum creatine kinase activity and the presence of mononuclear cell infiltrates in the muscle biopsy. Polymyositis, dermatomyositis and inclusion body myositis are differentiated on the basis of the distribution of muscle weakness, and specific histopathological features. Many specialties may see these patients as the clinical presentation can vary widely and may be atypical, requiring further diagnostic procedures. A 40-year-old man with a heliotrope rash and periorbital oedema, but no muscle involvement, was diagnosed with dermatomyositis sine myositis. He was successfully treated with corticosteroids but died later of cardiac failure. A 72-year-old man with a pulmonary malignancy subsequently developed the clinical features of dermatomyositis. Steroid therapy diminished the complaints but he died of pulmonary embolism. A 54-year-old woman with the clinical features of inclusion body myositis did not have rimmed vacuoles in her muscle biopsy specimen and was initially erroneously diagnosed with polymyositis, for which she was treated with corticosteroids, but without beneficial effect.
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PMID:[Three patients with divergent presentations of idiopathic inflammatory myopathy]. 1203 18

Tumor necrosis factor receptor-associated periodic syndrome (TRAPS) is a dominantly inherited autoinflammatory syndrome that results from mutations in TNFRSF1A, the gene that encodes the 55-kd tumor necrosis factor receptor. Clinically, patients present with recurrent episodes of fever in conjunction with localized inflammation at various sites. Myalgia is one of the most characteristic features of this syndrome and is frequently associated with an overlying erythematous, macular rash that, together with the myalgia, displays centrifugal migration. This has previously been believed to occur as a result of myositis. We describe herein the case of a 60-year-old man with TRAPS, in whom magnetic resonance imaging of the left thigh demonstrated edematous changes in the muscle compartments and surrounding soft tissues. A full-thickness wedge biopsy was performed, and hematoxylin and eosin staining and immunohistochemistry analysis of the specimen demonstrated normal myofibrils but a severely destructive monocytic fasciitis. These results suggest that the myalgia experienced by individuals with TRAPS is due to a monocytic fasciitis and not to myositis.
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PMID:Monocytic fasciitis: a newly recognized clinical feature of tumor necrosis factor receptor dysfunction. 1279 52

The hallmark of the inflammatory myopathies is muscle weakness. Although this feature can lead to significant disability and impairment of activities of daily living, its initial presentation may not be recognized early. Older individuals, in particular, may feel that the changes caused by myositis reflect the effects of aging rather than those of a disease process, and diagnosis, therefore, may be delayed. This factor has negative impact on the response to therapy. Inclusion body myositis, with its insidious onset in older people, and laboratory findings which may not be markedly abnormal, presents a diagnostic challenge. DM, with its characteristic symptomatic rash, is generally brought to medical attention more quickly. Another area of diagnostic concern occurs when associated organ involvement precedes myopathy. This has been observed, for example, with interstitial lung disease, and again represents a challenge to physicians. In this connection, the antisynthetase syndrome presenting with fevers, Raynaud's features, arthritis, or pulmonary involvement may not initially be recognized as a manifestation of inflammatory muscle disease. Each subgroup of IIM may present with a variety of extramuscular features that can complicate diagnosis and alter therapy and prognosis. This is particularly true for the pulmonary, GI, and cardiac manifestations and when cancer is associated with myositis. For these reasons, such features of IIM should be carefully evaluated, treated, and monitored over the course of the illness; in some cases these may play a greater role in determining the outcome of patients with IIM than the muscle involvement itself. It is hoped that in the future increased familiarity with the manifestations of the inflammatory myopathies, together with a better understanding of the underlying pathogenesis, will lead to more rapid diagnosis and more effective treatments.
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PMID:Clinical presentation of the idiopathic inflammatory myopathies. 1250 74

Twenty-eight patients with Japanese spotted fever were clinically investigated. The diagnosis was determined by confirming an increase of specific antibody. All patients were treated with minocycline, and all recovered, excluding one patient with a fulminant course. Fever and exanthema were observed in all patients, and an eschar was pointed out in 20 (71%) patients. The platelet count was 10 x 10(4)/microl or lower in 8 (28%) patients. The fibrin degradation product (FDP)-level was abnormally high, 10 microg/ml or more, in 16 (57%) patients. The creatine kinase (CK) value was high in 14 of 22 patients, suggesting the presence of myositis. The leukocyte count, FDP, C-reactive protein, and soluble interleukin 2 receptor (sIL2-R) levels were significantly higher in severe cases. In the group without concomitant steroid therapy, mean times of 54.7 h and 101.4 h were required to reduce the temperature to 38 degrees C and 37 degrees C or lower, respectively, after the initiation of tetracycline treatment. There were 6 severe cases: 1 with disseminated intravascular coagulation, 2 with multiorgan failure, 1 with acute respiratory distress syndrome, and 2 with meningoencephalitis. These severe cases formed a group that required 6 or more days to initiate therapy after the onset (P < 0.005 vs non-severe group), showing that delay in diagnosis and therapy is the major cause of aggravation. In the 2 patients complicated by multiorgan failure, the sIL2-R level, produced by activated lymphocytes, was 10,000 U/ml or higher, suggesting that an sIL2-R level of more than 10,000 U/ml can be used as a marker of poor prognosis. It may be better that moderate to severe cases are treated with minocycline plus short-term steroid therapy.
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PMID:Clinical study of Japanese spotted fever and its aggravating factors. 1267 13

We present two cases of interstitial pneumonia with anti-Jo-1 antibodies in the absence of myositis. The first patient was a 38-year-old woman and the second, a 59-year-old woman. Both patients were admitted to our hospital complaining of dry cough and dyspnea on effort. The diagnosis of interstitial pneumonia was made from chest radiography, computed tomography and surgical lung biopsy. Anti-Jo-1 antibodies, which were highly specific for polymyositis and dermatomyositis (PM/DM), were detected in both patients. However, the serum creatine kinase concentrations and electromyographic findings in both patients were normal, and no clinical signs (including muscle weakness, rash and arthralgia) were found. In the first patient, oral prednisolone (PSL) treatment (20 mg day) improved the interstitial pneumonia, but PSL has now been tapered to 17.5 mg day. In the second patient, oral PSL treatment (40 mg day) improved interstitial pneumonia, and the dose was tapered to 5 mg day. The second patient was followed for more than 10 years after treatment, but she has never shown any signs of clinical myositis. Further investigation will be required, because no pathophysiological relation between anti-Jo-1 antibodies and interstitial pneumonia with PM DM has yet been established.
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PMID:[Two cases of interstitial pneumonia with anti-Jo-1 antibodies in the absence of myositis]. 1458 96

Transient weakness of the legs developed in a 17 year-old male high school student three weeks after the initial injection of a hepatitis B vaccine. Seventeen days after the second injection of the vaccine, low-grade fever, a pruritic maculopapular rash appeared and weakness of the legs recurred. This was associated with elevation of the creatinine kinase to 2,406 U/L. The day after admission he became afebrile and in the subsequent four days the rash improved but leg weakness persisted. One-month later, muscle strength had returned; and the creatinine kinase had returned to normal levels. The only case of dermatomyositis associated with hepatitis B vaccination and the findings in the six reported cases of surface antigen-positive hepatitis associated with polyomyositis or dermatomyositis are briefly reviewed. Hepatitis B vaccination should be encouraged, but it is important to be aware that, rarely, dermatomyositis, polymyositis or neurovascular complications may occur. Polymyositis associated with the administration of the hepatitis B vaccine or with hepatitis B virus infection is a rare occurrence. A Medline Search performed from 1960 to January 2002 associating hepatitis B vaccine or hepatitis B virus with myopathy, myositis, polymyositis and dermatomyositis, showed only one case of dermatomyositis related to the hepatitis B vaccine, and six case reports relating polymyositis to hepatitis B virus infection. We present a case where a causal relationship between polymyositis and hepatitis B vaccination appears quite likely.
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PMID:Polymyositis: rare complication of hepatitis B vaccination. An unusual cause of toxic shock syndrome. 1544 86

Several reports over the past 15 years describe severe group A streptococcal infections causing septic shock, soft-tissue necrosis, and multiple organ failure; a phenomenon known as streptococcal toxic shock-like syndrome (TSLS). However, primary peritonitis associated with TSLS is rare. We report the case of a 40-year-old man admitted with pain in both thighs, hypotension, and severe abdominal pain. His daughter had been diagnosed with streptococcal pharyngitis 3 days earlier. We performed an emergency laparotomy for peritonitis, and culture of the ascites was positive for group A beta -hemolytic streptococcus (GAS). Further serotyping of the isolated GAS strain revealed the T-type 22 and the pyrogenic exotoxin gene, spe-C. The criteria for TSLS were clearly met, including the isolation of GAS from ascites, hypotension, liver failure, renal failure, coagulopathy, myositis, and a generalized erythematous macular rash with desquamation.
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PMID:Primary peritonitis associated with streptococcal toxic shock-like syndrome: report of a case. 1558 Mar 92

The patient is a 56-year-old Japanese woman who suffered from breast cancer and ovarian cancer at intervals of 6 years, and was also complicated by two episodes of dermatomyositis, each of which occurred simultaneously with each of two cancers. When she was 51 years old, she developed dermatomyositis for the first time 6 months after the resection of breast cancer, whose histological type was tubular adenocarcinoma. The dermatomyositis remitted without oral corticosteroids in 2 months, and the remission had continued for 6 years. However, at the age of 56, dermatomyositis abruptly recurred with a pruritic generalized rash, Gottron's papules and elevated serum CK levels. Examination for malignancy revealed an ovarian tumor, which was diagnosed as serous papillaly adenocarcinoma, and the surgery was performed. After the resection of the ovarian cancer, skin rash was improved dramatically and CK levels were normalized again without oral corticosteroids. Since there were no evidences of recurrence of the breast cancer, it was considered that each episode of dermatomyositis was associated with each of the cancers, respectively. We report this rare and interesting case to consider the etiology of cancer-associated myositis as a paraneoplastic syndrome, since the two cancers have different histological types.
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PMID:[A case of dermatomyositis associated with different types of cancers at intervals of six years]. 1567 98

The idiopathic inflammatory myopathies (IIM) of childhood are rare, multisystem autoimmune disorders, of which the most common is juvenile dermatomyositis (JDM). The criteria currently used to diagnose the paediatric IIMs, including both JDM and other childhood autoimmune conditions in which myositis may be a prominent feature, are somewhat outdated in relation to paediatric practice. Controversies surrounding the criteria for diagnosis have resulted in an international effort to define both the diagnostic and classification criteria in light of modern investigation and practice. Clinical features of these IIMs include muscle weakness and skin rash; however, these may be absent at disease onset. JDM patients require careful assessment of multiple organ systems, which can divided into musculoskeletal and extra-musculoskeletal, and examination should include validated disease measurement tools such as the Childhood Myositis Assessment Scale. Investigations include blood tests to assess generalised markers of inflammation as well as more specific markers of muscle inflammation; organ-specific investigations, such as MRI, and muscle biopsy are also often used. Treatment and management protocols include corticosteroids, methotrexate and other disease-modifying agents such as ciclosporin (cyclosporin) and intravenous immunoglobulin, as well as newer treatments such as tumour necrosis factor blockade or B-cell depletion. Management of children with JDM requires a multidisciplinary approach, including specialist physiotherapy, occupational therapy and nursing input. Two major international projects, the International Myositis and Clinical Studies Group (IMACS) and Paediatric Rheumatology International Trials Organisation (PRINTO) aim to standardise the assessment of these patients and measurement of their disease. The efforts of these large collaborative groups should provide much needed networks for mulitcentre trials in the future.
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PMID:Paediatric idiopathic inflammatory muscle disease: recognition and management. 1597 68


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