Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027121 (myositis)
 4,538 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Myositis is recognized as one of several neurologic complications encountered in Kawasaki disease. We report an unusual patient with Kawasaki disease which was complicated by severe myositis and respiratory failure secondary to weakness. Comparison of our patient with previously reported cases leads us to conclude that myositis in Kawasaki disease usually is present within 1-3 weeks of illness; the symptoms usually are mild and probably remain unnoticed by most physicians, but weakness can be severe and respiratory failure may occur. The degree of creatine kinase elevation may be useful in predicting the severity of myopathy and in alerting the physician to the need for close respiratory monitoring. Therapy is supportive and complete recovery is to be expected.
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PMID:Myositis in Kawasaki disease. 234 31

During the last several years, there has been increasing interest in the use of intravenous immune globulin for immunosuppression. Although the mechanism(s) of action remains to be delineated, immune globulin therapy has been shown to be effective in some antibody-mediated disorders. In Rh disease, antibody-induced cytopenias, myasthenia gravis, and the clotting disorder associated with anti-factor VIII antibody, intravenous immune globulin has had therapeutic benefit. It was of interest that intravenous immune globulin may partially ameliorate the tendency toward dilation of the coronary vessels in Kawasaki disease. If this disorder represents a vasculitis of the small feeding vessels of the coronary arteries, could this agent influence other forms of collagen vascular disease? Pilot studies in dermatomyositis and polymyositis and systemic juvenile rheumatoid arthritis indicate benefit from intravenous immune globulin therapy. In these studies we used a high-dose protocol consisting of 1 gm/kg/day of immune globulin for 2 days every 4 weeks. In patients with either myositis or systemic juvenile rheumatoid arthritis, beneficial effects were seen. In the former group of patients, increased proximal muscle strength and reduction in creatine kinase levels were observed. In the latter group of patients a marked reduction in systemic features was observed. The amount of corticosteroids required was reduced in both groups of patients. These studies indicate the potential for intravenous immune globulin in collagen vascular disorders and the need for carefully controlled trials of this form of therapy.
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PMID:The use of intravenous immune globulin in collagen vascular disorders: a potentially new modality of therapy. 279 2

Systemic pathological alterations were studied in thirty-seven autopsied patients with Kawasaki disease. Systemic vasculitis was the most characteristic pathological finding and was present in all the patients. In addition to the vasculitis, there was a high incidence of inflammatory lesions in various organs and tissues: in the heart, endocarditis, myocarditis, and pericarditis; in the digestive system, stomatitis, sialoduct-adenitis, catarrhal enteritis, hepatitis, cholangitis, pancreatitis, and pancreas ductitis; in the respiratory system, bronchitis and segmental interstitial pneumonia; in the urinary system, focal interstitial nephritis, cystitis, and prostatitis; in the nervous system, aseptic leptomeningitis, choriomeningitis, gangliontis, and neuritis; in the hematopoietic system, lymphadenitis, splenitis, and thymitis. Dermatitis, panniculitis or myositis were also observed in some patients. Therefore, Kawasaki disease is a systemic inflammatory disease which mainly affects the cardiovascular system. These systemic inflammatory lesions are considered to correspond to the variegated clinical manifestaitions. The relationship between Kawasaki disease and infantile polyarteritis nodosa (IPN) were discussed, based on the clinicopathological characteristics.
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PMID:General pathology of Kawasaki disease. On the morphological alterations corresponding to the clinical manifestations. 744 9

We present two cases of polymyositis (PM) associated with interstitial pneumonia (IP) whose sera contain autoantibodies to OJ (isoleucyl tRNA synthetase). The first patient is a 51 year-old female who was diagnosed as rheumatoid arthritis (RA) and treated with gold and corticosteroid at another hospital. She was admitted to Keio University Hospital due to worsening of dyspnea on exertion and polyarthritis. Laboratory findings revealed elevation of serum CK and LDH. A diagnosis of PM was made based on the myogenic pattern of EMG and pathological feature by muscle biopsy. Chest radiography and CT showed interstitial fibrosis. Because of clinical deterioration, the dose of corticosteroid was increased (prednisolone 50 mg/day) and her symptom was stabilized. The second patient, a 62 year-old male, was admitted to Kawasaki Municipal Hospital because of dyspnea on exertion, polyarthritis, and fever. He was diagnosed as PM associated with IP on the basis of his clinical and laboratory findings, and chest radiography. He was treated with methylprednisolone pulse therapy (800 mg/day for three days) and his symptoms were improved. Both patients were found to have autoantibodies to OJ. Autoantibodies to aminoacyl tRNA synthetase have been described to be associated with myositis and/or IP. In North American, it was reported that all patients with anti-OJ had either myositis or IP or both. This suggests that anti-OJ was commonly associated with the anti-synthetase syndrome observed with other anti-synthetases. This is the first report of Japanese patients with anti-OJ antibody. The clinical features of these patients were likely to be similar to those observed in North American patients. However, further studies are necessary to clarify the precise clinical significance of this antibody.
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PMID:[Two cases of polymyositis associated with interstitial pneumonia with anti-OJ (isoleucyl tRNA synthetase) antibodies]. 972 63

Kawasaki's disease is an inflammatory syndrome of young children that affects multiple organ systems. The most common ophthalmologic manifestations of Kawasaki's disease are bilateral conjunctival injection and nongranulomatous iridocyclitis. To our knowledge, this patient is the first with Kawasaki's disease to demonstrate extraocular muscle palsy and orbital myositis.
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PMID:Orbital myositis due to Kawasaki's disease. 1041 94

This review article describes the imaging finding of the connective tissue disorders in children. The radiological features of the following conditions are described; the spondyloarthropathics, systemic lupus erythematosus (SLE), dermatomyositis, scleroderma, the vasculitides, Kawasaki disease, synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO), and focal myositis. The features on several integrated imaging techniques are described.
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PMID:Overview of the radiology of connective tissue disorders in children. 1071 8

Initially used as replacement therapy in patients with hypogammaglob-ulinemia, intravenous gamma-globulin (IVIg) preparations are increasingly being used as treatment for various autoimmune disorders. Although expensive, IVIg has become first-line or adjunctive therapy in the treatment of disorders as diverse as autoimmune or post-transfusion thrombocytopenia, autoimmune hemolytic anemia, Kawasaki's disease, inflammatory myositis, myasthenia gravis, pediatric acquired immune deficiency syndrome, bone marrow transplantation, and inflammatory de-myelinating polyneuropathy. IVIg remains the first line of therapy in patients with humoral immunodeficiency syndromes. Clinical studies support the selective use of this therapy in the above disorders; it has also been tried as therapy in many other diseases, with varied results. IVIg therapy has received Food and Drug Administration approval for use as maintenance treatment of patients with primary humoral immunodeficiencies and as therapy for acute or chronic autoimmune thrombocytopenic purpura.
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PMID:Intravenous gamma-globulin therapy. 1907 96

Severe, organ-threatening and life-threatening manifestations of inflammatory rheumatic diseases, such as diffuse alveolar hemorrhage in the context of small vessel vasculitis, sometimes inadequately respond to immunosuppressive treatment. In the case of an immanent or already occurring organ failure, immunosuppressive treatment may need to be supplemented with rapidly effective rescue treatment procedures. Due to the rarity of many rheumatic diseases, the evidence for the use of rescue treatment, such as plasmapheresis, extracorporeal membrane oxygenation (ECMO) and the administration of intravenous immunoglobulins (IVIG), is relatively low for many indications. The use of plasmapheresis is considered useful in acute anti-glomerular basement membrane (GBM) disease (Goodpasture's syndrome) or catastrophic antiphospholipid antibody syndrome (APS). The use of ECMO treatment may be considered for persistent respiratory failure despite mechanical ventilation due to diffuse alveolar hemorrhage or acute respiratory distress syndrome (ARDS). Administration of IVIG is indicated for acute cardiac involvement in Kawasaki's disease and may be considered in catastrophic APS and refractory myositis.
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PMID:[What are the indications for rescue procedures? : Systemic rheumatic diseases in the intensive care unit]. 3148 28

Together with heart-healthy lifestyle habits, statins serve as the cornerstone of primary and secondary prevention of atherosclerotic cardiovascular disease in adults. Several conditions, most notably familial hypercholesterolemia (FH), cause early dyslipidemia and vascular disease, contributing to the development and progression of atherosclerosis from childhood and increased cardiovascular risk. In recent decades, studies increasingly have evaluated the safety and efficacy of statins in such high-risk youth. The strongest evidence for pediatric statin use is for the heterozygous FH population, whereby statin use has been shown to lower low-density lipoprotein cholesterol effectively, slow the progression of atherosclerosis and vascular dysfunction, and significantly reduce cardiovascular risk in early adulthood. Numerous meta-analyses and Cochrane reviews have demonstrated that attributed adverse effects, including liver toxicity, myositis, and rhabdomyolysis, occur no more frequently in youth receiving statins than placebos, with no impact on growth or development. However, further studies evaluating the long-term safety of pediatric statin use are required. In the current review, we summarize the pediatric experience of statin use to date, focusing on its utility for FH, Kawasaki disease, post-heart transplantation, and other at-risk populations. Current guidelines and indications for use are summarized, and the short- and medium-term safety experience is reviewed. Finally, a clinical approach to the indications, initiation, and monitoring of statins in youth is provided.
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PMID:The Rationale, Indications, Safety, and Use of Statins in the Pediatric Population. 3273 68