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)

The use of all-trans retinoic acid (ATRA) is now standard therapy for the treatment of acute promyelocytic leukaemia (APML). There have been increasing reports of ATRA-induced myositis, with its frequent association with retinoic acid syndrome and Sweet's syndrome. We report a case of a young man with APML who developed ATRA-induced myositis characterized by unexplained fevers, bilateral leg swelling and a non-painful purpuric, petechial rash, with prompt resolution of symptoms and signs with high-dose steroids and cessation of ATRA. Rapid recognition of this adverse reaction and prompt institution of steroids is of prime importance given its potentially fatal course.
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PMID:A case of all-trans retinoic acid-induced myositis in the treatment of acute promyelocytic leukaemia. 1630 43

Interstitial lung disease in patients with antisynthetase syndrome and no evidence of myositis is rare and may precede other disease manifestations. We report a patient who initially presented with symptoms primarily related to lung involvement. The diagnosis of the antisynthetase syndrome without myositis was made many months later when he developed a characteristic hand rash (mechanic's hands), which was confirmed by positive antibodies to Jo-1. With treatment, both the hand rash and the interstitial lung disease improved. Antisynthetase syndrome should be considered in patients presenting with interstitial lung disease with no evidence of myositis. Appropriate laboratory testing with measurement of specific autoantibodies may help in the early diagnosis and treatment of the syndrome.
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PMID:Interstitial lung disease in a patient with antisynthetase syndrome and no myositis. 1632 94

Autoantibodies against aminoacyl-tRNA synthetase (ARS) are known to associate with myositis, arthritis, and interstitial lung disease (ILD). Anti Jo-1 antibody is a common diagnostic tool of this syndrome and is available on a commercial basis, while other anti-ARS antibodies such as anti-EJ, anti-PL7 and anti-OJ antibodies are less commonly examined. The aim of this study was to investigate the clinicopathological characteristics of 9 ILD patients who were positive for anti-ARS antibodies including anti-EJ, anti-PL7 and anti-Jo-1 antibodies. In physical examination, muscle pain and/or skin rash (including mechanic's hand) were observed frequently. In laboratory findings, levels of myogenic enzymes were slightly elevated, and anti-nucleus antibody was detected in most cases. Air-space consolidation with traction bronchiectasis, ground glass opacities and characteristic volume loss of both lower lobes were observed in radiological examination. Organization in alveolar spaces and lymphoid alveolitis that did not destroy existing pulmonary structures were main pathological findings. Immunosupressive therapy was effective and the prognosis appears satisfactory in these cases. Although anti-ARS antibodies had been measured as a marker of myositis, we suggest that they are useful as markers of interstitial pneumonia.
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PMID:[Clinical and pathological findings of patients with interstitial lung disease associated with antisynthetase]. 1636 63

Idiopathic inflammatory myopathies (IIMs) are systemic autoimmune diseases characterized by chronic muscle inflammation resulting in progressive weakness and frequent involvement of internal organs, mainly the pulmonary, gastrointestinal and cardiac systems which considerably contribute to the morbidity and mortality of the IIMs. Aim of this study was to present clinical characteristics, disease course, frequency of relapses and survival in patients with juvenile dermatomyositis (DM). A national registry of patients with juvenile IIMs was elaborated by the authors in Hungary. We have summarized data of the register according to signs and symptoms, disease course, frequency of relapses and survival of patients with juvenile IIM. Analysis was performed using data of 44 patients with juvenile DM diagnosed between 1976 and 2004 according to Bohan and Peter's criteria. Survival probability was calculated by Kaplan-Meier method. Data of patients with juvenile DM were compared with data of 66 patients with adult DM. The most frequent cutaneous features were facial erythema and heliotrope rash. Extramuscular and extraskeletal manifestations of the disease were more frequent in adult patients. The most common extramuscular feature was arthralgia in both groups of patients with juvenile or adult DM. Cardiac manifestation of the disease was not observed in juvenile patients. Respiratory muscle involvement and interstitial lung disease (ILD) were more frequent among adult DM patients than cardiac manifestation of the myositis. In view of the disease course, the authors found that frequency of polycyclic and monophasic subtypes of the disease were mainly similar. The hazard of relapse was found higher during the first year after the remission. None of the juvenile patients died. Among adult patients four disease-specific deaths occurred. There was no correlation between relapse free survival and initial therapeutic regimen. Many of our patients had polycyclic or chronic disease. As relapses can occur after a prolonged disease-free interval, patients should be followed up for at least 2 years. Although we found favourable survival probability, further investigations are needed to assess functional outcome.
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PMID:National registry of patients with juvenile idiopathic inflammatory myopathies in Hungary--clinical characteristics and disease course of 44 patients with juvenile dermatomyositis. 1676 56

The idiopathic inflammatory myopathies are a heterogeneous group of diseases that can involve various systems. Antibodies directed against aminoacyl-tRNA synthetases, such as anti-Jo-1 antibodies, are strongly associated with a syndrome which consists of myositis, interstitial lung disease (ILD), arthritis and Raynaud's phenomenon. Forty-one patients with various forms of idiopathic inflammatory myopathies were assessed: 14 patients with anti-Jo-1 antibodies and 27 patients without anti-Jo-1 antibodies as a control group. We retrospectively analysed clinical symptoms, treatment and outcome in both groups. Patients with anti-Jo-1 antibodies more often had ILD (64.2 vs. 11.1%), arthritis (64.2 vs. 18.1%) and Raynaud's phenomenon (38 vs. 0%). Patients without the anti-Jo-1 antibody presented worse muscle strength and more frequently myalgia (37 vs. 21%), cutaneous rash (18.5 vs. 7%), heliotrope rash (29% vs. 7%) and periungueal changes (22 vs. 0%) than the anti-Jo-1-positive patients. Outcome was good in both groups. Improvement was achieved in the 14 (100%) Jo-1 positive patients, and in 25 (92.5%) controls. Two (7.5%) patients from control group achieved remission.
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PMID:Clinical features and prognosis of patients with idiopathic inflammatory myopathies and anti-Jo-1 antibodies. 1676 58

Skin involvement in dermatomyositis does not always parallel muscle disease and can be the most active or severe component of the disease, failing to respond to therapeutic interventions that are adequate for myositis and other systemic involvement. This case refers to a resistant paraneoplastic amyopathic dermatomyositis rash which readily resolved with intravenous immunoglobulin (IVIG), practically in the form of monotherapy. This observation gains particular significance considering that IVIG is still regarded as a second-line therapeutic regimen in the treatment of dermatomyositis despite its prevalence in matters of safety.
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PMID:Successful treatment of refractory rash in paraneoplastic amyopathic dermatomyositis. 1677 4

Myositis has been reported as a rare manifestation of Lyme disease, and Lyme myositis can be an important consideration in the differential diagnosis of unusual cases, especially in patients who live in or travel to endemic areas. We report the case of a 43-year-old man who presented with focal myositis of the proximal lower extremity and was subsequently diagnosed as having Lyme myositis. The patient had previously experienced a febrile illness and rash, but had no ongoing symptoms of Lyme disease. Myositis was confirmed by magnetic resonance imaging and muscle biopsy; Borrelia burgdorferi infection was confirmed by Lyme serology and polymerase chain reaction testing of synovial fluid and biopsy material. The current case is reviewed in the context of findings from previous case descriptions.
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PMID:Lyme myositis. 1687 48

Azathioprine, an analog of 6-mercaptopurine has been used as a steroid-sparing agent in the treatment of inflammatory bowel disease for over 30 years. Hypersensitivity reactions to azathioprine including fever, myalgia, arthralgia, rash are well documented in the literature. Here, we report 2 cases of azathioprine hypersensitivity in patients with inflammatory bowel disease manifesting with the unusual symptom of profound muscular weakness resulting in inability to perform simple tasks such as lifting even light objects, sitting upright, and walking a few steps. Development of severe weakness raised concern about myositis, rhabdomyolysis, myopathy, and sepsis in these patients. Discontinuation of azathioprine resulted in prompt improvement of muscular weakness, and rechallenge led to recurrence of similar symptoms within hours. These observations suggest that the development of muscular weakness in patients on azathioprine should be considered as an adverse effect of the drug. Failure to recognize this adverse outcome can lead to prolonged periods of muscular weakness in this group of patients.
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PMID:Severe muscular weakness: an unusual adverse effect of azathioprine therapy. 1691 6

A well documented case of erythema infectiosum is being reported here for the first time from India which was associated with myositis that has not been reported globally. A 9-year-old child presented with moderate to high grade fever, mild anemia, and erythematous rash involving face, trunks and limbs associated with arthralgia, myalgia and myositis. Parvovirus B19 infection was confirmed by detection of IgM antibodies (inhouse ELISA) and DNA (nested PCR) in patient's serum.
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PMID:Detection of parvovirus B19 in a case of erythema infectiosum with myositis. 1703 21

Juvenile dermatomyositis is an inflammatory disease of unknown etiology that primarily affects skin and muscles. The pathognomonic Gottron's sign consists of symmetric macules and papules on the dorsal aspect of the interphalangeal joints and exterior areas of the big joints. A periorbital violaceous (heliotrope) skin rash is also characteristic. There may be a discordance in time of presentation of the skin and muscle disease, and a small subset of patients apparently do not develop muscle disease at all. The absence of muscle involvement is termed 'amyopathic dermatomyositis.'We describe two children who presented with the characteristic rash of juvenile dermatomyositis but with no clinical evidence of muscle involvement. One developed muscle weakness 3 years later. Neither patient had a full muscle work-up at the onset of the disease, which left questions about diagnoses and whether or not there may have been subtle muscle involvement. On the basis of our literature review, the outcome of these patients is uncertain, although it appears that myositis develops in many, maybe most, affected children. We suggest that in the absence of muscle disease, application of sunscreen and administration of hydroxychloroquine sulfate may ameliorate the rash. More aggressive treatment will need to be given when muscle involvement can be demonstrated.
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PMID:Amyopathic dermatomyositis in children: a diagnostic and therapeutic dilemma. 1703 28


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