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)

Drug rash eosinophilia with systemic symptoms (DRESS syndrome) presents as an acute febrile illness with leukocytosis, eosinophilia, lymphadenopathy, skin rash with acute hepatitis, renal failure, myositis, or systemic organ involvement. Aromatic anticonvulsants like phenytoin, carbamazepine, and phenobarbital cause drug-induced hypersensitivity or DRESS syndrome. However, sodium valproate being nonaromatic compound although known hepatotoxic drug in preexisting chronic liver disease has never been reported to cause DRESS syndrome alone. Here we report an interesting case of DRESS syndrome caused by valproate, which presented as an acute hepatitis illness with rash, renal dysfunction, and typical hematological features of DRESS syndrome within 2 months of the introduction of the drug in an epileptic patient. Patient initially showed a good response to intravenous steroids with improvement in the liver and renal dysfunction. However, later on, developed pancytopenia either due to steroid-induced sepsis or DRESS syndrome-related secondary hemophagocytosis (HPS) due to involvement of bone marrow as a rare occurrence and succumbed to illness.
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PMID:Valproate-induced Drug Rash Eosinophilia with Systemic Symptoms Syndrome: An Unknown Hepatotoxicity. 3211 99

Eosinophilic granulomatosis with polyangitis (EGPA) is a systemic necrotizing small-vessel vasculitis that presents heterogeneously as a multi-organ disease. EGPA evolves through three phases: (1) prodromic phase with asthma, atopy and sinusitis, (2) eosinophilic phase characterized by peripheral eosinophilia and eosinophilic infiltration without necrosis, and (3) vasculitic phase involving organ damage. EGPA often presents with asthma, mononeuritis multiplex, lung infiltrates, sinusitis and constitutional symptoms. Although myalgias are common, EGPA rarely presents with true weakness with elevated creatinine kinase (CK). We describe a rare case of a patient presenting with eosinophilic myositis, who subsequently developed fulminant EGPA. The patient's diagnosis was supported by an initial clinical presentation of weakness and elevated CK, followed by fleeting pulmonary infiltrates and mononeuritis multiplex, peripheral eosinophilia, and strongly positive myeloperoxidase anti-cytoplasmic antibody (MPO-ANCA). Muscle biopsy revealed eosinophilic myositis. The patient responded well to high-dose glucocorticoids and cyclophosphamide with improved symptoms and biochemical markers. Based on our literature review, there are only seven similar cases reported of EGPA presenting with myositis and confirmatory muscle biopsies. There is significant heterogeneity in their clinical findings, histopathology and treatments that were used. Our case report and literature review highlights the importance of recognizing myositis as an initial presenting symptom of EGPA, providing an opportunity for early diagnosis and treatment to reduce risk of further disease progression and morbidity.
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PMID:Eosinophilic granulomatosis with polyangiitis presenting with myositis: case based review. 3227 Feb 95

Eosinophilic myositis is characterised by peripheral blood eosinophilia and eosinophilic infiltration in muscles, and is comprised three subtypes: focal eosinophilic myositis (FEM), eosinophilic polymyositis, and eosinophilic perimyositis. Muscle involvement of FEM is usually limited to the lower legs, and pulmonary complications have not been reported. We report a rare case of FEM in the left adductor pollicis complicated by lung lesions. A 37-year-old woman developed swelling of the first web space in the left hand. Magnetic resonance imaging (MRI) of the left hand demonstrated increased signal on fat-suppressed T2-weighted imaging in the left adductor pollicis. A muscle biopsy specimen demonstrated perimysial and endomysial infiltration of mononuclear cells and eosinophils, and necrosis and regeneration of muscle fibres. Chest computed tomography (CT) revealed upper-lobe-dominant ground-glass opacities (GGO). Both focal myositis and pulmonary lesions improved without treatment. This case suggests that FEM could involve adductor pollicis and have pulmonary lesions. In this case, myositis and GGO resolved spontaneously. Some FEM cases treated with glucocorticoids were reported in the past. Further studies are required to determine whether patients with FEM require therapeutic intervention.
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PMID:Spontaneous resolution of focal eosinophilic myositis of the adductor pollicis complicated by lung lesions. 3308 52


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