Cases reported "Disease Progression"

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11/39. A severely disabling disorder: fibrodysplasia ossificans progressiva.

    Fibrodysplasia ossificans progressiva (FOP) is an extremely rare hereditary disorder characterised by progressive heterotopic ossification of the soft tissues. The resulting progressive immobilisation of the limbs, jaw and chest wall generally leads to severe disability. We present an 18-year-old girl with advanced FOP. She had three operative interventions to excise her ectopic bones but all resulted in failure. Treatment strategies for this disorder should include the avoidance of exacerbating factors.
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ranking = 1
keywords = fibrodysplasia ossificans progressiva, ossificans progressiva, fibrodysplasia ossificans, progressiva, fibrodysplasia, ossificans
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12/39. Human T cell leukaemia virus type I associated neuromuscular disease causing respiratory failure.

    polymyositis and inclusion body myositis have rarely been described in association with human T cell leukaemia virus type I (HTLV-I) infection. Most of such patients have coexisting HTLV-I associated myelopathy (HAM). Two patients with HTLV-I infection, myopathy, and respiratory failure are described. The muscle biopsy specimen of the first patient bore the histological features of inclusion body myositis and there was no evidence of concurrent myelopathy. The second patient had HAM, and her muscle biopsy showed non-specific myopathic and neuropathic changes. Both patients developed respiratory muscle weakness over eight years after diagnosis of myopathy, leading to hypercapnic respiratory failure requiring mechanical ventilatory support. Respiratory failure as a complication of HTLV-I associated myopathy has not previously been described.
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ranking = 0.00036421914801689
keywords = myositis
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13/39. Rapidly progressive interstitial lung disease in a dermatomyositis patient with high levels of creatine phosphokinase, severe muscle symptoms and positive anti-Jo-1 antibody.

    It has been reported that there is a subgroup of dermatomyositis (DM) patients with rapidly progressive interstitial lung disease (ILD) who have mild muscle symptoms, slightly increased levels of muscle enzymes, and absence of anti-Jo-1 antibody. A 51-year-old woman with DM was intubated requiring mechanical ventilation because of a rapidly progressing ILD in spite of the absence of the typical poor prognostic factors. A high dose or pulse therapy of corticosteroids was not effective, but additional treatment of cyclosporine gradually improved her respiratory condition. It is not clear why a rapidly progressive ILD occurred in this case lacking poor prognostic factors. However, if corticosteroid treatment is not effective, additional administration of cyclosporine in the early period of rapidly progressive ILD may rescue deteriorating cases.
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ranking = 0.00060703191336149
keywords = myositis
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14/39. Interstitial lung disease associated with juvenile dermatomyositis: clinical features and efficacy of cyclosporin A.

    OBJECTIVES: Interstitial lung disease (ILD) is a rare complication of juvenile dermatomyositis (JDM). The aim of this study was to clarify the clinical features of JDM-associated ILD and to evaluate the efficacy of cyclosporin A (CSA). methods: We reviewed clinical records of 10 cases of JDM that were admitted to Hokkaido University Hospital between April 1990 and March 2001. RESULTS: Five cases were complicated with ILD, three with interstitial pneumonia and two with bronchiolitis obliterans organizing pneumonia. ILD was associated with active JDM and progressed despite corticosteroid therapy. Testing for anti-Jo-1 antibody was negative in all cases. Respiratory symptoms were initially noticed in only one case. In the other cases, ILD was first detected by routine examination of chest X-ray. All the cases received CSA (3-5 mg/kg/day) in combination with prednisolone. One patient died of respiratory failure, but the others responded well to treatment with CSA. CONCLUSION: ILD should be evaluated carefully in all cases of JDM regardless of respiratory symptoms. CSA is a choice for steroid-resistant cases of JDM-associated ILD.
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ranking = 0.00060703191336149
keywords = myositis
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15/39. Prevention of progression of interstitial lung lesions by early combination therapy with corticosteroids and cyclosporine/cyclophosphamide in two patients with amyopathic dermatomyositis.

    Two patients with amyopathic dermatomyositis complicated by interstitial lung lesions were effectively treated with a combination of corticosteroids and cyclosporine and/or cyclophosphamide. A 48-year-old female patient was treated with pulse methylprednisolone and cyclosporine 2 months after onset of dermal symptoms. A 45-year-old male patient was treated with oral prednisolone and pulse cyclophosphamide 2 1/2 months after onset of dermal symptoms. Early evaluation of interstitial lung lesions and early extensive therapy may improve prognosis of interstitial lung lesions in patients with amyopathic dermatomyositis.
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ranking = 0.00072843829603378
keywords = myositis
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16/39. Successful treatment of dermatomyositis-related rapidly progressive interstitial pneumonitis with sequential oral cyclophosphamide and azathioprine.

    Interstitial lung disease (ILD) is a major complication of idiopathic inflammatory myopathies (IIM). We report the successful use of oral cyclophosphamide, followed by azathioprine maintenance, in the treatment of a patient with dermatomyositis-related rapidly deteriorating ILD, resistant to steroid. Prompt recognition and early aggressive immunosuppressive therapy may improve the outlook of this condition.
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ranking = 0.00060703191336149
keywords = myositis
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17/39. Case report: severe central nervous system involvement in juvenile dermatomyositis.

    We present 3 patients with juvenile dermatomyositis (JDM) and severe central nervous system (CNS) complications. All patients had at least 4 positive criteria of Bohan and Peter, which confirmed a definite diagnosis of JDM. They were all male, and had a relatively high creatinine kinase value at admission (1532-4260 U/l). Besides, progressive proximal muscle weakness and rash, one patient presented with rapid irreversible decline of vision. Ophthalmologic examination showed active vasculitis of the retina. After 2 weeks of treatment with immunosuppressive drugs and being in improved, relatively stable clinical condition, all 3 patients developed generalized tonic-clonic convulsions. Other causes of the neurological symptoms could be excluded. In all 3 patients, the course of JDM was fatal. The clinical symptoms and further investigations in our patients show CNS involvement in JDM. Although rarely reported, CNS vasculopathy can be a serious and life-threatening complication of JDM.
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ranking = 0.00060703191336149
keywords = myositis
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18/39. A case confirming the progressive stages of pyomyositis.

    A teenage boy presented in the early stage of pyomyositis. He had neck pain, tenderness, and fever. A computed tomography scan showed inflammation in the sternocleidomastoid muscle with no fluid collection. This progressed to a pus-filled drainable mass caused by Stapylococcus aureus. The authors describe this case to highlight the predictable stages and increase the index of suspicion to enhance its early recognition.
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ranking = 0.00060703191336149
keywords = myositis
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19/39. ankylosis of the jaw in a patient with fibrodysplasia ossificans progressiva.

    A case of fibrodysplasia ossificans progressiva (FOP) is presented. This uncommon connective tissue disease tends to produce progressing ectopic osteogenesis. Because there are no reported curative procedures for TMJ ankylosis occurring in this condition, a palliative surgical approach is described. Etiology, diagnosis, and prognosis of the disease is reviewed. Recent research in BMP cytokine-induced bone repair may allow new approaches to treating this debilitating disease in the future.
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ranking = 1.1513303450304
keywords = fibrodysplasia ossificans progressiva, ossificans progressiva, fibrodysplasia ossificans, progressiva, fibrodysplasia, ossificans
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20/39. High-grade neuroendocrine carcinoma of the lung presenting an unusual spread mimicking pleural mesothelioma associated with dermatomyositis.

    neuroendocrine tumors of the lung comprise a heterogeneous group of tumors that represents a spectrum of disease from typical carcinoid tumors to the high-grade neuroendocrine carcinomas (large-cell neuroendocrine carcinomas and small-cell carcinomas). The high-grade neuroendocrine carcinomas are characterized by early metastasis and poor prognosis. The peripheral location and especially the massive pleural spread are rare for a high-grade neuroendocrine carcinoma. We report a case in which a high-grade neuroendocrine carcinoma, associated with dermatomyositis, presented an unusual pattern of progression, mimicking malignant pleural mesothelioma on diagnostic imaging.
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ranking = 0.00060703191336149
keywords = myositis
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