Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0034067 (emphysema)
 11,506 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pneumomediastinum as a complication of interstitial pneumonia with leakage of air into the mediastinum or subcutaneous tissues is a rare complication of dermatomyositis (DM). Herein we report a case of pneumomediastinum complicating polymyositis (PM), which is usually associated with DM. A 61-year-old man was hospitalized in our department because of deterioration of interstitial pneumonia. Treatment with high-dose corticosteroid and cyclosporin A steadily improved his interstitial pneumonia. Two weeks later, he developed subcutaneous emphysema and chest X-ray showed pneumomediastinum. Both subcutaneous emphysema and pneumomediastinum improved gradually without any additional treatment.
 ...
PMID:A case of polymyositis complicated with interstitial pneumonitis and pneumomediastinum. 1662 27

A 38-year-old male nonsmoker presented with constitutional symptoms, polyarthritis, facial rash, and progressive dyspnea of more than 1-year duration. Chest radiology revealed bilateral reticular and ground glass infiltrates. Connective tissue disease investigation was unrevealing. Pulmonary function test was consistent with restrictive lung disease and decreased diffusion capacity. The patient was treated with corticosteroids for amyopathic dermatomyositis with no improvement of dyspnea. Flexible bronchoscopy was noncontributory. Four and 6 months after initial presentation, he developed spontaneous pneumomediastinum and extensive subcutaneous emphysema that resolved with conservative therapy. Surgical lung biopsy revealed cellular-fibrotic nonspecific interstitial pneumonia for which he was started on cytotoxic medication, with no improvement in respiratory symptoms. The frequency of interstitial lung disease (ILD)-related polymyositis/dermatomyositis (DM) has been reported to range between 5% and 30% and nonspecific interstitial pneumonia is seen in up to 80% of DM-related ILD and in almost all the reported patients with amyopathic DM. On account of the increased morbidity and mortality of ILD-related DM, investigations for the early detection of ILD should be performed during initial evaluation and during follow-up of patients with polymyositis/DM. Serum levels of anti-Jo-1 antibodies, chest roentgenogram, high-resolution computed tomography of the lungs, and pulmonary function tests, including diffusing capacity of the lung for carbon monoxide, should be routinely included. In those patients with evidence of DM-related ILD, a lung tissue biopsy should be considered for better prognostic stratification. Conversely, the presence of spontaneous pneumomediastinum, pneumothorax, and subcutaneous emphysema should prompt a search for underlying connective tissue diseases.
 ...
PMID:Spontaneous pneumomediastinum in a patient with facial rash. 2316 62

Interstitial lung disease (ILD) is a life-threating complication, commonly associated with polymyositis (PM), and dermatomyositis (DM). A subset of acute ILD associated with PM/DM patients are refractory to conventional treatment, and leads to a high rate of mortality. The efficacy of therapeutic plasma-exchange (TPE) as a PM/DM treatment to improve muscle involvement is controversial due to a lack of evidence. However, in recent reports, TPE has been effective in improving lung involvement. To evaluate the efficacy of this therapy, we retrospectively studied TPE treatment outcomes for in 18 acute PM/DM-ILD patients who were resistant to conventional therapies. Five patients were diagnosed with DM (27.8%), 11 with CADM (61.1%), and two with PM (11.1%). Among 18 patients, 11 (61.1%) achieved satisfactory improvement after four or more rounds of TPE, whereas seven died due to respiratory failure. We also analyzed risk factors to predict unresponsiveness to TPE in these patients. Notably, the prevalence of subcutaneous/mediastinal emphysema was significantly higher in the non-responsive group (6/7, 85.7%) than in the responsive group (2/11, 18.2%; P = 0.013); moreover, patients with this complication were mainly in the CADM subgroup (6/8, 75%). Subcutaneous/mediastinal emphysema and increased serum ferritin levels were shown to be poor prognostic factors, predictive of unresponsiveness to TPE, in PM/DM patients. No autoantibodies were found to be associated with TPE outcome, although we only investigated anti-Jo-1 and anti-Ro antibodies; the clinical significance of other myositis-specific autoantibodies, especially anti-melanoma differentiation-associated gene 5 (MDA5) antibody, is not known. Our results indicate that TPE might be an alternative treatment for acute PM/DM-ILD patients resistant to conventional therapies, except for those with subcutaneous/mediastinal emphysema and high serum ferritin levels.
 ...
PMID:Efficiency of Therapeutic Plasma-Exchange in Acute Interstitial Lung Disease, Associated With Polymyositis/Dermatomyositis Resistant to Glucocorticoids and Immunosuppressive Drugs: A Retrospective Study. 3178 64