Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0231807 (exertional dyspnea)
 3,402 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 55-year-old woman was referred to the department of urology in our hospital with left renal tumor, discovered during examinations at another hospital for fever and dyspnea on exertion. Because surgery was difficult due to severe hypoxemia, pulmonary function impairment (restrictive) and bilateral diffuse interstitial shadows on chest X-ray film, the patient was referred to our department. Interstitial pneumonitis was found on transbronchial lung biopsy, and serum GOT, LDH and CPK values were elevated. These symptoms and abnormalities of laboratory data were improved by administration of prednisolone 60 mg/day, and left nephrectomy was performed without any complications. Pathological examination of the surgical specimen showed clear cell carcinoma (Grawitz). Steroid therapy was tapered off and her clinical course was good. Six months after surgery, the patient developed a recurrence of fever, which was not responsive to antibiotics. Polymyositis was diagnosed on the basis of elevated serum GOT, LDH and CPK, electromyogram and muscle biopsy findings and positive anti-Jo-1 antibody. Polymyositis/dermatomyositis is sometimes associated with interstitial pneumonitis or malignant neoplasms, but rarely with both simultaneously. Moreover, renal cell carcinoma is very rare among the malignant neoplasms associated with polymyositis/dermatomyositis, and we therefore report this unusual case.
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PMID:[A case of interstitial pneumonitis associated with polymyositis complicated by renal cell carcinoma]. 146 89

We describe a 58-year-old female with BOOP associated with polymyositis. Four months prior to the appearance of distinctive manifestation of polymyositis, she presented with a two-week history of cough, dyspnea on exertion, and fever. Chest roentgenogram demonstrated bilateral basal infiltrative shadows. The patient was treated with prednisolone, 30 mg/day because of progressive hypoxemia. Open lung biopsy revealed organizing masses of granulation tissue extending from the respiratory bronchioles into the intra-alveolar spaces, which was consistent with BOOP. She developed muscle pain in her legs, fever, dry cough, hypoxemia, and high CPK value in the course of tapering of steroid dose. The findings of biopsy from the left rectus femoris muscle were compatible with polymyositis. Retrospective study of the patient's serum on admission showed positive anti Jo-1 antibody.
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PMID:[A case of bronchiolitis obliterans organizing pneumonia with positive anti Jo-1 antibody preceding polymyositis]. 163 59

A 60-year-old woman was admitted to our hospital with a two month history of dry cough and dyspnea on exertion. A chest roentgenogram revealed diffuse interstitial shadows with a reduction of lower lung volume. Laboratory examinations revealed an increase in CPK and aldolase. There was decreased proximal muscle power, and the findings of a biopsy of the right deltoid were compatible with polymyositis. Myositic symptoms were stable, but the respiratory symptoms worsened, and an open lung biopsy was performed for diagnosis and to determine the best treatment. The histological findings of biopsy materials demonstrated active interstitial pneumonia complicated by cellular interstitial pneumonia, bronchiolitis obliterans organizing pneumonia, usual interstitial pneumonia and lymphoid hyperplasia. The patient responded well to adrenocorticosteroid and immunosuppressive therapy, and is now attending as an out patient. It is well known that PM-DM can be associated with interstitial pneumonia, and this complication is an important prognostic factor clinically. The pathological patterns of interstitial pneumonia in PM-DM may be divided into usual interstitial pneumonia and bronchiolitis obliterans organizing pneumonia. Furthermore, it is well documented that these patterns are concurrent with the response to adrenocorticosteroid and prognostic factors. However, our case of PM-DM, in which various patterns such as rheumatoid arthritis (RA) were pathologically revealed, cannot be considered as having uniform pathological pattern. We consider that pulmonary pathological patterns of PM-DM are very varied, as with RA. It is a very important to evaluate the nature of these patterns and the subsequent clinical course in PM-DM with interstitial pneumonia.
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PMID:[A case of interstitial pneumonia of polymyositis-dermatomyositis with various pathological findings in open lung biopsy]. 833 51

In 1973, a 36-year-old black woman with dyspnea on exertion, Raynaud's phenomenon and arthralgia was first seen at the University of California at Los Angeles Medical Center. Her chest roentgenography showed bilateral basilar infiltration, open lung biopsy revealed bronchiolitis obliterans with organizing pneumonia (BOOP) and muscle biopsy was consistent with inflammatory myopathy. She has been followed for 18 years, has responded well to conservative treatment, initially with alternate day prednisone, with added azathioprine for the past 8 years, and remained fully employed. The favorable prognosis of this case is consistent with that of the BOOP category proposed by Tazelaar, et al in 1990. We conclude that a careful histological subclassification is suggested before initiating aggressive therapy in patients with polymyositis and interstitial lung disease.
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PMID:Bronchiolitis obliterans organizing pneumonia in polymyositis. A case report with longterm survival. 833 17

This is the first report on idiopathic inflammatory myopathies (IIM) in French Canadians. We reviewed retrospectively 30 French Canadian adults (20 women and 10 men) with IIM seen consecutively over 12 years. The median age at diagnosis was 45 years. The IIM were 8 (27%) primary polymyositis (PM), 9 (30%) primary dermatomyositis (DM), 5 (17%) IIM with neoplasia (lymphoma, breast, esophageal, colonic, and skin cancer) and 8 (27%) IIM with a connective tissue disease (4 with systemic sclerosis, 2 with mixed connective tissue disease, and 2 with rheumatoid arthritis). The most common presenting symptom was proximal muscle weakness (n = 10,33%). Of the remaining 20 patients, 6 (20%) had the onset of their weakness within 1 month of the presenting symptom. Only 3 (10%) patients did not have proximal muscle weakness. Twenty-six (87%) patients had weakness in the pelvic girdle, 25 (83%) in the shoulder girdle, and 7 (23%) in the neck muscles. Other common symptoms included dyspnea on exertion and dysphagia, each present in 13 (43%) patients. Gottron's papules and the heliotrope rash were the most common skin lesions documented in 11 (37%) and 10 (33%) patients, respectively. The serum creatine kinase (CK) level was between 171 and 1,000 U/L in 13 (43%) patients and between 1,001 and 6,000 U/L in 13 (43%) patients. Antinuclear antibodies (ANA) on HEp-2 cells were positive in 16 (53%) patients, of which 2 (13%) expressed autoantibodies to nuclear pore complexes. Autoantibody specificities were anti-La (n = 4, 13%), anti-U1RNP (n = 3, 10%), and anti-Ro (n = 2, 7%). None of the patients expressed anti-Jo-1, anti-topoisomerase I, or anticentromere antibodies. Twenty-eight (93%) patients received corticosteroid therapy, and 8 (27%) patients responded to prednisone alone. Thirteen (43%) patients were treated with methotrexate, and 9 (69%) responded. The mean follow-up was 62 months: 23 (77%) had their disease controlled, 3 (10%) patients were lost to follow-up, and 4 (13%) died (no death occurred because of IIM or its treatment). Therapy was discontinued because of remission in 5 (17%) patients. Cumulative survival rates at 2, 5, and 10 years were 89%, 89%, and 85%, respectively. The presence of autoantibodies to nuclear pore complexes and anti-La autoantibodies, the rare occurrence of anti-Jo-1 autoantibodies, the response to conventional therapies, and a high survival rate may distinguish IIM in French Canadians from that of other reported series.
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PMID:Distinctive features of idiopathic inflammatory myopathies in French Canadians. 887 Jan 12

A 61-year-old woman was admitted to our hospital with a two week history of knee-joint pain, dry coughing and dyspnea on exertion. A chest roentgenogram revealed bilateral patchy and infiltrative shadows. Laboratory examination revealed high CK and aldolase levels. Although myositic symptoms were absent, the respiratory symptoms rapidly worsened and respiratory failure developed. An open-lung biopsy and a muscle biopsy were done. The open-lung biopsy specimen showed bronchiolitis obliterans organizing pneumonia (BOOP), but the muscle biopsy specimen revealed non-specific findings. After 4 weeks of intravenous intermittent high-dose corticosteroid therapy, 50mg of cyclophosphamide was given daily, along with 20mg of prednisolone on alternate days. There were marked clinical, physiological and roentgenographic improvements. A test for anti-Jo-1 antibody was positive, which suggested that this patients had pulmonary manifestations of polymyositis/dermatomyositis. Although BOOP can have an indolent course and a good prognosis, it may rapidly worsen and respiratory failure may develop, in which case it should be treated aggressively with a combination of intravenous high-dose corticosteroids and immunosuppressants.
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PMID:[Rapidly progressing BOOP in a patient with positive anti-Jo-1 antibody: response to corticosteroid pulse and immunosuppressant therapy]. 895 14

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

A 33-year old man was admitted to our hospital because of an abnormal shadow on the chest radiograph, dry cough, and exertional dyspnea. Chest radiograph and high-resolution computed tomography (HRCT) on admission showed ground-glass opacities and bronchiectasis with volume loss in the bilateral dorsal areas. Thoracoscopic lung biopsy specimens showed mainly a pattern of NSIP (nonspecific interstitial pneumonia). We considered this case as hypersensitivity pneumonia or interstitial pneumonia (IP) associated with collagen disease. Oral prednisolone (PSL) was initiated at 55 mg/day (1 mg/kg). However he complained of proximal muscle weakness and pain and difficulty of breathing. He had heart failure due to the myocarditis. We established a diagnosis of IP associated with polymyositis and it was confirmed by his symptoms, muscle biopsy findings and elevation of serum CPK. We considered this case as the myocarditis due to polymyositis.
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PMID:[A case of myocarditis associated with polymyositis preceded by interstitial pneumonia]. 2207 14