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 40-year-old woman was admitted because of increasing exertional dyspnea. Right heart failure was suggested by the presence of hepatomegaly, pretibial edema and also echocardiographic findings. Physical examination and echocardiography showed no evidence of valvular disease or congenital heart disease except for right ventricular dilatation and tricuspid regurgitation. The ventricular septum deviated toward the left ventricle throughout the cardiac cycle, but left ventricular function was preserved. Severe pulmonary hypertension averaging 44 mmHg was revealed by cardiac catheterization. Digital subtraction angiography and pulmonary blood flow scintigraphy showed no evidence of pulmonary artery embolism, and no interstitial pulmonary lesions that might have caused pulmonary hypertension were recognized. Hypergammaglobulinemia suggested an autoimmune disorder, and signs of systemic lupus erythematosus (SLE), such as pleural effusion, proteinuria, lymphocytopenia, LE cell phenomenon and antinuclear antibodies were present. Several autoimmune diseases are known to be causative factors of pulmonary hypertension. However, only ten cases of SLE complicated by pulmonary hypertension have been reported the present one. These cases were characterized by a high incidence of Raynaud's phenomenon and positivity for anti-RNP antibody. In our present case, SLE activity was suppressed using prednisolone, but pulmonary hypertension persisted and the patient eventually died due to right cardiac failure. Judging from the clinical course of the ten reported cases of SLE-pulmonary hypertension, there seems to be no hope of improving the pulmonary hypertension once it has become established. Therefore it is important to detect and cure pulmonary hypertension as early as possible.
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PMID:[A case of lupus erythematosus preceded by right heart failure due to pulmonary hypertension]. 174 69

This is a case report of pulmonary hypertension in a woman with systemic lupus erythematosus who had taken an oral contraceptive. She was 16 yr old when diagnosed with SLE in July 1984, based on many clinical features and high DNA antibodies, RNP antibodies and CPK, and low complement. She improved slowly with prenisolone. She remained in remission for 7 months except for mild flare-ups involving synovitis, pleuritic chest pain and Raynaud's phenomenon. She began taking oral contraceptives 5 months later (30 mcg ethinyl estradiol and 150 mcg levonorgestrel). 7 months later she was readmitted with the same severe clinical and laboratory findings, but in addition exertional dyspnea. Pulmonary hypertension was evident, by x-ray, EKG, echocardiogram and right heart catheterization. Lupus anticoagulant and anticardiolipin antibodies were negative. She was treated with predniosolone, warfarin and nifedipine and remained stable for 6 months. She died of cardiac arrest after emergency surgery for ovarian cyst, complicated by shock and siezures. The author discussed the relationship between pulmonary hypertension and both oral contraception and SLE, since it is rare in either of these situations.
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PMID:Pulmonary hypertension, systemic lupus erythematosus, and the contraceptive pill: another report. 382 39

A 59-year-old woman was admitted to our hospital with exertional dyspnea. Linear and reticular opacities in the middle and lower fields of both lungs were observed on chest roentgenograms and chest computed tomograms (CT). The presence of anti RNP-antibody and Raynaud's phenomenon, sclerosis of the fingers, and leukopenia yielded a diagnosis of mixed connective tissue disease associated with interstitial pneumonia. The symptoms and lung involvement were alleviated after the administration of prednisolone (40 mg/day). However, the patient experienced the sudden onset of dyspnea during pulmonary function tests. A chest X-ray film disclosed mediastinal air around the left pulmonary artery, and a chest CT scan demonstrated some blebs in the left lower lung field. After tapering the dosage of prednisolone in 5 mg increments per week, the pneumomediastinum disappeared without treatment. The clinical features and laboratory data findings suggested the patient's interstitial pneumonia was associated with systemic lupus erythematosus rather than with progressive systemic sclerosis or dermatomyositis. The pneumomediastinum may have been due to the rupture of blebs secondary to interstitial pneumonia during pulmonary function tests or as a result of steroid therapy.
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PMID:[Mixed connective tissue disease-associated interstitial pneumonia complicated by pneumomediastinum during prednisolone therapy]. 1097 89

We have experienced a case of chronic active Epstein-Barr virus infection (CAEBV) complicated in systemic lupus erythematosus (SLE) and antiphospholipid antibody syndrome (APS). A 35-year-old woman was admitted to our hospital with complaints of fever and dyspnea on exertion. She was diagnosed as having SLE on the basis of arthritis, oropharyngeal ulcer, lymphopenia, and positive autoantibodies against DNA, RNP and SSA. The diagnosis of APS was also made because of positive anti-cardiolipin IgG antibodies and the existence of multiple pulmonary infarction with pulmonary hypertension. The administration of 30 mg/day of prednisolone and anti-coagulation significantly improved clinical symptoms. However, she was again admitted to the hospital four months later because of progressive liver damage and pancytopenia. Increment of prednisolone did not improve the clinical situation and she expired because of pulmonary hemorrhage. At autopsy, there were a significant increase of histiocytes with hemophagocytosis and a dense infiltration of atypical lymphocytes in the liver, spleen, lymph nodes and bone marrow. Infiltrated lymphocytes were positive for CD 3 and EBER 1 in immunohistochemical staining and EBVmRNA was detected by in situ hybridization. Final pathological diagnosis was CAEBV with hemophagocytic syndrome in association with lupus nephritis, pulmonary hemorrhage and pulmonary infarction.
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PMID:[An autopsied case of chronic active Epstein-Barr virus infection complicated in systemic lupus erythematosus and antiphospholipid antibody syndrome]. 1259 14

A 52 year-old woman noticed general fatigue, polyarthralgia, and muscle weakness of lower extremities in October 2001. In December, she felt difficulty in walking due to muscle weakness. In January 2002, she admitted another hospital because of dyspnea on exertion and edema of lower extremities. Laboratory test revealed leukocytopenia, the elevation of creatine kinase and positive anti-U1-RNP antibodies. Her chest computed tomography (CT) showed severe interstitial pneumonia. Cardiac echogram revealed that she had pericardial effusion and pulmonary hypertension. Then she was transferred to Keio University Hospital and she was diagnosed as having mixed connective tissue disease (MCTD) manifestating myositis, interstitial pneumonia, pulmonary hypertension and pericarditis. Prednisolone (PSL) 60mg daily following to methylprednisolone (mPSL) pulse therapy was begun and her symptoms were gradually improved. In middle of February, she complained of high fever over 39.0 degrees C. Bacterial culture tests were negative and laboratory data indicated pancytopenia and a high level of serum ferritin. Bone marrow aspiration revealed hemophagocytosis in bone marrow specimens and she was diagnosed as having hemophagocytic syndrome associated with MCTD. mPSL pulse therapy was not effective and intermittent cyclophosphamide pulse therapy (IV-CY) was performed resulting in improvement of the symptoms. This case suggested the effectiveness of IV-CY therapy in patients with corticosteroid-resistant HPS associated with connective tissue diseases.
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PMID:[A case of mixed connective tissue disease successfully treated for hemophagocytic syndrome with intermittent intravenous injection of cyclophosphamide]. 1555 24