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Query: UMLS:C0024141 (systemic lupus erythematosus)
 44,322 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A young woman with systemic lupus erythematosus developed portal hypertension and pulmonary hypertension. This is the first report of such a case. We suggest that the presence of the lupus anticoagulant may have been related to the development of these two unusual features of her illness.
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PMID:Portal and pulmonary hypertension in a case of systematic lupus erythematosus: possible relationship with a clotting abnormality. 644 58

An autopsy case with SLE suffering from Raynaud's phenomenon and pulmonary hypertension was reported. Histological examinations revealed systemically marked fibrous intimal thickening of arteries and arterioles with or without thrombus throughout the whole body, especially of the pulmonary arteries and arterioles. Pulmonary arterial changes in the present case were compared with those in 52 autopsied cases with SLE without pulmonary hypertension, but there were no cases with such marked arterial changes as the present case. In addition, the incidence of pulmonary thrombosis was significantly higher in the cases with Raynaud's phenomenon than the cases without this phenomenon. However, the relation between pulmonary hypertension and Raynaud's phenomenon, pulmonary thrombosis, fibrous pericarditis, or type of lupus nephritis in SLE could not be clarified with a significant difference.
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PMID:Vascular lesions in systemic lupus erythematosus (SLE) with pulmonary hypertension. 646 52

To assess the nature and distribution of cardiovascular abnormalities associated with mixed connective tissue disease, we studied 38 patients with overlapping clinical manifestations of systemic lupus erythematosus, progressive systemic sclerosis and polymyositis, and circulating antibodies to nuclear ribonucleoprotein. The protocol included taking a medical history and a physical echocardiogram, and pulmonary function tests. Cardiac catheterization was performed on 17 patients. Postmortem examination was performed on four of the five patients who died during follow-up. Acute pericarditis and/or pericardial effusion was detected in 11 patients (29%) and mitral valve prolapse was identified in 10 patients (26%). Marked intimal hyperplasia of coronary arteries was observed in all four hearts that were autopsied and perivascular and myocardial leukocytic aggregates were present in two hearts. Pulmonary vascular resistance was elevated in 11 of the 17 patients who underwent cardiac catheterization. In summary, cardiovascular abnormalities associated with mixed connective tissue disease include acute pericarditis and/or effusion, mitral valve prolapse, intimal hyperplasia of coronary arteries, perivascular and myocardial leukocytic infiltrates, and pulmonary hypertension.
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PMID:Cardiovascular manifestations of mixed connective tissue disease in adults. 664 Aug 71

Various pleuropulmonary manifestations are reported to occur in the course of systemic lupus erythematosus (SLE). Among these, pulmonary hypertension is one of the most uncommonly observed. The natural course of this involvement can be independent of the primary disease, and resembles primitive pulmonary hypertension. Various pathogenetic mechanisms might be responsible for this picture. In the case here reported, the pathological data were strongly suggestive of pulmonary vasculitis, emphasizing that SLE, like other connective tissue diseases, can be responsible for fatal pulmonary hypertension.
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PMID:Pulmonary hypertension and systemic lupus erythematosus. 668 Nov 42

Overt pulmonary hypertension in systemic lupus erythematosus (SLE) is rarely reported. We have documented 4 such patients, all of whom had severe extrapulmonary manifestations of the disease and also Raynaud's phenomenon. The relationship between Raynaud's phenomenon and pulmonary hypertension in SLE is borne out by a review of the literature. A common vasospastic aetiology is suggested.
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PMID:Pulmonary hypertension in systemic lupus erythematosus: a report of four cases. 668 Nov 41

Although pulmonary involvement commonly occurs in systemic lupus erythematosus (SLE), pulmonary vascular hypertension has rarely been reported. Two patients with SLE had pulmonary hypertension without underlying pulmonary parenchymal or cardiac disease. The first patient's condition was initially diagnosed as primary pulmonary hypertension (PPH), and full-blown SLE subsequently developed. The second patient had well-established SLE when respiratory symptoms secondary to underlying pulmonary hypertension developed. There is a possible relationship between connective tissue diseases and PPH.
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PMID:Systemic lupus erythematosus and pulmonary vascular hypertension. 670 30

In many cases of chronic microembolic pulmonary hypertension the source of the emboli is unknown. Disease or injury to the sinus node is usually accompanied by thrombus formation within the antrum atrii dextri, and this region is not often inspected carefully during necropsy. In the present study the hearts of 14 patients with right ventricular hypertrophy or documented pulmonary hypertension showed disease or injury in the sinus node, and each had both old and recent thrombosis in the antrum atrii dextri. In all 14 cases both lungs were available for histological examination. The group included six cases of rheumatic heart disease with mitral stenosis, four cases of disseminated lupus erythematosus, and four cases of so-called primary pulmonary hypertension. Every lung contained both new and old microemboli, as well as a wide variety of other histological abnormalities narrowing the small pulmonary arteries. Many of these narrowing lesions were recognisable as the consequence of prior microembolisation. For future postmortem examination of cases of chronic microembolic pulmonary hypertension, mural thrombi within the antrum atrii dextri should be considered as one important potential source for recurring microembolisation to the lungs.
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PMID:Thrombi in antrum atrii dextri of human heart as clinically important source for chronic microembolisation to lungs. 682 32

Pulmonary hypertension has been reported rarely in patients with systemic lupus erythematosus (SLE). During the past 31/2 yr we have observed pulmonary hypertension as a major clinical manifestation of their disease in four of 43 patients with well-documented SLE followed at out institution. Pulmonary hypertension could be attributed to underlying lung disease in three and was considered to be primary in the remaining patient. Neither hydralazine nor prednisone administration had any effect on the course of the pulmonary hypertension in these patients. The presence of pulmonary hypertension in the course of active SLE may be more common than previously recognized.
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PMID:Pulmonary hypertension in systemic lupus erythematosus: report of four cases and review of the literature. 702 13

Three autopsy cases of systemic lupus erythematosus with unique association of multiple nodular hyperplasia (MNH) of the liver, portal hypertension, and hypertensive pulmonary vascular disease are reported. None of the patients had received oral contraceptive or androgenic steroid, but they were treated with glucocorticoids for 2 to 11 years. Raynaud's phenomenon, sclerodactyly, and mild impairment of the kidney were the common clinical features. Macroscopically, MNH is characterized by many nodules scattered throughout the non-cirrhotic liver, and histologically, each nodule is made up of normal-appearing hepatocytes and not encapsulated. Portal tracts are scanty in the nodules. MNH seems to be a regenerative-hyperplastic process, but its true nature still remains unclear. Relationships between MNH and portal hypertension, MNH and pulmonary hypertension, and collagen disease and pulmonary hypertension are discussed. A brief review of the literature concerning multiple benign hepatocellular tumors similar to MNH is also presented.
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PMID:Systemic lupus erythematosus associated with multiple nodular hyperplasia of the liver. 710 12

Primary pulmonary hypertension is an irreversible and fatal disorder. Every effort should therefore be made to discover all the other treatable diseases which may be associated with pulmonary hypertension. The association of systemic lupus erythematosus and pulmonary hypertension was rarely reported in the past. We add another case in which pulmonary hypertension was the presenting symptom of systemic lupus erythematosus (SLE). In contrast to the previously reported cases, our patient responded well to corticosteroids. It is assumed that this favorable response was due to the relatively early stage of the disease, when the histopathologic pulmonary changes were still in the reversible inflammatory stage.
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PMID:Corticosteroid responsive pulmonary hypertension in systemic lupus erythematosus. 718 37


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