Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0042384 (vasculitis)
 20,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

150 cases of chronic inflammatory lung diseases of unknown aetiology and assumed hyperergic (immuno-reactive) pathogenesis were examined for hypertensive pulmonary arterial lesions and for chronic cor pulmonale. Hypertensive lesions of the small pulmonary arteries were found in more than half of the cases with chronic disorders of long duration, but were inconspicuous in diseases of acute progressive character. Hypertensive lesions were found regularly in chronic interstitial pneumonia, frequently in scleroderma and rheumatoid arthritis and occasionally in dermatomyositis and disseminated lupus erythematosus. Chronic Cor pulmonale occurred in 16% of the cases with hypertensive arterial lesions of grade I (hypertrophy of media) and in 50% of grade II/III (hypertrophy of media and intimal fibrosis). Interstitial lung fibrosis plays an important role in the pathogenesis of cor pulmonale: two thirds of the cases with interstitial lung fibrosis had developed cor pulmonale and all the cases with cor pulmonale also had interstitial lung fibrosis. Hypertensive arterial lesions of grade IV-VI according to Heath and Edwards (angiitis, plexogenic and angiomatoid lesions) have been described in severe cases of pulmonary hypertension (congenital cardiac shunts, primary pulmonary hypertension). In secondary forms of pulmonary hypertension, as represented by our material, these changes are of little importance.
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PMID:[Hypertensive lesions of pulmonary arteries in chronic inflammatory lung diseases (author's transl)]. 15 72

The pulmonary vascular changes in systemic lupus erythematosus (SLE) have been investigated from 20 autopsies performed at the Mount Sinai Hospiral from 1964 to 1973. Acute lesions included fibrinoid necrosis and vasculitis. Chronic lesions consisted of intimal fibrosis, medial hypertrophy, alteration of elastic laminae, periadventitial fibrosis, and, in one case, aneruysmal dilatation. These changes were found variously in arterioles, arteries and veins. The fibrotic and occlusive vascular lesions may account for the syndrome of "unexplained breathlessness" that occurs in SLE. These lesions may progress in certain individuals to overt pulmonary hypertension; the concept of coexisting primary pulmonary hypertension and SLE should be re-examined.
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PMID:Pulmonary vascular disease in systemic lupus erythematosus. 125 21

We describe a 16-year-old girl with systemic-onset juvenile arthritis who presented with pulmonary hypertension, without evidence of pleural or parenchymal involvement of the lung, pulmonary vasculitis, or immune deposition in the pulmonary vasculature. Pleuropulmonary involvement occurs occasionally in juvenile arthritis, but primary pulmonary hypertension has not, to our knowledge, been previously reported. Histocompatibility typing showed positivity for HLA-DR3 and DRw52, both of which are associated with idiopathic pulmonary hypertension in children, and with pulmonary hypertension among patients with systemic sclerosis. Treatment with cyclosporine and corticosteroids resulted in a marked improvement in the clinical findings and pulmonary function in our patient.
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PMID:Primary pulmonary hypertension in a patient with systemic-onset juvenile arthritis. 174 43

Pulmonary hypertension due to vasculitis is a rare complication of rheumatoid arthritis (RA). We describe 2 patients with RA and pulmonary hypertension who died with a clinical diagnosis of idiopathic pulmonary hypertension. In each case postmortem examination revealed severe pulmonary vasculitis as the actual cause of the hypertension. Subclinical systemic vasculitis was found in one case and hepatic nodular regenerative hyperplasia in both cases. Pulmonary vasculitis must be included in the differential diagnosis of pulmonary hypertension associated with RA. Open lung biopsy should be considered in this clinical setting in an attempt to identify this potentially treatable cause of pulmonary hypertension.
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PMID:The association of pulmonary hypertension with rheumatoid arthritis. 281 Feb 87

Five patients with classic hemophilia were found to have primary pulmonary hypertension, a disorder not previously recognized in this population. All patients had had their coagulation disorder treated for 10 years or more with self-administered lyophilized concentrates of factor VIII, and all had antibodies to human immunodeficiency virus (HIV). Primary pulmonary hypertension was confirmed by histologic means at autopsy in one patient and by lung biopsy findings in another. In the other three patients, the findings are in agreement with this diagnosis. No patient had underlying cardiac or pulmonary disease, or clinical or pathologic evidence of collagen-vascular disease, vasculitis, parasitic disorders, hemoglobinopathy, or exposure to anorexigenic agents. Whether the primary pulmonary hypertension was related to treatment with lyophilized factor VIII, or to the presence of antibodies to HIV, or both, is unknown.
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PMID:Primary pulmonary hypertension in patients with classic hemophilia. 280 26

Eight autopsy cases of primary pulmonary hypertension were studied. All cases were diagnosed as primary plexogenic arteriopathy. Microscopical study of the lungs showed in every case hypertensive arterial changes of Grade II-VI after Health & Edwards' criteria. Truncal pulmonary artery of three cases revealed aneurysm or severe dilatation with thrombus, and dilation was also recorded in another three cases. Histology of gross pulmonary artery in several cases presented disruption and defect of elastic fiber and alcian-blue positive microcystic change in media, lymphoid cell infiltration in outer media and inner adventitia, adventitial thickening, and intimal fibrosis. The liver of 7 cases showed secondary congestive changes and in one case intrahepatic portal vasculitis.
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PMID:Primary pulmonary hypertension. Study of eight autopsy cases. 361 16

An infant dying with pulmonary hypertension had a pulmonary vessel foreign body vasculitis as identified by light microscopy and characterized ultrastructurally by scanning electron microscopy and energy-dispersive x-ray analysis. The inclusions were of two distinct types: those containing silicon and titanium, and others consisting of talc. The possible sources of these inclusions and the importance of considering foreign body vasculitis in the pathogenesis of clinically idiopathic pulmonary hypertension are discussed.
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PMID:Infantile pulmonary hypertension associated with foreign body vasculitis. 722 22

The plexiform lesion in primary pulmonary hypertension is a glomeruloid structure forming channels in branches of the pulmonary artery. These lesions have been considered an abnormal growth of modified smooth muscle cells. We present immunohistochemical evidence in 10 cases of plexogenic pulmonary hypertension that the plexiform channels and the concentric obliterative arteriopathy associated with these channels represent abnormal growth of factor VIII-related antigen-positive endothelial cells. In addition, these cells strongly expressed vimentin, a growth- and differentiation-related intermediate filament. Morphologically and immunohistochemically, the lesions resembled the neovascularization associated with the brain tumor glioblastoma multiform. Furthermore, we noted an exclusively perivascular inflammatory cell infiltrate (but no vasculitis) in seven of the 10 cases with plexogenic arteriopathy composed of T cells, B cells, and macrophages. Our findings indicate that the plexiform lesion may result from a deregulated growth of endothelial cells. The presence of perivascular inflammatory cells suggested that cytokines and growth factors may further influence the development of the plexiform lesion.
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PMID:Exuberant endothelial cell growth and elements of inflammation are present in plexiform lesions of pulmonary hypertension. 1143 86

Involvement of the pulmonary arteries is common in systemic Takayasu arteritis and, in nearly all of the reported cases, the diagnosis has been based solely on clinical and angiographic evaluations of the patients. Isolated pulmonary Takayasu arteritis occurs rarely and can be diagnosed only after histologic examination of the appropriate tissue specimens; five such patients are described herein. The patients were two men and three women. Their age at diagnosis ranged from 25 to 66 years. The initial clinical diagnosis was thromboembolism in two patients, and primary pulmonary hypertension, pulmonary granulomatosis, or pulmonary tumor or sarcoidosis in the remaining three patients, respectively. All five patients underwent surgery for lung resection (three patients) or for reconstruction/bypass of the obstructed pulmonary arteries (two patients). Three types of vascular lesions were observed in the surgical specimens: the classic large-vessel granulomatous giant cell arteritis, a peculiar type of organized thrombus with prominent recanalization and neoangiogenesis, and plexogenic arteriopathy. Thus, the histopathologic findings of pulmonary Takayasu arteritis is distinctive and differs in many aspects from that of systemic Takayasu arteritis. Moreover, the vascular lesions of pulmonary Takayasu arteritis are distinguishable from those of pulmonary hypertension, sarcoidosis, and other types of pulmonary angiitis and granulomatosis.
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PMID:Isolated pulmonary Takayasu arteritis: clinicopathologic characteristics. 873 60

Pulmonary hypertension (PH) is a rare but potentially life-threatening complication of systemic lupus erythematosus (SLE). We reviewed the literature on this complication, its pathogenesis and clinical presentation, and treatment options. PH is reported in 0.5% to 14% of patients with SLE. The literature describes the cases of 105 patients, 90% of whom were female. Average age was 33 years, and overall mortality was 25 to 50% two years after PH diagnosis. As in patients with primary pulmonary hypertension, dyspnea is the most common presenting symptom of PH in patients with SLE. Up to 58% of patients with both PH and SLE have Raynaud's phenomenon. Echocardiography can show right ventricular hypertrophy and dilation, even before symptom onset. Right-heart catheterization, with an assessment of vasoreactivity, is a necessary part of the work-up and is also needed for treatment decision-making. PH in patients with SLE is associated with intimal hyperplasia, smooth-muscle hypertrophy and medial thickening, similar to the changes seen in primary PH. Several pathological mechanisms have been proposed for PH associated with SLE. They include vasoconstriction, vasculitis, thrombosis, anticardiolipin antibody and lupus anticoagulant. Endothelial dysfunction may be an important factor in the onset of PH, possibly by contributing to vasospasm. Higher serum endothelin levels are found in patients with SLE and pulmonary hypertension than in other SLE patients. Several specific antibody patterns have been reported in patients with PH and SLE. Oral calcium channel blockers are indicated for patients who respond to acute NO challenge. Continuous intravenous prostacyclin represents a therapeutic advance, although it appears less effective than in primary PH. Some patients have been improved by new oral endothelin receptor antagonists, usually combined with intensive immunosuppressive therapy. Patients with SLE have an increased risk of PH. Vigilance is therefore required to detect early signs of PH. Early diagnosis allows treatment to start before irreversible vascular lesions occur.
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PMID:[Pulmonary hypertension associated with systemic lupus erythematosus]. 1565 27


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