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Query: UMLS:C0409974 (lupus)
 22,386 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We sought to explore immunological factors in patients who died with rapidly fatal fibrosing lung diseases (Hamman-Rich syndrome). A retrospective review of cases of interstitial lung disease showed 12 recent deaths from Hamman-Rich syndrome. The mean age was 62, men outnumbering women 3 : 1. Five patients had proved collagen vascular disease (rheumatoid arthritis three, lupus two). Four others had a history of allergic disorders, syphilis, chronic eosinophilia, or hypersensitivity reactions. One patient showed disappearance of immunofluorescence as fibrosis advanced, which has not previously been reported. The study suggests a possible aetiological link between disorders of immunity and Hamman-Rich syndrome. The evidence also supports the notion that Hamman-Rich syndrome is an accelerated variant of the more indolent interstitial pneumonias.
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PMID:Rapidly fatal pulmonary fibrosis: the accelerated variant of interstitial pneumonitis. 16 92

Pleuropulmonary disease is a common feature of the following connective tissue diseases: systemic lupus erythematosis (SLE), rheumatoid arthritis (RA), progressive systemic sclerosis (PSS), mixed connective tissue disease (MCTD), polymyositis/dermatomyositis (PM/DM), and Sjogren's syndrome (SS). Features common to most of these disorders include pleurisy with effusion and interstitial lung disease. Pleural effusions caused by SLE and RA have certain characteristics on pleural fluid analysis that aid in diagnosis, but infection and other causes of effusion must be excluded. Interstitial lung involvement is usually indolent in onset, but a more rapidly progressive course over weeks to a few months may mimic infection. Several drugs used to treat connective tissue diseases may cause interstitial disease, increase susceptibility to infection, or both. This complicates differential diagnosis. Acute lupus pneumonitis and SLE-related alveolar hemorrhage are usually fulminant processes, often associated with fever. Diagnosis of these conditions always requires exclusion of infection. Rheumatoid nodules may mimic infectious and neoplastic lung diseases. Needle biopsy helps reduce the likelihood of infection or malignancy, but open lung biopsy is needed if a firm diagnosis of rheumatoid nodules is required.
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PMID:Respiratory manifestations of connective tissue disease. 305 17

Pulmonary problems are common in systemic lupus erythematosus, and may be the presenting feature of this multi-system disease. The clinical spectrum ranges from mild, self-limited, pleuritic chest pain to fulminant and rapidly fatal, diffuse, pulmonary hemorrhage. Accordingly, treatment must be individually tailored to the clinical features of each patient. Non-steroidal-anti-inflammatory drugs may be adequate therapy for pleuritic pain. High dose corticosteroids may be indicated in more severe cases of pleurisy with effusion, lupus pneumonitis, and diffuse interstitial lung disease. Immunosuppressive drugs such as azathioprine and cyclophosphamide should be considered in cases of lupus pneumonitis or interstitial lung disease unresponsive to steroids. Combined therapy with corticosteroids, immunosuppressives and plasmapheresis should be considered for fulminant cases of diffuse pulmonary hemorrhage attributed to lupus. There is no definitive therapy for pulmonary hypertension at this time. Decisions regarding treatment in each instance must be made with the recognition that there is little strong clinical evidence to support the use of any of these therapies. Finally, no pulmonary process should be attributed to lupus until infection has been rigorously excluded in these patients.
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PMID:Pulmonary disease in systemic lupus erythematosus. 390 99

Pleuropulmonary complications of systemic lupus erythematosus (SLE) occur in 50-70% of patients and include pleuritis, pleural effusions, acute lupus pneumonitis, diffuse interstitial lung disease, atelectasis, diaphragmatic dysfunction and bronchiolitis obliterans. Additionally, a syndrome of acute reversible hypoxemia has recently been documented. This seems to occur in patients hospitalized for exacerbations of SLE and may be due to pulmonary leukoaggregation. It has become clear that other groups of pulmonary complications may be specifically associated with the antiphospholipid antibodies, both in patients with SLE and in those suffering from the "primary" antiphospholipid syndrome. These include pulmonary embolism and infarction, both thromboembolic and perhaps nonthromboembolic pulmonary hypertension, pulmonary arterial thrombosis, pulmonary microthrombosis, adult respiratory distress syndrome, intraalveolar pulmonary hemorrhage, as well as a postpartum syndrome.
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PMID:Review: antiphospholipid antibodies and the lung. 769 84

A 58 year old woman developed systemic symptoms, interstitial lung disease, splenomegaly, leukopenia and anti-histone and anti-nuclear antibodies (ANA), while treated with hydralazine for hypertension. Five months after presentation she was admitted with high fever, skin rash and atypical lymphocytosis due to acute cytomegalovirus (CMV) infection. Worsening leukopenia and increased ANA were found, and high titres of anti-DNA antibodies, anti-cardiolipin antibodies and rheumatoid factors appeared. Hydralazine was stopped and the patient gradually became asymptomatic. All autoantibodies spontaneously disappeared (over 16 weeks), and the white cell count and spleen size became normal. The patient was found to be a slow acetylator and to have both HLA-DR4 and selective IgA deficiency. Thus, a multifactorial genetic susceptibility to develop drug-induced lupus was brought out in stages first by hydralazine and then by CMV, yet all manifestations and autoantibodies resolved spontaneously, demonstrating the complex interplay of varied environmental factors with a genetic predisposition in the pathogenesis of autoimmunity.
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PMID:Effect of acute cytomegalovirus infection on drug-induced SLE. 783 Nov 73

Pericardial and lung involvement in rheumatoid arthritis (RA), suspected to be less severe in a developing nation (Turkey), have been evaluated. We have studied clinical, echocardiographic and pulmonary findings (radiological and functional) in 93 consecutive Turkish patients with definite/classical RA. Findings were compared with those of a group of patients with osteoarthritis or local rheumatological conditions (n = 60) in a blind protocol. Fifty patients with systemic lupus (SLE) were studied as a high risk control group for pericardial involvement. While pericardial disease was detected in 5.5% (5/90) of RA patients, it was detected in 6.6% (4/60) of the control patients. SLE patients had a 26% (13/50) prevalence. Interstitial lung disease was found in 27.7% of RA patients but it was present in 6.6% (4/60) of the control patients. We observed that a group of patients with RA in Turkey had a low prevalence of pericardial disease. This is further evidence that RA has a mild course in developing countries.
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PMID:Pericardial and pulmonary involvement in rheumatoid arthritis in Turkey. 808 66

Intravenous pulse cyclophosphamide (IPC) was used to treat lupus interstitial lung disease in 2 patients. Vital capacity increased by 67-110% of baseline values with IPC treatment. Total lung capacity and diffusing capacity also improved. Dyspnea and other manifestations of lupus improved dramatically. This is the first report documenting the efficacy of the early use of IPC for acute and chronic interstitial lung disease associated with lupus.
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PMID:Treatment of lupus interstitial lung disease with intravenous cyclophosphamide. 812 99

The pleuropulmonary manifestation of systemic lupus erythematous (SLE) are pleuritis, acute lupus pneumonitis, chronic interstitial lung disease with fibrosis, alveolar hemorrhage, respiratory muscle and diaphragmatic dysfunction, atelectasis, bronchiolitis obliterans, pulmonary vascular disease with pulmonary hypertension, and pulmonary embolism. This article reviews these specific pleuropulmonary consequences of SLE while focusing on clinical, pathologic, and therapeutic considerations.
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PMID:Pleuropulmonary manifestations of systemic lupus erythematosus. 815 98

Takayasu arteritis (TA) is a chronic inflammatory and obliterative disease of large vessels particularly the aorta and its major branches. Recently, the disease has been shown to involve the parenchyma of various organs. Specific glomerular lesions reported in patients with TA are mesangial proliferative, membrano-proliferative, crescentic glomerulonephritis and amyloidosis. Dermatological manifestations of this disease are erythema nodosum, facial lupus rash and erythema induratum. Dilated cardiomyopathy, myocarditis and pericarditis have been reported in TA. Rarely, interstitial lung disease, pneumonic consolidation, idiopathic ulcerative colitis, rheumatoid arthritis and polymyositis have been described in association with TA. In this report, five patients of TA with primary parenchymatous involvement of kidneys, skin, heart and gastrointestinal tract have been described. An association of primary parenchymatous organ involvement and TA suggests an autosensitization to an unidentified antigen in the pathogenesis of TA.
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PMID:Systemic manifestations of Takayasu arteritis: the expanding spectrum. 911 17

Diverse pleuropulmonary manifestations including diaphragmatic dysfunction, pleural effusion, acute lupus pneumonitis, pulmonary hemorrhage, pulmonary hypertension, and diffuse interstitial lung disease have been described in patients with systemic lupus erythematosus (SLE). Bronchiolitis obliterans organizing pneumonia (BOOP) as an initial manifestation of SLE is rarely reported. We describe 2 patients who had SLE concurrent with the onset of BOOP. Their respiratory symptoms, followup pulmonary function tests, and radiologic findings showed much improvement after steroid therapy.
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PMID:Bronchiolitis obliterans organizing pneumonia as an initial manifestation in patients with systemic lupus erythematosus. 937 94


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