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

The clinicopathological features of four patients with systemic lupus erythematosus and pulmonary hemorrhage are described. Our study confirms that pulmonary hemorrhage may be a dominant clinical expression of lung involvement in this disease. Its clinical manifestations are usually quite characteristic. However, hemoptysis may be absent. Radiographically, bilateral alveolar infiltrates resembling pulmonary edema or infection may be seen. Pulmonary hemorrhage was a major contributing factor to the death of three of our patients. The possible pathogenetic mechanisms responsible for pulmonary hemorrhage in our patients and other patients previously recorded in the literature are reviewed. Evidence supporting an immune complex pathogenesis is presented. Our immunopathological and ultrastructural studies demonstrate deposition of immune aggregates in the lungs in the alveolar septa, large blood vessels, and bronchioles in a manner similar to that which has been observed in the experimental serum sickness model of immune complex mediated pulmonary injury. The histological abnormalities, although nonspecific, are consistent with this interpretation, and collectively show diffuse alveolar lining cell and endothelial cell injury. However, an immune complex pathogenesis may not completely explain the occurrence of pulmonary hemorrhage in SLE. Other factors, including bleeding disorders, pulmonary infection, oxygen toxicity, and the "shock lung" syndrome, may also have contributed to lung hemorrhage in some of these patients.
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PMID:Pulmonary hemorrhage in systemic lupus erythematosus. 36 23

In 7 years (1981-1988) at the Kenyatta National Hospital (KNH), Nairobi the diagnosis of systemic lupus erythematosus (SLE) was made in 67 patients. In 23 of these patients lupus nephritis complicated the SLE. Lupus nephritis was diagnosed through renal biopsy, haematuria and proteinuria in urine with positive lupus erythematosus (LE) cell phenomenon. The histology found in these patients included 5 patients with minimal lesion, 7 patients with membranous, 3 with focal, 4 with diffuse, 3 with crescenteric and one with membranoproliferative glomerulonephritis. While patients with minimal, membranous and focal nephritis had general good outlook on low dose maintenance or intermittent high dose steroid therapy the others with diffuse, crescenteric and membranoproliferative nephritis had poor prognosis. Patients with diffuse proliferative, membranoproliferative and crescenteric nephritis tended to have septicaemia, pulmonary oedema, fluid overload and chronic renal failure with poor prognosis. These patients responded poorly to oral and parenteral steroid therapy whether high or low dose.
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PMID:Management of lupus nephritis at the Kenyatta National Hospital. 227 66

Two cases of pathologically confirmed miliary pulmonary tuberculosis complicated with ARDS were presented. Both had systemic lupus erythematous and used maintenance dose of corticosteroid. Case one developed respiratory distress and severe hypoxemia one day postpartum and chest radiograph revealed nodular and miliary infiltrations and pleural effusion. The patient was intubated and placed on a volume-cycled ventilator. A FIO2 of 70% and a PEEP of 0.98 kPa were required to maintain the oxygen tension at 6.95 kPa. The effective compliance of the lung decreased progressively and the patient died 5 days later. Autopsy revealed disseminated tuberculosis extensively involving the lungs, the liver and kidney. The alveoli were filled with edematous fluid with formation of hyaline membranes and micro-atelectasis. Case two developed respiratory distress and pulmonary edema at the third month of pregnancy. Cardiopulmonary arrest occurred when trying to intubate the patient. Postmortem needle puncture of the lungs and liver revealed charges comparable with tuberculosis and ARDS. In considering the relatively high incidence of pulmonary tuberculosis in China, the percentage of miliary tuberculosis as a potential cause of ARDS might not be very low. It is important to maintain a high index of suspicion for this treatable precipitating disorder and initial appropriate therapy early enough in patients with ARDS.
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PMID:[The adult respiratory distress syndrome associated with miliary tuberculosis]. 273 72

The authors present a retrospective study of all the patient followed up for systemic disease in the rheumatology Department of Bichat hospital between 1975 and 1984 in whom aortic regurgitation developed. Only rare or previously undescribed associations were retained: two MacDuffie syndromes, one adult form of Still's disease, one Takayashu's disease, one association of rheumatoid arthritis and Takayashu's disease, one rheumatoid arthritis, one Cogan's syndrome and two cases of disseminated lupus erythematosis. The authors use these cases and a review of the literature to discuss the possible physiopathological mechanisms of the aortic regurgitation. This study confirms the value of regular clinical cardiovascular examination with echocardiography in cases with progressive symptoms. The evolution of the vascular disease seems to be more or less parallel to that of the systemic disease and in a significant number of cases it becomes sufficiently severe to become the main clinical problem. In our series, there was one sudden death, one death due to cardiogenic pulmonary oedema and three patients required aortic valve replacement.
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PMID:[Aortic insufficiency in certain so-called systemic diseases]. 288 May 72

The pathological findings in the lungs and related organs of 26 patients (21 female, 5 male) with systemic lupus erythematosus (SLE), with onset of disease before age 20 years, were reviewed. Several categories of lung lesions were found. Chronic interstitial pneumonitis was present in all 26 patients and was severe in 5. Acute pneumonia was present in 20, mild in 13, moderate in 2, and severe in 5. Alveolar hemorrhage, massive enough to cause death in 5, was seen in 18 patients, and pulmonary edema was found in 13. Fourteen patients had hyaline membranes indicative of acute alveolar damage (DAD), 12 had alveolitis obliterans, indicative of prior episodes of DAD, and 9 had bronchiolitis obliterans. Other parenchymal lesions were mild interstitial fibrosis in 12, alveolar hemosiderosis and alveolar overinflation in 10 each, and alveolar septal calcinosis with chronic renal insufficiency in 3. Pleural effusion, pleuritis, or pleural thickening were noted in 15 of 26, 6 of 23, and 7 of 23 evaluable patients, respectively. Vascular lesions were present in 16 as intimal thickening (9), thromboemboli (8), medial hypertrophy (6), calcinosis (3), and vasculitis (2). A previously unreported lesion was chronic (proliferative) peribronchitis, noted in 11 patients. Diaphragmatic lesions included mild variation in fiber size in 7, mild fibrosis in 2, and calcinosis in 1 of 13 evaluable patients. Correlation of the above lesions with previously described lung syndromes in SLE such as lupus pneumonitis, hemorrhagic lung disease, chronic interstitial fibrosis, lupus cor pulmonale, pleurisy, and "shrinking lung syndrome" are discussed.
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PMID:Pulmonary lesions in childhood onset systemic lupus erythematosus: analysis of 26 cases, and summary of literature. 360 12

We report a case of hypocomplementemic urticarial vasculitis syndrome (HUVS) with membranous glomerulopathy in a 62-year-old man who had a 2-month history of secondary iritis. He was transferred to our hospital because of uncontrollable edema and respiratory dysfunction. Physical examination revealed anasarca, pulmonary edema, hypertension and urticaria-like eruption on his arms. Urinalysis, blood chemistry and serological studies showed massive proteinuria (10.5g/day) with numerous granular casts, hypoalbuminemia (1.5g/dl), renal dysfunction (creatinine; 1.6mg/dl, BUN; 86mg/dl), hypercholesterolemia (total cholesterol; 455mg/dl), positive results for antinuclear factor, microsome test, thyroid test, lupus anticoaglant, antithyroglobulin test and rheumatoid factor, but LE cell or double-strand anti DNA antibody was negative. Serum complement levels were persistently low as CH50 of 13 U/ml and Clq of 6.0 micrograms/dl. The patient serum precipitated with normal human Clq by immunodiffusion analysis, indicating the presence of anti-Clq antibody. Renal biopsy revealed membranous glomerulopathy with prominent fine granular deposition of Clq along the glomerular basement membrane by immunofluorescent study and subepithelial dense deposit by electron microscopy. Corticosteroid treatment was ineffective for hypocomplementemia and nephrotic syndrome. Acute subendocardial infarction occurred on the 25th hospital day and he died of acute respiratory distress syndrome on the 45th hospital day. Autopsy revealed leucocytoclastic vasculitis in the alveolar wall. HUVS was confirmed by clinical symptoms, such as iritis and urticaria-like eruption, serum anti-Clq antibody, the absence of any specific autoantibody for systemic lupus erythematosus (SLE) and leucocytoclastic vasculitis in the alveolar wall.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Nephrotic syndrome due to membranous glomerulopathy in hypocomplementemic urticarial vasculitis syndrome;--a case report]. 807 26

Acute pulmonary edema is an unusual initial presentation for systemic lupus erythematosus. A 46-year-old woman required intensive care for life-threatening pulmonary edema of unknown etiology, which was unresponsive to conventional treatment. Her condition improved only when pulse corticosteroid therapy was initiated, with clinical and echocardiographic improvement in cardiac function. The diagnosis of systemic lupus erythematosus was then made, based on immunologic tests and renal biopsy. The patient's condition remained stable only with continuation of appropriate therapy for systemic lupus erythematosus.
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PMID:Acute pulmonary edema as the initial hospital presentation of systemic lupus erythematosus. 811 78

Twenty patients with rapidly progressing glomerulonephritis (RPGN) aged 16-50 underwent plasmapheresis (PA) as adjuvant to immunodepressants. Of these, 9 patients had lupus, 4 Bright RPGN, 4 and 3 patients combined glomerulonephritis with hemorrhagic vasculitis and Wegener granulomatosis, respectively. A total of 130 procedures were performed (3-13 per each patient). The results of PA treatment included complete recovery of renal function in 8 and partial in 10 patients (3 of them could stop hemodialysis), death of pulmonary edema (2 cases). The authors think advisable to combine immunodepressants with PA, especially when the drugs are contraindicated, in patients with malignant glomerulonephritis.
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PMID:[Plasmapheresis in the treatment of rapidly progressing glomerulonephritis]. 837 42

A 27-year-old Caucasian female, with a past history of recurrent spontaneous abortions, was admitted with pre-eclampsia at 26 weeks' gestation during her sixth pregnancy. She was previously known to have antiphospholipid antibodies since her fifth abortion, but had no clinical or serological evidence of systemic lupus erythematosus. A small-for-dates infant was delivered by emergency Caesarean section at 27 weeks for poor placental blood flow and fetal distress. She was transferred to the renal unit on the sixth post partum day with pulmonary edema, hypertension, disseminated intravascular coagulation and acute renal failure. Renal biopsy showed lesions compatible with thrombotic microangiopathy with diffuse glomerular necrosis. She was plasma exchanged and remained dialysis dependent for 7 months. Antiphospholipid antibodies were present in high titres and were the presumed cause of her acute renal failure. The patient now has stable renal function with a creatinine clearance of 30 ml/min for over two years. The late recovery of renal function is unique in the above circumstances.
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PMID:Reversible renal failure due to the antiphospholipid antibody syndrome, pre-eclampsia and renal thrombotic microangiopathy. 857 29

A 37-year-old man with systemic lupus erythematosus, who underwent an aortic valve replacement with a Carpentier-Edwards porcine valve for severe aortic insufficiency, was admitted to the hospital with pulmonary edema. Transesophageal echocardiography revealed severe aortic insufficiency arising from partial dehiscence of the valve sewing ring, as well as centrally from the valve cusp. In addition, marked thickening of the mitral valve was observed with severe eccentric regurgitation. At surgery, valvulitis of the native mitral and bioprosthetic aortic valves was demonstrated, with a perforation of the porcine valve cusp. After replacement of both valves, the patient had a stormy postoperative course with recurrent communications between the left ventricle and atrium requiring multiple surgeries and eventually died. This case illustrates the severity of valvulopathy and ensuing complications that can affect patients with systemic lupus erythematosus and demonstrates that the valvulopathy can affect bioprosthetic valves, a finding that has significant implications as to the type of valve replacement in these patients.
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PMID:Valvulitis involving a bioprosthetic valve in a patient with systemic lupus erythematosus. 867 31


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