Exudative enteropathy in disseminated lupus erythematosus. Report of 3 cases and review of the literature

Protein-losing enteropathy (PLE) is characterized by loss of essentially protein substances into the gastrointestinal tract. Few reports of PLE supervening in patients who have systemic lupus erythematosus (SLE) have appeared in the literature. We report three new cases. All three were women who had a severe form of SLE involving several organs. PLE was diagnosed on the basis of an increased clearance of alpha 1 antitrypsin. The severeness of the clinical picture in all three patients justified the use of immunosuppressive agents (corticosteroids and pulse cyclophosphamide therapy) which were effective. These cases are compared to the 24 previously reported. The frequency of PLE during an SLE flare-up is probably underestimated. It should be looked for in SLE patients who have edema by means of the simple alpha 1 antitrypsin test. PLE is often found in severe clinical forms of SLE and should be managed using corticosteroids either alone or in association with immunosuppressive drugs.     

Systemic lupus erythematosus and multiple myeloma: an uncommon association. Two cases and literature review

The association of systemic lupus erythematosus and multiple myeloma is uncommon. We report two cases of systemic lupus erythematosus associated to multiple myeloma. The cases are discussed in the light of a review of the literature. The clinical, laboratory and radiographic findings of the patients, as well as the subsequent therapeutic approach are discussed. A systematic review of all the other cases of this association is performed. We report two female patients of 50 and 35 years old who developed a multiple myeloma seven and three years respectively after the diagnosis of systemic lupus erythematosus. In the second case, systemic lupus erythematosus was associated to monoclonal gammopathy. One patient died after three months and one patient is still in remission after three years of the diagnosis of multiple myeloma. The coexistence of systemic lupus erythematosus and multiple myeloma is very rare and the possible pathogenetic mechanisms underlying this association remain unclear.     

Myelopathy revealing lupus. Two cases and review of the literature

INTRODUCTION: Myelopathy is a rare manifestation of systemic lupus erythematosus, occurring most often during the course of the disease. EXEGESIS: We report two cases of women with myelopathy as the first manifestation of systemic lupus erythematosus; both had an unusual course. We review the literature for previously reported cases. CONCLUSION: The clinical presentation of myelitis is heterogeneous. Usually, neurologic deficits evolve within a few hours (typically acute transverse myelitis) and outcome is usually poor. However, chronic or recurrent transverse myelitis has also been reported, including relapsing myelitis that resolved spontaneously. Myelopathy can be the first manifestation of the disease and this might be more common than initially thought. Magnetic resonance imaging (MRI) findings depend on the timing of the examination and the stage of the disease; the MRI may therefore be normal. An association with optic neuritis is frequently reported in the literature and differential diagnosis with multiple sclerosis may be difficult. Overlapping features between both diseases have been termed "lupoid sclerosis" and are actually classified as demyelinating syndromes associated with lupus. Myelopathy does not appear to be consistently associated with antiphospholipid antibodies, as has been previously suggested. The best treatment protocol has not been determined; however, in recent years, pulses of methylprednisolone and cyclophosphamide have gained acceptance by most authors.     

Associated myasthenia and disseminated lupus erythematosus. A report on two cases and complete review of the published literature (author's transl)]

Two patients aged 47 and 48 years respectively were found to have associated immunity disorders: myasthenia and DLE in the first case, and erythroblastic anemia, myasthenia, a lupus syndrome, and a thymoma in the second case. The association of myasthenia and DLE has been reported 39 times in the published literature (20 times only if stricter biological criteria are applied). The association does not increase the severity of the patient's condition and a thymoma is not present more frequently. Studies on the major histocompatibility complex and lymphocyte levels are still insufficient in this context: the haplotype was HLA A1 B8 in three cases out of seven. The physiopathological data available cannot confirm the possibility of a common pathogenesis in which the thymus and lymphocytes could play a determining role.     

Posterior reversible encephalopathy syndrome during systemic lupus erythematosus: four new cases and review of the literature

Posterior reversible encephalopathy syndrome (PRES) associates various neurological manifestations (headaches, seizures, altered mental status, cortical blindness, focal neurological deficits, vomiting) and transient changes on neuroimaging consistent with cerebral edema. Posterior reversible encephalopathy syndrome mainly occurs in the setting of hypertension, eclampsia, renal failure and/or use of immunosuppressive drugs.We report four cases of PRES complicating systemic lupus erythematosus (SLE). In all our cases, renal involvement and hypertension were present. Neurological symptoms were typical. Magnetic resonance imaging showed posterior cerebral edema and in one case hemorrhagic complication. With symptomatic treatment and immunosuppressor withdrawal when they were previously used, symptoms fully resolved within 15 days in all cases, but one who had only partial regression related to cerebral hemorrhage.Including our cases, we reviewed a total of 46 patients with SLE and PRES. Their clinical and radiological presentation was not specific. The peculiar role of SLE itself in the occurrence of PRES was not clear, since hypertension (95%), renal involvement (91%), recent onset of immunosuppressive drugs (54%) and/or recent treatment with high intravenous dose of steroids (43%) were often present. The hypertension and other worsening factors should be treated. Finally, the evolution of this clinical and radiological spectacular syndrome is generally rapidly favorable.     

Transverse myelopathy in systemic lupus erythematosus. Report of three cases and review of the literature.

Three cases of transverse myelopathy associated with systemic lupus erythematosus were reported, and 23 similar cases previously reported were reviewed. A diagnosis of systemic lupus erythematosus was made in only 60% before the onset of transverse myelopathy. The time of onset of myelopathy was randomly distributed during the disease. The most common presenting neurologic symptom was numbness, or weakness of the legs, or both. A unique association between the acute stage of transverse myelopathy and marked reduction of cerebrospinal fluid glucose concentration was observed. Thirteen patients died, 9 had permanent neurologic deficits, and only 4 recovered nearly normal function. Eight patients had ischemic necrosis of the spinal cord at postmortem examination, and vascular lesions were found in the spinal cord of 3 additional patients. The value of steroid treatment was uncertain. Patients who were started on steroid therapy within 24 hours of the onset of myelopathy may have benefited.     

Myocardial infarction and disseminated lupus erythematosus

Myocardial infarction is an underestimated complication of disseminated lupus erythematosus (DLE). Its features, treatment and prognosis are poorly understood. From June 1988 to December 2002, out of 1572 consecutive patients admitted during the first hours of acute myocardial infarction with ST elevation, 7 (5 women, aged 38 +/- 7 years) had DLE. The commonest risk factor was smoking (N = 4). There was a higher incidence of anterior infarction (N = 5). The infarct occurred 7 +/- 5 years after diagnosis of DLE. There were other complications of DLE in all cases. Three patients had antiphospholipid syndromes. The culprit artery was usually the left anterior descending (N = 5). The lesions included stenosing atheroma (N = 5) and extensive thrombosis (N = 5). The coronary disease was usually limited to a single vessel (N = 5). Revascularisation procedures include pre-hospital thrombolysis (N = 3) followed by immediate angioplasty (N = 2) or primary angioplasty (N = 4). TIMI grade 3 flow was obtained in all cases, 278 +/- 162 min after the onset of symptoms. The clinical course was characterised by acute reocclusion in 3 patients, recurrent in 2 patients with an antiphospholipid syndrome, and death in 1 case. Acute myocardial infarction occurs in already complicated cases of DLE, in young patients, associating atherosclerosis and extensive thrombosis. The risk of early reocclusion after reperfusion is high, especially in cases with the antiphospholipid syndrome.     

以精神障碍为首发的系统性红斑狼疮1例并文献复习

正系统性红斑狼疮(systemic lupus erythematosus,SLE)是一种累及全身各系统的自身免疫性疾病,是由于遗传、内分泌、环境等因素相互作用,导致机体免疫失调引起的慢性炎性疾病。病程呈复发-缓解型,广泛器官损害的易感性。文献报道~[1],约14%~75%有中枢神经系统损害。SLE伴有精神异常和/或神经功能障碍,临床上称之为神经精神性系统性     

Pulmonary silicosis and disseminated lupus erythematosus

In a 59-year old sand-blaster, histologically proven silicosis was complicated by systemic lupus erythematosus (SLE) and focal glomerulonephritis with IgG, IgA and ClQ deposits. Nothing likely to facilitate SLE was detected by investigating the familial background, the HLA phenotype and the complement system. This type of SLE differs from drug-induced lupus-like syndromes by a high level of anti-double helix DNA antibodies and by the renal lesions observed. The connection between silicosis and SLE lies in changes in humoral immunity, i.e. polyclonal activation and production of antinuclear antibodies. A decrease in the number of suppressor T-cells may also be held responsible.     

Thrombosis in disseminated lupus erythematosus

In the course of disseminated lupus erythematosus (DLE), the development of arterial or venous thrombosis is sometimes observed. They are rare in the course of hematological forms of DLE, in which the more or less constant leucopenia is accompanied to variable extent by the affection of the other blood-lines: thrombocytopenia and/or thrombocytopathy and/or hemolytic anemia. The discovery of a circulating anticoagulant seems to be correlated with the existence of thrombosis. In fact it involves antiphospholipid antibodies, responsible for the protraction of certain coagulation tests, which shows up their existence. Recent studies have shown recognition of the membrane phospholipids of the vascular endothelial cell. A functional imbalance in this would be observed to encourage the synthesis of factors favorable to thrombosis.     

Pulmonary hypertension in systemic lupus erythematosus: Report of four cases and review of the literature

Pulmonary hypertension has been reported rarely in patients with systemic lupus erythematosus (SLE). During the past 3$\text{1}\text{2}$ 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 our institution.Pulmonary hypertension could be atrributed 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.     

Systemic lupus erythematosus and sickle hemoglobinopathies: a report of two cases and review of the literature

Two adolescent black females with sickle cell disease who developed systemic lupus erythematosus (SLE) are presented. The recognition of SLE was delayed because all new symptoms were initially attributed to their underlying sickle cell disease. Nine similar cases have been previously reported. The hypothesis that a deficiency of the alternative complement pathway in some patients with sickle hemoglobinopathies predisposes them toward immune complex disorders was not confirmed in our study. Our first patient had normal and our second increased activity of the alternative pathway of complement activation.     

Thymoma associated with systemic lupus erythematosus and immunologic abnormalities

The association between Systemic Lupus Erythematosus (SLE) and thymoma occurs with a greater frequency than dictated by coincidence alone. The immunologic effects of thymectomy on the appearance and/or the course of SLE are still to be elucidated. We report one case of SLE diagnosed at the same time as thymoma, and two cases of thymoma associated with immunologic disorders in the absence of clinical signs and symptoms diagnostic of SLE.