Evidence for a therapeutic effect of plasmapheresis in patients with systemic lupus erythematosus.

Fourteen patients with active systemic lupus erythematosus (SLE) have been treated with plasmapheresis at a rate of two litres daily on three to four days per week, over a period of two to three weeks. Plasma was replaced isovolemically with either fresh frozen plasma or with human plasma protein fractions. Ten patients were receiving treatment with prednisone at the time of plasmapheresis, and four had received no prior treatment. Eight patients showed evidence of either clinical improvement or clinical and immunochemical improvement, at the time of plasmapheresis. In the three patients who showed high levels of circulating complexes before treatment, there was a sudden fall in the level of circulating immune complexes, which was quantitatively greater than could be explained by the amount removed. This suggests that in some patients with SLE, clearance of complexes by the mononuclear phagocytic system is initially blocked by high levels of circulating complexes and that one effect of plasmapheresis may be to relieve this blockade. Five patients showed a clinical response to plasmapheresis despite the fact that tests for immune complexes were negative. Three patients showed no response to plasmapheresis, and three were regarded as unevaluable. In a limited number of patients, who show a high level of circulating immune complexes, and whose condition is deteriorating despite treatment with corticosteroids, there may be an important therapeutic role for plasmapheresis.     

Uterine-umbilical artery Doppler velocimetry in pregnant women with systemic lupus erythematosus.

We evaluated continuous wave uterine-umbilical artery Doppler velocimetry for predicting pregnancy outcome in women with systemic lupus erythematosus (SLE). Lupus anticoagulant (LAC) and anticardiolipin (ACL) antibody status also were correlated with Doppler results and outcome. Three Doppler vascular patterns were identified in 27 pregnancies of 26 women with SLE. Patients with normal flow velocity in both vessels had normal outcomes (n = 18). Reduced flow velocity of the umbilical artery only was present in five women, whose newborn infants were of lesser gestational age and birthweight, two being small for gestational age. In four pregnancies reduced flow velocity was noted in both vessels. These cases had the poorest outcome, with three perinatal losses and all fetuses being small for gestational age. Doppler velocimetry showed 100% sensitivity and negative predictive value in the detection of the small for gestational age fetus and abnormal antepartum fetal heart rate tracing. Fourteen of 18 women with normal Doppler studies did not have preeclampsia or SLE flare-ups, whereas all nine women with abnormal Doppler studies had such complications. In all 27 pregnancies the women were screened for LAC, and 21 women also were tested for the ACL antibody. Poor correlation was found between antiphospholipid antibody status and Doppler results in three of the six pregnancies with positive antibody testing the patients had normal Doppler studies and outcomes. Thus, Doppler velocimetry may help determine when these substances will affect the outcome adversely. In this study the umbilical-placental vascular system was affected more often. Uterine-umbilical arterial Doppler velocimetry uniquely identified the fetus at risk for adverse perinatal outcome in pregnancies complicated by SLE. Thus, it is a potentially valuable tool in clarifying the pathophysiology and in the management of SLE in pregnancy.     

Endobronchial carcinoid presenting as Focal bronchiectasis in a young woman with systemic lupus erythematosus

Non‐resolving pulmonary symptoms in a patient with SLE require evaluation to exclude rare pulmonary lesions, such as carcinoid tumors.     

Successful preoperative treatment of a Graves' disease patient with agranulocytosis and hemophagocytosis using double filtration plasmapheresis

Agranulocytosis is an uncommon but serious complication of Graves' disease under thionamide therapy. In some patients removal of circulating thyroid hormones and thyroid antibodies by plasmapheresis is an effective adjunctive therapeutic option. In perioperative settings, however, plasmapheresis may cause excess bleeding intraoperatively due to coagulation factor depletion unless fresh frozen plasma (FFP) products are used in the replacement fluid mix. Double filtration plasmapheresis (DFPP) in which only a small amount of albumin supplementation is used may be a potential alternative to conventional apheresis interventions where clotting factor depletion is problematic. We report a case of a patient with Graves' disease complicated with intravenous immunoglobulin responsive methimazole-induced agranulocytosis/hemophagocytosis who underwent successful preoperative DFPP treatment in preparation for thyriodectomy. In addition to conventional apheresis using FFP replacement, DFPP may offer an effective adjunct option in the management of hyperthyroid patients needing emergent surgical interventions.     

Recovery of both acute massive pulmonary hemorrhage and acute renal failure in a systemic lupus erythematosus patient with lupus anticoagulant by the combined therapy of plasmapheresis plus cyclophosphamide

Acute massive pulmonary hemorrhage (AMPH) is a rare and highly fatal complication in systemic lupus erythematosus (SLE). We report here survival in a case of AMPH in a SLE patient with both rapidly progressive glomerulonephritis and lupus anticoagulant. The AMPH occurred while the nephritis was refractory to 2 courses of pulse methylprednisolone therapy. After combined therapy with plasmapheresis plus cyclophosphamide, circulating immune complex levels declined, AMPH recovered, and serum creatinine levels returned to normal. In conclusion, the combined therapy of plasmapheresis plus cyclophosphamide should be considered for treating AMPH especially in those SLE patients with rapidly progressive glomerulonephritis.     

Antisynthetase syndrome with refractory lung involvement and myositis successfully treated with double filtration plasmapheresis

Antisynthetase syndrome (ASS) is characterized by inflammatory muscle disease, pulmonary and joint involvement, and antisynthetase autoantibodies, with anti‐Jo‐1 antibody being the most common. Despite the use of immunosuppressive drugs, the prognosis of lung involvement seems poor. Herein, we report a case of refractory ASS, which maintained long‐term remission by double filtration plasmapheresis (DFPP) combined with immunosuppressive therapy. For a 65‐year‐old woman, who was diagnosed with ASS, immunosuppressive therapy was initiated and plasmapheresis (PP) was performed five times due to acute interstitial pulmonary disease and inflammatory myopathy. She remained in remission for eight months following PP. Increase in interstitial involvement was identified by lung tomography when the patient presented again with complaint of progressive increase in dyspnea and muscle pain. Although the immunosuppressive therapy was increased for the patient with elevated creatine phosphokinase (CPK) (2776 IU/mL), a rapid decrease in diffusion capacity of the lung for carbon monoxide (DLCO) was observed and the patient underwent PP. After four sessions of therapy, insufficient clinical and laboratory response was obtained (control CPK 1797 IU/mL) and because of that issue DFPP using a 2A filter was performed to the patient. There was a marked improvement in complaints of the patient, DLCO, and laboratory findings (control CPK 508 IU/mL) after three sessions of DFPP. The patient, who continued the immunosuppressive therapy after DFPP procedure, is being followed for 12 months in remission. Although our experience is limited with only one patient, DFPP seems promising as a treatment option for ASS with severe lung involvement. J. Clin. Apheresis, 28:422–425, 2013. © 2013 Wiley Periodicals, Inc.     

Cyclosporin and plasmapheresis in treatment of progressive systemic lupus erythematosus (pSLE)

The prognosis of patients suffering from progressive systemic lupus erythematosus (pSLE) is poor, despite treatment with intensive drug regimens with combinations of corticosteroids, azathioprine and cyclophosphamide. Side-effects such as infections and malignomas often occur. In the present trial, 21 patients (four male, 17 female, aged 37.9 +/- 12.8) suffering from pSLE for 9.4 +/- 2.6 years, were treated for 2.3 +/- 1.8 years with drug regimens as mentioned above. Then, over a period of 6.4 +/- 2.6 (range 1-8) years, in addition to conventional therapies, cyclosporin (2.5 +/- 0.6 mg/kg body wt/d) and, in active stages of the disease with extremely high concentrations of anti-ds-DNA-, anti-nuclear antibodies and circulating immunocomplexes, plasmapheresis (therapeutic plasma exchange (TPE)) have been applied. Compared with previous treatment modalities, significantly (P < 0.05) more effective and rapid reduction of antibodies was achieved. Clinical symptoms improved within 2 to 4 weeks. Under the new therapeutic regimen all patients reported increased performance and a better quality of life. After 5 to 48 (17.5 +/- 13.8) months, cyclosporin was established as mono-therapy for 8/21 patients. In] the other cases, corticosteroids, azathioprine and cyclophosphamide were reduced by 40 to 100%. No severe side-effects were seen. In acute stages of pSLE and in forms with persistently high antibody levels, the addition of TPE to conventional therapy was very effective, with regard to improving both clinical and laboratory parameters.     

The patient with systemic lupus erythematosus.

SLE is an illness of unknown cause(s) affecting multiple organ systems, whose diagnosis depends upon a high index of suspicion and knowledge of its clinical manifestations. The American Rheumatism Association criteria may be used as a guide to help direct one's clinical and laboratory approaches. Survivorship appears to be improving and a wider spectrum of disease is appreciated today.     

Psychiatric illness associated with systemic lupus erythematosus.

Estimates of the frequency of mental changes associated with systemic lupus erythematosus (SLE) range from 3% to 65%. Variations in classifying and reporting symptoms account for much of the difficulty in determining the true incidence. Other problems in study design also make it difficult to compare results of various studies, although it appears that psychiatric illness associated with SLE falls into two categories: organic and functional disorders. The former occur in 22% to 29% of patients having SLE and rheumatoid arthritis and are associated with a poorer prognosis and higher mortality. The latter occur in 18% to 35% of SLE patients and slightly less in patients with rheumatoid arthritis. Several possible causes for mental changes of SLE have been proposed. The case of a patient with SLE having an acute psychotic episode illustrates the possible roles of premorbid personality, mental and environmental stress, and steroid therapy in precipitating psychiatric illness in SLE.     

Listerial infections in patients with systemic lupus erythematosus.

Infection with Listeria monocytogenes is known to occur more frequently in immunosuppressed patients, including those receiving high-dose prednisone or cytotoxic therapy for collagen vascular disease. We reviewed three cases of listeriosis and systemic lupus erythematosus (SLE) seen at our institution, in addition to five cases reported in the English literature. Seven of the eight patients had non-CNS listerial infections. All patients but one had associated risk factors of either renal failure or pregnancy. From our review, we found that listeriosis is uncommon in SLE, and patients without renal failure or pregnancy do not seem to be at increased risk for listeriosis. Although most patients were treated with high-dose prednisone, with or without cytotoxic drugs, the role of immunosuppression by these drugs as a risk factor for listeriosis remained unclear.     

Transverse myelopathy in systemic lupus erythematosus.

There have been 28 reported cases of transverse myelopathy associated with systemic lupus erythematosus. In a 6-month period, the authors saw 2 new cases. Both of these patients were young women (29 and 31 years old) who had received a diagnosis of systemic lupus erythematosus prior to the onset of the transverse myelopathy. The case histories of the 2 patients are reported in detail and the findings are compared with those of previously reported cases. The rehabilitation program and outcome are described. Systemic lupus erythematosus should be considered in the differential diagnosis of transverse myelopathy because there may be many unsuspected cases.     

系统性红斑狼疮合并妊娠14例临床分析

目的分析并探讨系统性红斑狼疮(SLE)患者的妊娠情况,孕期产期并发症及妊娠结局。方法回顾性分析卫生部北京医院收治的14例系统性红斑狼疮合并妊娠患者的临床资料,分析妊娠对SLE患者病情的影响及对母亲胎儿的影响。结果 14例患者中,3例(21%)患者妊娠诱发系统行红斑狼疮发病,5例(36%)患者妊娠诱发系统性红斑狼疮活动,其余6例病情稳定(43%)。14例患者中,3(21%)例流产,11(79%)例分娩。在11例(72%)成功分娩者中,有4例(29%)患者母婴平安。7例(50%)出现围生期母婴并发症,新生儿出现1例胎盘血流灌注,2例宫内窘迫,1例低体质量儿;产妇1例出现左心力衰竭,1例妊娠高血压,2例贫血,产后出血。病情活动组患者的SLE疾病活动指数最高值,母婴并发症发生的例数均明显高于病情稳定组(均P<0.05)。结论 SLE妊娠后可诱发系统性红斑狼疮发病及病情活动,围生期可能出现母儿并发症。     

Cognitive bias in systemic lupus erythematosus.

Evidence of cognitive bias in depression and anxiety has sparked an increasing interest in the potential for pain-related bias in patients suffering from chronic pain and/or illness. Research to date has been somewhat inconsistent, and the vast majority has been conducted on just two patient populations: rheumatoid arthritis (RA) and chronic pain patients. The present study investigates cognitive bias in Systemic Lupus Erythematosus (SLE) patients, particularly in relation to disease activity and depression. Forty-three SLE patients are compared to RA patients and healthy controls on their endorsement and recall of pain/illness words relative to depression and control words. Patients are first divided according to their disease activity, and secondly according to depression. SLE and RA patients self-endorsed more negative illness words and fewer positive illness words than did healthy controls, regardless of disease activity or depression status. Groups did not differ in their recall patterns, although all groups demonstrated a recall bias for positive words and illness words. Post-hoc analyses revealed a significant recall bias for disability-related illness words compared to sensory pain words in ill, depressed patients compared to ill, non-depressed patients and healthy controls. Consistent with the most recent research, it appears to be both the nature of the illness stimuli and the depression status of the patient that determines cognitive bias in chronically ill populations.