Lupus
-
Hemophagocytic syndrome is an unusual but fatal disorder characterized by pancytopenia and activation of macrophages. We describe one case of acute systemic lupus erythematosus with an unusual presentation of hemophagocytic syndrome not related to infection. The patient presented with pancytopenia related to increasing hemophagocytic activity of histiocytes in the bone marrow. ⋯ She underwent immunosuppressive therapy with recovery from the hemophagocytic syndrome. Therefore, diagnosis of acute lupus hemophagocytic syndrome was made. The clinical presentation, laboratory diagnosis, and management of the patient are discussed and the literature was reviewed and presented, with emphasis on a possible distinct lupus subset, which includes a more aggressive systemic disease with heart involvement.
-
Combined central retinal artery occlusion and central retinal venous occlusion have been rarely reported in patients with systemic lupus erythematosus and anti-phospholipid syndrome. The impact of this severe vaso-occlusive disease on vision is usually devastating and permanent in spite of vigorous treatment. ⋯ The retina regained a normal appearance with her vision recovering to 6/6 2 weeks after the episode of temporary vision loss. Her rapid recovery suggests that continued anti-coagulation therapy and close follow-up to prevent severe complications and recurrent thrombosis is warranted.
-
The objectives of this study were to identify risk factors associated with mortality in patients with systemic lupus erythematosus (SLE) admitted to the intensive care unit (ICU) and to evaluate the usefulness of Acute Physiologic and Chronic Health Evaluation (APACHE) II score to predict outcomes in these patients, through the use of a retrospective patient record review from a multidisciplinary intensive care unit in a teaching hospital. One hundred and four patients with SLE admitted to the ICU were included in the study. The mean age of patients was 32.44 years, 96.2% were female and 61.5% were admitted with infection. ⋯ The most common cause of admission was infection. The factors associated with mortality were high APACHE II score and the use of inotropics/vasopressors. APACHE II score was unable to accurately predict mortality.
-
Live vaccines are not safe for immuno-compromised patients and should not be given to patients with systemic lupus erythematosus. In addition, all vaccines are not recommended for systemic lupus erythematosus patients when their disease is very active and mainly for patients with very active lupus nephritis. ⋯ However, vaccines may trigger the generation of autoantibodies which is usually short term and has no clinical significance. In individual cases vaccines exacerbate systemic lupus erythematosus; however, no specific clinical or laboratory variables have been identified to be associated with flare of systemic lupus erythematosus following vaccination.
-
The aim of this study was to explore whether polymorphisms of the Fcgamma receptors (FcgammaRs) IIB T/I232 and FcgammaRIIIB NA1/NA2, confer susceptibility to systemic lupus erythematosus (SLE) and lupus nephritis (LN). The authors conducted a meta-analysis on associations between the FcgammaRIIB T/I232 and FcgammaRIIIB NA1/NA2 polymorphisms and SLE and LN susceptibility as determined using 1) allele contrast, 2) recessive, 3) dominant models and 4) contrast of homozygotes. A total of 16 separate comparisons were considered, consisting of 2887 SLE patients and 3105 controls. ⋯ However, in Europeans no such association was found. In contrast, no association was found between SLE or LN and the FcgammaRIIIB NA1/NA2 polymorphism in all subjects, or in European and Asian populations. This meta-analysis shows that the FcgammaRIIB T/I232 polymorphism confers susceptibility to SLE, especially in Asian-derived populations.