Cerebellar ataxia in systemic lupus erythematosus: three case reports.

Three patients presented with cerebellar ataxia among 350 cases of systemic lupus erythematosus (SLE) seen over the last 14 years. Cerebellar signs were unilateral in one and bilateral in the other two patients. Other neurological findings were present in all three patients. One initially presented with only cerebellar ataxia; other features of SLE appeared a few years later. Lupus anticoagulant test was positive in one patient. Corticosteroids given in the early stages appeared to benefit these patients by ameliorating cerebellar dysfunction.     

Refractory hemophagocytic syndrome in systemic lupus erythematosus successfully treated with intermittent intravenous cyclophosphamide: three case reports and literature review

We experienced three patients with refractory or severe hemophagocytic syndrome associated with the activity of systemic lupus erythematosus, so-called acute lupus hemophagocytic syndrome (ALHS). All patients were successfully treated with intermittent intravenous injections of cyclophosphamide (IVCY). In each patient, hemophagocytosis was found during bone marrow examination, and infectious causes of hemophagocytic syndrome were carefully excluded. Patients 1 and 2 were refractory to combination therapy with a high-dose corticosteroid and rituximab or cyclosporine and were successfully treated with additive IVCY. Patient 3 had very severe ALHS that developed after cardiac surgery but was successfully treated with a high-dose corticosteroid and IVCY. We conducted a literature review of adult ALHS and analyzed 54 cases reported from 2006 to 2013. Although the clinical and laboratory features were diverse, fever, bicytopenia or pancytopenia, and hyperferritinemia were almost universal. Including our 3 patients, a total of 16 have reportedly been treated with IVCY-containing regimens, and ALHS was successfully controlled in most of them without switching or adding other therapies. We suggest that combination therapy with IVCY and corticosteroids may be useful to treat severe or refractory ALHS.     

Optic neuropathy as a presenting feature of systemic lupus erythematosus: two case reports and literature review

Systemic lupus erythematosus (SLE) may affect the eyes and/or visual system in up to a third of patients; however, optic nerve involvement has been rarely reported. SLE presenting as optic neuropathy is exceptional. We report two new cases of optic neuropathy as a presenting feature of SLE. The first patient presented with an ischemic optic neuropathy and antiphospholipid antibodies, and the second presented with optic neuritis. A literature review for previous cases of SLE presenting as optic neuropathy was performed. Early diagnosis of SLE-associated optic neuropathy is warranted and leads to a better prognosis.     

Subarachnoid hemorrhage in systemic lupus erythematosus in Japan: two case reports and a review of the literature

We report 51- and 43-year-old Japanese female patients with systemic lupus erythematosus (SLE) associated with subarachnoid hemorrhage (SAH) due to rupture of intracranial saccular aneurysms. We also review the literature of Japanese SLE patients with SAH. SAH in Japanese SLE patients is more frequent than in patients from Western countries, has different features from the general population, and can occur regardless of SLE disease activity. Clinicians must pay attention to SAH in all SLE patients.     

Subarachnoid hemorrhage in systemic lupus erythematosus in Japan: two case reports and a review of the literature

Abstract

We report 51- and 43-year-old Japanese female patients with systemic lupus erythematosus (SLE) associated with subarachnoid hemorrhage (SAH) due to rupture of intracranial saccular aneurysms. We also review the literature of Japanese SLE patients with SAH. SAH in Japanese SLE patients is more frequent than in patients from Western countries, has different features from the general population, and can occur regardless of SLE disease activity. Clinicians must pay attention to SAH in all SLE patients.     

Hemophagocytic syndrome as one of main manifestations in untreated systemic lupus erythematosus: two case reports and literature review

Hemophagocytic syndrome (HPS) is an unusual but fatal disorder characterized by pancytopenia and activation of macrophages. We describe two cases of untreated systemic lupus erythematosus (SLE) with HPS that presented as one of the manifestations of SLE. The onset of HPS was after parturition for one patient, and after abortion for the other. Bone marrow examination revealed severe hemophagocytosis in both patients. One patient responded to pulsed methylprednisone alone, and the other responded to pulsed methylprednisone plus intravenous immunoglobulin (IVIG). We believe accurate diagnosis, intensive therapy, and sufficient supportive cares are essential in improving patients’ prognosis.     

Late-onset systemic lupus erythematosus in Northwestern Spain: differences with early-onset systemic lupus erythematosus and literature review

To further investigate into the epidaemiology of systemic lupus erythematosus (SLE) in Southern Europe, we have assessed the incidence, clinical spectrum and survival of patients diagnosed with late-onset SLE (age?≥?50 years) according to the 1982 American College of Rheumatology (ACR) classification criteria at the single hospital for a well-defined population of Lugo, Northwestern (NW) Spain. Between January 1987 and December 2006, 51 (39.3%) of the 150 patients diagnosed as having SLE fulfilled definitions for late-onset SLE. The predominance of women among late-onset SLE (4:1) was reduced when compared with that observed in early-onset SLE (7:1). However, the incidence of late-onset SLE was significantly higher in women (4.2 [95% confidence interval (CI): 3.1-5.6] per 100,000 population) than in men (1.3 [95% CI: 0.6-2.2] per 100,000 population) (p?相似文献   

Life-threatening spontaneous splenic rupture with systemic lupus erythematosus: case report and literature review

Although involvement of the reticuloendothelial system in systemic lupus erythematosus (SLE) is a well-recognised concomitant of the disease, spontaneous splenic rupture is an unusual occurrence. We observed a 54-year-old woman with SLE who had spontaneous splenic rupture during the late course of the disease and showed some changes histopathologically. The courses and the clinical characteristics of such patients are reviewed, and the diagnosis and treatment of these cases are discussed. Early evaluation for SLE patients with spontaneous splenic rupture should be considered and included in the differential diagnosis of acute abdomen, as it may affect follow-up and treatment, although the condition is rare.     

接种流感病毒疫苗后触发系统性红斑狼疮一例并文献复习

目的：探讨外界环境因素在系统性红斑狼疮（SLE）发病机制中的作用。方法：报道1例男性14岁患者在接种流感病毒疫苗后触发了SLE疾病的发生，并对患者的一级亲属的血清进行了免疫学检测。结果：患儿父亲血清中有高滴度的抗核抗体。结论：外界环境因素如流感病毒疫苗免疫注射可能触发有内在遗传基因背景的个体发生SLE。对有SLE易感倾向的个体及SLE患者应减少非计划内免疫接种的次数。     

Cardiac wall rupture in systemic lupus erythematosus: a case report and review of the literature

Clinical Rheumatology - Cardiac wall rupture (CWR) is a serious and often fatal complication of myocardial infarction (MI). Despite an increase in the incidence of MI in patients with systemic...     

Visceral muscle dysmotility syndrome in systemic lupus erythematosus: case report and review of the literature

Intestinal pseudo-obstruction (IPO) is not uncommon in systemic lupus erythematosus (SLE), and IPO in SLE has an apparent association with ureterohydronephrosis. However, hepatobiliary dilatation without mechanical obstruction presenting together with IPO and ureterohydronephrosis is much more scarce in SLE. Here, we named this rare triad of IPO, ureterohydronephrosis, and biliary tract dilatation as visceral muscle dysmotility syndrome (VMDS). It always imitates an acute abdomen and is even life-threatening if treated incorrectly. To diagnose a VMDS, infections and mechanical obstructions should be ruled out carefully. Here, we report a 24-year-old Chinese woman with SLE who presented of VMDS that associated with corticoids tapering induced SLE flare. In this case, early vigorous immunosuppressive treatment conquered the triad timely and thus yielded a good outcome.     

Multiple tendon rupture in systemic lupus erythematosus: case report and review of the literature.

Tendon rupture in systemic lupus erythematosus (SLE) is a rare complication that appears to occur in patients receiving corticosteroid therapy. A case is presented with sequential bilateral rupture of Achilles tendon and unilateral rupture of a patellar tendon. Six more published cases are reviewed. Tendon rupture in SLE has affected both males and females between the ages of 24 and 63 years. It occurred exclusively in the weight-bearing tendons: in 6 instances the patellar tendon was severed, in 2 the Achilles, and in one the quadriceps. All 7 patients were on corticosteroid therapy at or just before the time of injury. The deleterious effect of this treatment in loosening connective tissue is discussed.     

Silica-associated systemic lupus erythematosus with lupus nephritis and lupus pneumonitis: A case report and a systematic review of the literature

IntroductionSeveral epidemiological studies have shown that silica exposure triggers the onset of systemic lupus erythematosus (SLE); however, the clinical characteristics of silica-associated SLE have not been well studied.Patient concernsA 67-year-old man with silicosis visited a primary hospital because of a fever and cough. His respiratory condition worsened, regardless of antibiotic medication, and he was referred to our hospital.DiagnosisThe patient showed leukopenia, lymphopenia, serum creatinine elevation with proteinuria and hematuria, decreased serum C3 level, and was positive for anti-double stranded DNA antibody, anti-nuclear antibody, and direct Coombs test. He was diagnosed with SLE. Renal biopsy was performed, and the patient was diagnosed with lupus nephritis (class IV-G(A/C) + V defined by the International Society of Nephrology/Renal Pathology Society classification). Computed tomography revealed acute interstitial pneumonitis, bronchoalveolar lavage fluid showed elevation of the lymphocyte fraction, and he was diagnosed with lupus pneumonitis.InterventionsPrednisolone (50 mg/day) with intravenous cyclophosphamide (500 mg/body) were initiated.OutcomesThe patient showed a favorable response to these therapies. He was discharged from our hospital and received outpatient care with prednisolone slowly tapered off. He had cytomegalovirus and herpes zoster virus infections during treatment, which healed with antiviral therapy.Review:We searched for the literature on sSLE, and selected 11 case reports and 2 population-based studies. The prevalence of SLE manifestations in sSLE patients were comparative to that of general SLE, particularly that of elderly-onset SLE. Our renal biopsy report and previous reports indicate that lupus nephritis of sSLE patients show as various histological patterns as those of general SLE patients. Among the twenty sSLE patients reported in the case articles, three patients developed lupus pneumonitis and two of them died of it. Moreover, two patients died of bacterial pneumonia, one developed aspergillus abscesses, one got pulmonary tuberculosis, and one developed lung cancer.ConclusionClose attention is needed, particularly for respiratory system events and infectious diseases, when treating patients with silica-associated SLE using immunosuppressive therapies.     

系统性红斑狼疮合并膀胱炎及尿路梗阻一例并文献复习

目的提高对系统性红斑狼疮(SLE)合并膀胱炎及尿路梗阻的认识。方法报告1例特殊而临床少见的SLE合并膀胱炎、尿路梗阻及假性肠梗阻病例并复习相关文献。结果患者为37岁女性，SLE病史4年，出现膀胱刺激征1年，腹胀1个月；中段尿培养阴性；B超及膀胱镜示：膀朊壁增厚，膀胱容量减少，黏膜充血、水肿。X线及造影检查证实肠梗阻及尿路梗阻；激素及环磷酰胺治疗后膀胱炎及肠梗阻症状减轻。结论SLE合并的膀胱炎临床少见，属于间质性膀胱炎，是SLE合并尿路梗阻的最常见原因；SLE尿路梗阻常与假性肠梗阻同时出现，可能属于SLE的一个独特临床亚型；SLE合并肠梗阻病情严重，病死率高，临床上应予重视。     

Spinal cord involvement in pediatric systemic lupus erythematosus: case report and literature review.

Transverse myelitis is a rare but serious complication of systemic lupus erythematosus (SLE). We describe the youngest patient with transverse myelitis ever recorded in SLE. Clinical improvement was observed after prednisone and cyclophosphamide therapy. The English language literature from 1966 to the present on the subject are reviewed.     

Acute intermittent porphyria and systemic lupus erythematosus: report of a case and review of the literature

The case of a Greek woman with acute intermittent porphyria (AIP) associated with systemic lupus erythematosus (SLE) is described and the literature on this association is reviewed. The coexistence of these two diseases may be determined by unknown mechanisms or could be fortuitous.