Transverse myelopathy in systemic lupus erythematosus: an analysis of 14 cases and review of the literature

OBJECTIVE: To give a comprehensive review of transverse myelopathy (TM), a rare but serious condition reported in 1-2% of patients with systemic lupus erythematosus (SLE). METHODS: 14 patients with SLE and TM were evaluated and 91 additional cases published in the English and German literature reviewed. RESULTS: TM presented either as the initial manifestation or within five years of the diagnosis of SLE. Most patients presented with a detectable sensory deficit at the thoracic level. In our 14 patients, 22% of the patients showed complete neurological recovery, whereas in the total patient population of 105 (our cases plus those reviewed in the literature), complete recovery was observed in 50%, partial recovery in 29% and no improvement or deterioration in 21%. Treatment with intravenous methylprednisolone followed by cyclophosphamide seemed to be most effective. Seventy per cent of the total patient population had abnormal magnetic resonance imaging findings. In our group of 14 patients, those with higher disease activity (measured by the SLAM) at onset of TM were treated more aggressively (for example, with plasmapheresis and intravenous pulse cyclophosphamide). TM in our patients was associated with antiphospholipid antibodies in 43% of the cases as compared with 64% of the total patient population. Optic neuritis occurred in 48% of the total patient population with SLE and TM, suggesting an association. CONCLUSIONS: TM in SLE is a poorly understood entity. Outcome might be more favourable than previously suggested. There is an association of TM with antiphospholipid antibodies in SLE patients. Treatment including intravenous cyclophosphamide may improve the final outcome. This report emphasises the need for multicentre trials to establish guidelines for optimal treatment.     

Aspectic meningitis in systemic lupus erythematosus. Report of three cases.

Three patients with aseptic meningitis were subsequently diagnosed as having lupus erythematosus. One patient had a single meningitic episode, another had chronic meningitis, and the third two acute episodes 5 years apart. All 3 patients developed further neurophychiatric manifestations of SLE, leading to death in 1. Aseptic meningitis appears to be an early manifestation of SLE and may herald more serious brain damage. No new cases of aseptic meningitis occurred in this series after initiation of therapy for SLE. In contrast, bacterial meningitis did occur as a late complication of the disease.     

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.     

系统性红斑狼疮合并横贯性脊髓炎八例临床分析

目的横贯性脊髓炎(transversemyelitis,TM)是系统性红斑狼疮(systemiclupuserythe-matosus,SLE)少见的严重合并症。本文报道8例并结合文献复习,对其临床表现、治疗方法、预后进行分析。方法回顾了本院1998—2005年的住院和门诊随访病例,报道8例SLE合并TM,并文献复习。结果8例(7例女性,1例男性)SLE发病年龄中位数为19岁(15￣32岁),SLE距TM发病的中位数时间为8个月(0￣12年),就诊时间距TM发病的中位数为3.3个月(0￣7年)。脊髓病变1例发生在颈髓,余7例均定位在胸髓(T1￣T9)。脊髓磁共振成像(MRI)有助于诊断,其中3/7例呈T2加权长条形病变,4/7例呈斑点状病变。其他临床表现、血清学(其中2例存在抗磷脂抗体)、脑脊液均无特征性提示。4/8例遗留明显的神经系统后遗症。其中3例进行了地塞米松鞘内注射治疗,仅1例有短暂疗效。另外4例完全恢复或基本恢复,其中3例在TM发生早期(<10d)接受了大剂量激素治疗,1例有自愈倾向。结论TM是SLE少见的严重合并症,倾向于早年、早期出现,多累及胸髓。早期积极治疗可能改善预后,肌力Ⅲ级以上者预后较好。     

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.     

Pure red cell aplasia in procainamide induced systemic lupus erythematosus. Report and review of the literature

We describe a patient who developed pure red cell aplasia as part of a lupus-like syndrome while taking procainamide. Pure red cell aplasia resolved spontaneously after discontinuation of this drug. Although pure red cell aplasia has been reported in several patients with idiopathic systemic lupus erythematosus (SLE), this represents the first such report in a patient with drug induced SLE.     

Deep fungal infection in systemic lupus erythematosus - three cases reported, literature reviewed.

Three patients with systemic lupus erythematosus (SLE) and deep fungal infection are described. Two patients had disseminated cryptococcal infection and the third disseminated histoplasmosis. Allwere receiving corticosteroids at the time fungal infection developed. One patient with disseminated cryptococcosis improved after treatment with amphotericin B and 5-fluorocytosine. The other patient with disseminated cryptococcosis died before adequate therapy could be given. The patient with histoplasmosis responded satisfactorily to amphotericin B. A survey of the literature revealed 30 additional cases of deep fungal infection in patients with SLE, most of whom were on corticosteroid therapy. The majority of the patients had candidiasis (14 patients); 11 patients had severe cryptococcal infection. Other fungal infections reported were histoplasmosis, aspergillois, coccidioidomycosis, and maduromycosis caused by Allerscheria boydii. Twenty-seven patients died; in 22 death was related to the fungal infection. The fungal infection was not diagnosed until necropsy in at least 11 persons. Deep fungal infections should be considered whenever patients with SLE have fever of unknown origin, diffuse pulmonary infiltrates, or unexplained CNS symptoms.     

The spectrum of liver disease in systemic lupus erythematosus: Report of 33 histologically-proved cases and review of the literature

The charts of 238 patients with systemic lupus erythematosus (SLE) were reviewed. Although not routinely screened for biochemical evidence of liver disease, 124 of 206 patients tested had at least one abnormal result, and 43 met strict criteria for the existence of liver disease. In most patients, a specific viral or drug etiology could not be implicated. The spectrum of liver disease in 33 patients from whom liver tissue was available included cirrhosis, chronic active hepatitis, granulomatous hepatitis, chronic persistent hepatitis and steatosis. Three of four cirrhotic patients demonstrated a peculiar form of cholestasis which resembled a “canalicular cast” of bile. Of the nine patients who had serial liver biopsies, four showed progression of their disease. Three patients died of liver failure. Liver involvement in SLE is more common than previously recognized. Severe and even fatal liver disease can occur.     

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   

Collapsing glomerulopathy occurring in HIV-negative patients with systemic lupus erythematosus: report of three cases and brief review of the literature

Collapsing glomerulopathy (CG), once recognized as a distinct renal disease in HIV-positive patients, has since been described in association with a number of other conditions, including autoimmune diseases. Among the latter, CG has been reported in Still's disease. Extensive search of the literature revealed only three patients with renal biopsy diagnosis of CG in the presence of clinical and serological evidence of SLE (HIV-negative). This case report presents three adult female HIV-negative patients with SLE who were detected as having significant proteinuria and underwent renal biopsy. In all three cases, renal biopsy showed features of diffuse lupus nephritis. In addition, variable numbers of glomeruli showed segmental collapse of glomerular tuft with hyperplasia of overlying visceral epithelial cells and were diagnosed as CG. The patients were managed with aggressive immunosuppressive therapy, including intravenous steroids in two patients and tacrolimus and cyclophosphamide in one patient. All three patients achieved partial remission of proteinuria with maintained renal function at the last follow-up. Collapsing glomerulopathy is a rare occurrence in patients with SLE, which should be considered in patients with lupus nephritis and significant proteinuria. A timely performed renal biopsy allows accurate diagnosis and appropriate therapeutic modifications.     

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     

Hydroxychloroquine cardiotoxicity in systemic lupus erythematosus: a report of 2 cases and review of the literature

BACKGROUND: Hydroxychloroquine (HCQ) is extensively used in the long-term treatment of systemic lupus erythematosus (SLE). Although considered by clinicians to be relatively safe, serious side effects have been documented in the literature. Retinotoxicity has received the most attention, whereas neuromyotoxicity and cardiotoxicity have been described in isolated case reports. We present 2 cases of potential cardiotoxicity occurring in patients with SLE while receiving long-term HCQ therapy. OBJECTIVE: To review the incidence, presentation, and mechanism of serious antimalarial toxicity, and to discuss the impact of HCQ on cardiac health in SLE. METHODS: The authors reviewed the English-language literature from 1948 to December 2002 using Medline databases. RESULTS: In addition to our patients, there are 2 published cases of biopsy-proven HCQ cardiotoxicity in the English-language literature. Both occurred in patients with SLE. The literature indicates that antimalarial cardiotoxicity may be of particular importance in patients with SLE given their already increased cardiac risk due to primary heart disease and accelerated atherosclerosis. Endomyocardial biopsy reveals a constellation of findings including vacuolar myopathy, myeloid bodies, and curvilinear bodies. CONCLUSIONS: As HCQ use among SLE patients increases, clinicians should be alert to the possibility of antimalarial cardiotoxicity. The potential severity and reversibility of this complication underscore the importance of timely diagnosis. The cases presented here, one with biopsy and one without, illustrate the utility of endomyocardial biopsy in HCQ-treated SLE patients with cardiac complaints to ensure accurate diagnosis and appropriate management.     

Association between myasthaenia gravis and systemic lupus erythematosus: three case reports and review of the literature

The coexistence of systemic lupus erythematosus (SLE) and myasthaenia gravis (MG) has been reported previously. Because of their shared clinical characteristics and autoantibody-mediated pathogenesis, an SLE expert panel decided to include MG as one of the 19 neuropsychiatric SLE syndromes. This study reports a cluster of three cases of SLE/MG overlap from our cohort and a review of the published data concerning this overlap of SLE and MG. A systematic Medline review revealed 13 cases described in eight publications from 1994 to 2009. In summary, 12 of the 16 patients (three from our cohort and 13 from the reported cases) were women with an average age of 34 years. The most common SLE manifestations were polyarthritis (15 out of 16 patients), skin rashes (5/16), serositis (5/16), and cytopaenias (10/16). All of the patients were anti-nuclear antibodies (ANA) positive and 15/16 were anti-dsDNA positive. Proximal muscle weakness was the most frequent MG-related symptom (9/16), while 11/16 patients were anti-acetylcholine receptor (anti-AChR) antibody positive and 9/16 had diagnostic electromyography (EMG). These data suggest that MG should to be included in the differential diagnosis of lupus patients with fatigue and muscular weakness together with inflammatory and drug-induced myopathy.