Neuropsychiatric lupus

Sixty-six systemic lupus erythematosus (SLE) patients followed prospectively had 77 episodes of neuropsychiatric lupus (NPL). Patients with NPL had more SLE manifestations than patients without NPL. Serologic data and cerebrospinal fluid analysis were not helpful in identifying an episode of NPL. Electroencephalogram was abnormal in more than 1/2 and brain scans in more than 3/4 of patients. In 74 episodes with a known outcome, 54 improved, 44 having been treated with increased corticosteroids. Patients with NPL had a higher incidence of deaths compared to patients without NPL.     

Lamotrigine-induced lupus

A 57-year-old woman was under treatment for epilepsy with lamotrigine 2 mg/kg/d since 1996. In April 1998 she was admitted to the Rheumatology Unit for arthralgias affecting the small joints of the hands, wrists and knees, erythematosus skin rash and myalgias. She referred a vascular abnormality at the hands exposed to cold, compatible with Raynaud's phenomenon. Serum antinuclear antibodies (ANA) were positive (1:320, speckled pattern); moreover, a positivity for anti-Ro/SSA was observed. Rheumatoid factor was negative, serum complement was normal. LAC and anticardiolipin antibodies were negative. Drug-related lupus diagnosis was made with resolution of symptoms and gradual normalisation of positivity of ANA and anti-Ro/SSA upon lamotrigine withdrawal. To our knowledge, this is the first case report of an association between lamotrigine and the onset of SLE (systemic lupus erythematosus).     

Neuropsychiatric lupus

Nervous system disease in patients who have systemic lupus erythematosus (SLE) spans a wide spectrum of neurologic (N) and psychiatric (P) features that may be attributed to a primary manifestation of SLE, complications of the disease or its therapy, or a coincidental disease process. The etiology of primary NP disease is multifactorial and includes vascular injury of intracranial vessels, autoantibodies to neuronal antigens, ribosomes and phospholipid-associated proteins, and the intracranial generation of cytokines. In the absence of a diagnostic gold standard for most of the NP-SLE syndromes, a range of investigations are employed to support the clinical diagnosis and determine the severity of NP disease. Treatment remains largely empiric in the absence of controlled studies, and current strategies include the use of immunosuppressive therapies, appropriate symptomatic interventions, and the treatment of non-SLE factors.     

Major lupus organ involvement: severe lupus nephritis

Lupus nephritis is a common and severe complication of systemic lupus erythematosus. A number of patients have nephritis as a presenting feature that, in its severe form, can shortly lead to end-stage renal disease and/or death. Renal flare usually occurs a few years after the first episode and is remarkably predominant in the Asian population. Frequent monitoring for renal flare enhances early recognition and timely treatment. The mainstay therapy continues to be the prolonged use of cytotoxic/immunosuppressive drugs that have a number of undesirable effects, particularly ovarian failure and development of opportunistic infections. This review will focus on the pathogenesis and the unique genetic factors found in Asian patients with lupus nephritis. Here, we propose an appropriate management scheme for the treatment of lupus nephritis in Asian patients.     

Neonatal lupus

Neonatal lupus erythematosus is characterized by congenital atrioventricular heart block and/or transient lupus skin lesions frequently similar to those seen in patients with subacute cutaneous lupus erythematosus. The mothers have anti-SSA (Ro) and/or anti-SSB (La) antibodies. The syndrome is important to recognize because newborns may develop complete heart block with congestive heart failure. However, in the majority of cases, neonatal lupus erythematosus is a relatively benign disorder and cutaneous lesions generally disappear completely by 6 months of age.     

Neuropsychiatric lupus

Neuropsychiatric abnormalities in patients with systemic lupus have been recognized for more than a century. Although the prognosis of lupus has improved, involvement of the nervous system continues to be a major feature, with some abnormality recognized in 50 to 66 per cent of lupus patients. Diagnosis and therapy of neurologic disease remain the most difficult clinical challenges in the management of SLE.     

Neuropsychiatric lupus

Neuropsychiatric (NP) manifestations of systemic lupus erythematosus (SLE) have been recognized for more than 100 years but continue to pose a diagnostic and therapeutic challenge for rheumatologists and other physicians involved in the care of SLE patients. NP-SLE includes a plethora of clinical manifestations and the reported prevalence has varied widely between 14% and 75%. The recent introduction of a standard nomenclature, case definitions, and diagnostic criteria for19 NP-SLE syndromes should facilitate a more consistent approach to the classification of patients and to the execution of large multicenter clinical studies. The etiology of NP-SLE is likely multifactorial and includes microangiopathy, autoantibody production, and the intrathecal production of proinflammatory cytokines. Newer imaging modalities of brain structure and function provide novel ways of understanding pathogenic mechanisms. The use of standardized neuropsychometric techniques to evaluate cognitive function has identified a high prevalence of cognitive impairment in SLE patients. The management of patients with NP-SLE includes symptomatic and immunosuppressive therapies, evidence for which is largely limited to uncontrolled clinical trials and anecdotal experience. Multicenter initiatives are required to address important issues on prognosis and management.     

Impact of recurrent lupus nephritis on lupus kidney transplantation

This study was conducted to delineate the frequency of recurrent lupus nephritis in a Chinese kidney transplant cohort and to estimate its impact on long-term transplant outcomes. A total of 32 lupus transplant patients were enrolled in this study, and the medical records were retrospectively reviewed. Patients with unexplained graft abnormalities were subjected to allograft biopsy. Recurrent lupus nephritis was diagnosed by light microscopy, immunofluorescence, and electron microscopy. In addition, to determine the clinical manifestations of recurrent lupus GN in these patients, serum original systemic lupus erythematosus disease activity index (SLEDAI) scores while undergoing allograft biopsy were evaluated. In total, six out of 32 patients (18.8%; mean age, 40.5 ± 9.1 years) were diagnosed as having recurrent lupus nephritis and the mean time at diagnosis was 5.1 ± 4.9 years post-transplantation. According to the International Society of Nephrology/Renal Pathology Society (ISN/RPS) 2003 criteria, three of the six cases (50%) were defined as class I, one was class II, one was class IV, and one was class III + V. The graft and patient survival rates of recurrent lupus nephritis (n = 6) were not different from those of patients with other diagnostic entities. Although recurrent lupus nephritis was not uncommon, it did not appear to have a strong negative impact on long-term outcome in Chinese kidney transplant patients. The recurrence was potentially treatable and should not be precluded for receiving transplantation.     

三种自身抗体联合检测对狼疮疾病活动和狼疮肾炎的价值

目的探讨联合检测抗C1q抗体、抗核小体抗体(AnuA)和抗dsDNA抗体对狼疮活动和狼疮肾炎(LN)的价值。方法 90例系统性红斑狼疮(SLE)分为疾病活动和疾病稳定组、LN和非LN组, 酶联免疫吸附试验(ELISA)检测血清抗C1q抗体和AnuA水平,间接免疫荧光法比较三抗体单个和联合对疾病活动和LN的价值。结果抗C1q抗体、AnuA和抗dsDNA抗体阳性对疾病活动的敏感性分别为 71．4％、75．0％和66．1％,特异性分别为75．5％、70．6％和88．2％;抗dsDNA抗体阴性患者分别有36．7％抗 C1q抗体阳性和26．5％AnuA阳性。疾病活动组三抗体阳性率和抗体水平显著高于疾病稳定组;三抗体与 SLE疾病活动指数(SLEDAI)、血沉(ESR)、IgG、球蛋白水平显著正相关,与C3、C4及白蛋白水平显著负相关。LN组三抗体水平显著高于非LN组。结论三抗体都是狼疮疾病活动的指标,都与LN有关,联合检测可以提高疾病活动检出率。     

Systemic lupus erythematosus associated with recurrent lupus enteritis and peritonitis

We describe the case of a 41-year-old woman with systemic lupus erythematosus (SLE) who suffered from repeated reversible lupus enteritis characterized by marked edematous thickening of the small intestine. Ultrasonography (US) and computed tomography (CT) manifested as an accordion-like appearance and a target-like appearance, respectively. Resolution of gastrointestinal tract wall thickening was observed on follow-up US performed a week after the increase in predinosolone (PSL). We conclude that careful evaluation of sonographic and radiographic findings helps to establish the diagnosis of lupus enteritis.Abbreviations CT Computed tomography - FANA Fluorescent antinuclear antibody - GI Gastrointestinal - SLE Systemic lupus erythematosus - US Ultrasound