Narcolepsie révélant un lupus érythémateux systémique

Many neurologic and psychiatric manifestations have been associated with systemic lupus erythematosus. Narcolepsy, currently hypothesized as related to an autoimmune process, has been rarely associated with systemic lupus erythematosus. We report a 36-year-old woman who presented with narcolepsy and who subsequently developed systemic lupus erythematosus. Excessive daytime sleepiness resolved after the administration of four intravenous bolus of cyclophosphamide and methylprednisolone followed by maintenance therapy with hydroxychloroquine, aspirine and prednisone. Narcolepsy should be included in the neuropsychiatric manifestations of systemic lupus erythematosus and it may have a parallel clinical course to the activity of the lupus.     

Pseudo-obstruction intestinale au cours du lupus érythémateux systémique

Intestinal pseudo-obstruction (IPO) is an uncommon and severe complication of systemic lupus erythematosus (SLE). We report a 24-year-old female with a 2 year SLE duration who presented with abdominal pain, vomiting, constipation and abdominal distention. Plain abdominal radiograph showed multiple air-fluid levels of the small bowel. Computed tomographic scan of the abdomen revealed dilated small bowel loops without mechanical obstruction. Urinary tract involvement was also demonstrated. IPO was diagnosed and the patient responded well to immunosuppressive treatment. IPO is a recently recognized manifestation of SLE that may be the presenting manifestation of the systemic disease or occur more commonly during disease course. Early recognition of IPO is necessary to institute appropriate medical treatment and to avoid inappropriate surgical intervention.     

La pyomyosite : une complication infectieuse du lupus érythémateux systémique

Pyomyositis is a primary pyogenic muscular infection. It has been originally described in the tropics. Presenting signs and symptoms are not specific. It is frequently misdiagnosed in the early stages. We report the case of a 47-year-old woman with systemic lupus erythematosus treated with corticosteroids who presented a thigh pyomyositis evidenced by echography and MRI. Outcome was uneventful after antibiotics and surgical drainage. Pyomyositis is increasingly described among immunocompromised people, including lupus patients treated with corticosteroids.     

Syndrome de Barraquer-Simons au cours d’un lupus érythémateux systémique

Barraquer–Simons syndrome is a rare disorder characterized by a partial lipodystrophy. It is often associated with positive C3 nephritic factor and various glomerular nephropathy. Its association with some autoimmune diseases has also been reported. We report a 30-year-old woman with partial lipodystrophy, lupus erythematosus, hypothyroidism and vitiligo.     

Infection aiguë à cytomégalovirus révélatrice d’un lupus érythémateux systémique

Systemic lupus erythematosus (SLE) remains of unknown origin. Herpes viridae infections seem to play a role in the pathogenesis of this disease. We report a 31-year-old man who presented an acute cytomegalovirus (CMV) infection with persistent fever and myopericarditis as the presenting manifestation of SLE. This case report emphasizes a difficult differential diagnosis between SLE and an acute CMV infection and suggests a possible role of this virus in the pathogenesis of SLE.     

L’entérite : une manifestation peu fréquente et corticosensible du lupus érythémateux systémique

Introduction

. Lupus enteritis is a rare manifestation of systemic lupus erythematosus. The clinical manifestations are variable including abdominal pain, diarrhea, nausea and vomiting. Lupus enteritis is thought to be related to vasculitis.

Case reports

. We report here three new cases. All three patients aged of 45, 24 and 43 years (two females and one male) were admitted for abdominal pain, vomiting and diarrhea, and fulfilled the ACR criteria of systemic lupus erythematosus. The diagnosis of lupus enteritis was retained on the CT scan findings and the favorable outcome on corticosteroids after infectious etiologies were excluded.

Conclusion

. Lupus enteritis is thought to be one of the most common causes of acute abdominal pain in systemic lupus erythematosus. The diagnosis is based on clinical, radiological and biological findings. A good response to corticosteroids is usually reported.     

Rupture spontanée du tendon d’Achille à la phase précoce d’un lupus érythémateux systémique

Spontaneous tendon rupture is rare in the course of systemic lupus erythematosus (SLE). Its incidence rate remains unknown. The pathogenesis of this manifestation is complex and poorly understood. We report a 39-year-old woman who presented with a spontaneous Achille's tendon rupture as the presenting presentation of SLE, before any corticosteroid therapy. All the patients previously published were receiving corticosteroids and reported in some an associated traumatism. Risk factors are prolonged disease duration, chronic therapy with corticosteroids, deforming arthropathy of the hands, and inactive disease.     

Télomères et télomérase : intérêts et perspectives dans le lupus érythémateux systémique

Telomeres are specialized structures that cap and protect the end of chromosomes. Telomeres progressively shorten after each cellular division unless an enzyme, the telomerase, counteracts. Telomeres are implicated in cellular senescence, acting like a biological clock. Telomere length and telomerase activity are important in the physiopathology of cancer. In the past years, research has focused on them in order to find new therapeutic targets. Yet, oxidative stress, inflammation and increased leucocytes renewal are major environmental factors associated with telomeres shortening acceleration and thus in concordance with biological age. Thus, telomeric erosion induces cell apoptosis; indeed, apoptotic cell clearance is impaired in systemic lupus. Considering these elements and data resulting from oncology, telomere/telomerase couple was studied during the last decade in systemic lupus erythematosus. The objective was to know if this couple could have an implication in the physiopathology of this disease. A systematic review of literature is proposed about telomere and/or telomerase in systemic lupus erythematosus in order to discuss their physiopathological implication. Among 273 tested patients, telomere seems to be eroded and telomerase activity insufficiently increased but correlated to the activity of the disease. The analysis of telomere length and telomerase activity could be useful as prognosis factor or disease activity index. Telomere erosion could reflect an accelerated replicative senescence of the immune system. The role of the regulator T lymphocytes has not yet been precised. Standardized studies on larger population could be realized in systemic lupus and open new avenues of research and/or therapy based upon the telomere/telomerase biology.     

Lymphocytes T régulateurs et maladies auto-immunes systémiques : lupus érythémateux systémique,polyarthrite rhumatoïde et syndrome de Gougerot-Sjögren primaire

Regulatory/suppressor T cells (Tregs) maintain immunologic homeostasis and prevent autoimmunity. They are the guardians of dominant tolerance. Recent research reveals quantitative and/or functional defect of Tregs in systemic autoimmune diseases. In this article, past and recent studies of Tregs in human systemic lupus erythematosus (SLE), rheumatoid arthritis (RA) and primary Sjögren's syndrome (pGSS) are reviewed. Most studies report that Tregs are decreased in peripheral blood of subjects with active SLE. A population of CD4+CD25−Foxp3+ is specifically described in SLE. Tregs functions are still discussed. Tregs counts in peripheral blood of RA patients vary across studies. Enrichment of synovial fluid in Tregs contrasts with inflammation. Tregs suppressive effects are altered in vivo in RA secondary to proinflammatory cytokines environment and resistance of effector T cells to Tregs. In pGSS, the conflicting place of Tregs in the balance prevention of autoimmunity/antitumor immunity is unspecified. Immunosuppressive treatments, like corticosteroids and anti-TNF, modulate Tregs cells population. There is increasing interest in the use of Tregs as a biological therapy to preserve and restore tolerance to self-antigen. However, difficulties to characterize these lymphocytes and controversies in the results of studies refrain their use in current clinical practice.     

Neurolupus (2 partie). Description des outils diagnostiques et thérapeutiques devant une manifestation psychiatrique ou neurologique centrale au cours du lupus érythémateux systémique

Neurological and psychiatric manifestations of systemic lupus erythematosus are a heterogenous set of clinical manifestations grouped under the term of “neuropsychiatric systemic lupus erythematosus”. The classification of these manifestations published in 1999 has harmonized the definitions cases used in the studies but did not help the clinician to positively identify a specific manifestation of lupus or a neurological or psychiatric event occurred independently of the disease. Published cases series help us to identify neurological or psychiatric manifestations of lupus but modern diagnosis tools contribution have to be evaluated in order to optimize diagnosis management of such manifestations and to distinguish specific events related to lupus and independent manifestations. In this second part of our literature review about neuropsychiatric lupus, we propose to identify arguments, which could be in favor of lupus responsibility in front of a neurological or psychiatric event, and immunosuppressive treatments which are recommended.     

Neurolupus (1 partie). Description et démarche diagnostique et thérapeutique dans les manifestations neurologiques centrales et psychiatriques au cours du lupus érythémateux systémique

Systemic lupus erythematosus (SLE) is an autoimmune disease, which primarily affects skin and joints. Peripheral neurologic syndrome and central nervous system (CNS) manifestations are common in lupus patients but are not always attributable to lupus itself. A classification, published in 1999 by the American College of Rheumatology (ACR) research committee, described 12 CNS syndromes and seven peripheral neurologic syndromes compatible with “neuropsychiatric systemic lupus erythematosus” (NPSLE). Despite this consensus, studies which have been published since 1999 have reported a prevalence of NPSLE varying from 20 to 97 %, which shows the diagnosis difficulty and the heterogeneity of neuropsychiatric manifestations in SLE. In order to understand the limits of this classification, we propose in this first part an exhaustive review of publications describing neuropsychiatric manifestations according to the ACR 1999 classification. We also detail case definitions, prevalence and risk factors, clinical characteristics and diagnosis of each lupus-related psychiatric and CNS manifestation.     

Polyradiculonévrite aiguë révélant un lupus érythémateux systémique : une présentation inhabituelle d’évolution fatale

Introduction

Neurological manifestations of systemic lupus erythematosus are common and numerous. They mainly involve the central nervous system, peripheral involvement being rare. Acute polyradiculoneuropathy is very uncommon.

Case report

We report a 44-year-old man, who presented with acute polyradiculoneuropathy revealing systemic lupus erythematosus. Outcome was fatal despite treatment with corticosteroids and immunoglobulin.

Conclusion

Acute polyradiculoneuropathy is a very rare manifestation of systemic lupus erythematosus and can compromise functional and life prognosis. Early diagnosis and management are crucial.     

Le risque cardiovasculaire au cours du lupus systémique

Multiple factors contribute to the increased cardiovascular risk observed in patients with systemic lupus erythematosus (SLE). Among these are the so-called classical cardiovascular risk factors, the disease itself through its activity, treatments, and complications, and the thrombotic risk due to antiphospholipid antibodies (aPL). Observational studies suggest that most classical cardiovascular risk factors are observed more frequently in SLE patients than in the general population, and that these are insufficient to explain the increased cardiovascular risk observed in most studies. Given this high risk, adequate management of cardiovascular risk factors should be recommended in SLE patients. Paradoxically, the benefit due to the anti-inflammatory properties of treatments such as corticosteroids may exceed, in certain cases, their pro-atherogenic effect. Importantly, the tools that were developed for the estimation of cardiovascular risk at the individual level among the general population cannot be used reliably in SLE patients, as these tools appear to underestimate the true cardiovascular risk. The adequate indications and targets of cardiovascular treatments are therefore not fully known in SLE. A better understanding of the determinants of the cardiovascular risk in SLE will allow the identification and more tailored management of these high-risk patients.     

Évaluation des attentes des patients pour un programme d’éducation thérapeutique au cours du lupus systémique

Purpose

The aim of this study was to collect information to design a patient education program (PEP) for patients with systemic lupus erythematosus (SLE), based as much as possible on their expectations.

Patients and methods

Three different approaches were used for addressing patients’ needs: 1) A questionnaire on their expectations in terms of a PEP was sent to the members of SLE associations and offered to patients at the French reference center for SLE, 2) A patients’ focus group was conducted, and 3) After the teaching sessions, satisfaction questionnaires were also evaluated.

Results

The patients who answered the expectation questionnaire (n = 422, women/men sex-ratio: 12.6) indicated a major interest in the PEP (70.4%). Their expectations were broad, and covered the topics of pregnancy (90% of the women under the age of 40), the outcome of the disease (80.8%), the respective roles of the different treatments (70.4%), and also the management of everyday symptoms: fatigue and pain (66.4%). The focus group (eight people) highlighted the need for improving how the diagnosis of the disease was delivered, and also revealed the loneliness and the guilty feeling experienced by some patients toward their relatives. Satisfaction questionnaires confirmed these expectations for the PEP, and even extended them to new topics: the mechanisms behind SLE, travel and leisure, and possible accommodations in the workplace.

Conclusions

The direct consultation of patients with SLE targeted by a specific PEP program allowed us to confirm and adapt the topics and the content of a program designed by medical staff.