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.     

La pancréatite lupique : une série de six cas

Purpose

The occurrence of acute pancreatitis in systemic lupus erythematosus (SLE) is known but rare, and is exceptionally the presenting manifestation. Its pathogenesis is multifactorial, and it is difficult to separate what belongs to vasculitis, thrombotic phenomena in the context of an associated antiphospholipid syndrome, or iatrogenic complications. We report on six cases of lupus pancreatitis.

Methods

This is a retrospective monocenter study of 110 patients with SLE. The diagnosis of lupus pancreatitis was established after exclusion of other causes of pancreatitis.

Results

Five women and one man (5.4%) with a mean age of 36.3 years presented with lupus pancreatitis. In four patients the pancreatitis was concurrent with the diagnosis of SLE and it occurred later during an exacerbation of the disease in the two remaining patients. In all patients, pancreatic manifestations were associated with other organ involvement. Clinical manifestations were: abdominal pain (n = 6), vomiting (n = 3), and fever (n = 3). Elevated pancreatic enzyme was noted in all cases. All patients were treated by high doses of glucocorticoids. The outcome was favorable in five patients, and one patient died.

Conclusion

Pancreatitis may be the presenting manifestation of SLE. Its pathogenesis is often multifactorial. The outcome is usually favorable with corticosteroids.     

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.     

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.     

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.     

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.     

Physiopathologie du lupus systémique : le point en 2014

Systemic lupus erythematous is a chronic autoimmune disease characterized by the inflammation of several tissues and the production of auto-antibodies directed against nuclear antigens. Complex genetic disorders and environmental factors are at the origin of the disease but the precise cause of the auto-immune process is still unknown. Both innate and adaptive immune systems are involved. Apoptosis seems to be the main source of auto-antigens. The interactions between apoptotic cells, dendritic cells and lymphocytes activate the production of pathogenic antibodies and T lymphocytes. Amplification loops sustain the auto-immune process and the chronic inflammation. Several data point out B-lymphocytes and several cytokines involved in their homeostasis as new promising therapeutic targets.     

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.     

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.     

Les internistes et l’éducation thérapeutique du patient

Therapeutic education is not a recent idea. It arouses renewed interest thanks to the article 84 of French law no. 2009-879 from July 21, 2009 reforming the hospital and concerning patients, health and territory. This article inserts in the public health code no less than five articles devoting patient therapeutic education. Nevertheless, the conditions imposed by the legislator raise questions, which should be outstripped in favor of a widespread implementation of integrated care pathways in which therapeutic education is enclosed. In France, internists should be involved in the development of new programs of therapeutic education in the field of rare diseases, especially systemic autoimmune diseases. A pilot project for systemic lupus erythematosus is ongoing, before an expected geographic extension through referent centers, as well as the progressive development of additional programs aiming all rare diseases. Content, funding and evaluation are key points that remain to be defined.     

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.     

Pulmonary arterial hypertension in systemic lupus erythematosus

Pulmonary hypertension (PH) is a serious complication of connective tissue diseases. The prevalence of PH in systemic lupus erythematosus (SLE) ranges from 0.5 to 17.5%, depending on whether echocardiography or right heart catheterization is used as the gold standard for diagnosis. The recent guidelines for the diagnosis and treatment of pulmonary hypertension include several potential causes of PH in SLE, including: a primary vasculopathy similar to idiopathic pulmonary arterial hypertension (PAH); left heart diseases; post-thromboembolic disease; hypoxia and fibrosis resulting from interstitial lung disease; and the infrequent SLE-associated pulmonary veno-occlusive disease. The pathogenesis of PAH associated with lupus is yet unclear, but likely includes a role for the genetic background, the presence of antiphospholipid antibodies, and some level of endothelial dysfunction. The evolution of SLE-associated PH is highly variable and difficult to elicit because the published series have used heterogeneous inclusion criteria. Optimal therapeutic management of PAH associated with lupus is unclear because no dedicated randomized controlled trial is yet available. Treatment usually includes arterial pulmonary vasodilators and immunosuppressive agents when the patients have NYHA functional class II, III or IV dyspnea.     

Lupus hepatitis: a case series of 12 patients

Purpose

Several liver manifestations have been reported in systemic lupus erythematosus (SLE) and are usually non specific. We report on our experience of lupus hepatitis.

Methods

A retrospective monocenter study of 73 patients with SLE. The diagnosis of lupus hepatitis was established after exclusion of other causes of hepatitis and hepatic vein thrombosis.

Results

Liver involvement was noted in 12 patients (16.4%). There were nine female and three male patients; the mean age of these patients was 29 years. In seven patients liver involvement was concurrent with the diagnosis of SLE and it occurred later during an exacerbation of the disease in the five remaining patients. In all patients, liver manifestations were associated with other organ involvement. Clinical manifestations were: hepatomegaly (n = 4), jaundice (n = 4), abdominal pain (n = 3), ascitis (n = 2), portal hypertension (n = 1) and hepatic failure with encephalopathy (n = 1). Elevated liver enzyme was noted in 11 cases and liver cholestasis in eight cases. Presence of anti-ribosomal P antibodies was noted in one case. Liver biopsy was performed in five patients, and revealed chronic active hepatitis in three cases, chronic hepatic granulomas in one case and nonspecific inflammation in one case. The outcome was favorable in 11 patients without relapse, and one patient died of encephalopathy and liver failure.

Conclusion

Liver involvement associated with SLE is not uncommon. It is frequently asymptomatic and limited to liver test abnormalities. The role of anti-ribosomal P autoantibodies remains uncertain.