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Because Systemic Lupus Erythematosus (SLE) is a rare disease, and due to the significant prognostic impact of early management, a diagnosis confirmed by a physician with experience in SLE is recommended, for example from an expert center. Once the diagnosis is confirmed, existing manifestations should be identified in particular, renal involvement by an assessment of proteinuria, disease activity and severity should be determined, potential complications anticipated, associated diseases searched for, and the patient's socioprofessional and family context noted. Therapeutic management of SLE includes patient education on recognizing symptoms, understanding disease progression as well as when they should seek medical advice. Patients are informed about routine checkups, treatment side effects, and the need for regular vaccinations, especially if they are receiving immunosuppressive treatment. They are also advised on lifestyle factors such as the risks of smoking, sun exposure, and dietary adjustments, especially when they are receiving corticosteroids. The importance of contraception, particularly when teratogenic medications are being used, and regular cancer screening are emphasized. Support networks can help relieve a patient's isolation. The first-line medical treatment of SLE is hydroxychloroquine (HCQ), possibly combined with an immunosuppressant and/or low-dose corticosteroid therapy. The treatment of flares depends on their severity, and typically involves HCQ and NSAIDs, but may be escalated to corticosteroid therapy with immunosuppressants or biologic therapies in moderate to severe cases. Because there is no curative treatment, the goals of therapy are patient comfort, preventing progression and flares, and preserving overall long-term health and fertility. The frequency of follow-up visits depends on disease severity and any new symptoms. Regular specialized assessments are necessary, especially when treatment changes, but a frequency of every 3 to 6 months is recommended during periods of remission and monthly during active or severe disease, especially in children. These assessments include both clinical and laboratory tests to monitor complications and disease activity, with specific attention to proteinuria., (Copyright © 2024. Published by Elsevier Masson SAS.)

Hematopoietic stem cell transplantation (HSCT) for severe ADs was developed over the past 25years and is now validated by national and international medical societies for severe early systemic sclerosis (SSc) and relapsing-remitting multiple sclerosis (MS) and available as part of routine care in accredited center. HSCT is also recommended, with varying levels of evidence, as an alternative treatment for several ADs, when refractory to conventional therapy, including specific cases of connective tissue diseases or vasculitis, inflammatory neurological diseases, and more rarely severe refractory Crohn's disease. The aim of this document was to provide guidelines for the current indications, procedures and follow-up of HSCT in ADs. Patient safety considerations are central to guidance on patient selection and conditioning, always validated at the national MATHEC multidisciplinary team meeting (MDTM) based on recent (less than 3months) thorough patient evaluation. HSCT procedural aspects and follow-up are then carried out within appropriately experienced and Joint Accreditation Committee of International Society for Cellular Therapy and SFGM-TC accredited centres in close collaboration with the ADs specialist. These French recommendations were performed according to HAS/FAI2R standard operating procedures and coordinated by the Île-de-France MATHEC Reference Centre for Rare Systemic Autoimmune Diseases (CRMR MATHEC) within the Filière FAI2R and in association with the Filière MaRIH. The task force consisted of 3 patients and 64 clinical experts from various specialties and French centres. These data-derived and consensus-derived recommendations will help clinicians to propose HSCT for their severe ADs patients in an evidence-based way. These recommendations also give directions for future clinical research in this area. These recommendations will be updated according to newly emerging data. Of note, other cell therapies that have not yet been approved for clinical practice or are the subject of ongoing clinical research will not be addressed in this document., (Copyright © 2024 The Authors. Published by Elsevier Masson SAS.. All rights reserved.)

Before age 35, Holman hit over 0.500 at the University of California Los Angeles (UCLA); was recruited by professional baseball; led the Association of Interns and Medical Students and the International Union of Students in Denmark; had his passport confiscated; was stripped of a prestigious internship; shadowed by the Federal Bureau of Investigation ; grilled before a Senate committee on subversive activities; made a major medical discovery; and was recruited to be the new Chief of Medicine at Stanford. Holman was involved in building a leading academic institution. He expanded what medical students and graduates learned and what they researched. Holman saw the collision course between the technological capacity to do more and the growing expectations of the public. Moreover, he anticipated the monetization of health care and how it would widen the gap between what we know and what we practice in health care. He reinvented himself in population health. In contrast to reductionist laboratory-based research, his work embraced complexity and made action researchable and research action-oriented. Some innovations did not survive as originally conceived, but their ethos became mainstream. These included evidence-based management, shared physician-patient decision-making, self-management, critical evaluation of medical technology and diagnostics, and chronic disease management. Through the rise of the twentieth century American biomedical medicine, medical education, and slow-motion health care delivery crises that still occur, Holman changed the debate in a time when the funding, the people, the technology, and the need made all things seem possible., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Introduction: Castleman disease is a rare lymphoproliferation, which may mimic systemic lupus. Conversely, systemic lupus sometimes presents like an hematological malignancy. In these cases, a "Castleman-like" histology has been exceptionally described., Observation: A 55-year-old female treated by methotrexate for systemic lupus with skin and joint involvement presented weight loss, polyadenopathy and clinical signs of lupus flare. Biology showed pancytopenia, complement activation, and positive anti-DNA antibodies. PET/CT showed hypermetabolic polyadenopathy. The lymph node biopsy showed "Castleman-like" features. Treatment with corticosteroids and azathioprine resulted in complete remission., Conclusion: Systemic lupus and Castleman disease may share common clinical, biological, and histological features. The presence of specific elements of systemic lupus flare and the remission obtained by low-dose corticosteroids results in considering the diagnosis of Castleman-like systemic lupus and avoiding treatment intensification., (Copyright © 2022 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.)

Systemic lupus erythematosus (SLE) is a complex multifactorial autoimmune disease depending on both intrinsic and environmental factors. Among the latter, the Epstein-Barr Virus (EBV) has long been suggested as one of the responsible factors for the onset and activity of lupus disease. It is a herpes virus with a very specific tropism for B lymphocytes and therefore closely linked to the immune system. EBV infection almost always precedes the onset of lupus disease and in vitro data and animal models suggest that anti-EBV response may favor the development of autoantibodies and lupus disease in some subjects. Also, there are abnormalities in humoral and cellular responses to EBV and lupus patients have impaired control of EBV, with higher blood viral loads. Interstingly, this virus seems to be able to promote disease activity, by promoting the survival of autoreactive B lymphocytes and the production of interferon-α, which are two pivotal mechanisms in the pathophysiology of lupus disease., (Copyright © 2022 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.)

The assessment of systemic lupus erythematosus (SLE) disease activity in clinical trials has been challenging. This is related to the wide spectrum of SLE manifestations and the heterogeneity of the disease trajectory. Currently, composite outcome measures are most commonly used as a primary endpoint while organ-specific measures are often used as secondary outcomes. In this article, we review the outcome measures and endpoints used in most recent clinical trials and explore potential avenues for further development of new measures and the refinement of existing tools., Competing Interests: Disclosure The authors have nothing to disclose., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Secondary forms of immune thrombocytopenia (ITP) represent approximately 20% of all ITP cases in adulthood and this rate increases with age. Since some causes may influence both the prognosis and outcome but also the management of ITP, a minimal workup must be performed at ITP diagnosis to look for an associated or underlying cause. Among adults, B-cell lymphomas and mainly chronic lymphocytic leukemia, systemic auto-immune diseases such as systemic lupus or primary immunodeficiencies mainly represented by common variable immunodeficiency are the most frequent causes of secondary ITP. Whereas first-line therapy used for secondary ITP is usually similar to the one commonly used in primary ITP and relies mostly on corticosteroids±intravenous immunoglobulin according to the severity of bleeding, second and third-line treatments must take into account the type and degree of activity of the underlying disease., (Copyright © 2020 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.)

Purpose: Therapeutic education (TE) intends to help patients with systemic lupus erythematosus to better understand their disease and to improve their quality of life. The objective of this study was to assess illness perceptions of the person to provide a motivational environment for TE., Methods: Systemic lupus erythematosus patients followed in the department of internal medicine in Lille university hospital responded to a questionnaire assessing five dimensions of the person as proposed by Giordan: the cognitive (knowledge), perceptual (fatigue and pain), affective (anxious and depressive symptoms), infra-cognitive (intimate reasoning) and metacognitive (worldview) dimensions. The quality of life was also evaluated., Results: One hundred and twenty-four patients (114 women (92%); mean age 44.3±14.3 years) responded to the questionnaire. Regarding the cognitive dimension: quantity of information at the time of diagnosis was considered insufficient for 57 patients (46%). The median adherence evaluated by a scale had a median 97mm [88-100]. Regarding the perceptual dimension: pain was assessed at 59mm [44-78] and fatigue at 66mm [50-79] at visual scales. Regarding the affective dimension: prevalence of anxiety symptoms was 67% (83/124) and 28% (35/124) for depressive symptoms. Regarding the infra-cognitive dimension 78 patients (63%) had an external control place. Concerning the metacognitive dimension, systemic lupus erythematosus had repercussions on professional and family life. The quality of life was impacted., Conclusion: Multiple dimensions of systemic lupus erythematosus patient have to be considered for an optimal motivational environment for the practice of TE., (Copyright © 2018 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.)

Fertility preservation is routinely performed in cancerology but less systematically used in the field of immune diseases, even though the use of gonadotoxic treatments in young patients may be required and even though the disease itself can alter fertility. This review aimed to clarify the indications and methods of fertility preservation in this context. Cyclophosphamide is the only immunosuppressive drug requiring fertility preservation in women. In men, fertility preservation should be proposed before treatment with cyclophosphamide, methotrexate, mycophenolate mofetil or mTOR inhibitors. Other factors inherent to the disease or the patient may alter fertility. Thus, screening for infertility and fertility preservation have to be implemented as much as possible to increase the chances of successful procreation in patients with immune disease. For women, the choice between the different preservation methods depends on the patient's age, disease activity, the time available before the start of treatment, the possibility of future pregnancy and the woman's and even couple's wishes. Before puberty, the only accepted method is cryopreservation of ovarian tissue. After puberty, the first-line method is the cryopreservation of mature oocytes. If the treatment has to be started in an emergency, if ovarian hyperstimulation/oocyte retrieval is contraindicated or if the patient refuses this option, cryopreservation of ovarian tissue or GnRH agonists could be proposed. For men, the accepted method is sperm cryopreservation. For prepubertal boys, the cryopreservation of spermatogonia after testicular biopsy is still experimental., (Copyright © 2018 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.)

Significant advances in the understanding of the molecular basis of innate immunity have led to the identification of interferons (IFNs), particularly IFN-α, as central mediators in the pathogenesis of Systemic Lupus Erythematosus. Therefore, targeting of IFNs and of their downstream pathways has emerged as important developments for novel drug research in SLE. Based on this, several specific interferon blocking strategies using anti-IFN-α antibodies, anti-type I interferon receptor antibodies, Interferon-α-kinoid, or anti-IFN-γ antibodies have all been assessed in recent clinical trials. Alternative strategies targeting the plasmacytoid dendritic cells (pDCs), Toll-Like Receptors (TLRs)-7/9 or their downstream pathways such as the myeloid differentiation primary-response protein 88 (MYD88), spleen tyrosine kinase (Syk), Janus-kinases (JAKs), interleukin-1 receptor-associated kinase 4 (IRAK4), or the Tyrosine Kinase 2 (TYK2) are also investigated actively in SLE, at more preliminary clinical development stages, except for JAK inhibitors which have reached phase 2 studies. In a near future, in-depth and personalized functional characterization of IFN pathways may provide further guidance for the selection of the most relevant therapeutic strategy in SLE, tailored at the patient-level., (Copyright © 2017 Elsevier B.V. All rights reserved.)

The association between thymoma and autoimmunity is well known. Besides myasthenia gravis, which is found in 15 to 20% of patients with thymoma, other autoimmune diseases have been reported: erythroblastopenia, systemic lupus erythematosus, inflammatory myopathies, thyroid disorders, Isaac's syndrome or Good's syndrome. More anecdotally, Morvan's syndrome, limbic encephalitis, other autoimmune cytopenias, autoimmune hepatitis, and bullous skin diseases (pemphigus, lichen) have been reported. Autoimmune diseases occur most often before thymectomy, but they can be discovered at the time of surgery or later. Two situations require the systematic investigation of a thymoma: the occurrence of myasthenia gravis or autoimmune erythroblastopenia. Nevertheless, the late onset of systemic lupus erythematosus or the association of several autoimmune manifestations should lead to look for a thymoma. Neither the characteristics of the patients nor the pathological data can predict the occurrence of an autoimmune disease after thymectomy. Thus, thymectomy usefulness in the course of the autoimmune disease, except myasthenia gravis, has not been demonstrated. This seems to indicate the preponderant role of self-reactive T lymphocytes distributed in the peripheral immune system prior to surgery. Given the high infectious morbidity in patients with thymoma, immunoglobulin replacement therapy should be considered in patients with hypogammaglobulinemia who receive immunosuppressive therapy, even in the absence of prior infection., (Copyright © 2017 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier SAS. All rights reserved.)

Introduction: Late-onset systemic lupus erythematosus represents a specific sub-group of the disorder, beginning after 50 years of age. The incidence is rarer and the course of the disease is considered to be more benign. Digital gangrene is an uncommon complication of systemic lupus erythematosus reported especially among middle-aged patients with long disease duration., Observation: We report a 53-year-old man, who presented with systemic lupus erythematosus revealed by an extensive digital gangrene., Conclusion: Digital gangrene is a rare complication of late onset systemic lupus erythematosus. Clinicians should be aware of this complication in order to initiate early and aggressive treatment., (Copyright © 2017 Elsevier Masson SAS. All rights reserved.)

The pulmonary-renal syndrome is a rare and life-threatening condition. It is defined as the association of a diffuse alveolar hemorrhage and a rapidly progressive glomerulonephritis. The characteristic histological lesion common to all underlying diseases is a necrotizing and crescentic glomerulonephritis. The pulmonary-renal syndrome is a diagnostic and therapeutic emergency: any delay in its management will lead to death or serious functional damage as pulmonary and renal impairment. ANCA-associated vasculitis and Goodpasture's disease are the main disorders associated to pulmonary-renal syndrome. More rarely systemic lupus, cryoglobulinaemia, Henoch-Schonlein purpura or subacute endocarditis may induce a pulmonary-renal syndrome. Differential diagnosis can sometimes be difficult, highlighting some ambiguity in the definition of the syndrome. Initial treatment usually associates systemic corticosteroid, cyclophosphamide and plasma exchange. The role of biotherapy as first line therapy remains to be determined., (Copyright © 2013 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.)