When obstetrics-gynecology specialists need to call an ophthalmologist urgently: a case report.

BACKGROUND: We report here a case of a healthy 23-year-old female patient who was assessed at the gynecology emergency department for genital ulcers, fever, and blurred vision. After suspicion of herpes simplex virus-2 lesions, the diagnosis of Behçet's disease was made. We report this case with the aim of including Behçet's disease in the differential diagnosis of genital ulcers, and emphasize the emergency of the vision loss that can be irreversible. CASE PRESENTATION: A healthy 23-year-old European female patient was assessed by gynecology in the emergency department for genital lesions associated with fever and blurred vision. At first, these lesions were suspected to be primary herpes simplex virus-2 infection One day later, she experienced decreased visual acuity in both eyes. After 4 days of worsening genital ulcers and persistent blurred vision, the patient was referred to the ophthalmology department. Fundoscopic examination showed retinal hemorrhages that were consistent with the first presentation of Behçet's disease. CONCLUSIONS: This case demonstrates that genital ulcers can be the very initial symptom of this ophthalmologic emergency. The differential diagnosis of genital ulcers is challenging. Behçet's disease should be included, especially when associated with systemic or ocular manifestations, and should be considered an emergency for the gynecologist to prevent long-term vision loss.

No association of complement mannose-binding lectin deficiency with cardiovascular disease in patients with Systemic Lupus Erythematosus.

Cardiovascular (CV) morbidity is the major cause of death in patients with Systemic Lupus Erythematosus (SLE). Previous studies on mannose-binding lectin (MBL) gene polymorphisms in SLE patients suggest that low levels of complement MBL are associated with cardiovascular disease (CVD). However, as large studies on MBL deficiency based on resulting MBL plasma concentrations are lacking, the aim of our study was to analyze the association of MBL concentrations with CVD in SLE patients. Plasma MBL levels SLE patients included in the Swiss SLE Cohort Study were quantified by ELISA. Five different CV organ manifestations were documented. Of 373 included patients (85.5% female) 62 patients had at least one CV manifestation. Patients with MBL deficiency (levels below 500 ng/ml or 1000 ng/ml) had no significantly increased frequency of CVD (19.4% vs. 15.2%, P = 0.3 or 17.7% vs. 15.7%, P = 0.7). After adjustment for traditional CV risk factors, MBL levels and positive antiphospholipid serology (APL+) a significant association of CVD with age, hypertension, disease duration and APL+ was demonstrated. In our study of a large cohort of patients with SLE, we could not confirm previous studies suggesting MBL deficiency to be associated with an increased risk for CVD.

Churg-Strauss syndrome with cardiac involvement: case illustration and contribution of CMR in the diagnosis and clinical follow-up.

This report summarises three cases of Churg-Strauss syndrome (CSS) illustrating the diagnostic challenges associated with the cardiac manifestation of this disease. Here, we illustrate the role of cardiac magnetic resonance (CMR) for diagnosis and follow-up of CSS with a focus on new non-contrast T2-weighted imaging sequences for quantification of myocardial scar tissue and quantitative T2 mapping techniques, which allow the detection of myocardial edema.

ESCMID Study Group for Infections in Compromised Hosts (ESGICH) Consensus Document on the safety of targeted and biological therapies: an infectious diseases perspective (Immune checkpoint inhibitors, cell adhesion inhibitors, sphingosine-1-phosphate receptor modulators and proteasome inhibitors).

BACKGROUND: The present review is part of the European Society of Clinical Microbiology and Infectious Diseases (ESCMID) Study Group for Infections in Compromised Hosts (ESGICH) consensus document on the safety of targeted and biological therapies. AIMS: To review, from an infectious diseases perspective, the safety profile of immune checkpoint inhibitors, LFA-3-targeted agents, cell adhesion inhibitors, sphingosine-1-phosphate receptor modulators and proteasome inhibitors, and to suggest preventive recommendations. SOURCES: Computer-based Medline searches with MeSH terms pertaining to each agent or therapeutic family. CONTENT: T-lymphocyte-associated antigen 4 (CTLA-4) and programmed death (PD)-1/PD-1 ligand 1 (PD-L1)-targeted agents do not appear to intrinsically increase the risk of infection but can induce immune-related adverse effects requiring additional immunosuppression. Although CD4+ T-cell lymphopenia is associated with alefacept, no opportunistic infections have been observed. Progressive multifocal leukoencephalopathy (PML) may occur during therapy with natalizumab (anti-α4-integrin monoclonal antibody (mAb)) and efalizumab (anti-CD11a mAb), but no cases have been reported to date with vedolizumab (anti-α4ß7 mAb). In patients at high risk for PML (positive anti-JC polyomavirus serology with serum antibody index >1.5 and duration of therapy ≥48 months), the benefit-risk ratio of continuing natalizumab should be carefully considered. Fingolimod induces profound peripheral blood lymphopenia and increases the risk of varicella zoster virus (VZV) infection. Prophylaxis with (val)acyclovir and VZV vaccination should be considered. Proteasome inhibitors also increase the risk of VZV infection, and antiviral prophylaxis with (val)acyclovir is recommended. Anti-Pneumocystis prophylaxis may be considered in myeloma multiple patients with additional risk factors (i.e. high-dose corticosteroids). IMPLICATIONS: Clinicians should be aware of the risk of immune-related adverse effects and PML in patients receiving immune checkpoint and cell adhesion inhibitors respectively.

[Whipple's disease, when to think about it?]. / Maladie de Whipple: quand y penser?

Whipple's disease is a chronic, systemic, bacterial infection caused by Tropheryma whipplei. Its cardinal symptoms include intermittent and recurrent arthralgia or arthritis together with chronic diarrhoea, abdominal pain and weight loss. It may mimick many chronic inflammatory diseases, and the diagnosis remains a challenge. Salivary and faecal quantitative PCR for T. whipplei should be ensued, if positive, by an upper endoscopy for duodenal biopsies. The treatment consists of a combination of oral doxycycline and hydroxychloroquine for 12 months followed by life-long doxycycline. Whipple's disease, although rare, is an entity that should be considered regularly, as its progression may be fatal if left untreated.

[Myelodysplastic syndromes and autoimmunity]. / Syndromes myélodysplasiques et auto-immunité.

Myelodysplastic syndromes (MDS) are characterized by inefficient hematopoiesis and result in peripheral cytopenia. This heterogenous disease group arises from clonal disorders of hematopoietic stem cells. Several cohort studies and numerous case reports have highlighted a link between MDS and autoimmune disorders. Patients with MDS are more prone to develop particular systemic inflammatory diseases. On the other hand, patients suffering from autoimmune diseases are at higher risk to develop MDS. The scope of this article is to review the association between myelodysplasia and autoimmunity. It gives practical clues when to look for MDS in a patient suffering from autoimmunity and lists the main autoimmune diseases which may complicate the course of a MDS.

[Giant cell arteritis: guidelines of the University Hospital of Lausanne]. / Artérite de Horton: recommandations Lausannoises de prise en charge.

Giant cell arteritis (GCA) is a subacute/chronic vasculitis and represents the most common form of systemic vasculitis in people over the age of 50 years. The absence of clear and specific diagnostic criteria with the highly variable clinical presentation is a diagnostic challenge requesting a multidisciplinary approach. Yet, GCA is an emergency and the treatment must be initiated very rapidly due to the risk of blindness. This article presents a review of GCA as well as the diagnostic and therapeutic institutional guidelines of the University Hospital of Lausanne.

Prevalence of vascular disease in systemic lupus erythematosus compared with type-1 diabetes mellitus: a cross-sectional study of two cohorts.

OBJECTIVES: Systemic lupus erythematosus (SLE) is associated with considerable cardiovascular morbidity that has not yet been directly compared with other diseases with known cardiovascular risk. METHODS: Two hundred and forty-one patients of the multicentre Swiss SLE cohort study (SSCS) were cross-sectionally assessed for coronary heart disease (CHD), cerebrovascular disease (CVD) and peripheral artery disease (PAD). SLE patients were compared with a cohort of 193 patients with type-1 diabetes mellitus being followed at the University Hospital Basel. A subgroup analysis of 50 age- and sex-matched patients from the University Hospital Basel was performed. RESULTS: Of patients within the SSCS 13.3% had one or more vascular events: 8.3% CHD, 5% CVD and 1.2% PAD. In type-1 diabetes mellitus patients, 15% had vascular events: 9.3% CHD, 3.1% CVD and 5.6% PAD. In the matched subgroup, 26% of SLE patients had vascular events (14% CHD) compared with 12% in type-1 DM patients (2% CHD). Cardiovascular risk factors were similar in both groups. Vascular events in SLE patients were associated with age, longer disease duration, dyslipidaemia, and hypertension. CONCLUSION: Cardiovascular morbidity in SLE is at least as frequent as in age- and sex-matched type-1 diabetes mellitus patients. Therefore, aggressive screening and management of cardiovascular risk factors should be performed.

[Food allergy or food intolerance?]. / Allergie ou intolérance alimentaire?

Adverse food reactions can be classified into two main categories depending on wether an immune mechanism is involved or not. The first category includes immune mediated reactions like IgE mediated food allergy, eosinophilic oesophagitis, food protein-induced enterocolitis syndrome and celiac disease. The second category implies non-immune mediated adverse food reactions, also called food intolerances. Intoxications, pharmacologic reactions, metabolic reactions, physiologic, psychologic or reactions with an unknown mechanism belong to this category. We present a classification of adverse food reactions based on the pathophysiologic mechanism that can be useful for both diagnostic approach and management.

[Anaphylaxis to blue dyes]. / Anaphylaxie aux colorants bleus.

In medicine, vital blue dyes are mainly used for the evaluation of sentinel lymph nodes in oncologic surgery. Perioperative anaphylaxis to blue dyes is a rare but significant complication. Allergic reactions to blue dyes are supposedly IgE-mediated and mainly caused by triarylmethanes (patent blue and isosulfane blue) and less frequently by methylene blue. These substances usually do not feature on the anesthesia record and should not be omitted from the list of suspects having caused the perioperative reaction, in the same manner as latex and chlorhexidine. The diagnosis of hypersensitivity to vital blue dyes can be established by skin test. We illustrate this topic with three clinical cases.

[Systemic and neurological uses of immunosuppressive agents]. / Immunosuppresseurs: usages systémiques et neurologiques.

Involvement of the central or peripheral nervous system, frequently present in systemic inflammatory immune disorders, has to be considered a severe threat and requires aggressive immunosuppressive treatment to achieve rapid remission. This is usually obtained with high-dose systemic corticosteroids combined with cyclophosphamide. Once remission is obtained, immunosuppressive agents with a more favorable safety profile are needed to exert a corticosteroid-sparing effect and minimize adverse events. New therapeutic approaches are currently developed to treat autoimmune diseases, mostly linked to the definition of new indications for biological agents such as TNF-alpha antagonists and rituximab.

[Management of adverse events induced by TNFalpha blocking agents]. / Allergologie: 1. Anti-TNFalpha: effets indésirables et implications pratiques.

TNFalpha blocking agents are effective and essential tools in the management of many inflammatory conditions including rheumatoid arthritis, spondylarthropathies and chronic inflammatory bowel disease. With time, some known side-effects have gained in importance and others have appeared. This article focuses on the potential risks of infection and autoimmunity induced by TNFalpha blocking agents and on the strategy to prevent and treat such adverse events.

[Systemic mastocytosis--new therapeutic strategies]. / Allergologie: 2. Mastocytose systémique--nouvelles stratégies thérapeutiques.

Systemic mastocytosis is characterized by an excessive proliferation of mast cells and their accumulation in different organs. Avoidance of trigger factors leading to anaphylaxis is a general measure valid for all forms of mastocytosis. A premedication is necessary in case of surgery, anesthesia or administration of radiocontrast agents. Symptomatic treatment comprises antihistamines, anti-leukotrienes, proton pump inhibitors and topical corticosteroids. Indolent mastocytosis with refractory symptoms, the rare cases of aggressive mastocytosis with organ dysfunction and the even rarer mast cell leukemia require cytoreductive therapy. First-line agents are interferon alpha 2b and imatinib, a tyrosine kinase inhibitor. To date there is no curative treatment.

[Hypersensitivity to proton pump inhibitors]. / Hypersensibilité aux inhibiteurs de la pompe à protons.

Hypersensitivity to proton pump inhibitors (PPI) is rare but potentially severe. An increase in hypersensitivity reactions to PPI is expected as these drugs are widely prescribed and some have become available over-the-counter. Allergy to PPI has to be considered in patients presenting anaphylaxis, late-type skin rashes, interstitial nephritis and other organ involvement suspicious to be drug-induced. Cross-reactivity between PPI is of concern, as they are closely related in structure. Skin prick- and intradermal testing are reliable tools to confirm immediate-type allergy to one PPI compound and search for safe alternatives. We review the literature on immediate and delayed hypersensitivity to these drugs.

[Central neuropsychiatric involvement in systemic lupus erythematosus]. / Atteintes neurologiques centrales du lupus érythémateux systémique.

About one third of patients with systemic lupus erythematosus (SLE) will present neurological or psychiatric symptoms in the course of the disease. Before attributing neuro-psychiatric illness to SLE, other causes have to be excluded, such as infections and treatment-related toxicity. Some manifestations of neuropsychiatric SLE (NPSLE) reflect active systemic disease, while other arise from chronic damage and may occur during apparent remission. Many clinical pictures are associated with antibodies, such as those targeting phospholipids, N-methyl-D-aspartate-receptor and anti-ribosomal P protein. This review discusses clinical manifestations and pathogenesis of central NPSLE, with a focus on the principal antibodies associated and other diagnostic tools at hand.

Hypereosinophilia in patients with multiple sclerosis treated with natalizumab.

OBJECTIVE: To report asymptomatic hypereosinophilia as a potential side effect in patients treated with natalizumab, an α-4 integrin blocking agent. METHODS: A case series of 3 patients treated with natalizumab for relapsing-remitting multiple sclerosis including functional and phenotypic characterization of their peripheral blood lymphocytes and eosinophils is presented. RESULTS: Marked peripheral blood eosinophilia with more than 2,000 cells/mm(3) emerged in all 3 patients after the fourth natalizumab infusion and was asymptomatic. Hypereosinophilia was associated with enhanced Th2 activity, ceased with drug discontinuation, and in 2 of 3 patients recurred with drug resumption. Despite persistently high eosinophil counts, there were no signs of end-organ damage. CONCLUSIONS: Hypereosinophilia may occur during treatment with natalizumab. It seems to reflect enhanced Th2 activity and recedes with systemic corticosteroids. If the patient is asymptomatic, natalizumab may be continued, provided that other causes of eosinophilia are excluded and the patient is carefully monitored.

[Adult onset Still's disease]. / Maladie de Still de l'adulte.

Adult onset Still's disease (AOSD) is a rare systemic inflammatory disorder affecting mainly young adults. AOSD is characterized clinically by spiking fever, arthritis, evanescent rash, sore throat, enlargement of lymph nodes and splenomegaly, and biologically by neutrophilic leukocytosis, high levels of ferritine and elevated liver enzymes. None of these features are specific, and although several classification criteria have been proposed, AOSD remains a diagnosis by exclusion. Its causes and pathomechanism are still unknown, although there is increasing evidence of dysregulated innate immune response. Treatment mainstays are systemic corticosteroids and methotrexate. Blockade of proinflammatory cytokines may be effective in the substantial proportion of patients with poor response to classical immunosuppressants.

Efficacy of TNF {alpha} blockade in cyclophosphamide resistant neuro-Behçet disease.

Behçet disease is a chronic relapsing inflammatory condition, predominantly affecting young adults, characterised by recurrent bipolar aphtae and systemic manifestations for which tumour necrosis factor (TNF) alpha blockade has recently emerged as an effective treatment. We report the case of a patient presenting with mucocutaneous and ocular manifestations who in the course of his disease developed CNS parenchymal involvement. While being treated with pulsed cyclophosphamide and corticosteroids, he suffered a relapse of his CNS involvement that was efficaciously controlled by infliximab. No disease activity was observed during a full year of TNF blockade, associated with azathioprine, colchicine, and corticosteroids. However, 7 months after the last administration of infliximab and still under immunosuppressant agents, CNS lesions recurred. Infliximab was successfully reintroduced and since continued with no side effects. The sequence of events observed in this patient suggests that TNF blockade is efficacious in suppressing neuro-Behçet disease and once introduced should be maintained for a prolonged period of time.