[Connective tissue diseases and pregnancy]. / Connectivites et grossesse.

Connective tissue diseases are rare usually affecting women of reproductive age. Patients should be informed about the potential obstetrical risks associated with their disease as well as potential exacerbations during pregnancy, while simultaneously being reassured with the prospect of a positive pregnancy outcome. In recent years, significant progress has been made in medical treatments, giving women the opportunity to consider pregnancy. Preconception counseling is critical to pregnancy planning. An effective contraceptive measure should be recommended based on disease activity and teratogenic medications should be modified. Pregnancy monitoring are managed based on specific clinical and serological markers (such as the presence of anti-SSA/SSB or anti-phospholipid antibodies). A multidisciplinary approach is essential for a safe pregnancy.

Les connectivites touchent souvent des femmes en âge de procréer. Les patientes doivent être informées des risques obstétricaux ou d'aggravation potentiels de leur maladie, mais également être rassurées quant à leur chance de succès de grossesse. En effet, grâce aux progrès de la médecine, il est possible de mener à bien une grossesse chez ces patientes en prenant certaines précautions. Une contraception efficace doit être mise en place en cas de maladie active, de prise de médicaments tératogènes ou fœto-toxiques. Une adaptation des traitements est donc souvent nécessaire avant la grossesse. Le suivi durant celle-ci, l'accouchement et le postpartum est conditionné par le type de maladie, son activité, ainsi que les profils sérologiques à risques (anti-SSA/SSB, anticorps antiphospholipides). Une approche multidisciplinaire est donc essentielle.

[Insulin allergy: how to manage it?] / Allergie à l'insuline : comment faire ?

Insulin allergy is a rare entity, complex to manage. Several types of hypersensitivity reaction are described, depending on the allergens (insulin itself vs additives). Type I, so-called immediate, IgE-mediated reactions are the most common. Their management requires a careful history and examination, as well as an allergological consult. If an IgE-mediated allergy is confirmed, insulin avoidance is recommended whenever possible. If insulin treatment is mandatory, another type of insulin may be offered. In case of failure, desensitization should be discussed, either via a dedicated protocol, or via insulin pump. In this article, we summarize the available data from the literature.

L'allergie à l'insuline est une entité rare, complexe à prendre en charge. Plusieurs types de réactions d'hypersensibilité sont décrits, selon les agents allergènes (insuline même vs additifs). Les réactions de type I, dites immédiates, IgE (immunoglobulines E) médiées sont les plus fréquentes. Leur prise en charge nécessite une anamnèse et un examen minutieux, ainsi qu'un avis allergologique. En cas de confirmation d'allergie IgE médiée, une éviction des insulines est dans la mesure du possible recommandée. Si la poursuite des traitements insuliniques est inévitable, un autre type d'insuline peut être proposé. En cas d'échec, une induction de tolérance devrait être discutée, soit via un protocole dédié, soit via la mise sous pompe à insuline. Nous résumons dans cet article les données de la littérature à disposition.

[Egg allergy in children and adults]. / Allergie aux œufs chez l'enfant et l'adulte.

Hen's egg allergy is the second major cause of food allergy in children and is rare in adults. It can present with diverse and sometimes severe symptoms that can be challenging for patients and clinicians. Allergy history represents the main diagnostic tool, and can be completed with skin tests, specific IgE testing and in selected cases with an oral challenge test. All patients with egg anaphylaxis should benefit from an emergency kit in case of reaction and specialized allergologic evaluation. Strict eviction of eggs can be difficult to achieve because of hidden allergens. For safety purpose, an oral tolerance induction is helpful selected patients. A pre-vaccination assessment is required in rare selected cases.

L'allergie aux œufs représente la deuxième cause d'allergie alimentaire la plus fréquente chez le jeune enfant mais elle est nettement plus rare chez l'adulte. Ses manifestations diverses et parfois graves représentent un défi pour les patients et le clinicien. L'outil diagnostique principal est l'histoire clinique, suivi si nécessaire par des tests cutanés, le dosage des immunoglobulines E spécifiques et/ou un test de provocation. Les patients présentant une anaphylaxie doivent être équipés d'une trousse d'urgence et adressés au spécialiste. L'éviction stricte des œufs est indiquée mais peut être difficile à respecter en raison des allergènes cachés. Une induction de tolérance orale chez des patients sélectionnés offre la perspective future d'une meilleure sécurité au quotidien. Un bilan avant vaccination est requis dans de rares cas sélectionnés.

Anti-apolipoprotein A-1 autoantibodies correlate with disease activity in systemic lupus erythematosus.

OBJECTIVES: Apolipoprotein A-1 (ApoA-1) is a protein fraction of the high-density lipoproteins with anti-inflammatory and antioxidant properties that play a major role in reverse cholesterol transport. The presence of anti-ApoA-1 IgG has been reported in SLE to be variably associated with disease activity or cardiovascular events (CVEs). We assessed the clinical performance of anti-ApoA-1 IgG and of antibodies directed against its immunodominant F3L1 peptide (F3L1 IgG) in a well-characterized Swiss SLE cohort study. METHODS: A total of 354 biological samples and interviews from 176 individuals were studied. SLEDAI, clinical characteristics, anamnestic CVEs and therapy details were recorded. Sera were tested for the presence of anti-ApoA-1 IgG, anti-F3L1 IgG, anti-dsDNA IgG and aPL. RESULTS: Anti-ApoA-1 and anti-F3L1 IgG positivity was associated with higher SLEDAI, mostly due to concomitant positivity of dsDNA IgG and low complement. Variations in time of anti-ApoA-1 IgG correlated positively with variations of anti-dsDNA IgG and inversely to variations of C3 levels. No cross-reactivity was found between anti-ApoA-1 and anti-dsDNA IgG. Positivity for anti-Apo-A1 IgG was more frequent in individuals receiving 10 mg/day or more of prednisone. We did not find any significant association between anti-ApoA-1 IgG positivity and CVEs. CONCLUSION: Anti-ApoA-1 and anti-F3L1 IgG in SLE correlate strongly with laboratory markers of activity, particularly with the presence and titre of dsDNA IgG. These results confirm and extend previous findings and support the use of anti-ApoA1 IgG in the clinical setting. Their role in CVEs deserves further investigation.

[ENT and head and neck manifestations of the IgG4-related disease]. / Atteintes de la sphère ORL et cervicofaciale dans la maladie à IgG4.

IgG4-related disease is a rare multi-organic inflammatory disease that frequently involves the ENT and head and neck areas. In these cases, salivary gland and lacrimal gland involvement is very common and includes enlargement, infiltration, and formation of pseudotumours. Diagnosis of IgG4 related disease remains a challenge and relies on several clinical, serological, radiological and histopathological criteria to differentiate from other diseases with similar clinical presentation. Histology reveals IgG4 positive lymphoplasmocytic infiltrates, storiform fibrosis and obliterative phlebitis. Glucocorticoids are the first line of treatment and can be combined with other immunosuppressants. The prognosis is favorable if treatment is initiated early. Recurrences are common. Delay in diagnosis can have severe multi-organic consequences.

Rare et encore peu connue, la maladie à immunoglobuline G4 (IgG4) est multiorganique et se manifeste fréquemment dans la sphère oto-rhino-laryngologique et cervico-faciale. Dans ces cas, les glandes lacrymales et salivaires sont les plus fréquemment touchées, présentant des tuméfactions, infiltrations et pseudotumeurs. Le diagnostic reste un défi ; il repose sur des critères cliniques, sérologiques, radiologiques et histopathologies qui visent à la distinguer des nombreuses maladies de présentations cliniques similaires. À l'histologie, on retrouve un infiltrat lymphoplasmocytaire riche en plasmocytes IgG4+, une fibrose storiforme et des phlébites oblitérantes. Les glucocorticoïdes sont la première ligne de traitement et peuvent être associés à d'autres immunosuppresseurs. Le pronostic est favorable si le traitement est initié rapidement. Les récidives sont courantes. Un retard diagnostique peut avoir des conséquences multiorganiques sévères.

Diagnostic accuracy of multi-lead ECGs obtained using a pocket-sized bipolar handheld event recorder.

BACKGROUND: Handheld ECG event recorders are useful for rhythm monitoring but only record a single lead, which may limit interpretation. We sought to investigate if a multi-lead ECG may be reconstituted using this device, and aimed to evaluate diagnostic accuracy. METHODS: A commercially-available handheld bipolar ECG event recorder was used to reconstruct a 9-lead ECG (leads I, II and III, V1-6). Tracings were analyzed independently by a cardiologist and a fellow. RESULTS: A total of 52 patients were evaluated. Accuracy was excellent (85-98%) for identifying atrial fibrillation, atrioventricular block, bundle branch block and left ventricular hypertrophy, but lower (77-88%) for ST-segment changes and prolonged QTc. CONCLUSIONS: A 9-lead ECG can be reconstituted using a handheld single-lead ECG event recorder, and provides good diagnostic accuracy for common findings.

Reclassifying Anaphylaxis to Neuromuscular Blocking Agents Based on the Presumed Patho-Mechanism: IgE-Mediated, Pharmacological Adverse Reaction or "Innate Hypersensitivity"?

Approximately 60% of perioperative anaphylactic reactions are thought to be immunoglobulin IgE mediated, whereas 40% are thought to be non-IgE mediated hypersensitivity reactions (both considered non-dose-related type B adverse drug reactions). In both cases, symptoms are elicited by mast cell degranulation. Also, pharmacological reactions to drugs (type A, dose-related) may sometimes mimic symptoms triggered by mast cell degranulation. In case of hypotension, bronchospasm, or urticarial rash due to mast cell degranulation, identification of the responsible mechanism is complicated. However, determination of the type of the underlying adverse drug reaction is of paramount interest for the decision of whether the culprit drug may be re-administered. Neuromuscular blocking agents (NMBA) are among the most frequent cause of perioperative anaphylaxis. Recently, it has been shown that NMBA may activate mast cells independently from IgE antibodies via the human Mas-related G-protein-coupled receptor member X2 (MRGPRX2). In light of this new insight into the patho-mechanism of pseudo-allergic adverse drug reactions, in which as drug-receptor interaction results in anaphylaxis like symptoms, we critically reviewed the literature on NMBA-induced perioperative anaphylaxis. We challenge the dogma that NMBA mainly cause IgE-mediated anaphylaxis via an IgE-mediated mechanism, which is based on studies that consider positive skin test to be specific for IgE-mediated hypersensitivity. Finally, we discuss the question whether MRGPRX2 mediated pseudo-allergic reactions should be re-classified as type A adverse reactions.