[Autoimmune and inflammatory pathologies associated with systemic scleroderma: Clinical, serological and prognostic profiles. Bi-centric retrospective series in the PACA region]. / Pathologies auto-immunes et inflammatoires associées à la sclérodermie systémique : profils cliniques, sérologiques et pronostiques. Série rétrospective bi-centrique en région PACA.

INTRODUCTION: Systemic sclerosis (SSc) is a rare auto-immune disease, affecting principally women between 40 and 60 years old. It is caracterised by a cutaneous and visceral fibrosis, an alteration of the microvascular network and the presence of autoantibodies. SSc can be associated with another connectivite tissue disease or to other autoimmune diseases, thus defining the overlap syndrome. The goal of our study is to describe these overlap syndromes. METHODS: We have analysed the data of a retrospective and bicentrique cohort, from the internal medicine unit of Hôpital Nord in Marseille and from the internal medicine unit of the Hôpital Sainte-Anne in Toulon, of patients followed for a SSc between January 1st, 2019 and December 1st, 2021. We have collected clinical, imunological features, associated auto-immune and inflammatory diseases with its morbidity and mortality. RESULTS: The cohort included 151 patients including 134 limited cutaneous SSc. Fifty-two (34.4%) patients presented at least one associated auto-immune or inflammatory disease. The association of two connectivite tissue diseases including SSc was found in 24 patients (15.9%), a third with Sjögren's syndrome and a third with autoimmune myositis. The principal associated disease to SSc was the autoimmune thyroiditis found in 17 patients (11.3%). The occurrence of complications (hospitalization, long-term oxygene therapy, death) was not significantly different depending on the existence or not of an overlap syndrom. CONCLUSION: SSc is often associated with other autoimmune diseases. This interrelation between associated pathologies and SSc, modifying sometimes the evolution of SSc, enhances the need of a personalized follow-up.

Acute Myocarditis after COVID-19 vaccination: A case report.

INTRODUCTION: The etiology of myocarditis often remains undetermined. A large variety of infectious agents, systemic diseases, drugs, and toxins can cause the disease. We report the case of a 19-year-old man who developed myocarditis three days after Pfizer-BioNTech COVID-19 booster vaccination. CASE REPORT: A 19-year-old man, presenting with troponin-positive acute chest pain, was referred to our department. He had received the Pfizer-BioNTech COVID-19 vaccine three days prior to his admission. The diagnosis of acute myocarditis was confirmed by cardiovascular magnetic resonance imaging. Patient hemodynamic status remained stable during hospitalization. The left ventricular ejection fraction was preserved during hospital stay and at one-month follow-up. We found no evidence for another infectious or autoimmune etiology. CONCLUSION: Although imputability of the vaccine cannot be formally established on the basis of this case report, the findings raise the possibility of an association between mRNA COVID-19 vaccination and acute myocarditis.

[Diagnostic issues of Whipple's disease during chronic inflammatory rheumatism: About three cases]. / Difficultés diagnostiques de la maladie de Whipple au cours des rhumatismes inflammatoires chroniques : trois observations.

INTRODUCTION: Whipple's disease (WD) can mimic chronic inflammatory rheumatism leading to incorrect prescription of tumor necrosis factor inhibitors (TNFI). Several complicated cases of WD have been reported during TNFI treatment which is strongly suspected to modify the host-pathogen relationship. Tropheryma whipplei asymptomatic carriage is high in the general population, making the diagnosis of WD more difficult face to unexplained arthritis. OBSERVATIONS: We report three observations that illustrate situations for which the detection of T. whipplei might be valuable to investigate the differential diagnosis of inflammatory rheumatism. CONCLUSION: The decision to check for T. whipplei infection should rely on individual clinical assessment. It should be considered in the absence of clinical response or in case of worsening of an inflammatory rheumatism under TNFI treatment, especially in front of atypical features. A systematic screening for T. whipplei before anti-TNF treatment seems unjustified since asymptomatic carriers are frequent.

Early detection with the ß-LACTA™ test of extended-spectrum ß-lactamase-producing Enterobacteriaceae in blood cultures.

Effective antibiotic therapy is crucial for the outcome of septic patients and requires early diagnosis of ß-lactam resistance in cases of Gram-negative bacteremia. Here, we report high sensitivity of the ß-LACTA™ test in rapid detection of extended-spectrum ß-lactamase-producing Enterobacteriaceae in blood cultures positive for Gram-negative bacilli.

Gadolinium enhancement in brain magnetic resonance imaging in progressive multifocal leukoencephalopathy after natalizumab monotherapy: is it really atypical?

Progressive multifocal leukoencephalopathy (PML) generally occurs in patients with impaired cellular immunity. Monoclonal antibodies also predispose the patient to PML as they depress the immune system. PML was classically characterized by a lack of inflammation and absence of gadolinium enhancement. However, gadolinium enhancement of PML lesions was first described in HIV-positive patients under therapy. We present a case of gadolinium enhanced PML lesions occuring after natalizumab monotherapy of a relapsing multiple sclerosis. Radiologists must be aware of this particular feature, as confirmation of the diagnostic of PML becomes more challenging. Namely, distinction between starting PML and multiple sclerosis enhanced additional active lesion is difficult and diagnosis must be established by combined analysis of full clinical evolution, brain MRI scans, and polymerase chain reaction of cerebrospinal fluid.

[Pleuropulmonary manifestations of necrotising vasculitis]. / Manifestations pleuropulmonaires des vascularites nécrosantes.

The pleuropulmonary manifestations of necrotising vasculitis are frequent and polymorphic. If the existence of extrapulmonary signs and the presence of neutrophil polynuclear anticytoplasmic antibodies are helpful for the diagnosis of a bout of vasculitis, the existence of pleuropulmonary symptoms can also make for discussion of infections or iatrogenic effects induced by immunosuppressive treatments.