A case of VEXAS with microcytic anemia: don't be mislead by an associated condition!

Background: VEXAS syndrome encompasses a wide range of rheumatological and hematological manifestations, which often features myelodysplastic syndrome accompanied by either macrocytic anemia or macrocytosis.

Case Report: A 61-year-old Sicilian male was referred for a microcytic anemia associated with skin lesions, recurrent fever, involuntary weight loss, recurrent superficial venous thrombosis, migratory polyarthritis and a lung nodule. A hemoglobin electrophoresis uncovered a minor beta-thalassemia contributing to the anemia in addition to the chronic inflammation and vitamin B9/B12 deficiencies.

Segmental Arterial Mediolysis and its Mimickers: A Case Report and Review of the Literature.

Unlabelled: This case report addresses segmental arterial mediolysis (SAM), a rare non-inflammatory vasculopathy. A 51-year-old man presented at the emergency department for epigastric and left upper quadrant pain. He had a history of arterial hypertension and had recently received methylprednisolone for knee pain.

Gastrocnemius Myositis Associated with Behçet's Syndrome.

Unlabelled: Behçet's syndrome is a complex vasculitis involving vessels of all sizes. The typical clinical presentation consists of recurrent oral ulcers associated with genital ulcers, and/or intra-ocular inflammation and/or cutaneous lesions. It may also involve the joints, central nervous system, cardiovascular system and gastrointestinal tract.

Giant cell arteritis with adventitial pattern in a 51-year-old man: A case report.

Clinical presentation as well as histological or biological findings can sometimes make the diagnosis of giant cell arteritis difficult. Histopathological features of temporal artery biopsy from giant cell arteritis patients are also challenging because of the various described appearances or even finding of clinically normal temporal artery biopsy does not rule out the diagnosis. We here describe the case of a 51-year-old man with temporal artery biopsy showing lymphocytes infiltrates in the adventitia corresponding to the so-called adventitial pattern of giant cell arteritis according to Hernandez-Rodriguez et al.

COVID-19 impact on an oncologic emergency department visits: observational study.

Objective: This study aimed at evaluating the impact of COVID-19 on emergency department (ED) visits in a tertiary cancer centre and providing information on the features of the unplanned events during the first wave of COVID-19 pandemic.

Methods: This retrospective observational study based on data from ED reports was divided into three periods of 2 months each around the first lockdown announcement of 17 March 2020: pre-lockdown, lockdown and post-lockdown.

Results: A total of 903 ED visits were included in the analyses.

Isolated adrenocorticotropic hormone deficiency and sialadenitis associated with nivolumab: a case report.

Background: Immune checkpoint inhibition with anti-PD(L)1 and anti-CTLA4 antibodies has significantly changed cancer treatment during the last 10 years. Nevertheless, boosting the immune system with immune checkpoint inhibition can result in immune-related adverse events, affecting different organ systems, among which the endocrine system is the most affected. However, there are few descriptions of the association of immune-related adverse events, and the pathophysiology of some is still lacking.

Atypical acute disseminated encephalomyelitis with systemic inflammation after a first dose of AztraZaneca COVID-19 vaccine. A case report.

Background: Only a few cases of acute disseminated encephalomyelitis (ADEM) following coronavirus disease 19 (COVID-19) vaccination have been described since the beginning of the vaccination campaign.

Results: Here we report the first case of central nervous system (CNS) demyelination with systemic inflammatory findings on whole body 19-fluorodeoxyglucose positron emission tomography with computed tomography (FDG-PET/CT) following the ChAdOx1 nCoV-19 vaccine.

Conclusions: Clinicians should stay aware of potential new adverse events after immunization.

Recurrent 'Occult' F-FDG Uptake in an Atypical Case of Anti-Myeloperoxidase Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.

Unlabelled: A 72-year-old woman, with anti-myeloperoxidase antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), presented with two episodes of spinal pachymeningitis (at two different levels 9 years apart, cervical in 2011 and dorso-lumbar in 2020) associated with aortitis and only demonstrated by F-18 fluorodeoxyglucose positron emission tomography/computed tomography (F-FDG PET/CT). This association between aortitis and pachymeningitis in AAV appears exceptional. Moreover, the relapse of aortitis and pachymeningitis in 2020 was not accompanied by an increase in ANCA.

A Rare Case of a Metastatic Malignant Abrikossoff Tumor.

Abrikossoff tumor, also called granular cell tumor (GCT), is a neoplasm of the soft tissues which is most commonly a solitary, painless, and benign tumor. However, 2% of Abrikossoff tumors can be malignant. We report here the case of a 75-year-old male who presented a local recurrence of Abrikossoff tumor of the left thigh.

Characteristics of thrombocytopenia, anasarca, fever, reticulin fibrosis and organomegaly syndrome: a retrospective study from a large Western cohort.

Idiopathic multicentric Castleman disease (iMCD) is a non-clonal inflammatory lymphoproliferative disorder of unknown origin. Recently, TAFRO syndrome (thrombocytopenia, anasarca, fever, reticulin fibrosis and organomegaly) emerged as a singular variant of iMCD in Asia and was associated with a severe course and a poor outcome. The present study describes the first large Western cohort of TAFRO syndrome patients (n = 25) meeting the All Japan TAFRO Syndrome Research Group diagnostic criteria.

Sequential development of ANCA-associated vasculitis and anti-GBM disease: A report of two cases.

In case of AAV with kidney involvement, physicians should explore anti-GBM antibodies and be aware of the possible sequential development of AAV, especially with MPO-ANCA, and anti-GBM glomerulonephritis. This sequential disease history is associated with a poor renal outcome, highlighting the need for urgent diagnosis and management.

Intrinsic Capacity Assessment by a Mobile Geriatric Team During the Covid-19 Pandemic.

In the autumn of 2020, the second wave of the COVID-19 pandemic hit Europe. In this context, because of the insufficient number of beds in geriatric COVID units, non-geriatric wards were confronted with a significant number of admissions of geriatric patients. In this perspective article, we describe the role of a mobile geriatric team in the framework of the COVID-19 pandemic and specifically how it assisted other specialists in the management of hospitalized geriatric patients by implementing a new approach: the systematic assessment and optimization of Intrinsic Capacity functions.

Rare Features of Dermatomyositis in a Patient with Ovarian Cancer.

Unlabelled: Despite having a classic presentation of dermatomyositis, a patient with ovarian cancer demonstrated several uncommon features: (i) unexpected onset of dermatomyositis in spite of cancer remission, (ii) development of Evans' syndrome and subcutaneous oedema, and (iii) dysphagia. We discuss the occurrence of these conditions as well as their treatment.

Learning Points: This case illustrates a mode of onset of dermatomyositis that could challenge its classification as a 'paraneoplastic' syndrome, as the dermatomyositis appeared when the patient was in complete metabolic remission.

Hydroxychloroquine in a G6PD-Deficient Patient with COVID-19 Complicated by Haemolytic Anaemia: Culprit or Innocent Bystander?

Unlabelled: Hydroxychloroquine has been used worldwide as a first-line treatment for patients hospitalized with COVID-19. Little is known about COVID-19 and its effects on patients with congenital red blood cell disorders. We report a case of haemolytic anaemia in a 32-year-old patient and a fortuitous highlighting of G6PD deficiency.

Exercise-induced torsades de pointes as an unusual presentation of cardiac sarcoidosis: A case report and review of literature.

Background: Sarcoidosis is a rare multisystem disease characterized histologically by non-caseating granuloma formation in the affected organ. While cardiac sarcoidosis is found on autopsy in up to 25% of sarcoidosis cases, it is still underdiagnosed and is associated with a poor prognosis. Although the etiology of sarcoidosis remains unclear, an antigen triggered exaggerated immune response has been hypothesized.

ANCA-associated vasculitides: Recommendations of the French Vasculitis Study Group on the use of immunosuppressants and biotherapies for remission induction and maintenance.

Treatment of vasculitides associated with anti-neutrophil cytoplasm antibodies (ANCA) (AAVs) has evolved dramatically in recent years, particularly since the demonstration of rituximab efficacy as remission induction and maintenance therapy for granulomatosis with polyangiitis and microscopic polyangiitis. In 2013, the French Vasculitis Study Group (FVSG) published recommendations for its use by clinicians. Since then, new data have made it possible to better specify and codify prescription of rituximab to treat AAVs.