Mesenteric inflammatory veno-occlusive disease of the spleen metasynchronous with two arterial thrombotic events in systemic lupus erythematosus.

Vasculitides, particularly those affecting small vessels, are known to complicate systemic lupus erythematosus (SLE); however, isolated venulitis of the mesenteric bed has rarely been reported. Here we relate the case of a 46-year-old woman with SLE who presented with acute abdominal pain due to artery thrombosis and extended splenic ischemia requiring splenectomy. The histological examination revealed diffuse venulitis in the absence of arterial vasculitis consistent with the definition of mesenteric inflammatory veno-occlusive disease (MIVOD). Furthermore, arterial wall thickening suggestive of uncomplicated atherosclerosis was observed. Two months later, the patient suffered of severe myocardial infarction (MI) resulting from thrombosis of the anterior interventricular coronary artery with otherwise no signs of coronary disease at coronarography. Extensive work-up to establish the cause of MI was negative, with the exception of marginal, isolated and transient elevation of cardiolipin IgG (14.5 GPL, n.v. 0-5 GPL). This patient's SLE history is dramatically marked by the previously non-described association of MIVOD and two arterial thrombotic events (splenic and coronary) occurring within a two months period, and stresses the need of better understanding and prevention of vascular complications in SLE.

[The anti-synthetases syndrome: diagnostic and treatments]. / Syndrome des antisynthétases: diagnostic et traitements.

The anti-synthetases syndrome is a rare disease with a specific constellation of clinical symptoms present in a subset of patients with inflammatory myopathy. Besides constitutional symptoms and myositis, it is associated with mechanic's hands, Raynaud phenomenon, and non-erosive arthritis. This syndrome is characterized by the presence of one of eight auto-antibodies to aminoacyl-transfer ribonucleic acid synthetase enzymes in the serum. Interstitial lung disease is more frequent in this subpopulation of inflammatory myopathy and worsens the patient's prognosis.

[Systemic vasculitides: novel nomenclature and novel therapeutic approaches]. / Vascularites systémiques: nouvelles nomenclature et approches thérapeutiques.

A novel nomenclature of systemic vasculitides is proposed by the 2012 Chapel Hill Consensus Conference. It aims at substituting established eponyms and introducing new terms and groups closer to our current understanding of vasculitis pathophysiology. In parallel, a therapeutic revolution is taking place partially based on the concept of re-induction of immune tolerance for ANCA-associated vasculitis (AAV). Two major multicentric randomized studies have shown that rituximab (RTX), monoclonal antibody capable of selectively killing B lymphocytes, is not inferior when compared to cyclophosphamide (CYC) to induce remission in AAV, and superior in the case of disease relapse. Thus, a hot debate is taking place whether or not to maintain CYC or use RTX in AAV. An individual-based choice may be wise for the moment being.