Large-vessel involvement in ANCA-associated vasculitis: A multicenter case-control study.

OBJECTIVE: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) primarily affects small vessels. Large-vessel involvement (LVI) is rare. We aimed to describe the characteristics of LVI, to identify associated risk factors, and to describe its therapeutic management. METHODS: This multicenter case-control (1:2) study included patients with AAV according to the ACR/EULAR classification and LVI as defined by the Chapel Hill nomenclature, together with controls matched for age, sex, and AAV type. RESULTS: We included 26 patients, 15 (58 %) of whom were men, with a mean age of 56.0 ± 17.1 years. The patients had granulomatosis with polyangiitis (n = 20), or microscopic polyangiitis (n = 6). The affected vessels included the aorta (n = 18; 69 %) supra-aortic trunks (n = 9; 35 %), lower-limb arteries (n = 5; 19 %), mesenteric arteries (n = 5; 19 %), renal arteries (n = 4; 15 %), and upper-limb arteries (n = 2; 8 %). Imaging showed wall thickening (n = 10; 38 %), perivascular inflammation (n = 8; 31 %), aneurysms (n = 5; 19 %), and stenosis (n = 4; 15 %). Comparisons with the control group revealed that LVI was significantly associated with neurological manifestations (OR=3.23 [95 % CI: 1.11-10.01, p = 0.03]), but not with cardiovascular risk factors (OR=0.70 [95 % CI: 0.23-2.21, p = 0.60]), or AAV relapse (OR=2.01 [95 % CI: 0.70-5.88, p = 0.16]). All patients received corticosteroids, in combination with an immunosuppressant in 24 (92 %), mostly cyclophosphamide (n = 10, 38 %) or rituximab (n = 9, 35 %). CONCLUSION: Regardless of distinctions based on vessel size, clinicians should consider LVI as a potential manifestation of AAV, with the aorta commonly affected. The risk of developing LVI appears to be greater for clinical phenotypes of AAV with neurological involvement. Standard AAV treatment can be used to manage LVI.

[A case of chronic idiopathic purpura]. / A propos d'un cas de purpura idiopathique chronique.

This paper focuses though on the medical management of the chronic ITP, as well as on the controversies generated by it and the therapeutic progress. Even if the idiopathic thrombocytopenic purpura is a rather frequent cause of childhood thrombocytopenia, it remains an exclusion diagnosis to be considered only in cases of persistent isolated thrombocytopenia observed in previously healthy children (other etiology excluded by the physical examination and medical history). Idiopathic thrombocytopenic purpura (ITP) is secondary to the premature platelet lysis by antibodies targeting the platelet membrane glycoproteins. The chronic evolution is defined by a prolongation of symptoms for more than 12 months. The management of chronic ITP remains controversial. Novel treatments are on the spot, such as the rituximab and the thrombopoetin receptors' agonists (the most up-to-date therapies). We present the case of a 10 years old girl admitted in our department for recurrent diffuse purpuric lesions in the context of a chronic idiopathic purpura. The clinical aspects were classical: diffuse purpuric rash, without any other symptoms. An extended check-up was performed: it confirmed the presence of anti-platelets antibodies.

NF-kappaB inhibition impairs the radioresponse of hypoxic EMT-6 tumour cells through downregulation of inducible nitric oxide synthase.

Hypoxic EMT-6 tumour cells displayed a high level of inducible nitric oxide synthase (iNOS) and an increased radiosensitivity after a 16 h exposure to lipopolysaccharide, a known activator of nuclear factor-kappaB (NF-kappaB). Both iNOS activation and radioresponse were impaired by the NF-kappaB inhibitors phenylarsine oxide and lactacystin. Contrasting to other studies, our data show that inhibition of NF-kappaB may impair the radioresponse of tumour cells through downregulation of iNOS.

A fatal case of Mycoplasma hominis meningoencephalitis in a full-term newborn.

We report the case of a 20-day-old full-term baby, born to a mother who had had an uncomplicated pregnancy and delivery, who died 13 days after the onset of meningitis. Mycoplasma hominis was the sole agent repeatedly recovered from cerebrospinal fluid and from postmortem brain tissue.