Kocuria massiliensis sp nov, a new bacterial species isolated from a patient with foot osteomyelitis

International audienceMost of the species from the genus Kocuria are environmental or commensals of mammalian skin and oral bacteria, and had rarely been associated with human infection. However, recent reports showed an increase of the clinical role of these bacteria in human infectious diseases. Most of the cases occurred in hospitals and were device related. They included bacteremia, peritonitis, abscess, endocarditis and ocular infection. We here describe the main characteristics and the draft genome of Kocuria massiliensis sp. nov., strain P3598(T) (CSURP3598), a new Kocuria species that caused foot osteomyelitis in a 78-year-old woman. The improvement of diagnostic tools for the identification of bacteria in microbiological laboratories, including MALDI-TOF MS and 16S rRNA sequencing, largely contributed to the emergence and to the expansion of the clinical spectrum of infections caused by Kocuria spp. To the best of our knowledge, we report here the first case of osteomyelitis with a bacterial species from the genus Kocuria

Nailfold videocapillaroscopy alterations in dermatomyositis, antisynthetase syndrome, overlap myositis, and immune-mediated necrotizing myopathy

International audienceIntroduction/objectives The aim of our study was to investigate possible differences in nailfold videocapillaroscopy (NVC) features between patients with dermatomyositis (DM), overlap myositis (OM), antisynthetase syndrome (ASS), and immune-mediated necrotizing myopathy (IMNM). Methods We performed a cross-sectional monocentric study. All patients with inflammatory myopathies (IMs) over a 6-month period were analyzed by NVC for giant and ramified capillaries, tortuosities, capillary density, disorganization, and scleroderma pattern. Clinical, biological, and pathological characteristics were retrospectively recorded. Patients were classified as having DM, OM, ASS, or IMNM for comparison. Patients were also compared with a group of patients with systemic sclerosis (SSc). Results NVC was analyzed in DM (n = 17), OM (n = 8), ASS (n = 12), and IMNM (n = 6). Vascular disorganization and avascular zones were observed only in DM (11.8%) and OM (62.5%). The percentage of patients with giant capillaries was higher in OM (n = 4/8) than in DM (n = 3/17) and absent in ASS and IMNM. Frequency of ramified capillaries, tortuosities, hemorrhages, or thrombosis was not different between subgroups. A scleroderma pattern was only observed in OM patients. Conclusion In this limited series of patients, we observed that DM and OM NVC abnormalities are different from ASS and IMNM. We could not determine NVC specific patterns associated with myositis-specific antibody subtypes of DM because of the small number of patients

Autologous haematopoietic stem cell transplantation (AHSCT) in autoimmune disease adult patients in France: analysis of the long-term outcome from the French Society for Bone Marrow Transplantation and Cellular Therapy (SFGM-TC)

International audienc

Safety of Intravenous Immunoglobulin (Tegeline®), Administered at Home in Patients with Autoimmune Disease: Results of a French Study

The efficacy of intravenous immunoglobulins (IVIg) in patients with autoimmune diseases (AID) has been known for several decades. Majority of these patients received IVIg in hospital. A retrospective study was conducted in 22 centers in France to evaluate the feasibility of the administration of Tegeline, an IVIg from LFB Biomedicaments, and assess its safety at home, compared to in hospital, in patients with AID. The included patients were at least 18 years old, suffering from AID, and treated with at least 1 cycle of Tegeline at home after receiving 3 consecutive cycles of hospital-based treatment with Tegeline at a dose between 1 and 2 g/kg/cycle. Forty-six patients with AID, in most cases immune-mediated neuropathies, received a total of 138 cycles of Tegeline in hospital and then 323 at home. Forty-five drug-related adverse events occurred in 17 patients who received their cycles at home compared to 24 adverse events in hospital in 15 patients. Serious adverse events occurred in 3 patients during home treatment, but they were not life-threatening and did not lead to discontinuation of Tegeline. Forty-five patients continued their treatment with Tegeline at home or in hospital; 39 (84.8%) were still receiving home treatment at the end of the study. In conclusion, the study demonstrates the good safety profile of Tegeline administered at home at high doses in patients with AID who are eligible for home administration of Tegeline

Temporal Arteritis Revealing Antineutrophil Cytoplasmic Antibody-Associated Vasculitides: Case-Control Study of 50 Cases.

BACKGROUND: Temporal arteritis (TA) is a typical manifestation of giant cell arteritis (GCA). Rarely, ANCA-associated vasculitides (AAV) can be revealed by TA, leading to misdiagnoses of GCA and inappropriate treatments. METHODS: We retrospective included patients with TA revealing AAV (TA-AAV), and compared them with classical GCA patients. RESULTS: Fifty patients with TA-AAV (median age 70 years) were included. Thirty-three (66%) patients presented atypical symptoms for GCA (ENT, renal, pulmonary and neurological involvement in 32%, 26%, 20% and 16%, respectively). ANCA were screened at disease onset in 33 cases, and were positive in 88%, leading to diagnosis of early TA-AAV in 20 cases. AAV diagnosis was delayed in 30 cases after a median interval of 15 months. Compared to GCA, TA-AAV patients were younger (70 vs. 74 years), more frequently men (48 vs. 30%), had high frequency of atypical manifestations and higher CRP levels (10.8 vs. 7.0 mg/dL). Temporal artery biopsy (TAB) from TA-AAV showed fibrinoid necrosis and small branch vasculitis in 23% each, whereas it did not in GCA. Treatment failure-free survival was comparable between early TA-AAV and GCA, whereas those with delayed TA-AAV patients had a significant higher risk of treatment failure compared to GCA [HR 3.85 (1.97-7.51), P<0.0001)]. CONCLUSION: TA revealing AAV should be considered in case of atypical manifestations for GCA, GCs refractoriness or early relapse. Analysis of TAB for small branch vasculitis and/or fibrinoid necrosis could be useful. Detection of ANCA should be performed in case of suspected GCA with atypical clinical features and/or TAB.status: Published onlin

Untreated patients with type 1 Gaucher disease: who are they? Resultas from the Gaucher Non-Treated study (GANT study)

International audienc

A Cross-Sectional Retrospective Study of Non-Splenectomized and Never-Treated Patients with Type 1 Gaucher Disease

International audiencePatients with type 1 Gaucher disease (GD1) present thrombocytopenia, anemia, organomegaly, and bone complications. Most experts consider that the less aggressive forms do not require specific treatment. However, little is known about the disease course of these forms. The objective of this cross-sectional retrospective study was to compare the clinical, radiological, and laboratory characteristics of patients with less severe GD1 at diagnosis and at the last evaluation to identify features that might lead to potential complications. Non-splenectomized and never-treated patients (19 women and 17 men) were identified in the French Gaucher Disease Registry (FGDR). Their median age was 36.6 years (2.4–75.1), and their median follow-up was 7.8 years (0.4–32.4). Moreover, 38.7% were heterozygous for the GBA1 N370S variant, and 22.6% for the GBA1 L444P variant. From diagnosis to the last evaluation, GD1 did not worsen in 75% of these patients. Some parameters improved (fatigue and hemoglobin concentration), whereas platelet count and chitotriosidase level remained stable. In one patient (2.7%), Lewy body dementia was diagnosed at 46 years of age. Bone lesion onset was late and usually a single event in most patients. This analysis highlights the genotypic heterogeneity of this subgroup, in which disease could remain stable and even improve spontaneously. It also draws attention to the possible risk of Lewy body disease and late onset of bone complications, even if isolated, to be confirmed in larger series and with longer follow-up

Analysis of risk factors for complications and adverse obstetrical outcomes in women with Takayasu arteritis: a French retrospective study and literature review

International audienceObjective Takayasu arteritis (TAK) is a large vessel vasculitis affecting young women of childbearing age. The outcome of pregnancies in TAK patients, factors associated with maternal and foetal complications and adverse outcomes were analysed. Methods All pregnancies in women with a TAK diagnosis were retrospectively included from 20 French hospitals providing care for TAK, until August 2015. Results The study consisted of 43 pregnancies in 33 women, including 29 with a pre-existing TAK diagnosis and 4 diagnosed during pregnancy. Complications were observed in 20 pregnancies (47%), including 35% with arterial hypertension (n = 15), 9% with pre-eclampsia (n = 4), 2% with HELLP syndrome (n = 1) and 14% with intrauterine growth restriction (IUGR, n = 6, leading in one case to a medically indicated termination of pregnancy). There were 42 live births (98%) at a median term of 38 [27-42] weeks gestation including 9 before 37 weeks (21%). The median birth weight was 2940 [610-4310] grams. Five children (12%) required transfer to a neonatal intensive care unit. One premature boy (27 weeks gestation) died after 2 days. Treatment during pregnancy included steroids (n = 25/43; 58%), azathioprine (n = 9/43; 21%) and infliximab (n = 1/43; 2%). The risk of developing arterial hypertension during pregnancy was associated with previous chronic arterial hypertension and with an infra-diaphragmatic vasculitis injury (P = 0.01 and P = 0.04, respectively). No correlation was reported between TAK activity and any of the obstetrical complications described in the study. Conclusion This study showed a high rate of adverse obstetrical complications without significant impact on live birth rates. Pregnancy did not appear to influence TAK disease activity..Pregnancy did not appear to influence TA disease activity