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Human blood DCs encompass pDCs and two subsets of mDCs: CD1c(+) mDCs and CD141(+) mDCs. The rare CD141(+) DC population is thought to be the equivalent of mouse CD8α(+) cDCs that play a significant role in antigen cross-presentation. Here, we analyzed by Q-PCR TLR1-10 expression in blood DC subsets. Whereas CD1c(+) DCs express all TLR except TLR9, CD141(+)(More)
The reversible cerebral vasoconstriction syndrome (RCVS) is an under-estimated transient acute cerebrovascular disorder. It has long been mistaken as central nervous system vasculitis whereas it is now believed to result from an acute but prolonged vasospasm of cerebral arteries. This disorder can be precipitated by postpartum or vasoactive drug. However,(More)
Erdheim-Chester disease (ECD) is a rare form of non-Langerhans histiocytosis, with noncodified therapeutic management and high mortality. No treatment has yet been shown to improve survival in these patients. We conducted a multicenter prospective observational cohort study to assess whether extraskeletal manifestations and interferon-α treatment would(More)
Schnitzler syndrome (SS) is a rare clinical entity, which belongs to the spectrum of monoclonal gammapathy-associated systemic disorders. Its pathophysiology remains elusive, even if it is tempting to consider it as a late onset and probably acquired auto-inflammatory syndrome. SS mainly occurs in the fifth and sixth decade, and present with an urticariform(More)
OBJECTIVE To analyse the efficacy and tolerance of infliximab in refractory Takayasu arteritis (TA). METHODS French multicentre retrospective study that included patients with TA. Clinical disease activity was defined as new vascular and/or constitutional signs. RESULTS Fifteen patients with TA [median age 41 (range 17-61) years; 13 women] were(More)
AIM To investigate the presentation, disease course and long-term outcome of a western cohort of idiopathic granulomatous mastitis (IGM) and to analyse the impact of different therapeutic strategies. METHODS Multicentre retrospective study of 23 women followed over an extended period. Patients were recruited in nine French internal medicine departments.(More)
OBJECTIVES To report cases of cat scratch disease with vertebral osteomyelitis. METHODS We describe clinical features, diagnostic, treatment, and outcome of 2 patients with vertebral osteomyelitis due to Bartonella henselae and provide a review of the relevant literature. RESULTS A 47-year-old man was investigated for fever, splenomegaly, and cervical(More)
OBJECTIVE Studies have shown that aortitis may be present in half the patients with recent-onset giant cell arteritis (GCA). We assessed whether aortitis at diagnosis affects longterm outcome in patients with GCA. METHODS We retrospectively analyzed the longterm outcome of a prospective cohort of 22 patients with biopsy-proven GCA who all had aortic(More)
The aim of this study is to assess the long-term effectiveness and safety of IL1Ra in Schnitzler syndrome (SchS). Between 2010 and 2012, we performed a nationwide survey among French internal medicine departments to identify SchS patients. We retrospectively analyzed the long-term efficacy and safety of IL1Ra and the outcome of patients that did not receive(More)
OBJECTIVE Mucosal associated invariant T cells (MAIT) and innate lymphoid cells (ILCs) have immunoregulatory functions at mucosal sites and have been involved in various inflammatory and autoimmune diseases. The aim of this study was to assess their frequencies in blood in ANCA-associated vasculitis (AAV). METHODS The frequencies and function of MAIT(More)