Coxiella burnetii endocarditis on bioprosthetic aortic valve, with peripheral arterial embolism.

Acute limb ischemia related to Coxiella burnetii endocarditis is rare. We report an original case of a 68-year-old man hospitalized for recurrent acute left limb ischemia in a context of atrial flutter, which revealed C. burnetii endocarditis. This case illustrates that even if embolic events are uncommon, septic embolisms must be systematically searched for in case of C. burnetii endocarditis. Conversely, extensive etiologic explorations must be performed in case of systemic embolism. New molecular techniques represent a major step forward in infective endocarditis diagnosis. Finally, diagnosis must be suspected in case of unexplained fever, inflammatory syndrome, or embolic event, especially in patients at risk. Conversely, in case of chronic Q fever, an immunodeficiency cause must be researched.

Ultrasonographic assessment of the internal jugular vein for the estimation of central venous pressure in hemodialysis patients: A preliminary study.

PURPOSE: To investigate a new noninvasive method to assess central venous pressure (CVP) in hemodialysis patients, based on the ultrasonographic measurement of the collapsing point of the internal jugular vein (CVPni). MATERIALS AND METHODS: In this preliminary, noninterventional, single center study, we enrolled 22 dialyzed patients with an indwelling jugular catheter. CVPni was compared to the gold-standard invasive measurement of CVP using the central venous catheter (CVPi). Agreement between CVPi and CVPni was assessed by Bland and Altman Method. Correlation was assessed by linear regression. RESULTS: A strong correlation was observed between CVPi and CVPni (OR = 3.47 [2.96; 4.07], P < .0001). For overloaded patients, the area under the curve for the operating characteristic curve was 0.971 (IC95: 0.915; 1.000). For under-loaded patients, area under the curve was 0.971 (IC95: 0.917; 1.000). The mean bias between intra-individual CVPi and CVPni measures was 0.57 cm H2 O (SD: 3.1 cm H2 O). CONCLUSION: CVPni appears as a noninvasive and reliable technique. Further studies are required to confirm these results and to assess the direct clinical impact of this new method.

Severe ADAMTS13 deficiency in adult idiopathic thrombotic microangiopathies defines a subset of patients characterized by various autoimmune manifestations, lower platelet count, and mild renal involvement.

The significance of ADAMTS13 deficiency in adult thrombotic microangiopathy (TMA) remains controversial. In an attempt to define the characteristics of adult TMA with severe ADAMTS13 deficiency, we determined 2 groups of patients on the basis of ADAMTS13 activity (undetectable or detectable). Clinical presentation, laboratory values, autoimmune manifestations, and outcome were compared between the groups. Patients were included retrospectively from 12 centers. All fulfilled the diagnosis criteria of TMA. Patients with a history of transplantation, cancer and chemotherapy, and Centers for Disease Control and Prevention (CDC) stage C human immunodeficiency virus (HIV) infection were not included. Forty-six patients were included. Thirty-one patients had an undetectable ADAMTS13 activity (<5%), and the remaining 15 patients had ADAMTS13 activity of >25%. Severe ADAMTS13 deficiency was associated with a plasmatic inhibitor in 17 cases (55%), suggesting an immune-mediated mechanism. Patients with undetectable ADAMTS13 were more frequently of Afro-Caribbean origin than patients with detectable ADAMTS13 activity (48.4% vs 13.3%, respectively; p = 0.03). As opposed to patients with detectable ADAMTS13 activity, patients with severe ADAMTS13 deficiency displayed various autoimmune manifestations that consisted of nondestructive polyarthritis (4 cases) associated in 1 case with malar rash and extramembranous glomerulonephritis, discoid lupus (3 cases), and autoimmune endocrinopathies, Raynaud phenomenon, and sarcoidosis-like disease (1 case each). In patients with severe ADAMTS13 deficiency, antinuclear antibodies, anti-double-stranded DNA antibodies, and anticardiolipin antibodies were positive in 22 (71%) cases, 3 (9.7%) cases, and 1 (3.2%) case, respectively. One patient fulfilled the criteria for the diagnosis of systemic lupus erythematosus. During follow-up, 1 patient with severe ADAMTS13 deficiency developed antinuclear antibodies, and 3 others developed anti-double-stranded DNA antibodies, in association with neurologic manifestations and anticardiolipin antibodies in 1 case. Patients with severe ADAMTS13 deficiency also had a lower platelet count (12 x 10(9)/L; range, 2-69 x 10(9)/L) and less severe renal failure (estimated glomerular filtration rate: 78 mL/min; range, 9-157 mL/min) than patients with detectable ADAMTS13 activity (49.5 x 10(9)/L; range, 6-103 x 10(9)/L; p = 0.0004, and 15.8 mL/min; range, 5.6-80 mL/min; p < 0.0001, respectively). End-stage renal failure occurred in 1 patient with severe ADAMTS13 deficiency and in 3 patients with detectable ADAMTS13 activity (3.2% vs 21.4%, respectively; p = 0.08). Flare-up and relapse episodes and survival were comparable between the groups. Taken together, these data indicate that adult idiopathic thrombotic thrombocytopenic purpura, as defined by severe ADAMTS13 deficiency, may occur preferentially in a particular ethnic group, and is characterized by severe thrombocytopenia, mild renal involvement, and a wide spectrum of autoimmune manifestations that may be completed during follow-up. Indeed, apparently idiopathic thrombotic thrombocytopenic purpura may be considered a specific autoimmune disease.

A three-arm study comparing immediate tacrolimus therapy with antithymocyte globulin induction therapy followed by tacrolimus or cyclosporine A in adult renal transplant recipients.

BACKGROUND: Induction therapy with antithymocyte globulin (ATG) reduces the incidence of acute rejection after transplantation. A study was undertaken to assess the efficacy and safety of ATG induction on tacrolimus-based and cyclosporine A (CsA)-based therapies compared with immediate tacrolimus triple therapy in kidney transplant recipients. METHODS: In a 6-month, open-label, randomized, prospective study conducted in 30 European centers, 555 renal transplant patients were randomly assigned to tacrolimus triple therapy (Tac triple, n=185), ATG induction with tacrolimus (ATG-Tac, n=186), or ATG induction with CsA microemulsion (ATG-CsA, n=184); all were combined with azathioprine and corticosteroids. The primary endpoint was incidence and time to first acute rejection episode confirmed by biopsy. RESULTS: Patient demographics and clinical parameters at baseline were similar. Patient and graft survival rates were similar in all groups. The incidence of clinically apparent acute rejection was significantly higher (P=0.003) for Tac triple (33.0%) compared with ATG-Tac (22.6%) and the incidence for ATG-Tac was significantly lower (P=0.004) than for ATG-CsA (37.0%). The incidences of acute rejection confirmed by biopsy (primary endpoint) were 25.4%, 15.1%, and 21.2% for Tac triple, ATG-Tac, and ATG-CsA, respectively (Tac triple vs. ATG-Tac, P=0.004). The incidences of corticosteroid-resistant acute rejection were 7.0% (Tac triple), 4.8% (ATG-Tac), and 10.9% (ATG-CsA) (ATG-Tac vs. ATG-CsA, P=0.038). In the ATG groups, the incidences of leukopenia, thrombocytopenia, serum sickness, fever, and cytomegalovirus infection were significantly higher (P<0.05). CONCLUSIONS: Acute rejection was significantly lower in the ATG-Tac group compared with the ATG-CsA and Tac triple groups. Significantly more hematologic and infectious adverse events were observed in both ATG induction groups.

Control of a relapse and induction of long-term remission of Wegener's granulomatosis by cyclosporine.

We report a case of severe Wegener's granulomatosis that caused end-stage renal failure and recurrent flares of sinusitis and pulmonary manifestations. To avoid the cumulative toxicity of iterative treatments with cyclophosphamide pulses and glucocorticoids, treatment with cyclosporine as a single agent was instituted in the early phase of the third relapse, before any pulmonary involvement. Cyclosporine enabled rapid control of the disease and induced a complete remission of more than 30 months.