Vascular expression of annexin A2 in lupus nephritis.

AIMS: To evaluate vascular expression of annexin A2 (ANXA2) and its subunit S100A10 in lupus nephritis (LN). METHODS: The present histological study included 14 patients with LN and 11 controls (patients with non-lupus kidney diseases). Kidney biopsies from patients with lupus were scored for lupus glomerulonephritis (according to the International Society of Nephrology/Renal Pathology Society 2003 classification) and vascular lesions (such as microthrombi and antiphospholipid syndrome nephropathy (APSN)). ANXA2 and S100A10 expression in glomerular and peritubular capillaries was evaluated by immunohistochemistry on tissue sections. The staining intensity score ranged from 0 (no expression) to 4 (intense expression). RESULTS: In patients with LN, the median age (range) at first kidney biopsy was 36 (18-49). Vascular lesions were observed in six patients (including two with APSN). We observed intense expression of ANXA2 in glomerular and peritubular capillaries while expression of S100A10 was weaker. However, one of the patients with APSN showed strong S100A10 expression. Patients with LN and controls differed significantly in terms of S100A10 expression in peritubular capillaries. We also observed a statistical difference between patients who had LN with renal vascular lesions and those without renal vascular lesions in terms of ANXA2 expression in peritubular capillaries. CONCLUSIONS: The presence of vascular lesions in LN appears to be associated with significant differences in the vascular expression of ANXA2. Vascular expression of ANXA2 was somewhat higher in LN. Vascular expression of S100A10 was somewhat lower in LN (except one of the two patients with APSN). Further studies of ANXA2's putative value as a biomarker of active LN or of vascular lesions in LN are required.

Determinants of hydroxychloroquine blood concentration variations in systemic lupus erythematosus.

OBJECTIVE: Blood concentrations of hydroxychloroquine (HCQ) vary widely among patients with systemic lupus erythematosus (SLE). A pharmacokinetic/pharmacodynamic relationship has been found in different situations, and a very low blood concentration of HCQ is a simple marker of nonadherence to treatment. Therefore, interest in blood HCQ concentration measurement has increased, but little is known about factors that influence blood HCQ concentration variability. This study was undertaken to analyze determinants of blood HCQ concentrations. METHODS: We conducted a retrospective analysis of patient data, including data from the Plaquenil Lupus Systemic (PLUS) study, to determine the association of epidemiologic, clinical, and biologic factors with blood HCQ concentrations. Data for nonadherent patients (blood HCQ concentration <200 ng/ml) were excluded. RESULTS: To examine homogeneous pharmacologic data, we restricted the analyses of the PLUS data to the 509 SLE patients receiving 400 mg/day. We found no association of ethnicity or smoking with blood HCQ concentrations and no pharmacokinetic drug-drug interaction with antacids or with inhibitors or inducers of cytochrome P450 enzymes. On multivariate analysis, high body mass index (P = 0.008), no treatment with corticosteroids (P = 0.04), increased time between the last tablet intake and measurement of blood HCQ concentrations (P = 0.017), low platelet count (P < 0.001), low neutrophil count (P < 0.001), and high estimated creatinine clearance (P < 0.001) were associated with low blood HCQ concentrations. In 22 SLE patients with chronic renal insufficiency (median serum creatinine clearance 52 ml/minute [range 23-58 ml/minute]) who received 400 mg/day HCQ, the median blood HCQ concentration was significantly higher than that in the 509 patients from the PLUS study (1,338 ng/ml [range 504-2,229 ng/ml] versus 917 ng/ml [range 208-3316 ng/ml]) (P < 0.001). CONCLUSION: We provide a comprehensive analysis of determinants of blood HCQ concentrations. Because this measurement is increasingly being used, these data might be useful for clinicians.

[Cervical lymph node tuberculosis: diagnosis and treatment]. / Tuberculose lymphonodale cervicale: prise en charge diagnostique et thérapeutique.

OBJECTIVES: The purpose of this study was to evaluate the advantages of surgery for diagnosis and treatment of cervical lymph node tuberculosis. MATERIAL AND METHODS: This was a retrospective study from 1st January 1998 to 31st December 2007 including 30 patients with cervical lymph node tuberculosis. The population included 60% autochthones with a mean age of 47.1 years and a female predominance (73.33%). RESULTS: The lymph nodes were most often supraclavicular, unilateral, firm, and a mean 3 cm at its largest span. Lymph nodes were excised for diagnosis in 22 patients, which demonstrated specific granulomatous and giant cell lesions with caseous necrosis in 21 patients out of 22. Five abscessed adenopathies required surgical drainage, and three cases required repeated lymph node cleaning after well-conducted medical treatment. CONCLUSION: Surgery retains an important place in the diagnosis and treatment of cervical lymph node tuberculosis.

Procalcitonin at the onset of giant cell arteritis and polymyalgia rheumatica: the GRACG prospective study.

OBJECTIVES: An epidemic pattern has been reported for GCA and PMR. Immunological studies have shown that an unknown antigen activates the dendritic cells of the adventitia and the type 4 toll-like receptors. Procalcitonin (PCT) is an early marker of bacterial infection. The goal of the study was to assess the level of PCT in GCA and PMR at the onset of the disease. METHODS: Patients diagnosed during the 2002-06 period were randomly selected. All the 46 patients fulfilled the ACR or the Hunder criteria, and all blood samples were taken before steroid therapy. RESULTS: PCT was normal in all patients. PCT was slightly increased in men (0.087 +/- 0.023 microg/l) compared with women (0.066 +/- 0.027 microg/l) (P = 0.009), and in PMR (0.092 +/- 0.027 microg/l) compared with GCA (0.068 +/- 0.026 microg/l) (P = 0.018). There was no significant correlation with inflammation markers. CONCLUSIONS: These results are not in favour of a bacterial trigger for GCA or PMR. Increased PCT levels in patients with inflammatory syndrome, GCA-PMR symptoms and negative temporal artery biopsy may rule out the diagnosis of GCA and PMR.

Clinical significance of autoantibodies recognizing Sjögren's syndrome A (SSA), SSB, calpastatin and alpha-fodrin in primary Sjögren's syndrome.

The aim of our study was (i) to compare the clinical and biological characteristics of 148 (137 women, 11 men) primary Sjögren's syndrome (pSS) patients at diagnosis as a function of their sex and (ii) to assess the prognostic value of anti-calpastatin and anti-alpha-fodrin autoantibodies. In addition, the presence of anti-nuclear antibodies (ANA), anti-52- and 60-kDa Sjögren's syndrome A (SSA), anti-Sjögren's syndrome B (SSB), anti-cyclic citrullinated peptide (CCP) antibodies and rheumatoid factors (RF) of IgA, IgG and IgM isotypes was sought in sera collected at pSS onset. Raynaud's syndrome, significantly more frequent in women, was the only systemic manifestation of pSS whose frequency differed significantly as a function of the patient's sex (P = 0.02). ANA (P = 0.001) and anti-60-kDa SSA autoantibodies (P = 0.03) were significantly more common in women, while men never synthesized detectable levels of anti-SSB, anti-calpastatin or IgG anti-alpha-fodrin autoantibodies. In addition, anti-CCP autoantibodies were found in low percentages of pSS patients (4% F/18% M). The absence of autoantibodies does not exclude the diagnosis of pSS in men that will be based mainly on the anatomopathological findings of a minor salivary gland biopsy. Positivity of anti-60-kDa SSA, anti-SSB, anti-calpastatin, IgA and IgG anti-alpha-fodrin antibodies is not associated with pSS clinical and biological severity.

[Treatment in primary Streptococcus pneumoniae peritonitis in adult: a case report and review of the literature]. / Prise en charge thérapeutique de la péritonite primitive à Streptococcus pneumoniae de l'adulte: étude d'un cas et revue de la littérature.

INTRODUCTION: Streptococcus pneumoniae primary peritonitis is rare. The diagnosis is uneasy and the treatment is not standardised. CASE REPORT: We report a single case of S. pneumoniae primary peritonitis needing surgical treatment. DISCUSSION: S. pneumoniae primary peritonitis can be medically treated. Surgery is needed in case of sepsis, associated digestive injuries or failure of medical treatment.

[Incidence of cardiovascular events in giant cell arteritis: preliminary results of a prospective double cohort study (GRACG)]. / Incidence des accidents cardiovasculaires au cours de la maladie de Horton: résultats préliminaires de l'étude prospective en double cohorte GRACG.

UNLABELLED: Giant cell arteritis is the most frequent vasculitis. Cardiovascular events such as cerebrovascular accident or ischemic heart disease may occur in patients with giant cell arteritis. However, their real incidence, as well as their relative risk compared to the general population, remains unknown. PURPOSE: To assess in a prospective, double cohort study, the incidence of cardiovascular events in giant cell arteritis patients compared to controls, after controlling for cardiovascular risk factors. PATIENTS AND METHODS: We included on predefined criteria 432 newly diagnosed patients with giant cell arteritis, each assigned to sex- and age-matched controls randomly selected from the general population. Cardiovascular risk factors (high-blood pressure, diabetes, smoking, hypercholesterolemia and preexisting peripheral vascular disease) were collected at inclusion. During the 24-month follow-up, all cardiovascular events were collected. After stratification for cardiovascular risk factors, a log-rank test was performed to compare cases and controls. A parametric survival model was used for multivariate analysis. RESULTS: Cardiovascular events all combined were significantly increased in patients with giant cell arteritis (RR = 2.15 [1.21-3.81], P = 0.009), and were mainly associated with age (P = 0.0001), past history of cardiovascular disease (P = 0.023) but also with giant cell arteritis (P = 0.009). However, each subset of cerebrovascular accident (RR = 2.42 [0.84-7]) or ischemic heart disease (RR = 1.67 [0.72-3.89]) increased but did not significantly. CONCLUSION: Cardiovascular events incidence is increased in patients with giant cell arteritis, and prescription of preventive antiagregant treatment may be discussed.

[Aetiological spectrum of hyperferritinemia]. / Spectre étiologique des hyperferritinémies.

UNLABELLED: Serum ferritin levels may be increased in many conditions: renal diseases, liver diseases, human immunodeficiency virus infection. The purpose of this study was to assess the aetiological spectrum of high serum ferritin levels in a 1200-bed university hospital, to compare our results with the data already published and to assess a potential association between aetiology and ferritin levels. PATIENTS AND METHODS: Patients with a serum ferritin level higher than 600 microg/l were retrospectively included between 15 November 2003 and 15 January 2004, and their medical records were reviewed. RESULTS: Ninety-eight patients (38 women and 60 men; median age: 59,5 years [19-92]) were recruited in departments of hepatology and gastroenterology (22%), haematology (14%) and internal medicine (18%). Diagnosis performed were: non-HIV systemic infections (23,8%), haematological diseases (16,1%), alcoholism (11,2%) and malignancies (9,8%). Dialysed chronic renal failure, liver diseases, haemochromatosis and systemic inflammatory diseases counted for 4.2 to 5.2% of cases. Serum ferritin level lied between 600 and 1000 microg/l for 50 patients, between 1000 and 1500 microg/l for 24, and over 1500 microg/l for 24. There was no significant difference between the three groups as regards the etiological distribution. DISCUSSION: In our study, chronic renal failure was not a major cause of high ferritin level: this is probably due to the current use of erythropoietin, which has decreased the use of blood transfusions. The two major aetiology of hyperferritinemia were non-HIV infections and malignancies.

[Fistulisation of a tubercular adenopathy in the oesophagus. About a paucisymptomatic form]. / Fistulisation d'une adénopathie tuberculeuse à l'oesophage. A propos d'une forme frustre.

The authors present the case of a cervico-mediastinal mass which disappeared spontaneously. The clinical presentation and the image of a fistula during the oesophagoscopy are in favour of the fistulisation of a tubercular adenopathy in the oesophagus. This pathology is rare. This observation is original by the delay of diagnosis due to a dysphagie summarizing the clinical signs and the spontaneously favorable evolution of the fistula.

Auxiliary partial orthotopic versus standard orthotopic whole liver transplantation for acute liver failure: a reappraisal from a single center by a case-control study.

OBJECTIVE: To reappraise the results of auxiliary partial orthotopic liver transplantation (APOLT) compared with those of standard whole-liver transplantation (OLT) in terms of postoperative death and complications, including neurologic sequelae. SUMMARY BACKGROUND DATA: Compared with OLT, APOLT preserves the possibility for the native liver to recover, and to stop immunosuppression. METHODS: In a consecutive series of 49 patients transplanted for fulminant or subfulminant hepatitis, 37 received OLT and 12 received APOLT. APOLT was done when logistics allowed simultaneous performance of graft preparation and the native liver partial hepatectomy to revascularize the graft as soon as possible. Each patient undergoing APOLT (12 patients) was matched to two patients undergoing OLT (24 patients) according to age, grade of coma, etiology, and fulminant or subfulminant type of hepatitis. All grafts in the study population were retrieved from optimal donors. RESULTS: Before surgery, both groups were comparable in all aspects. In-hospital death occurred in 4 of 12 patients undergoing APOLT compared with 6 of 24 patients undergoing OLT. Patients receiving APOLT had 1 +/- 1.3 technical complications compared with 0.3 +/- 0.5 for OLT patients. Bacteriemia was significantly more frequent after APOLT than after OLT. The need for retransplantation was significantly higher in the APOLT patients (3/12 vs. 0/24). Brain death from brain edema or neurologic sequelae was significantly more frequent after APOLT (4/12 vs. 2/24). One-year patient survival was comparable in both groups (66% vs. 66%), and there was a trend toward lower 1-year retransplantation-free survival rates in the APOLT group (39% vs. 66%). Only 2 of 12 (17%) patients had full success with APOLT (i.e., patient survival, liver regeneration, withdrawal of immunosuppression, and graft removal). One of these two patients had neurologic sequelae. CONCLUSIONS: Using optimal grafts, APOLT and OLT have similar patient survival rates. However, the complication rate is higher with APOLT. On an intent-to-treat basis, the efficacy of the APOLT procedure is low. This analysis suggests that the indications for an APOLT procedure should be reconsidered in the light of the risks of technical complications and neurologic sequelae.

[Adult to adult living-related liver transplantation. The Paul-Brousse Hospital preliminary experience]. / Transplantation hépatique de l'adulte à partir de donneur vivant.

AIM: Liver-graft shortages justify the development of adult living-related liver transplantation. The preliminary experience with this technique at Paul-Brousse Hospital is reported. PATIENTS ET METHODES: From January to July 2000, 7 adult to adult living-related liver transplantations were performed. Donors were 5 females and 2 males aged 20 to 53 years old (median: 41). A right liver graft was harvested in all cases. Recipients were 5 males and 2 females aged from 17 to 58 years old (median: 50) transplanted for viral cirrhosis (4 cases including 2 with hepatocellular carcinoma), subfulminant hepatitis (1 case), hepatocellular carcinoma on a healthy liver (1 case), and epithelioid hemangioendothelioma (1 case). Follow-up ranged from 41 to 157 days (median: 117 days). RESULTS: One donor had a biliary fistula that healed spontaneously. One donor had asterixis for 24 hours. The 7 donors are alive at home without any late complications. One recipient was retransplanted for hepatic artery thrombosis and 2 recipients had a biliary fistula that healed spontaneously. The 7 recipients are alive at home with normal liver function. CONCLUSION: Our experience and other reports suggest that adult to adult living-related liver transplantation is feasible with rare mortality and low morbidity in donors. Results in recipients are comparable to those obtained with cadaveric grafts. For a given patient the possibility of living related donation might extend the indications for transplantation without penalizing patients waiting for a cadaveric graft.

Neoadjuvant transjugular intrahepatic portosystemic shunt: a solution for extrahepatic abdominal operation in cirrhotic patients with severe portal hypertension.

BACKGROUND: Major abdominal surgery, although technically feasible per se, can be contraindicated in some cirrhotic patients because of severe portal hypertension. The present study reports our experience of seven such patients who were prepared for major abdominal surgery by transjugular intrahepatic portosystemic shunt (TIPS). STUDY DESIGN: There were seven cirrhotic patients (six men and one woman aged 47 to 69 years) with portal hypertension. Portal hypertension was considered severe because of the presence of at least one of the following: history of variceal bleeding (five of seven patients), varices at risk of bleeding (red signs or cardial location of varices; four of seven patients), or intractable ascites (three of seven patients). The planned operations included colon, gastroesophageal, kidney, and aortic procedures in three, two, one, and one patient, respectively. Because portal hypertension was the leading cause of surgical contraindication, the following "two-step strategy" was applied to the seven patients: first, TIPS to control portal hypertension, followed, after a delay of at least 1 month, by abdominal surgery. RESULTS: The TIPS procedure was successfully performed in all patients without complications. The hepatic venous pressure gradient decreased from 18+/-5 to 9+/-5 mm Hg (p<0.01). All patients were operated on with a delay ranging from 1 month to 5 months after TIPS (2.9+/-1.3 months; median 3 months). The planned operation was performed in six of the seven patients. One patient with cancer of the cardia did not have resection because of extensive abdominal spreading of the tumor. Intraoperative transfusion was necessary in only two patients. Operative mortality occurred in one patient, 36 days after resection of a left colon cancer. CONCLUSIONS: The minimally invasive nature of TIPS allows us to propose the following two-step management of cirrhotic patients with severe portal hypertension needing abdominal surgery: decompression of the portal system by TIPS followed by elective surgery.

[Pharyngeal Wegener's disease: a case report]. / A propos d'un cas de forme pharyngée de maladie de Wegener.

We report a case of Wegener's disease with an exceptional 10-year course before diagnosis subsequent to the development of severe ENT features. Mucosal ulcerations involving the mouth and the pharynx were highly extensive and resolved completely, but temporarily, with cyclophosphamide and corticosteroid therapy.

[Hepatic disorders related to Lyme disease. Study of two cases and a review of the literature]. / L'atteinte hépatique dans la maladie de Lyme. Etude de deux cas et revue de la littérature.

We report two cases of Lyme disease, revealed by hepatic damage in a 71- and a 59-year old man. In the first case, the disease was revealed by febrile jaundice whereas, in the second case, results of liver tests showed cytolytic and cholestatic abnormalities with fever. Lyme disease is a zoonosis due to infection by Borrelia burdorferi transmitted by ticks. The multiple phases of the disease explain the polymorphism of the clinical manifestations. Usually, extrahepatic symptoms are first observed, including neurological tropisms of Borrelia burdorferi. On the contrary, hepatic impairment due to Lyme disease is rare, often asymptomatic and with biological manifestations only.

Percutaneous portal vein embolization increases the feasibility and safety of major liver resection for hepatocellular carcinoma in injured liver.

OBJECTIVE: To assess the influence of preoperative portal vein embolization (PVE) on the long-term outcome of liver resection for hepatocellular carcinoma (HCC) in injured liver. SUMMARY BACKGROUND DATA: On an healthy liver, PVE of the liver to be resected induces hypertrophy of the remnant liver and increases the safety of hepatectomy. On injured liver, this effect is still debated. METHODS: During the study period, 10 patients underwent preoperative PVE and 19 patients did not before resection of three or more liver segments for HCC in injured liver (cirrhosis or fibrosis). PVE was performed when the estimated rate of remnant functional liver parenchyma (ERRFLP) assessed by computed tomographic scan volumetry was less than 40%. RESULTS: In all patients, PVE was feasible. There were no deaths or complications. The ERRFLP after PVE was significantly increased compared with the pre-PVE value. Liver resection was performed after PVE in 9 of 10 patients, with surgical death and complication rates of 0% and 45%, respectively. PVE increased the number of resections of three or more segments by 47% (9/19). Overall actuarial survival rates with or without previous PVE (89%, 67%, and 44% vs. 80%, 53%, and 53% at 1, 3 and 5 years, respectively) and disease-free actuarial survival rates (86%, 64%, and 21% vs. 55%, 17%, and 17% at 1, 3, and 5 years respectively) after hepatectomy were comparable. CONCLUSION: With the use of PVE, more patients with previously unresectable HCC in injured liver can benefit from resection. Long-term survival rates are comparable to those after resection without PVE.

Association of transjugular intrahepatic portosystemic shunt with embolization in the treatment of bleeding duodenal varix refractory to sclerotherapy.

BACKGROUND: Bleeding from duodenal varices are often severe (mortality as high as 40%), and more difficult to sclerose than esophageal varices. We report a patient with a bleeding duodenal varix, refractory to sclerotherapy, successfully treated by the association of portosystemic shunt placement and varix embolization, via the same transjugular intrahepatic route. METHODS: A 40-year-old Black male underwent emergency TIPS and duodenal varix embolization after failure of endoscopic sclerotherapy. The portosystemic pressure gradient droped from 16 to 9 mm Hg following TIPS. At 5 months from TIPS, the patient is well, with a patent shunt at Doppler ultrasound. CONCLUSION: The present report of successful control of duodenal varix, actively bleeding and refractory to sclerotherapy, by means of combined TIPS and embolization, supports the role of TIPS and suggests that its association to embolization can be valuably considered in the difficult setting of portal hypertension with bleeding duodenal varices.