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L Guillevin

Publications and source records attributed to L Guillevin.

At least 19 recordsLinked to original sources

Lack of superiority of steroids plus plasma exchange to steroids alone in the treatment of polyarteritis nodosa and Churg-Strauss syndrome. A prospective, randomized trial in 78 patients.

OBJECTIVE: To define the most effective treatment for polyarteritis nodosa (PAN) and Churg-Strauss syndrome (CSS). METHODS: We conducted a prospective, randomized, multicenter trial in which 78 patients were randomly assigned to receive either prednisone and plasma exchange (group A; n = 36) or prednisone alone (group B; n = 42) as first-line treatment of PAN and CSS. Patients with hepatitis B virus-related PAN were not included in this study. The end point of the study was control of the disease (recovery and remission) or death. RESULTS: Clinical symptoms and laboratory findings did not differ statistically in the 2 groups at study entry. Initial control of the disease was similar in both groups. The assigned treatment was stopped in 16 patients because of lack of efficacy. Oral cyclophosphamide or dapsone therapy reversed the disease evolution in 7 of these 10 group A patients and in 4 of these 6 group B patients. At 7 years of followup, 56 patients had completely recovered (27 in group A, 29 in group B), 7 patients were in clinical remission, and 15 patients had died (19.2%; 6 group A patients and 9 group B patients). The prednisone-plasma exchange combination was no more beneficial than corticosteroids alone in preventing relapses over the long term. There was no significant difference in the 7-year cumulative survival rates of the two groups (83% and 79%, respectively). CONCLUSION: Based on our data, we conclude that combined treatment with prednisone and plasma exchange is not superior to treatment with prednisone alone and must not be systematically employed for initial treatment of PAN and CSS. In most cases, cyclophosphamide as second-line treatment is effective and well tolerated.

Adult

Pharmacokinetics of vidarabine in the treatment of polyarteritis nodosa.

The pharmacokinetics of vidarabine were studied in 8 patients with polyarteritis nodosa related to hepatitis B virus infection. The drug was administered by continuous infusion for three weeks at doses of 15 (1 week) and 7.5 (2 weeks) mg/kg per day, during which time 15 plasma exchanges were performed. Plasma was assayed for vidarabine and its principal metabolite, hypoxanthine arabinoside by high pressure liquid chromatography. Vidarabine was not detected in the plasma of any patients. Hypoxanthine arabinoside levels were used to evaluate vidarabine kinetics. The serum levels of hypoxanthine arabinoside ranged from 3.6 to 21.5 mg/l. The mean elimination half-life (+/- SD) was 3.0 +/- 1.7 h. The plasma clearance (mean +/- SD) was 195 +/- 270 ml/min when the dose was 7.5 mg/kg per day and 66.3 +/- 47 ml/min for a 15 mg/kg per day/dose (NS). Except for the elimination half-life, these results were not fully consistent with those observed in other studies. The influence of multiple plasma exchanges on vidarabine kinetics is limited and dosage adjustment is not required based on the continuous infusion of vidarabine.

Humans

Giant cell arteritis of the female genital tract with temporal arteritis.

The clinical and pathological features of a patient with giant cell arteritis of the uterus and ovaries are described. A 61 year old woman had fever and weight loss over a period of eight months. A hysterectomy with bilateral salpingo-oophorectomy was performed for a large cystic ovarian mass. Histological examination showed a benign ovarian cyst and unexpected giant cell arteritis affecting numerous small to medium sized arteries in the ovaries and myometrium. The diagnosis of temporal arteritis was confirmed by a random temporal artery biopsy, despite the absence of symptoms of temporal arteritis. This observation is compared with previously reported cases and the relation between granulomatous arteritis of the genital tract and temporal arteritis is discussed. The main differential diagnosis in this localisation was represented by Wegener's granulomatosis and periarteritis nodosa.

Female

AIDS and the brain.

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Acquired Immunodeficiency Syndrome

Treatment of polyarteritis nodosa and Churg-Strauss syndrome. A meta-analysis of 3 prospective controlled trials including 182 patients over 12 years.

To define the most effective treatment for polyarteritis nodosa (PAN) and Churg-Strauss syndrome (CSS), we undertook 3 consecutive prospective therapeutic trials including 182 patients and tried to answer several important questions: should cyclophosphamide (CYC) be given as the first-line treatment? what is the place of plasma exchanges (PE) in the treatment of systemic vasculitis? does hepatitis B virus (HBV)-related PAN require specific treatment? Our first randomized trial in 71 patients compared the association of CYC with corticosteroids (CS) and PE to CS and PE, in order to evaluate the efficacy of CYC given as the first-line treatment to control disease activity and subsequent survival of PAN and CSS patients. Between December, 1983, and December, 1988, we conducted two trials simultaneously: one aimed at patients without HBV markers and the second at patients with HBV markers. In 78 patients without HBV markers, we compared prednisone and PE to prednisone alone as the initial therapeutic regimen. In 33 patients with PAN related to HBV, a new therapeutic strategy was applied as an alternative to long-term steroid and immunosuppressive therapy: short-term steroid therapy and PE were used to control the evolution of PAN and anti-viral therapy was administered to suppress the etiological agent of the vasculitis. Twelve years after the beginning of the trials on PAN and CSS patients, we think that the therapeutic strategy should be as follow: in PAN without HBV and CSS: Prednisone in association with CYC improves the control of the disease despite infectious side effects which may be reduced by better CYC dose adaptation. It is also possible that CYC could be more effective in some subgroups of PAN, for instance those with clinical symptoms of poor prognosis. We are presently attempting to optimize the CYC prescription (pulses of CYC) in PAN and CSS in the hope of improving prognosis; in PAN related to HBV: The first-line treatment should be the association of anti-viral agents and PE. This treatment was effective and cured a majority of patients within 2 to 3 months; half of them seroconverted. The length of HBV infection before its diagnosis, delay before initiation of treatment and previous immunosuppressive therapy led to a poor seroconversion rate; the role of PE in the treatment of systemic necrotizing angiitis: PE are obviously useful in PAN related to HBV where immune complex deposition has been demonstrated.(ABSTRACT TRUNCATED AT 400 WORDS)

Adolescent

Polyarteritis nodosa related to hepatitis B virus. A retrospective study of 66 patients.

In an attempt to establish the characteristics, circumstances leading to infection and development of polyarteritis nodosa (PN) related to hepatitis B virus (HBV), prognostic factors and outcome, and to define the most effective treatment, 66 patients observed between 1972 and 1989 were analyzed. Hepatitis was clinically present in 19/66 patients before PN. In most cases, PN occurred less than 6 months after infection. Clinical manifestations of PN were comparable to those observed in patients without HBV infection except for orchitis which was present in 13.6% and for pulmonary signs which were absent. Transaminases were normal in 38 cases for SGOT and 31 for SGPT and twice the normal range or more in the other cases. Antineutrophil cytoplasmic antibodies (ANCA) were tested in 22 patients and present in 2 (9%). Twenty-eight patients were treated with prednisone +/- oral cyclophosphamide +/- plasma exchanges. Thirty-eight patients were given a short-term treatment with prednisone followed by the association of vidarabine, 15 mg/kg bw/d for one week and 7.5 mg/kg bw/d for 2 weeks, and plasma exchanges: 14 sessions during the 3 weeks of vidarabine infusion, then tapered until stopping treatment after 2 to 3 months depending upon the clinical results obtained. The mean duration of follow-up was 50.3 +/- 46.1 months. At the end of follow-up, 13 of the 28 patients (46.4%) treated with steroids +/- cyclophosphamide +/- plasma exchanges died and 7/38 (18.4%) of those treated with vidarabine and plasma exchanges (p < 0.001) died. HBe/anti-HBe seroconversion was observed in 2 patients treated with prednisone +/- cyclophosphamide +/- plasma exchanges who were alive at the time of final analysis and in 16 patients receiving the other regimen. The outcome of patients treated with a few days of prednisone, vidarabine and plasma exchange was good and, therefore, we propose this protocol as the first viable treatment for polyarteritis nodosa related to HBV, surpassing the conventional treatment with steroids and cyclophosphamide, which stimulates viral replication.

Adult

[Acute renal insufficiency associated with lymphoma. A new case].

We report on a case of acute renal failure secondary to relapse of a centrocytocentroblastic non-Hodgkin's lymphoma. Twenty-seven cases of acute renal failure due to lymphomatous infiltration of the kidneys have been so far reported. The clinical presentation is non-specific; the size of the kidneys appears normal or enlarged; diagnosis is confirmed by renal biopsy and/or CT scan examination. Chemotherapy and/or radiotherapy sometimes lead to dramatic improvement of renal function. However, prognosis is poor, mostly due to lymphomatous evolution.

Acute Kidney Injury

Plasmapheresis and subsequent pulse cyclophosphamide versus pulse cyclophosphamide alone in severe lupus: design of the LPSG trial. Lupus Plasmapheresis Study Group (LPSG).

A group of clinics are collaborating in the Lupus Plasmapheresis Study Group (LPSG) to investigate whether repeated plasmapheresis prior to pulse cyclophosphamide improves the therapeutical results in severe systemic lupus erythematosus (SLE). The underlying rationale is the hypothesis that plasmapheresis 1) eliminates pathogenic autoantibodies and immune complexes and 2) induces compensatory lymphocyte activation via feedback mechanisms between circulating antibodies and their respective clones ("antibody rebound"). It should be possible to utilize this enhanced activity for increased clonal deletion if pulse cyclophosphamide is applied shortly after plasmapheresis. Accordingly, in a randomized study, the LPSG will be comparing the repeated application of pulse cyclophosphamide alone with a treatment involving repeated plasmapheresis prior to the cyclophosphamide pulses in severe SLE. A third arm of the study will be gathering experience with a more intensified procedure. This overview summarizes the most important details of the planned study.

Combined Modality Therapy

Zidovudine removal during plasma exchange.

Pharmacokinetics of zidovudine (azidothymidine, AZT) were investigated after oral administration (300 mg every 8 hours) in a human immunodeficiency virus seropositive patient who was also treated with plasma exchange (60 ml/kg). Plasma AZT concentrations were measured using high performance liquid chromatography assay. Plasma exchange clearances reached 1-3.8% of the total clearance. The fraction of AZT removed from separated plasma accounted for only 1% of the administered dose. The contribution of plasma exchange to the elimination of AZT appears to be negligible. Solely on the basis of AZT pharmacokinetic data, no particular dose adjustment appears to be necessary in patients treated with multiple plasma exchanges.

Adult

Churg-Strauss angiitis. Arguments favoring the responsibility of inhaled antigens.

A 27-year-old man presented with relapsing vascular limb purpura, pulmonary infiltrates and cranial nerve palsies occurring after exposure to pigeons. A lung biopsy specimen showed Churg-Strauss angiitis. Actinomycetes were observed in pneumocytes. The circumstances preceding clinical manifestations and pathologic findings favored the diagnosis of pulmonary vasculitis as a consequence of inhaled antigen. Recovery was obtained after treatment with prednisone, cyclophosphamide, and plasma exchanges.

Adult

Longterm followup after treatment of polyarteritis nodosa and Churg-Strauss angiitis with comparison of steroids, plasma exchange and cyclophosphamide to steroids and plasma exchange. A prospective randomized trial of 71 patients. The Cooperative Study Group for Polyarteritis Nodosa.

We attempted to define the most effective treatment for polyarteritis nodosa and Churg-Strauss angiitis, with a prospective, randomized, multicenter trial of cyclophosphamide in conjunction with corticosteroids and plasma exchanges, compared to corticosteroids and plasma exchanges. A total of 71 patients who fulfilled clinical, histological and/or arteriographic diagnostic criteria were randomly designated to receive either prednisone and plasma exchanges (group A, n = 39) or cyclophosphamide, prednisone and plasma exchanges (group B, n = 32). The end points of the study were control of the disease (recovery and remission) and death. Upon study entry clinical and laboratory features did not differ in the 2 groups. Treatment was stopped in 19 patients because of ineffectiveness in 10 (9 in Group A) and side effects in 9 (8 in Group B). Initial control of the disease was similar in both groups. At 5 years, 27 patients had completely recovered and 14 patients were in clinical remission. The cyclophosphamide-prednisone-plasma exchange association was beneficial in preventing relapses during longterm followup. Nineteen deaths were reported during the followup period. There was no difference between the 10 year cumulative survival rates of the 2 groups (respectively, 72 and 75%). Thus, the association of cyclophosphamide with corticosteroids and plasma exchanges reduced the incidence of relapses and improved the quality of the clinical response to therapy.

Adolescent

[Vasculitis and neoplasms. 14 cases].

Fourteen cases of vasculitis associated with a neoplasm are reported. The vasculitides were classified as: leukocytolytic vasculitis 7, periarteritis nodosa 4, purpura rheumatica 1, cutaneous granulomatous vasculitis 1, microvasculitis nervosa 1. The neoplasms were diagnosed as: 10 hemopathies (including 4 cases of refractory anemia) and 5 solid tumors (1 patient had 2 tumors). The cutaneous manifestations (purpura, papules, subcutaneous nodules, etc.) and fever were the most common, while joint and neurological involvement were the rarest. The evolution of the vasculitis was usually corticosensitive and independent of the underlying neoplasm. A review of the literature revealed that these vasculitides (primarily leukocytolytic) were frequently associated with dysplastic myelogenous syndromes and hairy cell leukemia, while bronchopulmonary and colonic neoplasms were the most common solid tumors. The mechanisms giving rise to these vasculitides are discussed.

Aged