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Biomedical subjects

B Jarrousse

Publications and source records attributed to B Jarrousse.

At least 19 recordsLinked to original sources

Characteristics and outcome of 49 patients with symptomatic cryoglobulinaemia.

OBJECTIVE: To describe a population of patients with symptomatic cryoglobulinaemia, comparing manifestations and outcome as a function of hepatitis C virus (HCV) status. PATIENTS AND METHODS: A retrospective study on 179 patients who tested positive for cryoglobulins, seen between 1978 and 1998 in an internal medicine department. RESULTS: Among 179 cryoglobulin-positive patients, only 49 (18 men, 31 women; mean age 59.96+/-12 yr) had clinical manifestations attributable to cryoglobulinaemia. Thirty-three had HCV infection, 20 had systemic autoimmune diseases, two had haematological diseases, one had human immunodeficiency virus and HCV co-infection, one had HCV and HBV co-infection and six had essential mixed cryoglobulinaemia. The clinical manifestations and cryoglobulin levels in HCV(+) and HCV(-) patients did not differ significantly. Only arthralgias and elevated transaminases were significantly more frequent in HCV(+) patients (P<0.02 and <0.05, respectively). Five-year survival rates were comparable for HCV(+) and HCV(-) patients. Eight patients died (six HCV(+), two HCV(-)), with a median time between diagnosis and death of 38.7 months. CONCLUSION: Clinical manifestations of cryoglobulinaemia, except arthralgias, were comparable for HCV(+) and HCV(-) patients. When systemic manifestations are present, the prognosis is poor despite intensive or prolonged therapy.

Cryoglobulinemia↗

Long-term followup of polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome: analysis of four prospective trials including 278 patients.

OBJECTIVE: To determine the long-term outcome of patients with polyarteritis nodosa (PAN), microscopic polyangiitis (MPA), and Churg-Strauss syndrome (CSS), to compare the long-term outcome with the overall French population, to evaluate the impact on outcome of the type of vasculitis, prognostic factors, and treatments administered at diagnosis, and to analyze treatment side effects and sequelae. METHODS: Data from PAN, MPA, and CSS patients (n = 278) who were enrolled between 1980 and 1993 were collected in 1996 and 1997 and analyzed. Two prognostic scoring systems, the Five-Factors Score (FFS) and the Birmingham Vasculitis Activity Score (BVAS), were used to evaluate all patients at the time of diagnosis. RESULTS: The mean (+/- SD) followup of the entire population was 88.3 +/- 51.9 months (range 3 days to 192 months). Of the 85 deaths recorded, at least 41 were due to progressive vasculitis or its consequences. Death rates reflected disease severity, as assessed by the FFS (P = 0.004) and the BVAS (P < 0.0002), and the 2 scores were correlated (r = 0.69). Relapses, rarer in hepatitis B virus (HBV)-related PAN (7.9%) than in MPA (34.5%) (P = 0.004), occurred in 56 patients (20.1%) and did not reflect disease severity. Survival curves were similar for the subpopulation of 215 patients with CSS, MPA, and non-HBV-related PAN who were given first-line corticosteroids (CS) with or without cyclophosphamide (CYC). However, CS with CYC therapy significantly prolonged survival for patients with FFS scores > or =2 (P = 0.041). Relapse rates were similar regardless of the treatment regimen; only patients treated with CS alone had uncontrolled disease. CYC was associated with a greater frequency of side effects (P < 0.00001). CONCLUSION: Rates of mortality due to PAN (related or unrelated to HBV), MPA, and CSS reflected disease severity and were higher than the mortality rate in the general population (P < 0.0004). Rates of relapse, more common in MPA than HBV-related PAN patients, did not reflect disease severity. Survival rates were better among the more severely ill patients who had received first-line CYC. Based on these findings, we recommend that the intensity of the initial treatment be consistent with the severity of the disease. The use of the FFS and BVAS scores improved the ability to evaluate the therapeutic response.

Adult↗

Microscopic polyangiitis: clinical and laboratory findings in eighty-five patients.

OBJECTIVE: To retrospectively analyze the clinical symptoms, laboratory findings, and outcomes in patients with microscopic polyangiitis (MPA) who were enrolled in various clinical trials conducted by the French Vasculitis Study Group. METHODS: A cohort of 85 patients meeting the Chapel Hill criteria for MPA participated in the study. Seventy-one of them were included in prospective therapeutic trials. Eighty-one diagnoses were biopsy proven. In the other patients, diagnosis was based on clinical findings. RESULTS: Forty-seven men and 38 women, with a mean +/- SD age of 56.8 +/- 14.6 years, met the criteria for MPA. Their main clinical symptoms were renal manifestations (78.8%), weight loss (72.9%), skin involvement (62.4%), fever (55.3%), mononeuritis multiplex (57.6%), arthralgias (50.6%), myalgias (48.2%), hypertension (34.1%), lung involvement (24.7%; alveolar hemorrhage 11.8%), and cardiac failure (17.6%). The mean +/- SD serum creatinine level before treatment was 2.59 +/- 2.96 mg/dl; 47 patients had renal insufficiency (serum creatinine > 1.36 mg/dl). Eight patients underwent dialysis at the time of diagnosis, and long-term dialysis was necessary for 10 patients. Antineutrophil cytoplasmic antibodies (ANCA) were present in 38 of 51 patients (74.5%), of whom 33 had a perinuclear staining pattern (pANCA) and 5 had a cytoplasmic pattern. Antibodies to proteinase 3 were present in 4 patients and antibodies to myeloperoxidase were detected in 31, as determined by enzyme-linked immunosorbent assay. Of the 30 patients who underwent renal and celiac angiography, 4 had microaneurysms. Of the 29 patients (34.1%) who had relapses, 8 died during or after the relapse. During followup, 28 of the 85 patients (32.9%) died. The mean +/- SD duration of followup of the group was 69.9 +/- 60.6 months. Deaths were less frequent when patients had been treated with steroids and immunosuppressive drugs (13 patients [24.1%]) than with steroids alone (15 patients [48.4%]) (P < 0.01). The 5-year survival rate was 74%. CONCLUSION: This study demonstrated that MPA is a multisystemic disease in which renal symptoms are frequent, but the disease is also associated with general symptoms, arthritis, mononeuritis multiplex, and other manifestations that are also seen in various vasculitides. The rarity of abnormal angiogram findings and the high frequency of pANCA are characteristic of MPA. In most cases, the outcome is comparable with those of other systemic vasculitides, but relapses are frequent.

Abdominal Pain↗

Churg-Strauss syndrome. Clinical study and long-term follow-up of 96 patients.

Churg-Strauss syndrome (CSS) is a systemic vasculitis characterized by the presence of asthma, hypereosinophilia, and necrotizing vasculitis with extravascular eosinophil granulomas. In this retrospective study of 96 patients between 1963 and 1995, we analyzed clinical manifestations, identified prognostic factors, and assessed the long-term outcome. CSS was diagnosed when asthma, hypereosinophilia > 1,500/mm3 or > 10%, and clinical manifestations consistent with systemic vasculitis, with or without histologic evidence, were present. Asthma was the most frequently observed manifestation at presentation, with mononeuritis multiplex the second. Other common manifestations were weight loss, fever, myalgia, skin involvement, paranasal sinusitis, arthralgia, pulmonary infiltrate, and gastrointestinal involvement. Mean eosinophilia at presentation was 7.193 +/- 6.706/mm3; ANCA, present in 20 of 42 (47.6%) patients, predominantly gave the perinuclear labeling pattern. All the patients were treated with corticosteroids alone or in combination with cyclophosphamide or plasma exchanges. Clinical remission was obtained in 91.5%; 22 (25.6%) patients relapsed. Twenty-three patients died during follow-up: 11 of these deaths were directly due to vasculitis. The presence of severe gastrointestinal tract or myocardial involvement was significantly associated with a poor clinical outcome. The long-term prognosis of CSS is good and does not differ from that of polyarteritis nodosa, although most patients need low doses of oral corticosteroids for persistent asthma, even many years after clinical recovery from vasculitis.

Adolescent↗

Candidemia: a nosocomial complication in adults with late-stage AIDS.

We retrospectively analyzed 13 episodes of candidemia observed between July 1990 and July 1995 in human immunodeficiency virus (HIV)-infected adults. Candidemia was nosocomially acquired by 11 patients, among whom nine had a central venous catheter (CVC). Twelve cases were of stage C2/C3 according to the 1993 classification of the Centers for Disease Control and Prevention. The median CD4+ cell count was 10/mm3 (range, 3-400/mm3). Causative species were Candida albicans in nine episodes and Candida glabrata and Candida krusei in two episodes each. Eleven episodes occurred in 11 patients who had previously received fluconazole (mean total dose, 7.4 g), including the four episodes caused by non-albicans species. Outcome did not differ according to the administered antifungal therapy. CVCs were removed from seven patients (78%). The overall mortality was 38%. Candidemia is a potentially lethal nosocomial complication during late-stage AIDS and can be due to C. albicans and non-albicans strains.

AIDS-Related Opportunistic Infections↗

Human immunodeficiency virus-related vasculitis. Clinical presentation of and therapeutic approach to eight cases.

Vasculitides occurring during the course of human immunodeficiency virus (HIV) infection are heterogeneous in their clinical presentation, time of occurrence, histological findings and etiological factors. Some of them can be directly attributed to HIV infection and their treatment might require new therapeutic approaches, such as plasma exchanges, specific antiviral agents and/or vasodilators. We report 8 cases of vasculitis associated with HIV. Five of them met the classification criteria of polyarteritis nodosa and had symptoms compatible with microscopic polyangiitis. Etiological agents were not identified. Although hepatitis B virus, hepatitis C virus (HCV) and other viruses or opportunistic agents were detected in some patients, HCV was suspected of being the etiological factor in only 1 of them. Focusing on viral and immunological features, the possible mechanisms of vasculitis development in HIV+ patients are discussed. Long-term follow-up indicates a clear benefit of regimens including antiviral agents, vasodilators and plasma exchanges. No deterioration of AIDS status was observed with this treatment and no relapse was recorded.

Adult↗

[Prevention of opportunistic infections in HIV seropositive patients. Prevention of parasitic infections].

PNEUMOCYSTIS CARINII PNEUMONIA: Below a threshold 200 CD4 cont, the risk of P. carinii infection is sufficiently high to propose systematic prophylaxis. Trimethoprim-sulfamethoxazole is the treatment of choice for primary and secondary prevention. Pentamidine-isothionate can also be used, possibly with dapsone. TOXOPLASMOSIS: There is a consensus on primary prevention in patients with positive toxoplasmosis serology whose CD4 count is under 100. Trimethoprime-sulfamethoxazole is the first intention drug, but poor tolerance may require conversion to dapsone-pyrimethamine. For secondary prophylaxis, the consensus is for life-long treatment with low-doses after initiating treatment with sulfadiazine and pyrimethamine. LEISHMANIASIS: Secondary prevention is reasonable using monthly infusions of pentamidine.

AIDS-Related Opportunistic Infections↗

[Prevention of opportunistic infections in HIV seropositive patients. Prevention of bacterial infections].

TUBERCULOSIS: Primary prophylaxis is indicated in subjects at risks, i.e. those with a positive tuberculine test or a negative test and major immunodepression. BCG vaccine is definitely contraindicated in case of AIDS. Prescription of isoniazid in combination with vitamin B6 has been found to be effective. MYCOBACTERIUM AVIUM INTRACELLULARE: Prophylaxis concerns highly immunodepressed patients and is based on rifabutine. Prophylaxis is contraindicated in case of active tuberculosis. OTHER GERMS: Preventive care in food intake for potential digestive tract infections is required as well as antipneumococcal vaccine. Secondary prophylaxis is based on continuous antibiotics. The positive effect of intravenous immunoglobulins, especially in children, remains a question of debate.

AIDS-Related Opportunistic Infections↗

[Prevention of opportunistic infections in HIV seropositive patients. Prevention of viral infections and mycoses].

CYTOMEGALOVIRUS INFECTION: Primary prophylaxis is logical in high-risk subjects (CD4 < 50) who are asymptomatic; per os ganciclovir is under evaluation. Systematic secondary prophylaxis relies on intravenous ganciclovir or foscarnet. HERPES SIMPLEX AND ZOSTER: Secondary prevention with acyclovir is not recommended due to the risk of resistance. Herpes zoster retinitis is an absolute indication for continuous oral treatment with acyclovir. CRYPTOCOCCUS: Oral fluconazol is required for secondary prophylaxis. CANDIDIASIS: Due to the risk of resistant strains, neither primary nor secondary prophylasis is recommended. HISTIOPLASMOSIS: Risk of recurrence justifies secondary prophylaxis with itraconazole or fluconazole. COCCIDIODOMYCOSIS: Secondary prophylaxis with amphotericin B or fluconazole is mandatory. ASPERGILLOSIS: Itraconazole is the best agent when prophylaxis is needed.

AIDS-Related Opportunistic Infections↗

A prospective, multicenter, randomized trial comparing steroids and pulse cyclophosphamide versus steroids and oral cyclophosphamide in the treatment of generalized Wegener's granulomatosis.

OBJECTIVE: To investigate the effectiveness and side effects of oral versus pulse cyclophosphamide (CYC) in combination with corticosteroids (CS) in the treatment of systemic Wegener's granulomatosis (WG). METHODS: Patients with newly diagnosed systemic WG were enrolled in a prospective, randomized trial. At the time of diagnosis, prior to randomization, every patient received a daily injection of methylprednisolone for 3 days, followed by daily oral prednisone (1 mg/kg/day) and a 0.7-gm/m2 pulse of CYC. Patients were then randomly assigned to receive either prednisone plus intravenous pulse CYC (group A) or prednisone plus oral CYC (group B) as first-line treatment. CYC was given for at least 1 year and was then progressively tapered and discontinued. RESULTS: Fifty patients were included in the study: 27 in group A and 23 in group B. At 6 months, 24 group A patients (88.9%) were in remission, versus 18 group B patients (78.3%). At the end of the trial, 18 group A patients (66.7%) and 13 group B patients (56.5%) were in remission. In group A, 66.7% of the patients experienced side effects, versus 69.6% in group B. Infectious side effects were significantly more frequent in group B (69.6%) than in group A (40.7%) (P < 0.05). The incidence of Pneumocystis carinii pneumonia was higher in oral CYC-treated patients (30.4%) than in pulse CYC-treated patients (11.1%). Nine group A patients (33.3%) and 10 group B patients (43.5%) died. Actuarial curves showed that relapses were significantly more frequent in group A (59.2%) than in group B (13%) (P = 0.02). CONCLUSION: Our results indicate that pulse CYC is as effective as oral CYC in achieving initial remission of WG and is associated with fewer side effects and lower mortality. However, in the long term, treatment with pulse CYC does not maintain remission or prevent relapses as well as oral CYC.

Administration, Oral↗

Treatment of glomerulonephritis in microscopic polyangiitis and Churg-Strauss syndrome. Indications of plasma exchanges, Meta-analysis of 2 randomized studies on 140 patients, 32 with glomerulonephritis.

UNLABELLED: Although plasma exchanges (PE) have no added benefit in the treatment of vasculitides of the polyarteritis nodosa (PAN) group with steroids (CS) +/- cyclophosphamide (CY), this has not been demonstrated in patients presenting with glomerulonephritis (GN). We therefore reanalyzed the records of microscopic polyangiitis (MPA) or Churg-Strauss syndrome (CSS) patients presenting with GN. PATIENTS AND METHODS: Patients were included consecutively in 2 randomized trials: a) comparing CS vs CS + PE (n = 78) and b) comparing CS + pulse CY +/- PE in PAN and CSS with factors of poor prognosis (n = 62); 9-12 PE/patient were performed. RESULTS: 32 patients, 18 men and 14 women, presented with GN, 28 MPA and 4 CSS, mean age 53.2 +/- 17 years. Clinical/biological manifestations before treatment were comparable in both groups: weight loss 84.4%, fever 62.5%, mononeuritis multiplex 62.5%, purpura 28.1%, GI tract involvement 43.8%, arthritis 37.5%, asthma 12.5%, CNS manifestations 9.4%; cardiac involvement 9.4%; mean creatininemia was 303 +/- 286 mumol/l, proteinuria > 0.5 g/l or 1g/d was found in every case, microscopic hematuria in 20/32 patients, leukocyturia in 12/32. Eight out of/16 were ANCA-positive, ELISA detected anti-MPO antibodies in 5 and anti-PR3 in 3. HBV infection was never observed. After 1 year of treatment, creatininemia decreased from 374.4 +/- 352 to 290 +/- 352 mumol/l in the PE group and from 287 +/- 292 to 170 +/- 67 in the non PE group (NS). Six patients of the PE group and 2 of the non-PE group were dialyzed at onset of treatment. Four of the 6 PE patients and 1 of the 2 not treated with PE were off dialysis 1 year later. In addition 1 patient from the PE group developed a flare with renal failure and required chronic dialysis. The 5-year survival was higher in the PE group (4 deaths/19) than in the non PE group (7/13). The survival curve was 74% in the PE group vs 54% in the non-PE group (NS). CONCLUSION: This study confirms that PE have no added benefit in the treatment of GN in MPA and CSS.

Adolescent↗

Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients.

We undertook this study to determine the clinical, biologic, immunologic, and therapeutic factors associated with the prognoses of polyarteritis nodosa (PAN) and Churg-Strauss syndrome (CSS). Three hundred forty-two patients (260 with PAN, 82 with CSS) followed from 1980 to 1993 were included in a prospective study on prognostic factors. Two hundred eighty-eight of these patients were included in the prospective studies on PAN and CSS. Items to be considered for analysis were collected at the time of diagnosis, during the acute phase of the disease. A survival curve was plotted for each clinical and biologic symptom observed in PAN or CSS. Each treatment arm of the prospective therapeutic trials was also tested: 1) prednisone (CS) + oral cyclophosphamide (CYC) + plasma exchanges (PE) versus CS E, 2) CS + PE versus CS, 3) CS + oral CY versus CS + pulse CY, 4) CS + pulse CY + PE versus CS + pulse CY in severe PAN and CSS, and 5) PE + antiviral agents after short-term CS in hepatitis B virus-related PAN. Of the parameters thus evaluated, the following had significant prognostic value and were responsible for higher mortality: proteinuria > 1 g/d (p < 0.0001; relative risk [RR] 3.6), renal insufficiency with serum creatinine > 1.58 mg/DL (p < 0.02; RR 1.86), GI tract involvement (p < 0.008. RR 2.83 for surgery). Cardiomyopathy and CNS involvement were associated with a RR of mortality of 2.18 and 1.76, respectively; these were not statistically significant. Similar survival rates were obtained with the prospectively tested therapies. The five-factors score (FFS) we established considered the prognostic factors creatinemia, proteinuria, cardiomyopathy, GI tract involvement, and CNS signs. Multivariate analysis showed that proteinuria (due to vascular or glomerular disease) and GI tract involvement were independent prognostic factors. When FFS = 0 (none of the 5 prognostic factors present), mortality at 5 years was 11.9%; when FFS = 1 (1 of the 5 factors present), mortality was 25.9% (p < 0.005); when FFS > 2 (3 or more of the 5 factors present), mortality was 45.95% (p < 0.0001 between 0 and 2, p < 0.05 between 1 and 2). We conclude that an initial assessment of PAN or CSS severity enables outcome and mortality to be predicted. The FFS is a good predictor of death and can be used to help the clinician choose the most adequate treatment. Renal and GI signs are the most serious prognostic factors.

Adolescent↗

Treatment of mixed cryoglobulinemia with recombinant interferon alpha and adjuvant therapies. A prospective study on 20 patients.

In order to evaluate the efficacy of interferon alpha (IFNa) in mixed cryoglobulinemia (MC), a prospective multicenter clinical trial was conduced in April 1992. It consisted of treating 20 clinically symptomatic MC patients with IFNa for 26 weeks. Hepatitis C virus (HCV) infection was detected in 16 patients. A complete or partial clinical remission was obtained in 12 patients (60%). Eleven of these 12 responders (91.6%) experienced a clinical relapse less than 12 months after the end of therapy. Side effects were noted in 10 patients (50%). It was concluded that subcutaneously administered IFNa does not provide long-term remission.

Adrenal Cortex Hormones↗

Corticosteroids plus pulse cyclophosphamide and plasma exchanges versus corticosteroids plus pulse cyclophosphamide alone in the treatment of polyarteritis nodosa and Churg-Strauss syndrome patients with factors predicting poor prognosis. A prospective, randomized trial in sixty-two patients.

OBJECTIVE: To define the most effective treatment for severe polyarteritis nodosa (PAN) and Churg-Strauss syndrome (CSS) and to investigate the indication for plasma exchange treatment. METHODS: We conducted a prospective, randomized, multicenter trial in which 62 patients were randomly assigned to receive either prednisone plus cyclophosphamide (intravenous bolus) (group A; n = 28) or prednisone plus cyclophosphamide (intravenous bolus) plus plasma exchanges (group B; n = 34) as first-line treatment for severe PAN or CSS. Factors predicting poor prognosis were renal symptoms, gastrointestinal tract involvement, cardiomyopathy, central nervous system involvement, weight loss > 10% of body weight, and age > 50 years old. 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 or remission) or death. RESULTS: Clinical symptoms and laboratory findings did not differ significantly in the 2 groups. Initial control of the disease was similar in both groups. Relapse after initial control of the disease was observed in 7 patients (4 in group A and 3 in group B). The mean +/- SD followup period was 31.1 +/- 20 months for group A and 35.9 +/- 16.8 months for group B. At 5 years of followup, 38 patients (61.3%) were cured (16 in group A and 22 in group B), and 5 (8.1%) were in remission without treatment but had not yet completed the cure-defining period of 18 months (3 in group A and 2 in group B). Eight (12.9%) (2 in group A and 2 in group B) were considered to be in clinical remission and required a maintenance regimen of low-dose corticosteroids. Eleven patients died during the study period (7 in group A [25%], 4 in group B [11.8%]). Uncontrolled vasculitis was responsible for 4 deaths (2 in each group), and treatment side effects caused the death of 1 patient in group A. There was no significant difference between the 5-year cumulative survival rates of the 2 groups (75% and 88%, respectively). CONCLUSION: Based on our data, combined treatment with prednisone, cyclophosphamide, and plasma exchanges is not superior to treatment with prednisone and cyclophosphamide alone, and plasma exchanges should not be systematically proposed for initial treatment of severe PAN or CSS.

Adolescent↗

Polyarteritis nodosa related to hepatitis B virus. A prospective study with long-term observation of 41 patients.

Hepatitis B virus (HBV)-related polyarteritis nodosa (PAN) is a rare disease whose frequency has been decreasing over the past 10 years. We evaluated 41 patients with HBV-related PAN to determine the circumstances leading to infection, the clinical features of vasculitis, the prognostic factors, and the response to therapy. Most patients were first treated briefly with corticosteroids, and all were included in 2 nonrandomized prospective therapeutic trials of an antiviral agent (35 patients with vidarabine, 6 patients with interferon-alpha 2b) and plasma exchanges. The mean duration of follow-up was 69.6 +/- 44.8 months. At the end of the study, 21 (51.2%) patients had seroconverted to anti-HBeAb and 10 (24.4%) also had seroconverted to anti-HBsAb. In all, 23 (56%) patients no longer expressed serologic evidence of HBV replication. All 33 (80.5%) patients still alive at the end of follow-up recovered from PAN. Nineteen also recovered from HBV infection and were considered to be cured; 13 patients had persistent HBV infection and were considered to be in clinical recovery; and 1 patient was in remission, maintained with steroid therapy. Eight patients died during the study period; 3 deaths were directly attributable to PAN. HBV-related PAN is an acute disease, occurring shortly after infection and sharing the characteristics of classic PAN. It is not an antineutrophil cytoplasm antibodies (ANCA)-mediated vasculitis. The outcome was good for patients treated with short-term steroid therapy, antiviral agents, and plasma exchanges. We propose this protocol as the first treatment for HBV-related PAN, because it surpasses the conventional treatment with corticosteroids and cyclophosphamide, which facilitates viral replication and the development of chronic HBV infection.

Adolescent↗