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Biomedical subjects

F Lhote

Publications and source records attributed to F Lhote.

At least 55 records · Page 3Linked to original sources
PubMed1995

Gastrointestinal tract involvement in polyarteritis nodosa and Churg-Strauss syndrome.

OBJECTIVE: To study the nature and incidence of gastrointestinal (GI) manifestations in polyarteritis nodosa (PAN) and Churg-Strauss syndrome (CSS) and define their therapeutic and prognostic implications. METHODS: Fifty-three patients (29 males, 24 females) affected with PAN or CSS and followed in our institution were included in a retrospective study. Patients were divided into 2 groups: patients without GI manifestations (group A) and patients with GI manifestations (group B). Among patients with GI manifestations we have studied a subgroup with a possibly poorer prognosis in whom the following symptoms were present: GI tract hemorrhage, intestinal perforation, digestive tract surgery due to PAN manifestations, intractable abdominal pain and weight loss greater than 20% of normal weight due to GI tract ischemia. RESULTS: The clinical manifestations were those that are classically encountered in PAN and CSS. Every patient fulfilled the American College of Rheumatology (ACR) criteria for PAN and CSS. Thirty-five patients without GI manifestations were included in group A and 18 patients (34%) with GI manifestations in group B. The mean age of the group at the time of diagnosis was 56.9 +/- 19.1 years (range: 21-71 years) in group A and 47.5 +/- 16.8 years (range: 12-82) in group B. GI manifestations were considered as one of the symptoms revealing PAN in 7 (13.2%) cases. Six of the 18 patients with GI manifestations had definite organ involvement related to vasculitis. Abdominal pain without characteristic organ involvement or surgical emergency was present in 12/18 patients. HBV infection was more frequently observed in group B than in group A. Survival curves showed that at 10 years, 80% of the patients in group A were alive versus 67% in group B (P not significant). For the 9 patients with severe GI manifestations, the survival curves showed that, at 10 years, 44% of them were alive versus 80% in the other group A (p < 0.001). CONCLUSIONS: GI manifestations are frequent in PAN and CSS and were present in 34% of our patients. Prognosis of PAN with GI manifestations is not statistically different than in PAN without GI involvement, except for patients with severe digestive complications.

Adolescent↗
PubMed1995

Distinguishing polyarteritis nodosa from microscopic polyangiitis and implications for treatment.

The vasculitides are a heterogeneous group of disorders for which classification is needed to facilitate diagnosis and treatment. Recent studies based on a more comprehensive clinical analysis of symptoms and virologic investigations favor the recognition, in the polyarteritis nodosa (PAN) group, of a distinct form of systemic vasculitis called microscopic polyangiitis (MPA). This distinction may be confusing, so the features of each condition need to be more precisely defined. Differentiation between PAN and MPA cannot be based on only histologic criteria. Clinical manifestations (especially lung and kidney involvement), biologic signs (antineutrophil cytoplasmic antibodies and hepatitis B and C infection), and angiographic data must be taken into consideration. Therapeutic strategy should differ dramatically, depending on whether primary or secondary MPA or PAN has been diagnosed, and treatment optimization is certainly the best reason for improving the classification of the systemic vasculitides.

Diagnosis, Differential↗
PubMed1995

Polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome. Clinical aspects and treatment.

In this article, three systemic vasculitides--polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss syndrome--are reviewed, focusing on advances in the understanding of the pathogenesis, new classification nomenclature, and major clinical features of these vasculitides. Distinguishing criteria between polyarteritis nodosa and microscopic polyangiitis are also presented in detail, and recent advances in the clinical management of these diseases according to appropriate therapeutic strategies are recommended.

Churg-Strauss Syndrome↗
PubMed1995

Antineutrophil cytoplasmic antibodies (ANCA) and abnormal angiograms in polyarteritis nodosa and Churg-Strauss syndrome: indications for the diagnosis of microscopic polyangiitis.

Microscopic polyangiitis (MPA) is one of the vasculitides previously included in the polyarteritis nodosa (PAN) group. A diagnosis of MPA is usually considered when glomerulonephritis and/or lung hemorrhage are present. Small-sized vessels are involved and this sign is considered, for some authors, to be the main diagnostic criterion. The present study attempted to define clinical, radiological and immunological characteristics of MPA and to separate them from classic PAN (c-PAN) and Churg-Strauss syndrome. We have shown that, in most cases, patients presenting microaneurysms and/or multiple vessel stenoses, which reflect medium-sized vessel involvement, do not have ANCA. Conversely, patients with glomerulonephritis have almost never had abnormal angiograms. Furthermore, the clinical characteristics of ANCA-positive patients also indicate small-sized vessel involvement. Although at present it is not possible to definitively separate MPA from c-PAN, our results show that ANCA should be considered diagnostic for MPA and, in most cases, should be an exclusion criterion for c-PAN.

Adult↗
PubMed1994

Treatment of polyarteritis nodosa and Churg-Strauss syndrome: indications of plasma exchanges.

To define the most effective treatment for polyarteritis nodosa (PAN) and Churg-Strauss syndrome (CSS), we undertook 4 consecutive prospective therapeutic trials including 236 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? and does hepatitis B virus (HBV) related PAN require treatment? Our first randomized trial in 71 patients (1981-1983) 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. In the last protocol including 56 patients and addressed to severe PAN without HBV markers or CSS we have shown that PE did not improve the prognosis and control of the disease. Twelve years after the beginning of the trials on PAN and CSS patients, we think that the therapeutic strategy should be as follows: 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. 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 vasculitis: PE are obviously useful in PAN related to HBV where immune complex deposition has been demonstrated. When PAN is not related to HBV and in CSS, even in severe cases, there is presently no argument supporting systematic administration of PE at the time of diagnosis.

Adolescent↗
PubMed1994

[Allergic angiitis with granulomatosis: the Churg and Strauss syndrome].

Churg-Strauss Syndrome is a disorder characterized by pulmonary and systemic necrotizing vasculitis, extravascular granulomas, and eosinophilia occurring almost exclusively in patients with asthma or a history of allergy. The clinical manifestations most commonly associated with asthma are mononeuritis multiplex, gastrointestinal, cutaneous, cardiac and renal involvement. ANCA are found in 2/3 of the patients with Churg-Strauss Syndrome and are usually p-ANCA. The treatment of Churg-Strauss Syndrome relies on corticosteroids and cyclophosphamide.

Churg-Strauss Syndrome↗
PubMed1994

Treatment of polyarteritis nodosa related to hepatitis B virus with interferon-alpha and plasma exchanges.

OBJECTIVES: To test the effectiveness of and tolerance to interferon-alpha 2b (INFa2b) in association with plasma exchanges for the treatment of polyarteritis nodosa (PAN) related to hepatitis B virus (HBV). METHODS: A prospective, non blinded, multicentre trial was carried out in which patients with multisystemic HBV-related PAN were included. Each patient received the association of INFa2b and plasma exchanges. The end point of the study was control of the disease (recovery or remission) or death. RESULTS: Six patients were included in the study. Each patient had histopathological or arteriographic evidence of vasculitis and was infected with actively replicating HBV. All patients were alive at the end of the study and no longer presented clinical or laboratory evidence of systemic vasculitis. HBeAg/anti-HBeAb seroconversion was observed in four patients (66.6%) and HBsAg/anti-HBsAb in 3/6 (50%). Two patients are still being treated with INFa2b because of chronic active hepatitis. CONCLUSIONS: It is considered that this new therapeutic approach to HBV-related PAN effectively cured systemic vasculitis and was associated with control of HBV infections. Antiviral therapy may have a role to play as the first line treatment regime of virus-induced vasculitis.

Adult↗
PubMed1993

[Synchronization of plasma exchange and cyclophosphamide in severe systemic diseases. A consecutive study of 10 patients].

Ten patients with severe systemic diseases, including systemic lupus erythematosus (n = 2), polymyositis (n = 2), essential mixed cryoglobulinaemia (n = 2), rheumatoid arthritis vasculitis (n = 3) and Wegener's granulomatosis (n = 1), were treated with 3 consecutive plasma exchanges synchronized with pulse cyclophosphamide. This therapeutic regimen was applied every 4 weeks initially and thereafter every 6 weeks in case of positive response after the first 3 cycles; it was combined in all patients with corticosteroid therapy. The treatment was administered for severe flare-up of the disease in 7 patients and for failure of previous treatments, including corticosteroids, cyclophosphamide and plasmapheresis, in 3 patients. Three kinds of response were observed: lasting complete remission without relapse after synchronization had ceased in 4 patients, partial clinical remission with post-synchronization relapse in 5 patients, and primary failure without any clinical response to treatment in only 1 patient. These results suggest that repeated plasma exchanges synchronized with cyclophosphamide are effective against progressive autoimmune diseases and in cases where conventionally administered immunosuppressive treatments had failed. However, this type of treatment cannot prevent long-term relapses, and only a prospective study can evaluate its success in terms of survival.

Adult↗
PubMed1993

Successful treatment of HIV-related vasculitis with peripheral neuropathy with short-term steroids followed by the association of zidovudine and plasmapheresis.

OBJECTIVE: treatment of HIV-related vasculitis, avoiding prolonged immunosuppressive therapy. DESIGN: prospective pilot study of HIV-related neurological vasculitis. PATIENTS: two HIV-infected patients with histologically proven vasculitis. INTERVENTION: short-term corticosteroid followed by zidovudine combined with plasmapheresis. MAIN OUTCOME MEASURES: clinical, biological, immunological and electromyographic evaluation. RESULTS: complete neurological recovery. CONCLUSION: excellent tolerance and efficacy of combined zidovudine and plasmapheresis therapy in peripheral neurological HIV-related vasculitis.

AIDS-Related Opportunistic Infections↗
PubMed1993

New application of myocardial antimyosin scintigraphy: diagnosis of myocardial disease in polymyositis.

Heart disease is a rare but important complication of polymyositis. Diagnosis of myocardial disease is usually based on non-specific clinical, electrocardiographic, and echocardiographic data. This paper reports a case of polymyositis with myocardial disease diagnosed by myocardial imaging with radiolabelled antibody to myosin, a specific marker of the necrotic myocardial fibre.

Aged↗
PubMed1993

Treatment of polyarteritis nodosa related to hepatitis B virus with short term steroid therapy associated with antiviral agents and plasma exchanges. A prospective trial in 33 patients.

OBJECTIVE: To test the effectiveness and tolerance of antiviral agents associated with short term immunosuppression in the treatment of polyarteritis nodosa (PAN) related to hepatitis B virus (HBV). METHODS: We conducted a prospective, nonblinded, multicenter trial in which patients with multisystemic PAN related to HBV were included. Every patient initially underwent a short term (2 weeks) treatment with prednisone and then received vidarabine (Vira A) and plasma exchanges. The end point of the study was control of the disease (recovery or remission) or death. RESULTS: Thirty-three patients were included. Every patient had histopathologic or arteriographic evidence of vasculitis and was infected with actively replicating HBV. Disease activity during the first 6 months was controlled in 26 patients (78.8%). Among the 25 patients still alive at the end of the study, 24 (72.7%) had completely recovered with no clinical or laboratory evidence of systemic vasculitis after at least 18 months without treatment. Eight patients died during the study period; 3 of treatment failure, usually early in the course of the disease. One patient died of fulminant hepatitis 3 months after the entry in the study at time of seroconversion. The survival curve showed that at 7 years, 76% of the patients were alive. HBeAg/anti-HBeAb seroconversion was observed in 12 patients (36.3%) after one Vira A cycle. When a 2nd cycle of Vira A or alpha interferon was prescribed, HBeAg/anti-HBeAg seroconversion occurred in 15 patients (45.4%). Two other patients who underwent a 2nd cycle of Vira A administration, had lost HBeAg and no longer expressed serological evidence of replication as assessed by HBV DNA spot hybridization. At the end of the study, 17 (51.5%) no longer expressed serological evidence of HBV replication. This treatment was effective and only minor side effects were noted. CONCLUSIONS: We conclude that this new therapeutic approach to PAN related to HBV effectively controlled systemic vasculitis and was associated with a higher number of recoveries from chronic HBV infections. The development of new antiviral agents, such as interferon alpha 2b, allows us to hope that antiviral therapy will have a role to play as a first line treatment regimen of virus induced vasculitis.

Adult↗
PubMed1993

[Meningoradiculitis disclosing T-lymphocyte leukemia in HTLV-1 infection].

A case of HILV1-associated adult T cell leukaemia/lymphoma (ATLL) in à 21-year olf African woman is reported. The patient presented with lymphomatous meningoradiculopathy. The usual clinical features of ATLL were absent. Lumbar MRI showed a pial enhancement by DTPA-gadolinium of the conus medullaris which extended to the proximal cauda equina. Under systemic chemotherapy coupled with intrathecal chemotherapy the patient progressively improved, and at the second MRI examination complete disappearance of the lumbar enhancement was observed. MRI of the brain using axial and coronal T2-weighted sequences detected multifocal lesions of high-intensity signal in the subcortical white matter. ATLL is unusual in people of African origin. The ATLL-strongyloïdes infestation association has previously been reported, suggesting that parasitic infestation may be an important co-factor leading to the development of ATLL.

Adult↗
PubMed1992

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↗
PubMed1992

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↗
PubMed1992

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↗