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Biomedical subjects

L Guillevin

Publications and source records attributed to L Guillevin.

At least 217 records · Page 12Linked to original sources

Identification of the T cell antigen receptor V beta gene products in labial salivary glands from patients with primary Sjögren's syndrome.

T lymphocytes are predominantly involved in the development of Sjögren's syndrome. Their repertoire has recently been claimed to be restricted. Analysis of T cell receptor V beta gene products within the labial salivary glands led us to identify V beta 2 and V beta 8. This study, using a new panel of anti-V beta product monoclonal antibodies and a tissue triple-staining technique, examined the distribution of V beta gene family products in activated as well as non-activated CD4+ or CD8+ T cells subsets in the salivary gland tissue of SS patients. Our results suggest that the V beta genes used by the T CD4+ or T CD8+ cells in situ are similar, irrespective of the activation status of these cells. T lymphocytes homing into exocrine tissues might thus be selected on the basis of their V beta repertoire rather than their activation state.

Adult↗

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↗

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↗

Therapy of resistant systemic necrotizing vasculitis. Polyarteritis, Churg-Strauss syndrome, Wegener's granulomatosis, and hypersensitivity vasculitis group disorders.

The systemic necrotizing vasculitides represent a difficult group of diseases from a therapeutic perspective. Even with successful therapy patients often experience a high degree of disease and treatment morbidity. Refractory disease, or that which fails to respond to standard therapy, is particularly challenging. A clinical approach to several vasculitic syndromes is outlined in this article with emphasis given to therapies supported by controlled investigations and those supported by consensus opinion where available.

Churg-Strauss Syndrome↗

[Vasculitis of viral origin. Pathogenesis and therapeutic implications].

Some viruses are unquestionably the cause of vasculitis, by different mechanisms: circulating immune complexes, cryoglobulinemia and/or direct infection of the blood vessel. The main viruses responsible for vasculitis are hepatitis B & C viruses, cytomegalovirus, parvovirus B19 and human immunodeficiency virus. Viral vasculitis are clinically protean, most of the time similar to idiopathic vasculitis. The manifestations due to the virus itself are sometimes hidden and vasculitis may reveal the viral infection. In some cases of viral vasculitis, particularly in hepatitis virus-induced vasculitis, antiviral therapy may help in controlling the disease. A viral etiology must be considered during atypical vasculitis.

Cytomegalovirus Infections↗

Regressing microaneurysms in 5 cases of hepatitis B virus related polyarteritis nodosa.

OBJECTIVE: To investigate the angiographic evidence of polyarteritis nodosa (PAN) related to hepatitis B virus (HBV) infection. METHODS: We reviewed retrospectively the medical files of 5 patients who had therapy for PAN. RESULTS: Each patient showed dramatic regression or disappearance of the aneurysms and stenoses; this reflected the clinical course and remission obtained with treatment, but not the disappearance of HBV. When a patient was considered clinically cured, i.e., no signs of vasculitis for 18 mo after discontinuation of treatment, microaneurysms had disappeared on angiography. CONCLUSION: From our experience and that reported by others, when clinical remission is achieved, arteriography is usually unnecessary because microaneurysms have disappeared or significantly regressed.

Adult↗

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↗

[Still disease in adults revealed by a digestive manifestation].

Adult Still's disease is characterized by typical spiking fever, oligopolyarthritis, neutrophilic leukocytosis and involvement of various organs. We report a case which illustrated typical digestive features of Still's disease as dysphagia, peritonitis and manifests the hitherto unreported complication of gastric ulcerations. Treatment with prednisone was started in order to control arthritis, resulting in improvement of both gastric ulcerations (partially resistant to omeprazole treatment) and arthralgia. After seven years of follow-up, the patient remains clinically and biochemically stable with steroid and methotrexate treatment.

Anti-Inflammatory Agents, Non-Steroidal↗

[Course of ischemic risk in treated atheromatous hypertensive patients. The PRIHAM study].

Hypertension is one of the major cardiovascular risk factors. However seldom occurs alone in a patient and the antihypertensive treatment should be taken into account when assessing global cardiovascular status. The PRIHAM study was an open study which enrolled 3,216 hypertensive atheromatous patients who already has had a clinical ischaemic accident. Patients were followed-up for 3 years. The lowering effect on cardiovascular risk, starting from the fifteenth month was confirmed to the end of 36-month study. The effect on blood pressure was observed from the third month. Stabilized blood pressure observed within the first year persisted throughout the three years without tachyphylaxis. It was effective on SBP (-14.4%) and on DBP (-15%) while maintaining a stable heart rate both in the supine position or immediately upon standing or after 3 minutes in the standing position. Amongst diabetic and dyslipidemic patients, an absence of deleterious metabolic effects was noticed, parallel with the effectiveness. Tolerance was globally judged as good or very good by the investigating practitioners and the patients expressed an improvement in their feeling of well-being. In conclusion, from the level of effectiveness obtained and the low incidence of adverse effects, urapidil appears well adapted and easy to manage in a population with a particularly high cardiovascular risk.

Adult↗

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↗

Serum lipid concentration with reference to the clinical and immunological status of HIV infected men.

We investigated the serum concentrations of free fatty acids (FFA), cholesterol, phopholipids and triglycerides in HIV-positive men (n = 50) from three behaviour groups: heterosexuals (n = 16), drug addicts (n = 18) and homosexuals (n = 16) and a control group of HIV-negative men (n = 25). The circulating concentrations of lipids were analyzed with reference to the clinical status of infection and the absolute CD4 cell count. According to the clinical progression of HIV infection the patients were divided into two groups (CDC 1987 criteria): stages II and III (n = 28) and stage IVC (n = 22). HIV-positive men had higher polyunsaturated fatty acids (PUFA) (+100%), p < 0.001) only in the II and III stages, lower cholesterol (-25% to -40%, p < 0.001) and lower phospholipids (-25%, p < 0.001) for the two stages than in the controls. The triglycerides were increased only in stage IVC patients compared to the controls (+110%, p < 0.001). According to their CD4 cell count, the patients were divided into four groups: > 400 (n = 11), 400-150 (n = 9), 150-50 (n = 9) and < 50 (n = 19). Regardless of the CD4 count, the PUFA were significantly higher (+50% to +125%) and cholesterol (-35% to -45%) and phospholipids (-25% to -30%) lower than in the controls in all HIV-infected men except the patients with 400-150 CD4. Only the HIV-positive patients with < 50 CD4 cells had elevated triglycerides (+97%, p < 0.001). There was a significant negative correlation between the CD4 cell count and the serum triglyceride concentrations (r = -0.31, p < 0.03). In conclusion, the most elevated PUFA occurred in HIV-positive patients with > 400 CD4, while hypertriglyceridaemia is prevalent in very advanced stages of infection (with < 50 CD4). This suggests that there is a relationship between the circulating PUFA and triglycride levels and the progression of infection and immune suppression. The disturbances in lipid metabolism must now be correlated with the underlying metabolic, hormonal and cytokine changes and their role in the development of significant malnutrition and immune perturbations.

Adult↗

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↗