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Biomedical subjects

L Guillevin

Publications and source records attributed to L Guillevin.

At least 235 records · Page 13Linked to original sources
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 systemic vasculitis with cyclophosphamide and steroids: daily oral low-dose cyclophosphamide administration after failure of a pulse intravenous high-dose regimen in four patients.

The efficacy of cyclophosphamide (CY) in the treatment of severe systemic vasculitis has been demonstrated, but its optimal administration regimen remains to be defined. The four patients described participated in prospective therapeutic trials of polyarteritis nodosa (PAN) without hepatitis B infection markers, Churg-Strauss syndrome (CSS) and Wegener's granulomatosis (WG) and received daily oral low-dose CY after failure of a monthly pulse regimen. These patients were included in this study because of failure to induce clinical remission (two patients) and relapse during the course of treatment (two patients). The results were good with initial clinical remission in three patients and stabilization in one. One patient with WG relapsed 10 months after changing therapeutics. The advantages of the different administration protocols are discussed.

Administration, Oral↗
PubMed1994

Pharmacokinetics of cyclophosphamide in patients with systemic necrotizing angiitis.

Cyclophosphamide pharmacokinetics were investigated following administration to patients with systemic necrotizing angiitis. Ten patients (eight women and two men) received cyclophosphamide as a 1-h-rate-constant intravenous infusion at doses ranging from 600 to 1200 mg. All patients received concomitant oral prednisone (1 mg/kg/d). Blood samples were collected at the end of drug infusion and 0.25, 0.5, 1, 1.5, 2, 3, 4, 6, 8, 12, 24 h later. Serum cyclophosphamide concentrations were assayed by high pressure liquid chromatography. The peak serum cyclophosphamide levels ranged from 15.7 to 29.4 mg/L. The mean cyclophosphamide elimination half-life was 6.2 +/- 1.3 h (mean +/- SD). The mean apparent volume of distribution and mean total plasma clearance were, respectively, 0.75 +/- 0.22 L/kg (mean +/- SD) and 83 +/- 22 mL/min (mean +/- SD). These results obtained in systemic vasculitic diseases were consistent with those observed in other studies with cancer patients receiving comparable doses of cyclophosphamide.

Adult↗
PubMed1994

Pharmacokinetics of ceftriaxone during plasma exchange in polyarteritis nodosa patients.

Plasma exchange (PE) is currently being used to treat a variety of disorders involving immune complexes, such as polyarteritis nodosa. This procedure removes endogenous toxic components that accumulate in patients with this disease, but it also removes drugs. Plasma-protein binding and the volume of distribution (V) are two kinetic parameters which strongly affect the efficiency of drug removal by PE. Drugs that are highly bound to plasma proteins and have a low V may show a marked decrease in plasma levels as a result of PE. Because ceftriaxone exhibits saturable plasma-protein binding, which influences its pharmacokinetic parameters, particularly its V, we evaluated its removal during PE therapy in this nonrandomized crossover study. Twelve polyarteritis nodosa patients undergoing PE were studied. Each patient was given ceftriaxone intravenously in doses of 1 and 3 g on days 4 and 11, respectively, immediately before (n = six patients; group I) and 6 h before (n = six patients; group II) PE. Plasma was assayed for ceftriaxone by high-pressure liquid chromatography. The mean amounts eliminated +/- standard deviations were 230.8 +/- 38.5 mg (1 g) and 750.0 +/- 168.5 mg (3 g) for group I and 161.0 +/- 66.0 mg (1 g) and 347.0 +/- 121.0 mg (3 g) for group II. The drug fractions eliminated by PE were 23.0% +/- 3.9% (1-g dose) and 24.9% +/- 5.6% (3-g dose) for group I (P > 0.05), and 16.6% +/- 5.9% (1-g dose) and 11.5% +/- 4.0% (3-g dose) for group II (P < 0.05). These results showed that the drug fraction eliminated decreased when V increased only when the distribution phase of ceftriaxone had been completed (group II). These findings suggest that PE may influence ceftriaxone disposition and that it would be better to administer the drug after PE to assure its therapeutic efficacy.

Adult↗
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↗
PubMed1994

[Deafness and auto-immunity: what is the role of antiphospholipid syndrome? Apropos of 35 cases].

UNLABELLED: Currently, the importance of autoimmune phenomenon in the genesis of inner ear disease is difficult to evaluate because the clinical and biological criteria of autoimmune deafness has not yet been well defined. Among patients with sensorineural hearing loss, is it possible to define a group presenting clinical and biological findings suggesting autoimmune disease? What are the most sensitive and selective biological tests for autoimmune disease of the inner ear? Do patients with autoimmune deafness present a particular clinical, genetic and for biological profile? In order to answer these questions, we retrospectively studied 35 patients followed in our service from June 1990 to August, 1992 for sensorineural hearing loss of an unknown origin. All patients had had a complete biological work-up including examinations which would detect inflammation (erythrocyte sedimentation rate immunoglobulin IgG, IgA, IgM assays, protein electrophonesis) or dysimmunity (circulating immune complexes, marked depression of serum complement levels the presence of antinuclear antiorgan antibodies, or anticardiolipid and type II anticollagen antibodies). HLA typing had also been done. RESULTS. Only 4 patients had a biological profile suggesting dysimmunity. These patients presented no common clinical or genetic characteristic which would distinguish them as a separate subgroup. Biologically, anticardiolipid antibodies were found in all 4 of these patients and only in these patients. These 4 patients also had circulating immune complexes and 3 of the 4 patients also had elevated levels of IgA and IgM. IN CONCLUSION: including assays for antiphospholipid antibodies in the work-up of sensorineural hearing loss could reveal an antiphospholipid syndrome affecting the inner ear. Theoretically, the use of anticoagulants and agents decreasing platelet aggregation could provide effective treatment.

Adult↗
PubMed1994

[Nodular and granulomatous form of periarteritis nodosa caused by the hepatitis B virus].

Polyarteritis nodosa (PAN) is, in rare cases, associated with subcutaneous nodules and pathology does not usually show the presence of peri and extravascular granulomas. When present in patients with hepatitis B virus (HBV) related PAN these facts demonstrate that classification of PAN is not homogeneous. CASE REPORT. A patient infected by HBV developed a PAN demonstrated by clinical symptoms and pathology. The disease was characterized by the presence of subcutaneous nodules and histologically by peri- and extra-vascular granuloma which surrounded necrosis of medium-sized vessels. Outcome was also unusual in the patient who did not respond to the association of plasma exchanges and antiviral agents and was only slightly improved by steroids and cyclophosphamide. COMMENTS. HBV-related PAN is considered to be an immune complex disorder. In the present case report granuloma were present as observed in Churg Strauss syndrome or Wegener's granulomatosis which are the consequence of other pathogenetic mechanisms as anticytoplasmic neutrophil antibodies (ANCA). This case reports underlines the heterogeneity of the PAN group of vasculitis and the probable role for various pathogenetic mechanisms.

Antiviral Agents↗
PubMed1994

[Plasmodium vivax malaria seizure more than 5 years after a stay in Meknès (Morocco)].

The authors report a case of Plasmodium vivax malaria which happened more than five years after a stay of the patient in Morocco. The late appearance of parasite blood forms and especially gametocytes, as observed in this case, could explain, at least partially, the awakening of apparently extinguished foci and the renewal of the transmission of the epidemic, mediated by the presence of Anopheles. This phenomenon certainly contributed to the failure of malaria eradication campaigns in some countries with endemic P. vivax.

Adult↗
PubMed1994

Plasmapheresis and subsequent pulse cyclophosphamide in severe systemic lupus erythematosus. An interim report of the Lupus Plasmapheresis Study Group.

The rarity with which therapy-free remission is achieved in patients with severe SLE demand the continued search for improved treatment strategies. Possibly, therapeutic results can be improved by means of a "synchronized" combination of plasmaphereses followed by pulse cyclophosphamide (Ctx). If the hypothesis is correct, that plasmapheresis induces a compensatory "rebound" proliferation of pathogenic clones, then pulse Ctx applied immediately after plasmapheresis should attack these activated clones when they are most vulnerable and, ideally, eliminate them. Employing a strict variant of this strategy in a pilot study, treatment-free remission lasting now for up to 7 years were achievable in 8 of 14 SLE patients. The Lupus Plasmapheresis Study Group (LPSG) is currently conducting a randomized international multicenter trial comparing a synchronized procedure with the administration of pulse Ctx alone. The present paper explains the rationale of synchronization and reports on the interim status of the LPSG Study.

Combined Modality Therapy↗
PubMed1994

[Microangiopathic hemolytic anemia associated with cancers. Symptomatic treatment by plasma exchange].

OBJECTIVE: To determine the interest of plasmapheresis in the management of cancer-associated microangiopathic haemolytic anaemia (MHA) not due to cancer treatment. MATERIAL AND METHODS: [corrected] National retrospective study using the file of the French Hemapheresis Society. RESULTS: We isolated 6 patients (5 men and a woman aged 32 to 69-year-old) who had prostatic (4 cases) or breast carcinomas or Hodgkin's disease. Diagnosis of cancer preceeded MHA in 3 patients (from 2 to 4 years). Bone or bone marrow metastases were demonstrated in 5 patients. The clinical presentation included anuria (4 cases), bleeding (4 cases), and laboratory findings were consistent with microangiopathic haemolysis and thrombopenia in all cases and intravascular coagulation (2 cases) and/or renal failure (4 cases). MHA symptomatic treatment included 4 to 10 courses of plasmapheresis, extra-renal epuration (4 patients), anticoagulation (4 patients) and/or antiagregant (3 cases), haemodialysis (4 cases) and vincristine (2 cases). Cancer treatment consisted of antitumoural chemotherapy (2 cases) and/or hormonotherapy (5 cases). In all cases, haemolysis, thrombopenia and intravascular coagulation were controlled within 30 days. MHA treatment was effective alone in 3 patients. No relapse were observed in 3 patients whereas the course of cancer continued in 2 patients. Two patients relapsed and died from MHA after 4 and 36 months. Two patients relapsed and died from MHA within a few months and 1 was lost to follow-up. CONCLUSION: Symptomatic treatment of cancer-associated MHA including plasmapheresis may be useful while waiting for an aetiologic management of the tumour.

Adenocarcinoma↗
PubMed1994

Long-term plasma exchange. Analysis of indications, outcome and side effects.

In order to determine the characteristics and the course of diseases treated with long-term plasmapheresis (e.g., more than 25 plasma exchanges), we retrospectively studied 850 patients who underwent plasmapheresis. Long-term plasma exchange was prescribed to 38 patients who failed to respond to conventional therapy; cryoglobulinemias, peripheral neuropathies and monoclonal gammopathies were their most frequent underlying diseases. Improvement was noted in 65.8% cases. Only minor side effects were observed and the risk/benefit ratio for such therapy was excellent.

Adult↗
PubMed1994

Pneumocystis carinii pneumonia in the course of connective tissue disease: report of 34 cases.

OBJECTIVE: To determine the circumstances, the clinical features and the outcome of Pneumocystis carinii pneumonia (PCP) in human immunodeficiency virus (HIV)-free patients with connective tissue diseases (CTD). METHODS: Retrospective analysis of all cases referred 10 medical units in the last 10 years. RESULTS: A total of 34 cases of PCP in patients with CTD were studied (Wegener's granulomatosis, n = 12; systemic lupus erythematosus, n = 6; polyarteritis nodosa, n = 4; poly/dermatomyositis, n = 5; others, n = 7). The majority of patients (25/34 patients; 74%) presented PCP during the first 8 months following the diagnosis of CTD. At the time of diagnosis of PCP, most patients (32/34; 94%) were receiving corticosteroids (mean prednisone equivalent dose: 1.2 mg/kg/day) associated in 24 cases with cytotoxic agents (cyclophosphamide, n = 19; methotrexate, n = 5). Most patients were lymphocytopenic at the onset of PCP: 91% (31/34) of patients had fewer than 1.5 x 10(9)/l circulating lymphocytes and 76% (26/34) had fewer than 0.8 x 10(9)/l. The mean duration of prodromal symptoms was 6 days: this is much shorter than for AIDS associated PCP. Half the patients required intensive care for respiratory failure. Mortality was high (11/34 patients; 32%) although deaths were partly due to infections acquired in intensive care units. Among the 23 survivors, 10 (43%) received secondary prophylaxis for PCP and 13 (57%), received the usual therapeutic regimen. No relapse has been observed in either group with a mean followup of 22 months. CONCLUSION: Although rare, PCP must be considered in patients with any type of CTD and receiving cytotoxic agents and corticosteroids, particularly if they are lymphocytopenic. Thus, bronchoalveolar lavage must be rapidly performed in patients with CTD presenting with fever, pulmonary infiltrates, hypoxemia and lymphopenia.

Adult↗