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Biomedical subjects

L Guillevin

Publications and source records attributed to L Guillevin.

At least 307 records · Page 17Linked to original sources
PubMed1990

[Anti-Scl-70 antibodies in systemic scleroderma].

We looked for anti-Scl-70 and anti-centromere antibodies in 109 patients (26 men and 83 women). Mean age was 43 +/- 15 years. Forty patients had systemic sclerosis according to ARA criteria. The extension of cutaneous involvement was defined by using Barnett and Coventry criteria: 12 patients were type I (sclerodactyly), 20 type II (acrosclerosis) and 8 type III (diffuse scleroderma). Among the 12 patients with type I, there were 8 cases of CREST syndrome defined as follows: presence of sclerodactyly, Raynaud's phenomenon and 2 of the 3 following criteria: oesophageal dysmotility, calcinosis, telangiectasia. Other organ involvement was recorded. Control patients had idiopathic Raynaud's phenomenon (n = 22), other connective tissue diseases (n = 20), and miscellaneous diseases (n = 28). Ninety-nine patients were prospectively included in this study. Patients' sera were stored at -20 degrees C. Ten previously stored sera obtained from patients with systemic sclerosis were also analyzed. Immunological tests were performed simultaneously and with no information on the diagnosis. When antinuclear antibodies were detected by indirect immunofluorescence, double immunodiffusion and immunoblotting were performed. Anti-Scl-70 antibodies were detected in systemic sclerosis only: 1 of 12 type I, 11 of 20 type II and 4 of 8 type III. One serum negative by immunodiffusion was positive using immunoblotting. We found that the specificity of anti-Scl-70 antibodies for systemic sclerosis was 100 p. 100 and their sensitivity 40 p. 100. There was a correlation between the presence of anti-Scl-70 antibodies and the presence of antinuclear antibodies (p less than 0.05) and the extent of cutaneous involvement (p less than 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1990

[Treatment of systemic diseases with pulse cyclophosphamide: 15 cases].

Fifteen patients suffering from severe systemic diseases were treated with monthly pulses administration of cyclophosphamide (0.7 g/m2 of body surface): 8 acute systemic lupus erythematosus, 2 Wegener's granulomatous, 1 polyarteritis nodosa, 1 rheumatoid vasculitis, 1 progressive systemic sclerosis, 1 relapsing uveitis and 1 dermatopolymyositis. The indications for cyclophosphamide were: glomerulonephritis (6 cases), resistance to previous treatments (7 cases) and undesirable side effects of corticosteroid therapy (2 cases). After 3 pulses, the disease was controlled in 12 patients (80%) and corticosteroids could be decreased in all 12 cases without an evolutive relapse of the disease. Five patients developed infections (2 septicemia, 1 zona, 1 herpes gingival stomatitis and 1 viral meningitis) which were treated without sequelae. One patient developed cystitis with hematuria after the 3rd pulse; association of mesna, a urinary tract protective agent, enabled the continuation of treatment without a cystitis relapse. At the end of our retrospective study, the efficacy of pulse cyclophosphamide administration seems to be satisfactory but the risk of undesirable side effects should limit its use to severe systemic diseases or those resistant to conventional therapies.

Adult↗
PubMed1990

Treatment of progressive systemic sclerosis by plasma exchange: long-term results in 40 patients.

The efficacy of plasma exchanges (PE) during the course of scleroderma has only been investigated for short periods. The aim of this study was to follow patients over a long enough period to observe the course of the clinical and paraclinical symptoms in the short, medium, and long term. Forty patients, 24 women and 16 men, were treated by PE and observed for 1-3, 3-12 and over 12 months. Immunological, biological and clinical course and any undesirable side effects were evaluated using a detailed questionnaire. Concomitant therapies were reported and most frequently consisted of corticosteroids, colchicine, factor XIII or vasodilators (nifedipine, captopril). The therapeutic effectiveness of PE was assessed on the basis of improvements in cutaneous, digestive, joint, muscular, lung, cardiovascular and renal lesions. Our findings confirmed the effectiveness of short-term PE on scleroderma (52% of the patients improved during the first 3 months). However, this improvement was transient (5% improvement between 3 and 12 months and only 2.5% over 12 months) and limited to the cutaneous and muscular lesions. Thus, PE cannot be recommended for the treatment of progressive systemic sclerosis.

Adolescent↗
PubMed1989

[Splenectomy can cure thrombocytopenia of systemic lupus erythematosus].

Between 1976 and 1988, 7 patients with systemic lupus erythematosus (SLE) underwent splenectomy for severe peripheral thrombocytopenia (platelet count below 30,000/mm3) which had resisted corticosteroid therapy (n = 7), high-dose immunoglobulins (n = 4), vincristine (n = 2), danazol (n = 1), cyclophosphamide and plasma exchanges (n = 1). No infective or non-infective complication of splenectomy was observed. One patient died of intracerebral haemorrhage 3 years later, after several recurrences of thrombocytopenia. The 6 survivors have normal platelet counts (more than 150,000/mm3) after splenectomy, over a mean follow-up period of 80 months. Two of these 6 patients never had recurrent thrombocytopenia and have not received any treatment for 17 to 102 months. Four patients are still under prednisone in doses lower than 30 mg/day. Among these, 2 had recurrent thrombocytopenia 2 and 60 months respectively after splenectomy and responded favourably to another course of corticosteroids associated, in one case, with cyclophosphamide. Thus, splenectomy is an effective treatment of the thrombocytopenia associated with SLE. However, the possibility of early or late recurrences makes it necessary to reserve splenectomy to those cases where medical treatment has failed or is contra-indicated.

Adolescent↗
PubMed1989

[Periarteritis nodosa linked to the hepatitis B virus].

Periarteritis nodosa is a necrotizing vasculitis which, in more than one-third of the cases, is consecutive to an hepatitis B virus infection. This aetiological form is usually characterized by the absence of respiratory manifestations and by a greater frequency of hepatic cytolysis. In terms of survival, its prognosis is similar to that of other forms, but a persistent viral replication facilitates the subsequent development of chronic liver diseases and cirrhosis. Its modern treatment consists of a short course of corticosteroids combined with antiviral agent and plasma exchanges. Long-term corticosteroid therapy and immunodepressants are reserved for cases where the above-mentioned treatment has failed. This therapeutic approach results in a survival rate of 80 p. 100 at 4 years.

Female↗
PubMed1989

Symptomatic urogenital manifestations of polyarteritis nodosa and Churg-Strauss angiitis: analysis of 8 of 165 patients.

To establish the clinical features and evolution of ureteral and testicular manifestations of polyarteritis nodosa, 8 cases among a group of 165 patients were reviewed. Urogenital manifestations are rare and were related to hepatitis B virus markers in 5 of the 8 patients. Malignant hypertension was present in 6 of 8 patients and renal insufficiency related to renal infarctions was noted in 5. Prognosis is good and regression generally is obtained with steroid therapy or with plasma exchange and vidarabine when polyarteritis is related to hepatitis B virus. In some cases ureteral stenosis may require an operation in association with steroids and/or immunosuppressive treatments.

Adult↗
PubMed1989

Serum cytokine changes in systemic vasculitis.

Cytokines are known to alter a number of vascular tissue cell functions. The aim of this retrospective study was to determine serum cytokine levels in patients with vasculitis and to analyse the possible relation to the severity of the disease. Tumour necrosis factor alpha (TNF alpha), interleukin-1 (IL-1)beta, IL-2, interferon (IFN)- and IFN-gamma were assayed in 33 patients with polyarteritis nodosa (PAN) or Churg and Strauss angiitis (CSA), and three with Wegener granulomatosis (WG). Serum cytokine changes were observed in most patients with active disease, i.e. before treatment was started. In the majority of patients with PAN or CSA, there was a marked increase in serum IFN-alpha and IL-2 levels, while TNF-alpha and IL-beta levels were moderately elevated. Serum IFN-gamma remained undetectable in all but one of these patients. In patients with WG, serum IFN-alpha and IL-2 levels were also elevated, whereas IL-1 beta, IFN-gamma and TNF alpha levels remained within normal limits. In paired samples of patients with PAN, IFN-alpha and IL-2 levels were significantly higher before than after treatment. These preliminary data suggest that a particular pattern of cytokine changes is associated with vasculitis and that cytokines might be involved in the pathogenesis of PAN/CSA and WG. Prospective studies are warranted to determine whether cytokines could be considered for the monitoring of disease activity and therapy.

Biological Factors↗
PubMed1989

[Takayasu's disease localized exclusively in the pulmonary artery].

A 35 year-old woman exhibited a stenotic lesion of the right pulmonary artery with a severe inflammatory syndrome. A diagnosis of Takayasu arteritis was made, although no aortographic abnormality was present. Involvement of pulmonary arteries during the course of Takayasu arteritis is frequent, but a localisation of the disease to the pulmonary arterial tree alone is a very rare condition.

Adult↗
PubMed1989

[Sensory neuropathy of the trigeminal nerve and scleroderma].

Sensory neuropathy of trigeminal nerve is one of the neurological complications of systemic diseases, particularly scleroderma. Three cases are reported in which onset occurred at different stages of the disease, the main symptom being painful dysesthesias. All three branches of the trigeminal nerve may be affected but a preference is apparent for V2 and V3. Signs are usually limited to hypoesthesia and the chronic course is influenced little by treatment.

Cranial Nerve Diseases↗
PubMed1989

[Prognostic factors of hypereosinophilic syndrome. Study of 40 cases].

Forty patients with hypereosinophilic syndrome (HES), defined according to the criteria established by Chusid et al., were studied retrospectively in order to determine the prognostic factors in this heterogeneous disease. A myeloproliferative syndrome (MPS) was diagnosed 17 times when splenomegaly and/or a greater than 5 times the normal vitamin B12, level existed. Cutaneous-visceral involvement was almost always present (95%), but could appear after several years of evolution. Twenty-three patients had cardiac involvement, determined by clinical, radiological, electrocardiographic and echocardiographic examinations; 12 of these had endomyocardiac fibrosis. The overall survival rate was 80% at 5 years and 42% at 10 and 15 years. Among the factors evaluated that are capable of influencing this survival, 5 were found to impair the prognosis; presence of an MPS; non-response of the hypereosinophilia to corticoids; existence of a cardiopathy; being male; and an elevated maximum eosinophilia; the last two factors were significantly associated with the cardiopathy. Because hematological or cardiac anomalies are not always present at the time of the first examination, HES patients require very close surveillance.

Actuarial Analysis↗