[Apropos of the article: "From sedimentation rate to inflammation profile"].
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Biomedical subjects
Publications and source records attributed to P Godeau.
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A register of systemic lupus erythematosus has been open between 1st January 1987 and 31 December 1992 in France. One hundred and seventeen cases of pregnancy from more than 40 centers origin have been prospectively collected. One hundred and three were analyzed. Pregnancy outcome was as following: full term birth (n = 28), premature birth (n = 48), fetal wastage (n = 18 i.e. 13 early spontaneous abortions, two late spontaneous abortions and three stillbirths), therapeutic abortion (n = 5), elective abortion for unwanted pregnancy (n = 4). Four preterm babies died in neo-natal period. Lupus activity was present at pregnancy diagnosis in 28 cases (27%). Of 75 patients with inactive lupus at pregnancy beginning, 27 relapsed during pregnancy and seven in post-partum period. Two patients with nephrotic syndrome died of opportunistic infection. Fetal prognosis is mostly related to proteinuria and absence of anti-SSA antibodies. History of fetal losses, lupus activity at pregnancy beginning, hypertension, use of 20 mg/d or more prednisone dosage during pregnancy influence prematurity. The fetal hypotrophy factors are short duration of pregnancy, lupus activity at beginning of pregnancy, low serum levels of C3 or C4, hypertension, absence of anti-SSA antibodies. Three out of 22 newborns from mother with anti-SSA antibodies developed neonatal lupus: two with cutaneous lupus and one with complete congenital auriculo-ventricular block.
We describe a primitive hyperparathyroid in a old woman due to a parathyroid adenoma localized by Technetium 99m, Thallium 201 scintigraphy within the thyroid gland. During surgery, this adenoma was found inside a thyroid adenoma. It is the second case reported on the literature in a such localization. We underline the interest of the scintigraphy Technetium 99m, Thallium 201, for the detection of ectopic parathyroid adenoma.
Progress in the treatment of systemic vasculitis have permitted a decrease of mortality but with an increase in iatrogenic morbidity. Steroids remain the cornerstone of the treatment but precise modalities and other concomitant treatments are depending upon the type of vasculitis. In most cases, systemic vasculitis are primary and the treatment, although important, is symptomatic. However, in some cases such as hepatitis B virus-induced polyarteritis nodosa or hepatitis C virus-induced cryoglobulinemia, the treatment can be etiologic and is directed against the antigen responsible for the systemic vasculitis. In the future, a better understanding of pathological mechanisms, particularly of etiologic factors, and new treatment such as monoclonal antibodies should increase the prognosis of systemic vasculitis.
We evaluated the value of serum amyloid P component scintigraphy (123I-SAP) and labial salivary gland biopsy in four patients with apparently localized amyloidosis. One patient had pharyngeal amyloidosis, the second laryngeal amyloidosis, the third retro-ocular amyloidosis and the fourth had carpal tunnel syndrome. Three patients (no 2, no 3, no 4) have abnormal whole body retention of 123I-SAP and positive labial salivary gland biopsy for amyloidosis. Only the first patient had effectively localized amyloidosis and local treatment was sufficient. Melphalan was given to the patients no 2 and no 3, colchicine was given to the patient no 4. Labial salivary gland biopsy and 123I-SAP scintigraphy may give positive argument for the diagnosis of systemic amyloidosis in patients with apparently localized amyloidosis.
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.
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In a multi-centre prospective study of systemic lupus erythematosus and pregnancy in France, 117 cases were identified from 1987 to 1992. We report significant morbidity and mortality for mother and fetus from an analysis of 103 cases. Pregnancy outcome was as follows: 28 full-term births, 48 premature births, 18 fetal losses (13 early and two late spontaneous abortions, three stillbirths), five therapeutic abortions and four elective abortions (for unwanted pregnancy). Four preterm neonates died. Lupus was active at pregnancy onset in 28 patients. Of 75 patients with inactive lupus at conception, 27 relapsed during pregnancy, and seven postpartum. Two patients with nephrotic syndrome treated with high-dose corticosteroids died from opportunistic infections. Fetal loss correlated with a history of proteinuria and absence of anti-SSA antibodies. Prematurity was related to a history of fetal loss, active lupus at pregnancy onset, hypertension and > or = 20 mg/day prednisone during pregnancy. Intrauterine growth retardation correlated with pregnancy of short duration, low serum C3 or C4, hypertension, and absence of SSA antibodies. Three of 22 newborns whose mothers had anti-SSA antibodies developed neonatal lupus: two with cutaneous involvement and one with complete atrioventricular block.
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Multiple subungual splinter hemorrhages have been initially described as an important sign of subacute endocarditis. Secondly, they were reported in various other conditions, especially in isolated cases of primary antiphospholipid syndrome. We report five patients with multiple fingernail subungual splinter hemorrhages occurring in the course of antiphospholipid syndrome. Antiphospholipid syndrome was secondary to systemic lupus erythematosus in two, to Fasciola hepatica infection in one and was considered as primary in two. In all patients multiple subungual splinter hemorrhages occurred concomitantly with thrombotic events of diverse arterial sites. The mechanism of subungual splinter hemorrhages is most probably thrombotic.
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OBJECTIVE: To study T, natural killer (NK) and B blood cell subsets in primary antiphospholipid syndrome (APS). METHODS: We studied 10 patients with primary APS and 12 healthy subjects. Blood lymphocytes counts, proportions of B cells (CD19+), total CD5 and CD5 B cells, NK cells (CD16+CD56+), T cells (CD3+), CD4+ helper (naive CD4+CD45RA+, memory CD4+CD45RO+ and CD4+CD29+, activated CD4+CD25+), CD8 (immunoregulatory CD8+CD57+, activated CD8+HLA-DR+) T cell subsets were measured by flow cytometry analysis. RESULTS: In the primary APS group, we observed a lower total lymphocyte count (p = 0.009), an expansion of naive CD4 cells (p = 0.025), a lower proportion of memory CD4 cells (p = 0.04) and an increased ratio of naive/memory CD4 cells (p = 0.015). CONCLUSION: Blood T cells phenotypes in primary APS differ markedly from healthy controls, indicating that immunologic abnormalities in primary APS might extend beyond autoantibody production.
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The antiphospholipid syndrome (APS) is usually defined by the association of a clinical manifestation (venous or arterial thrombosis or miscarriage) with the presence of antiphospholipid antibodies (lupus anticoagulant and/or anticardiolipin antibodies). It frequently occurs in the course of systemic lupus erythematosus but is also encountered as a "primary" disease. APS is responsible for diverse respiratory manifestations. Pulmonary embolism is common. The site of the causal venous thrombosis is frequently unusual. Pulmonary hypertension may be a consequence of repeated embolism or may belong to the primary idiopathic variety. Pulmonary manifestations may also result from left-sided heart failure due to mitral or aortic valve abnormalities, myocardial infarction or a specific myocardiopathy. APS is probably involved in the occurrence of some cases of adult respiratory distress syndrome. Long term secondary prevention of recurrent thrombosis is a central point in the management of APS.
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OBJECTIVE: Polymyositis (PM) and dermatomyositis (DM) are inflammatory muscle diseases of presumed autoimmune origin. Many possible interventions are available to treat these patients: corticosteroids, immunosuppressive drugs, plasmapheresis, and total body irradiation. But these therapies are not always effective and may be responsible for certain serious side effects. Polyvalent intravenous immunoglobulin (IVIG) has been tried with success in inflammatory myopathies after failure of traditional treatment. An attempt was made to evaluate the efficacy of IVIG as first line therapy in patients with PM or DM. METHODS: Eleven Caucasian patients [6 women, 5 men, mean age 55.6 (SD 10.1) years], with active recent inflammatory myopathy, were treated by high doses of IVIG as first choice. The average duration of inflammatory myopathy before IVIG was 9.6 months (SD 10.2 months, with a range of 1 month to 3 years). Five patients had PM and 6 had DM. None had myositis associated with connective tissue disease. Two patients had a history of malignant disease: 1 lymphoma and 1 breast tumor with relapse of the malignancy during the study. One patient had a probable lung carcinoma and in another patient, ovarian carcinoma was diagnosed a few months after the onset of IVIG. We used preparations of polyvalent human i.v. gamma globulins with intact IgG. All patients received 1 g/kg daily for 2 days each month. The mean course of treatment was 4 months. RESULTS: Clinical assessment, evaluated by proximal muscle power and biochemical tests, was carried out before each treatment period. Significant clinical improvement was noted in only 3 of the 11 patients (one with acute coxsackie virus B infection, and one with possible drug induced myopathy). Mean muscle power estimated for the 11 patients before and after IVIG therapy was not significantly improved. Eight patients showed significant biochemical improvement (more than 50%). Mean CK levels for the 11 patients showed a statistically significant decrease during IVIG therapy (p < 0.01). Minor IVIG side effects were noted in one patient. CONCLUSION: IVIG therapy seems effective rarely as first therapy in patients with inflammatory myopathy but may be considered especially in viral or drug induced myopathy. IVIG therapy as the first treatment may also be tried in patients with contraindication for steroids, and in mild myopathy, especially in the elderly, to avoid steroid induced side effects.
Neuromyositis defined as the association of dermatomyositis or polymyositis and a neuropathy without any found cause is a very controversial entity because of the possibility of, in one hand, muscular modifications caused by neurological involvement and, on the other hand, neurogenic type manifestations caused by polymyositis. The study of 4 cases seen in an Internal Medicine department and the review of the literature allowed us to show that the concept of neuromyositis corresponds to a clinico-pathological reality when the diagnosis is based on the association of definite criteria of both primary muscle and nerve involvement excluding muscular abnormalities that could be the consequence of nerve involvement and vice versa. The criteria, most relevant when associated are: a) for muscular involvement: high increase of muscular enzyme over 6 times the superior limit of the normal values, pseudomyotonic electrical discharges, perifascicular atrophy, intense inflammatory infiltrates and massive necrosis, b) for neurological involvement: early abolition of tendinous reflexes in a patient without notable muscular atrophy and with little or no myalgia, sensitive abnormalities in areas other than those of muscular involvement, especially when they are intense, early weakness of distal muscles, decrease of nerve conduction speed, target fibers and lesions of nerve trunks (and albuminocytological dissociation in the particular case of polyradiculoneuritis). Once the diagnosis of neuropathy settled, it is necessary to exclude an usual cause (alcoholism, diabetes...) before concluding to neuromyositis. When we apply these restrictive (but nevertheless necessary for the validity of diagnosis) criteria, only 6 cases of the literature respond to this entity. It is a peripheral neuropathy in 5 cases (like two of ours) and a polyradiculoneuritis in one case (like our two others). Among these 6 cases, there is a vasculitis in two, frequency much higher to what is observed in adult polymyositis, which suggest a possible causative role of vascular involvement in neuropathy arising. In the other cases we can just give pathogenic hypothesis making the neuropathy and the polymyositis the result of the same process (immunological disturbance, paraneoplastic origin, viral disease). In one of our four patients, who have shown an HTLV-I infection by polymerase chain reaction in situ hybridization was positive in muscle which suggest a direct pathogenic role of the virus. HTLV-I infection should be considered as a possible cause of neuromyositis especially in endemic areas.