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J-C Piette

Publications and source records attributed to J-C Piette.

46 records · Page 3Linked to original sources
PubMed2003

[Neonatal lupus syndrome: review of the literature].

PURPOSE: Neonatal lupus syndrome include skin lesions, hematological and hepatic disorders, and congenital heart block (CHB) in the absence of severe cardiac malformation. This rare disorder is closely linked to transplacental transport of anti-SSA/Ro and anti-SSB/La maternal antibodies. CURRENT KNOWLEDGE AND KEY POINTS: The prevalence of CHB in newborns of anti-Ro/SSA positive women with known connective tissue disease is 2% and the risk of recurrence ranges from 10 to 17%. Skin and systemic lesions are transient, whereas CHB is definitive and is associated with significant morbidity and mortality (estimated at 16-19%). A pacemaker must be implanted in 2/3 of cases. Myocarditis may be associated or may appeared secondarily. Mothers of children with CHB are usually asymptomatic or have Gougerot-Sjögren, or undifferentiated connective tissue disease. Mothers of children with cutaneous manifestations may present with more severe disease and systemic lupus erythematosus. In anti-Ro/SSA positive pregnant women, echocardiograms should be performed at least every 2 weeks from 16 to 24 weeks gestation. Electrocardiogram should be performed for all children. FUTURE PROSPECTS AND PROJECTS: The efficiency of prophylactic treatment of CHB is not established. Therapy for CHB detected in utero is not standardized and involves fluorinated steroids (especially betamethasone).

Causality↗
PubMed2003

Questions about dexamethasone use for the prevention of anti-SSA related congenital heart block.

BACKGROUND: Mothers with anti-SSA/Ro antibodies who have had a previous fetus with congenital heart block (CHB) have a risk of recurrence estimated to be up to 16%. OBJECTIVE: To improve the management of these "high risk patients" by determining (a) whether or not prophylactic treatment is efficient; (b) whether or not fluorinated steroids (betametasone and dexamethasone) that do cross the placenta in an active form are safe for the fetus; and (c) which prophylactic treatment should be used. METHODS: Retrospective study performed on seven mothers sent to a university hospital owing to a past history of one (six mothers) or two children (one mother) with CHB. RESULTS: 13 subsequent pregnancies occurred. No CHB was observed. All four pregnancies in women treated with 10 mg/day prednisone were uneventful. Three pregnancies in women receiving no steroids resulted in two early spontaneous abortions and one live birth. The six pregnancies in women treated with dexamethasone (4-5 mg/day) ended in one early and one late spontaneous abortion, two stillbirths, and two live births with intrauterine growth restriction and mild adrenal insufficiency. A histological study of one stillbirth disclosed intrauterine growth restriction and marked adrenal hypoplasia. CONCLUSION: Adverse obstetric outcomes were often seen here and major concerns have been raised by paediatricians about the safety of fluorinated steroids, owing to the results of animals studies, retrospective data, and randomised trials. Because fluorinated steroids have not been shown to improve prophylactic treatment of CHB in pregnant women at high risk, their use is questionable.

Adrenal Insufficiency↗
PubMed2003

Can procalcitonin measurement help in differentiating between bacterial infection and other kinds of inflammatory processes?

OBJECTIVE: To study the levels of procalcitonin (PCT) in various inflammatory states seen in an internal medicine department and to evaluate the possible discriminative role of PCT in differentiating bacterial infection from other inflammatory processes. METHODS: PCT, C reactive protein (CRP), and white blood cell count (WBC) were measured in patients admitted to the department for fever or biological inflammatory syndrome, or both. The serum of 173 consecutive patients was analysed according to the aetiological diagnosis. The patients were divided into two groups: group I (n=60) with documented bacterial or fungal infection; group II (n=113) with abacterial inflammatory disease. RESULTS: PCT levels were >0.5 ng/ml in 39/60 (65%) patients in group I. In group II, three patients with a viral infection had slightly increased PCT levels (0.7, 0.8, and 1.1 ng/ml) as did two others, one with crystal arthritis and the other with vasculitis (0.7 ng/ml in both cases). All other patients in group II had PCT levels <0.5 ng/ml. In this study a value of PCT >0.5 ng/ml was taken as the marker of bacterial infection (sensitivity 65%, specificity 96%). PCT values were more discriminative than WBC and CRP in distinguishing a bacterial infection from another inflammatory process. CONCLUSION: PCT levels only rose significantly during bacterial infections. In this study PCT levels >1.2 ng/ml were always evidence of bacterial infection and the cue for starting antibiotic treatment.

Aged↗
PubMed2003

Long term outcome of catastrophic antiphospholipid syndrome survivors.

BACKGROUND: Catastrophic antiphospholipid syndrome (APS) is defined as life threatening multiple organ thromboses developing simultaneously or over a short period. The survival rate of catastrophic APS is about 50%, but the long term outcome of patients who survive is unknown. OBJECTIVE: To determine the long term outcome of patients with catastrophic APS and provide further information on patients who survived. PATIENTS AND METHODS: The clinical characteristics and outcomes of 130 patients with catastrophic APS have been reported previously. Six new cases were recently added to this series. Based on these publications, the authors who reported patients who had survived were contacted. Each author was asked (a) what treatment they gave their patients after the catastrophic APS; (b) if their patients had any further thrombosis. RESULTS: 63/136 (46%) patients died at the initial event. Of the remaining 73 patients, information was available for 58 (79%). Thirty eight (66%) patients did not develop further APS related events during an average follow up of 67.2 months. Eleven (19%) patients developed further APS related events but were still alive. No patients developed further catastrophic APS. Nine (16%) patients died: due to multiple organ failure (three patients); myelofibrosis (one); pneumonia (one); and APS related events (four). CONCLUSION: Sixty six per cent of patients who survive an initial catastrophic APS event remained symptom free with anticoagulation during an average follow up of 67.2 months. Twenty six per cent of the survivors developed further APS related events and the mortality rate of these patients was about 25%.

Adolescent↗
PubMed2003

Venous thromboembolism in the antiphospholipid syndrome: management guidelines for secondary prophylaxis.

Venous thromboembolism (VTE) in patients suffering from the antiphospholipid syndrome (APS) has been reported in almost any location of the vessel tree and the risk of recurrences has been found in several studies to be more closely associated with the presence of lupus anticoagulant than with the positivity for anti-cardiolipin antibodies. The thrombophilic state of APS raises the problem of the secondary prophylaxis to avoid VTE recurrences. For APS patients with VTE, published data appear to support a longer warfarin treatment if compared with the standard management of antiphospholipid (aPL)-negative patients with VTE. The question of how long oral anticoagulant treatment should be continued for APS patients, however, remains unanswered. Concerning the intensity of anticoagulation, several authors recommend a target international normalized ratio (INR) between 3.0 and 4.0 to efficiently protect from VTE recurrences. A recent decision analysis study does support such a suggestion. On the contrary, in a few prospective studies regimens with lower target INRs appear to be effective, and some authors therefore recommend a target INR of between 2.0 and 3.0. Specific large and prospective trials are needed to address this question. Until such information becomes available, individualized treatment according to the patient's individual risk factors for both bleeding and thrombosis is the general practice.

Administration, Oral↗
PubMed2002

Lessons from the "Euro-Lupus Cohort".

The "Euro-Lupus Cohort" is composed by 1,000 patients with systemic lupus erythematosus (SLE) that have been followed prospectively since 1991. These patients have been gathered by a European consortium - the "Euro-Lupus Project Group". This consortium was originated as part of the network promoted by the "European Working Party on SLE", a working group created in 1990 in order to promote research in Europe on the different problems related to this disease. The "Euro-Lupus Cohort" provides an updated information on the SLE morbidity and mortality characteristics in the present decade as well as defines several clinical and immunological prognostic factors.

Adolescent↗
PubMedDate not supplied

Infliximab in refractory uveitis due to Behçet's disease.

OBJECTIVE: To report 4 cases of refractory panuveitis due to Behçet's disease treated with a novel therapy: infliximab. METHODS: Retrospective study of 3 women and 1 man of Causasian origin with Behçet's disease complicated with panuveitis. Their uveitis was relapsing from 48 to 96 months and was resistant to the combination of colchicine (n = 4), high-dose prednisone (n = 4), pentoxyphilline (n = 2) and various immunossuppressors and/or immunomodulators given successively: intravenous cyclophosphamide (n = 4), azathioprine (n = 3), interferon alpha (n = 3), cyclosporine A (n = 2), oral cyclophosphamide (n = 1), mycophenolate mofetil (n = 1), methotrexate (n = 1), high-dose immunoglobulin (n = 1). Combination with respectively 1, 3, 4 and 5 immunossuppressors and/or immunomodulators failed before institution of infliximab. After informed consent was obtained, infliximab was administered as a single infusion of 5 mg/kg (maximum dose: 400 mg) at day 1, at week 2, 6 and then every 8 weeks. RESULTS: With a follow-up ranging from 7 to 22 months, infliximab was efficient in all cases. The mean prednisone dose decreased from 45 mg to 13 mg daily. Total recovery of visual acuity was observed in half of the cases. Infliximab was well tolerated without fever, severe sepsis or autoimmune manifestation. CONCLUSION: Infliximab may be efficient in refractory uveitis due to Behçet's disease. The optimal dose, rhythm and duration of infliximab infusions need to be standardized.

Adolescent↗
PubMedDate not supplied

Subacute bacterial endocarditis presenting as polymyalgia rheumatica or giant cell arteritis.

OBJECTIVE: To report on several patients with subacute bacterial endocarditis who were initially presumed, incorrectly, to have polymyalgia rheumatica or giant cell arteritis. METHODS: We report 3 cases of subacute streptococcal endocarditis mimicking giant cell arteritis in 2 cases and polymyalgia rheumatica in one. We reviewed the literature through Medline search of French and English-language articles published between 1966 and 2005 and found 5 similar cases. RESULTS: Shoulder and/or pelvic girdle pain was associated with neck or back pain in all patients. Scalp tenderness, bilateral jaw pain, amaurosis fugax were present in 2 patients. One patient had no fever. Two patients were treated with corticosteroids with initial good clinical response in one. Appropriate antibiotic therapy resulted in the rapid disappearance of rheumatic complaints in 2 patients and achieved a definitive cure of endocarditis in all cases. CONCLUSION: Rheumatologic symptoms may hinder the correct diagnosis of infective endocarditis in patients who present with a clinical picture suggesting polymyalgia rheumatica or giant cell arteritis. In such cases, blood cultures should be systematically drawn.

Adrenal Cortex Hormones↗