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Pierre-Yves Hatron

Publications and source records attributed to Pierre-Yves Hatron.

14 recordsLinked to original sources
PubMed2006

Recovery from deafness in a patient with Muckle-Wells syndrome treated with anakinra.

Muckle-Wells syndrome (MWS) is a dominantly inherited autoinflammatory disease characterized by rashes, fever, arthralgia, sensorineural deafness, and the possible development of systemic AA amyloidosis. We used anakinra to treat a 22-year-old patient with MWS who had deafness and a high serum level of C-reactive protein (CRP). Following treatment with anakinra, the patient's CRP level normalized, and she recovered from deafness. The fact that this occurrence has never been previously reported strengthens the role of anakinra in MWS but also raises new questions about the physiopathology of such deafness.

Adult↗
PubMed2006

[Bosentan for treatment of active digital ulcers in patients with systemic sclerosis].

OBJECTIVES: To describe the effect of bosentan and its dual inhibition of endothelin-1 ETA and ETB receptors on digital ulcers in patients with systemic sclerosis (SSc). METHODS: Patients receiving bosentan for SSc-related digital ulcers were identified in eight centers, and their characteristics and follow-up were recorded. RESULTS: Nine (six with diffuse and three with limited cutaneous forms of SSc) patients (median age: 54 years) had received bosentan for digital ulcers. Complete healing occurred in seven (median time to improvement: 4 weeks). Another experienced a significant decrease in the number of ulcers (from 22 to 5) in 8 weeks, while one had no improvement. After a median follow-up of 24.3 months, only one recurrence was observed. Raynaud phenomenon improved in all but one patient. DISCUSSION: These data suggest that some patients may benefit from bosentan to treat digital ulcers. The short time to healing in these patients with rather chronic ulcers argues strongly in favor of its use. These results also strengthen the evidence that endothelin-1 plays an important role in the vascular manifestations of SSc. CONCLUSION: Bosentan can be effective in the treatment of digital ulcers in some SSc patients with SSc, probably especially those involving substantial ischemia. Bosentan is not a first-line drug in this indication yet and must be carefully used by specialists in SSc. Forthcoming results from the international RAPIDS-2 study should clarify the indications for bosentan in the treatment of SSc-related digital ulcers.

Adult↗
PubMed2006

[Takayasu arteritis].

Takayasu arteritis is an inflammatory arteritis affecting large vessels, predominantly the aorta and its main branches. Thickening of the vessel wall is an early hallmark of the disease and leads to stenosis, thrombosis, and sometimes aneurysm formation. Reported incidence ranges from 1.2 to 2.6/million/year. Women aged 20 to 40 are most likely to suffer from the disease than men. Manifestations are very polymorphous, with presentations ranging from asymptomatic to neurologic catastrophes. Prognosis depends essentially on complications (retinopathy, hypertension, aneurysm, aortic insufficiency) and initial disease aggressivity. Diagnosis is based on imaging methods. Doppler ultrasound, computed tomography, and magnetic resonance imaging are fast and reliable methods for assessing vessel anatomy and luminal status. Positron emission tomography with fluorodeoxyglucose appears to be a highly sensitive and effective method for detecting disease activity, especially since standard inflammatory markers seem ineffective. Until now, corticosteroids have been the treatment of choice. If remission does not occur, methotrexate is added. Percutaneous transluminal angioplasty and sometimes vascular surgery is necessary in cases of critical ischemia or threatening aneurysm. Duration of treatment, choice of second-line treatment, and protocol for tapering medication currently depend more on experience than on evidence-based medicine. Multicenter studies are needed to guide future practice.

Diagnosis, Differential↗
PubMed2006

Myelopathies secondary to Sjögren's syndrome: treatment with monthly intravenous cyclophosphamide associated with corticosteroids.

OBJECTIVE: Central nervous system manifestations in Sjögren's syndrome (SS) include focal deficits, optic neuritis, and myelopathies. Acute and chronic myelopathies are frequently severe and sometimes respond poorly to corticosteroids. The efficacy of intravenous (IV) cyclophosphamide (CYC) has been suggested in single case reports. METHODS: We describe the potential usefulness of IV CYC in SS patients with severe myelopathies. Fourteen patients [with acute (n = 6) and chronic (n = 8) myelopathies] were treated with monthly CYC infusions (700 mg/m2) in addition to 500 mg of corticosteroids for one year. We evaluated the disability before and after CYC treatment using a walking distance calculation and the Expanded Disability Status Scale (EDSS). RESULTS: CYC treatment was well tolerated in all cases without serious adverse events. Nine patients (including the 6 with acute myelopathy) were improved after CYC treatment. Three patients were stabilized and 2 patients with chronic myelopathies had moderate progression of disability. The mean walking distance increased from 48.2 m before to 180.4 m after CYC treatment (p < 0.02). Mean EDSS score decreased from 6.6 to 5.7 (not significant). We found a correlation between the length of time before CYC treatment and clinical improvement for both the walking distance (p < 0.02) and the EDSS score (p < 0.05). CONCLUSION: Although a randomized multicenter controlled study is warranted to confirm our findings, IV CYC infusions seem to be useful for the treatment of myelopathies secondary to SS, particularly in acute but also in progressive cases. This treatment should be strongly considered as soon as possible when disease progression is observed.

Acute Disease↗
PubMed2006

Significance of persistent antiphospholipid antibodies in the elderly.

OBJECTIVE: The prevalence of anticardiolipin antibodies (aCL) and of vascular diseases increases with age, and aCL may be associated with various diseases in the elderly. So the significance of aCL in the elderly remains difficult to determine. We sought to determine the significance of persistent antiphospholipid antibodies (aPL) in the elderly. METHODS: We retrospectively analyzed the files of 327 patients [149 patients with antiphospholipid syndrome (APS); 64 patients age >or= 65 yrs] with 2 positive aPL [lupus anticoagulant (LAC) and/or aCL]. RESULTS: The frequency of APS was 40.8% (n = 134) in our 263 young patients (< 65 yrs) and 23.4% (n = 15) in our 64 elderly patients (>or= 65 yrs). The clinical characteristics of patients with persistent aPL were the same in those under and over 65 years. LAC was positive in all but one elderly patient with APS, and occurred in this group more frequently than in the young patients (93.3% vs 44.6%; p < 0.006). The presence of LAC allowed to discriminate APS patients in our elderly population (93.3% in APS vs 48.9% in non-APS patients; p < 0.009). CONCLUSION: Interpretation of a positive determination of APL is difficult in the elderly; persistent LAC may be the most valuable biological marker of APS in the elderly.

Aged↗
PubMed2006

High resolution computed tomography in fibrosing alveolitis associated with systemic sclerosis.

OBJECTIVE: To investigate the use of high resolution computed tomography (HRCT) in diagnosis of patients with fibrosing alveolitis associated with systemic sclerosis (FA-SSc), and to determine predictors of disease progression. METHODS: We retrospectively studied 90 patients with SSc who had undergone an initial (Time 1) and followup (Time 2) clinical and HRCT evaluation, with a mean +/- SD interval of 5.14 +/- 2.98 years between T1 and T2. RESULTS: At T1, HRCT was normal in 40 patients; at T2, 34/40 (85%) continued to have a normal HRCT. For the 50 patients with FA-SSc on HRCT scan at T1, the overall disease progression comprised extension of lung changes toward the apices with worsening of lung fibrosis at T2. Among the 37 patients who had areas of isolated ground-glass opacities at T1, 25 (68%) had progression of lung fibrosis at T2. These 25 patients were mostly men, who showed a more marked decrease of diffusing capacity and a longer interval between T1 and T2. CONCLUSION: The results emphasize the good longterm prognosis indicated by a normal initial HRCT in SSc. Patients with FA-SSc with abnormal HRCT experienced progressive replacement of ground-glass opacities by honeycombing and/or traction bronchiectasis/bronchiolectasis. Ground-glass opacity is probably the first step of lung fibrosis in SSc, and treatment should be discussed even at this early stage.

Adult↗
PubMed2005

Use of low-molecular-weight heparin from the first trimester of pregnancy: a retrospective study of 111 consecutive pregnancies.

BACKGROUND: During the first trimester of pregnancy, unfractionated heparin is the standard anticoagulant treatment for pregnant women at high risk of thrombosis. OBJECTIVE: To observe maternal and fetal tolerance for low-molecular-weight heparin begun in the first trimester of pregnancy. METHODS: Observational study conducted from 1 January 1997 to 31 May 2001. All patients began treatment before the 15th week of pregnancy. The outcome measures were the incidence and causality of adverse events in mother and fetus. RESULTS: The study included 97 patients (and 111 pregnancies) at very high risk for thrombosis. Seven fetal losses (6.3%) were observed: three early spontaneous abortions, three late spontaneous abortions and one medically indicated abortion. Twenty-five (22.5%) bleeding events occurred during pregnancy, seven (6.3%) of which required medical intervention: five curettages for first trimester spontaneous abortions, one late abortion at 21 weeks and one placental abruption at 25 weeks. Of nine (8.1%) primary postpartum hemorrhages involving a blood loss > or = 500 mL, three involved losses of 1000 mL or more and one required embolization of the uterine arteries. Five patients had thrombocytopenia, but none was treatment-related. Local cutaneous reactions occurred in 33 (29.7%) patients. Six (5.4%) maternal thromboembolic complications occurred during pregnancy or postpartum. At birth, two children had non-chromosomal congenital malformations (pyelectasia, cleft lip and palate). No fetal or neonatal complication was attributed to the treatment. CONCLUSION: The use of low-molecular-weight heparin (LMWH) for patients requiring anticoagulant treatment from the first trimester appears safe for mother and fetus.

Abortion, Induced↗
PubMed2005

Opportunistic infections in polymyositis and dermatomyositis.

OBJECTIVE: To assess prevalence and characteristics of opportunistic infections in patients with polymyositis/dermatomyositis (PM/DM). To determine the predictive values for opportunistic infections on clinical presentation, biochemical findings, and paraclinical features of PM/DM to detect patients at risk of opportunistic infections. METHODS: The medical records of 156 consecutive PM/DM patients in 3 medical centers were reviewed. RESULTS: Eighteen PM/DM patients (11.5%) developed opportunistic infections. The majority of patients exhibited an opportunistic infection after the onset of PM/DM (89% of cases). Opportunistic infections occurred most frequently during the first year following PM/DM diagnosis (62.5%). The pathogen microorganisms responsible for opportunistic infections were various, i.e., Candida albicans, Pneumocystis carinii, Aspergillus fumigatus, Geotrichum capitatum, Mycobacterium avium-intracellulare complex, M. xenopi, M. marinum, M. tuberculosis, Helicobacter heilmanii, cytomegalovirus, and herpes simplex virus. Mortality rates were as high as 27.7% in these PM/DM patients. Higher mean daily doses of steroids, lymphopenia, and lower serum total protein levels were significantly more frequent in the group of PM/DM patients with opportunistic infections. CONCLUSION: Our study underscores the high frequency of opportunistic infections in PM/DM, resulting in an increased mortality rate. It also indicates that a great variety of microorganisms are responsible for opportunistic infections, although they were more often due to fungi (>50% of cases). Our series highlights a predominance of both lung and digestive opportunistic infections (89% of cases). In addition, our results suggest that PM/DM patients presenting with factors predictive of opportunistic infection may require closer monitoring.

Adult↗
PubMed2004

Association between systemic sclerosis and breast cancer: eight new cases and review of the literature.

Several studies have demonstrated an increased frequency of cancer in patients with systemic sclerosis (SSc), specially lung and breast cancers. The pathogenesis of the association between SSc and cancer is not fully established. The aim of this study was to describe new cases of the association between SSc and breast cancer and to perform a review of the literature. We retrospectively studied the medical files of eight patients followed in our institution for SSc and breast cancer. We analyzed them with data available in the literature for a total of 46 patients. Cutaneous extension of SSc was clearly mentioned in 17 cases: the SSc was limited in 10 cases and diffuse in 7 cases The median age at the diagnosis of cancer was 54 years (range: 40-71). The median duration between SSc onset and breast cancer diagnosis was 11.5 months (range: 0-288). The duration between SSc onset and breast cancer diagnosis was < or = 12 months in 27 of 44 patients (61.4%), and in 11 (25%) of them the diagnosis of both diseases was made simultaneously. It was clearly mentioned for 35 patients whether the diagnosis of breast cancer was made before or after the onset of SSc. The diagnosis of breast cancer was made before SSc onset in 17 of 35 patients (48.6%) and after SSc onset in 18 of 35 patients (51.4%). For 33 patients, the follow-up was available: 18 (54.5%) died, 11 (33.3%) of them within the 1st year after the diagnosis of the cancer. For none of the patients did the anticancer treatment improve the SSc. The close temporal relationship between SSc onset and breast cancer diagnosis is highly suggestive of a pathophysiological link. SSc is probably not a paraneoplastic disease since the anticancer treatment has no influence on the evolution of SSc. However, it can be suggested that SSc could be a disease facilitating breast cancer and/or metastases development.

Adult↗
PubMed2004

Neurologic manifestations in primary Sjögren syndrome: a study of 82 patients.

Neurologic involvement occurs in approximately 20% of patients with primary Sjögren syndrome (SS). However, the diagnosis of SS with neurologic involvement is sometimes difficult, and central nervous system (CNS) manifestations have been described rarely. We conducted the current study to describe the clinical and laboratory features of SS patients with neurologic manifestations and to report their clinical outcome. We retrospectively studied 82 patients (65 women and 17 men) with neurologic manifestations associated with primary SS, as defined by the 2002 American-European criteria. The mean age at neurologic onset was 53 years. Neurologic involvement frequently preceded the diagnosis of SS (81% of patients). Fifty-six patients had CNS disorders, which were mostly focal or multifocal. Twenty-nine patients had spinal cord involvement (acute myelopathy [n = 12], chronic myelopathy [n = 16], or motor neuron disease [n = 1]). Thirty-three patients had brain involvement and 13 patients had optic neuropathy. The disease mimicked relapsing-remitting multiple sclerosis (MS) in 10 patients and primary progressive MS in 13 patients. We also recorded diffuse CNS symptoms: some of the patients presented seizures (n = 7), cognitive dysfunction (n = 9), and encephalopathy (n = 2). Fifty-one patients had peripheral nervous system involvement (PNS). Symmetric axonal sensorimotor polyneuropathy with a predominance of sensory symptoms or pure sensory neuropathy occurred most frequently (n = 28), followed by cranial nerve involvement affecting trigeminal, facial, or cochlear nerves (n = 16). Multiple mononeuropathy (n = 7), myositis (n = 2), and polyradiculoneuropathy (n = 1) were also observed. Thirty percent of patients (all with CNS involvement) had oligoclonal bands. Visual evoked potentials were abnormal in 61% of the patients tested. Fifty-eight patients had magnetic resonance imaging (MRI) of the brain. Of these, 70% presented white matter lesions and 40% met the radiologic criteria for MS. Thirty-nine patients had a spinal cord MRI. Abnormalities were observed only in patients with spinal cord involvement. Among the 29 patients with myelopathy, 75% had T2-weighted hyperintensities. Patients with PNS manifestations had frequent extraglandular complications of SS. Anti-Ro/SSA or anti-La/SSB antibodies were detected in 21% of patients at the diagnosis of SS and in 43% of patients during the follow-up (mean follow-up, 10 yr). Biologic abnormalities were more frequently observed in patients with PNS involvement than in those with CNS involvement (p < 0.01). Fifty-two percent of patients had severe disability, and were more likely to have CNS involvement than PNS involvement (p < 0.001). Treatment by cyclophosphamide allowed a partial recovery or stabilization in patients with myelopathy (92%) or multiple mononeuropathy (100%). The current study underlines the diversity of neurologic complications of SS. The frequency of neurologic manifestations revealing SS and of negative biologic features, especially in the event of CNS involvement, could explain why SS is frequently misdiagnosed. Screening for SS should be systematically performed in cases of acute or chronic myelopathy, axonal sensorimotor neuropathy, or cranial nerve involvement. The outcome is frequently severe, especially in patients with CNS involvement. Our study also underlines the efficacy of cyclophosphamide in myelopathy and multiple neuropathy occurring during SS.

Adult↗
PubMed2004

Multiplex reverse transcription polymerase chain reaction assessment of sialyltransferase expression in peripheral blood mononuclear cells in systemic sclerosis.

OBJECTIVE: To assess sialyltransferase expression in peripheral blood mononuclear cells (PBMC) of patients with systemic sclerosis (SSc) and to correlate this expression with the clinical features of the disease. METHODS: Using a multiplex reverse transcription polymerase chain reaction (RT-PCR) method, we simultaneously measured the expression of 5 sialyltransferases (ST3Gal IV, ST3Gal III, ST3Gal I, ST3Gal II, and ST6Gal I) and of one reference housekeeping gene, Tata box binding protein (TBP), in PBMC of 28 patients with SSc and 18 healthy controls. Expression of each sialyltransferase was defined by the ratio sialyltransferase amplification product intensity/TBP amplification product intensity, and was evaluated according to the skin sclerosis extension and the presence of lung fibrosis and/or of pulmonary hypertension. RESULTS: ST3Gal I and ST6Gal I expressions were lower in patients with SSc than in healthy controls (median 0.48 vs 1.30, p < 0.0001, and 0.71 vs 1.96, p < 0.01, respectively). No difference was observed for ST3Gal III and ST3Gal IV expression. ST3Gal IV/ST6Gal I ratio was higher in SSc patients than in controls (0.37 vs 0.28, p = 0.03). ST3Gal II was either weakly or more often not expressed in both groups. Ten patients had isolated pulmonary hypertension. They had higher ST3Gal IV expression than patients without pulmonary hypertension (0.73 vs 0.29, p = 0.04) and a higher ST3Gal IV/ST6Gal I ratio than patients without pulmonary hypertension (1.03 vs 0.27, p = 0.03) and controls (1.03 vs 0.28, p = 0.02). No difference was found in the sialyltransferase expression and soluble E-selectin concentration according to the cutaneous sclerosis extension or the presence of lung fibrosis. CONCLUSION: Sialyltransferase expression is modified in PBMC of patients with SSc compared to healthy subjects. Low ST6Gal I expression associated with normal ST3Gal IV expression, resulting in a higher ST3Gal IV/ST6Gal I ratio, suggests an enhanced expression of Sialyl-LewisX at the surface of PBMC in SSc, and therefore an active interaction between activated endothelial cells and PBMC through the binding between E-selectin and Sialyl-LewisX. This suggested that higher expression of Sialyl-LewisX concerned patients with isolated pulmonary hypertension more specifically. Binding between PBMC surface Sialyl-LewisX and activated endothelial cell E-selectin might therefore play a role in the pathogenesis of SSc-related isolated pulmonary hypertension.

Adult↗
PubMed2004

Autoantibodies against alpha-fodrin in Sjögren's syndrome with neurological manifestations.

OBJECTIVE: To investigate the diagnostic value of autoantibodies against alpha-fodrin in patients with Sjögren's syndrome (SS) with neurological manifestations compared to SS patients without neurological manifestations, a control group, and patients with other neurological autoimmune diseases including systemic lupus erythematosus (SLE) with neurological manifestations and multiple sclerosis (MS). METHODS: We evaluated alpha-fodrin autoantibodies in 31 patients with SS with neurological manifestations, 53 SS patients without neurological symptoms, 38 patients with SLE, 60 with MS, and 160 controls. RESULTS: Twenty of the 31 SS patients with neurological manifestations (64.5%) had an increased concentration of IgA and/or IgG anti-alpha-fodrin. This was not statistically different from that of SS patients without neurological symptoms (73.6%), but was higher than the number with SSA/SSB antibodies, which were found in 15 (48%) of our SS patients without neurological manifestations. When the results of the 2 tests were combined, 28 of the 31 (90.3%) patients had positive autoantibodies (alpha-fodrin and/or SSA/SSB). Alpha-fodrin antibodies were increased in 8 (13.3%) of the 60 patients with MS, in 6 (15.7%) of 38 patients with SLE, and in 10 (6.3%) of 160 controls. CONCLUSION: Our results confirm that alpha-fodrin antibodies are an additional diagnostic tool for SS. This test is of particular interest for patients with SS with neurological manifestations, in whom anti SSA/SSB antibodies are less frequently found.

Adult↗