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E Hachulla

Publications and source records attributed to E Hachulla.

At least 55 records · Page 3Linked to original sources
PubMed2002

[Antiphospholipid syndrome].

Since 1987 the antiphospholipid syndrome has been recognized as a major cause of acquired thrombophilia, whether it is associated with systemic lupus erythematosus or occurs as a free-standing syndrome (primary form). This autoimmune condition associates in young patients recurrent thrombosis (both venous and/or arterial) and/or a variety of obstetric complications with the persistent presence of antiphospholipid antibodies (aPL). These traditionally comprise anticardiolipin antibodies and lupus anticoagulants, respectively detected by immunological and clotting tests. Despite their name aPL do not bind to phospholipids per se, but are directed at phospholipid-binding plasma proteins, especially beta 2-glycoprotein I and prothrombin. Because the risk of recurrence is high, the standard of care is prolonged and high-intensity warfarin (INR near 3) after a venous thromboembolic event, together with the management of associated vascular risk factors. Prevention of adverse obstetric outcomes is frequently achieved by a combination of low-dose aspirin and heparin.

Adult↗
PubMed2002

[Rheumatoid purpura in adults and parvovirus B19 infection: fortuitous association or parvovirus B19-induced vasculitis?].

INTRODUCTION: Henoch-Schoenlein purpura has been reported to be associated with parvovirus B19 infection, particularly in children and rarely in adults. We report the case of a 42-year-old patient presenting with this association. EXEGESIS: A 42-year-old patient was admitted to our medical center because of lower limb purpura. Henoch-Schoenlein purpura diagnosis was confirmed on histological findings (kidney biopsy) and concomitantly parvovirus B19 infection was proved by serological test (IgM+). Association of Henoch-Schoenlein purpura and parvovirus B19 infection has already been described. However, none of the reported studies demonstrated clearly the link between these two diseases. With regard to this observation, we wonder about the systematic use of the parvovirus B19 serological test in patients presenting first Henoch-Schoenlein purpura. Indeed, parvovirus B19-induced vasculitis is habitually controlled with intravenous immunoglobulins. CONCLUSION: A prospective study should explore the link between Henoch-Schoenlein purpura and primary parvovirus B19 infection. Moreover, we should evaluate intravenous immunoglobulins' efficacy in Henoch-Schoenlein purpura associated with active parvovirus B19 infection in order to improve the prognosis of this disease.

Adult↗
PubMed2002

[Camptocormia: a sign of axial myopathy. Report of 7 cases].

PURPOSE: Camptocormia or progressive lumbar kyphosis is an anterior bend of the trunk. It appears in orthostatism or while walking and is reducible in the decubitus position. It concerns patients older than 60 years of age. It is due to a fatty degeneration of the paravertebral muscles, although the physiopathology remains unclear. METHODS: We report seven cases of camptocormia revealing authentic myopathies. RESULTS: Our observations concern five women and two men of 55 to 72 years of age. All patients present lumbar kyphosis and had a fatty involution of the paraspinal muscles on the muscular MRI. Four patients fulfilled the Bohan and Peter criteria of polymyositis and dermatomyositis. In the other cases paravertebral muscular biopsies led to the diagnosis of a congenital myopathy, a mitochondrial myopathy and an amyloid myopathy. Four patients received a corticosteroid-immunoglobulins or cyclosporin regimen. An improvement in the camptocormia was observed in three cases. In the other cases the treatment consisted of chemotherapy on account of severe nephrotic syndrome, a coenzyme-Q treatment for the patient with mitochondrial myopathy and only physiotherapy in the case of congenital myopathy, but without positive effect on camptocormia. CONCLUSION: Camptocormia appears as a muscular symptom that may reveal an axial myopathy due to multiple and varied pathologies. Thus, the discovery of camptocormia requires an aetiological investigation in order to propose an adequate treatment, which should be associated with physiotherapy.

Aged↗
PubMed2002

[Unusual association: hepatic peliosis and Crohn's disease].

INTRODUCTION: Hepatobiliary disorders occurs in about 10 to 30% of inflammatory bowel diseases. Fatty liver and gallstones are preferentially described with Crohn's disease. Conversely, peliosis hepatis has been only exceptionally reported in association with inflammatory bowel diseases. We describe here a case of peliosis hepatis complicating a misdiagnosed Crohn's disease. EXEGESIS: A 24-year-old woman was referred because of a hepatomegaly and a deterioration of the general state. The patient complained about diarrhea for 3 months and about an episode of bloody stools. There was an aphthous-like lesion on the tongue. X-ray pull-through revealed a typical aspect of terminal ileitis as seen in Crohn's disease. The liver was heterogeneous. Liver biopsy revealed a peliosis hepatis. No other possible cause of peliosis hepatis was found. Therefore, in this case, peliosis hepatis, revealed by the hepatomegaly, was associated with Crohn's disease. CONCLUSION: Association between Crohn's disease and peliosis hepatis has only exceptionally been described. A priori, this complication is not a pejorative feature in inflammatory bowel disease. The pathophysiological significance of this association remains unknown.

Adult↗
PubMed2002

[The antisynthetase syndrome: a subgroup of inflammatory myopathies not to be unrecognized].

PURPOSE: Antisynthetase syndrome (AS) is frequently revealed by interstitial lung disease and arthritis. There are mechanic's hand, Raynaud's phenomenon and anti aminoacyl t-RNA synthetase antibodies. The anti JO-1 antibody is the most frequently identified. We report five cases of antisynthetase syndrome with particular clinical features and good response to corticosteroids. METHODS: There are three women and two men with a median age of 59 years at presentation (range: 44-77). Three patients progressively developed AS: the symptoms are dyspnea (three). Raynaud's phenomenon (one), purpura (one) and hyperkeratosis, scaling and fissuring on the lateral sides of the fingers (two). Patients always had skin signs: hyperkeratosis and scaling (five), purpura (one), Raynaud's phenomenon with normal capillaroscopy (two). Lung disease is present in the five cases with interstitial lesions in CT scans (five), trouble of CO diffusion (three/three) and lymphocytic alveolitis (two/two). Moderate muscular disorders are present in five cases (moderate elevated muscular enzyme: five, positive muscle histology: two). Anti-JO-1 antibodies are present in five cases. AS is associated with connective tissue diseases: rheumatoid polyarthritis in one case and Gougerot-Sjögren in three cases. No malignant tumour is associated. Patients have received oral corticosteroid treatment (five/five) with high doses of intravenous perfusions (three/five) with, initially, a good response. For only one patient, immunosuppressive treatment was necessary because of the articular relapse. The interstitial lung disease had a good response to corticosteroids therapy alone in four cases. Because of the relapse during the tapering off of corticosteroids, corticosteroids were increased in one case and immunosuppressive therapy was required in one case. CONCLUSION: The prognosis of AS depends of the interstitial lung disease. High doses of corticosteroids are required. In our study, the response to corticosteroids is good. Immunosuppressive agents must be added in severe and progressive form of interstitial lung disease in AS.

Adrenal Cortex Hormones↗
PubMed2002

[Antiphospholipid syndrome with only antiphosphatidylethanolamine antibodies: report of 20 cases].

PURPOSE: The association of antiphosphatidylethanolamine antibodies (aPE) as the only antiphospholipid antibody with antiphospholipid syndrome (APS) is discussed. The aPE was described as the sole antibody in many cases suggesting APS. aPE was not included in the Sapporo criteria for the classification of APS. METHODS: We investigated the clinical features of 20 patients with aPE only; 17 patients had symptoms potentially related to APS (group 1) and three had other manifestations (group 2). RESULTS: There were 15 women and five men, mean age was 35 +/- 12 years at the beginning. In group 1 (n = 17), ten patients presented arterial thrombosis, nine venous thrombosis (five had both), and six microvascular thrombosis (livedo reticularis, lacunar pathology). The aPE positivity was persistent in 13 patients. A subgroup of four patients (three women) presented arteriosclerosis with peripheral arteriopathy which started before 45 years of age. They had another atherosclerosis risk factor associated with aPE persistence. In group 2 (n = 3), there was no thrombotic event, one demyelinating pathology, one microvascular pathology, and one arterial dysplasia. The aPE positivity was never confirmed. Finally, 13 patients presented an APS with aPE only, confirmed at least 8 weeks later. CONCLUSIONS: Our study points out that testing for aPE would be of interest for patients when symptoms were potentially related to APS, particularly when other antiphospholipid antibodies were negative. This description questions the enlargement of the APS biological criteria defined in Sapporo. The role of aPE in atherosclerosis is considered.

Adult↗
PubMed2002

[Systemic sclerosis and pregnancy].

PURPOSE: Pregnancy in a patient with systemic sclerosis (SSc) may pose a double problem to the medical team: influence of SSc on pregnancy and consequences of pregnancy to SSc manifestations. CURRENT KNOWLEDGE AND KEY POINTS: Concepts have evolved. SSc was considered for a long time not only as not very propitious for pregnancy but also as a strict contraindication for procreation because risks for the mother and the baby were thought to be major. Currently, fertility is thought to be normal. Miscarriages and small-for-gestation age infants rate do not seem to be higher in SSc. Maternal and perinatal mortality is also not higher in SSc without severe visceral manifestations, i.e. without either pulmonary hypertension, or cardiac or respiratory insufficiency. Conversely, there is a significantly higher frequency of premature infants in SSc. As regards influence of pregnancy on SSc, the greatest fear is the occurrence of renal crisis, which may be life threatening for both mother and child. Each elevation of blood pressure, even if this increase is mild, should be considered as potentially very serious. However, pregnancy itself does not seem to increase the risk of renal crisis. Consequences of pregnancy to SSc manifestations are various but usually mild. FUTURE PROSPECTS AND PROJECTS: SSc is not a strict contraindication for pregnancy only if severe organ involvement, diffuse subset of SSc or recent onset of the disease has been ruled out. Physicians should be aware of specific problems, which SSc is possibly posing during pregnancy. Finally, it has been recently suggested that pregnancies could be involved in the pathogenesis of SSc through persisting microchimerism of fetal origin.

Acute Disease↗
PubMed2002

[Cogan syndrome or sarcoidosis?].

INTRODUCTION: The Cogan's syndrome is characterized by the association of vestibulo-auditory dysfunction, non syphilitic interstitial keratitis or another significant inflammatory eye lesion. Some authors consider this disease as a vasculitis, because it is frequently associated with systemic manifestations. Based on Cogan's diagnostic criteria, Cogan's syndrome may be part of other systemic diseases, as polyarteritis nodosa or Wegener's granulomatosis. EXEGESIS: We report the case of a patient who presented with a Cogan's syndrome and developed further sarcoidosis. CONCLUSION: If Cogan's syndrome is characterized as systemic disease because of its association with aortitis or other vasculitis, on the other hand, clinical presentation may be part of many other systemic diseases.

Diagnosis, Differential↗
PubMed2002

[Psychiatric complications of corticoid therapy in the elderly over 65 years of age treated for Horton disease].

OBJECTIVES: To analyse steroid psychiatric related complications in aged (> 65 years old) with temporal arteritis (TA). METHODS: Retrospective cohort study. PATIENTS: In a cohort of 126 elderly patients with a diagnosis of TA and followed with a mean period of 64 months, clinical and biological presentations, outcome and corticoid adverse effects were recorded throughout the follow-up period. RESULTS: Twenty patients (16%), (mean age: 73 +/- 7.9 years, male n = 6) exhibited corticosteroid related psychiatric complications. Symptoms appear to be dose dependent and generally begin during the first month of treatment. Psychiatric disorders were as follow: mood disturbances (n = 8), depression (n = 6), mania (n = 3), anxiety neurosis (n = 2) and dementia (n = 1). Three patients were hospitalized in psychiatric units and 2 in nursing home. Psychiatric adverse affects appears to be more frequent with prednisone than prednisolone (P < 0.05).

Aged↗
PubMed2002

[Relevance of anti-nucleosome antibodies detected by enzyme-based immunoassays in lupus diagnosis. Comparative analysis of four commercial kits].

Among the biological assays used for the diagnosis of systemic lupus erythematosus (SLE), the detection of anti-double strand DNA antibodies (dsDNA Ab) is regarded as highly specific. However this biological parameter is negative among 20 to 40% of patients. Recent studies have revealed potential interest of the anti-nucleosome antibodies in the diagnosis of the lupus, in particular when any anti-dsDNA antibody activity could be detected. We selected 80 sera in order to evaluate four commercial anti-nucleosome enzyme-based immunoassays (EIA) kits. Their sensitivity and specificity values were compared with those obtained by the detection of anti-dsDNA Ab, carried out with both a Farr assay and two EIA kits. No anti-nucleosome EIA kits reached performances of the Farr assay for the diagnosis of lupus. On the other hand, our results show an higher diagnostic value for some anti-nucleosome EIA kits compared with 2 anti-dsDNA EIA kits. Apart from SLE, anti-nucleosome antibodies can be observed in others auto-immune diseases, in particular Sjögren's syndromes, the primary antiphospholipid syndrome, the systemic sclerosis and the mixed connective tissue disease. Compared results of the four anti-nucleosome EIA kits highlight many discordances. These variations, testifying to the absence of standardization for this new parameter, must encourage with a careful interpretation of results, according to the clinical context.

Adolescent↗
PubMed2002

Novel promiscuous HLA-DQ HIV Nef peptide that induces IFN-gamma-producing memory CD4+ T cells.

We describe the highly conserved sequence 56-68 of the HIV Nef protein as the first promiscuous HLA-DQ HIV-derived peptide. The Nef peptide exhibits an albeit rare capacity to bind 6 different HLA-DQ molecules whereas no binding is observed with the 10 HLA-DR molecules tested. In agreement with these data, after immunization with the Nef peptide, HLA-DQ transgenic Abeta degrees mice display a vigorous cellular and humoral response while the specific immune response of HLA-DR expressing mice is minimal. The promiscuous potentiality of the Nef 56-68 peptide in humans has been confirmed by ex vivo immunization experiments with CD4+ T cells from 14 healthy donors expressing different HLA genotypes. Nef 56-68 specific CD4+ T cells rapidly acquire a memory cell phenotype and are characterized by the preferential usage of the TCR Vbeta 6.1 gene segment and predominant production of IFN-gamma. Taken together, these data indicate that the Nef 56-68 peptide constitutes an attractive component of vaccines aiming at inducing or enhancing HIV-specific T cell immunity.

Alleles↗
PubMed2001

Long-term follow-up of neurosarcoidosis.

The authors evaluated the long-term clinical outcome of neurosarcoidosis and determined predictive factors of disease course. Twenty-seven patients with neurosarcoidosis were followed for at least 5 years from the onset of neurologic symptoms. Patients with CNS involvement during the course of the disease had a higher Modified Oxford Handicap Scale score than those with peripheral nervous system involvement (p < 0.02). CNS involvement may be a predictive factor for a less favorable disease course. Early and intensive treatment should be considered in such cases.

Adolescent↗
PubMed2001

[Wegener's granulomatosis in the elderly patient].

PURPOSE: To determine aged-related variations in clinical and biological presentation and outcome in Wegener's granulomatosis. METHODS: In a retrospective cohort study of 35 patients with a diagnosis of Wegener's granulomatosis, 24 patients (69%) younger than 60 years of age and 11 (31%) aged 60 years or older were compared for clinical and biological characteristics. RESULTS: Clinical presentation was the same in the two groups; lymphopenia was more common in the elderly group (P > 0.05). Despite a similar treatment regime, outcome was significantly worse for the elderly group (> or = 60 years), with a mortality rate of 36% versus 8% in the control group. Mortality was essentially due to delayed infectious complications, raising the problem of a less intensive immunosuppressive treatment after remission.

Adult↗