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Biomedical subjects

E Hachulla

Publications and source records attributed to E Hachulla.

At least 109 records · Page 6Linked to original sources
PubMed2000

Human pre-alpha-inhibitor is a positive acute-phase protein that is more susceptible than inter-alpha-inhibitor to proteolysis by stimulated neutrophils.

BACKGROUND: Pre-alpha-inhibitor (PalphaI) is a human plasma serine-proteinase inhibitor that is structurally related to inter-alpha-inhibitor (IalphaI). It is composed of a heavy chain named H3 covalently linked to bikunin by means of a glycosaminoglycan chain. We developed an ELISA procedure making it possible to measure PalphaI for the first time and we investigated its levels in sera from patients with inflammatory diseases. MATERIALS AND METHODS: We generated rabbit anti-H3 immunoglobulins, which were used on solid phase and biotinylated antibikunin immunoglobulins to detect trapped PalphaI. RESULTS: We demonstrate that PalphaI is more susceptible than IalphaI to in vitro proteolysis by stimulated neutrophils. However, the degradation products thus released as well as the other members of the IalphaI family present in serum do not affect the ELISA test. In a panel of control sera we observed PalphaI concentrations of 25.6 +/- 7.8 mg L-1 (mean +/- SD; n = 30). These values increased to 64.2 +/- 16.06 mg L-1 (mean +/- SD; n = 15) in patients with inflammatory diseases, concording with the positive acute-phase protein nature of PalphaI. However, for all these patients, the serum concentrations of PalphaI and C-reactive protein poorly correlated (r = 0.476; P = 0.076). Indeed, four patients had a relatively weaker increase in their PalphaI level than that of C-reactive protein. More often than not their plasma elastase content was then elevated. CONCLUSION: During inflammatory diseases plasma PalphaI levels may be dependent on increased synthesis in combination with enhanced catabolism, perhaps implicating neutrophil or other proteinases.

Acute-Phase Proteins↗
PubMed2000

Paraoxonase activity is dramatically decreased in patients positive for anticardiolipin antibodies.

It has been reported that paraoxonase 1 (PON1) activity inhibits low-density lipoprotein (LDL) oxidation and modulates the risk of coronary heart disease. This study shows that autoantibodies (IgG) directed against modified LDL were increased in 71 patients positive for anticardiolipin antibodies. In a representative subgroup of these patients (n = 36) PON1 activity was dramatically decreased and the prevalence of the RR genotype of this enzyme tended to be increased in patients who had developed arterial thrombosis. This study suggests that PON1 abnormalities play a role in the antiphospholipid syndrome.

Adult↗
PubMed2000

Hypothenar hammer syndrome followed by systemic sclerosis.

We describe the first case of bilateral hypothenar hammer syndrome (HHS) followed by systemic sclerosis (SSc) that was associated with silica exposure (Erasmus syndrome). The patient was a woman smoothing tiles in an earthenware factory who presented with bilateral digital ischemia associated with Raynaud's phenomenon. HHS was diagnosed, based on an angiographic study showing aneurysm of the ulnar arteries and occlusions of multiple digital arteries. Pulmonary silicosis was also diagnosed on pulmonary tomodensitometry. Two years later digital swelling with acroosteolysis developed. The FANA test was positive (titer 1:640) and anticentromere antibody tests were also positive. Esophageal manometry showed dysmotility of the lower esophagus. These findings were consistent with a diagnosis of SSc.

Adult↗
PubMed2000

[Clinical manifestations and therapeutic approach in neurosarcoidosis].

Neurological impairment is a frequent cause of morbidity and mortality in patients with sarcoidosis. The aim of this study was to evaluate the clinical manifestations of the disease, the response to corticosteroids and alternative treatments. During a 5 year period, diagnosis of neurosarcoidosis was performed in 40 patients. We retrospectively analyzed clinical, laboratory data and response to treatments. Mean age was 41.3 years (range 17-72). Mean time of follow-up was 46 months. Neurologic signs were the first symptom in 50 p. 100 of cases and an isolated manifestation in 12.5 p. 100. Central nervous system impairment was seen in 60.7 p. 100, meningitis in 27 p. 100. Other clinical manifestations were cranial nerve palsies (27 p. 100), peripheral neuropathy (33 p. 100), myopathy (16 p. 100). Eighty percent of the patients were treated by corticosteroids. Because of a lack of efficacy 40 p. 100 of patient required alternative treatment (including methotrexate, cyclophosphamide, azathioprin, cyclosporin). Complete recovery was observed in only 27.5 p. 100 of cases confirming the severity of neurosarcoidosis. Forty percent of patients were clinically stable and 10 p. 100 worsened. No patient died. This study confirms that intensive initial treatment is often necessary to prevent irreversible lesions. Alternative treatment should be rapidly initiated in resistant forms.

Adolescent↗
PubMed2000

[What vascular events suggest a myeloproliferative disorder?].

Patients with polycethemia vera (PV) or essential thrombocythemia (ET) are at increased risk of arterial and venous thromboembolic events. Arterial ischemic complications occur in 24 to 43% of these patients, particularly those with cardiovascular risk factors (especially cigarette smoking). Non-atheromatous arterial thrombosis concerns all large and medium-sized vessels, particularly cerebral, limb, coronary and digestive arteries. Extensive complications have been described in patients with lower limb occlusive arteriopathy, particularly stent or bypass thrombosis, critical ischemia. Juvenile myocardial infarction or rapid postangioplasty coronary thrombosis may reveal certain myeloproliferative disorders, particularly ET. Venous thrombosis is more frequent in PV than in ET; superficial or deep venous thromboses are seen. Thromboembolic events occur in about 25-30% of the patients and account for one-third of the deaths. Mesenteric vein thrombosis, portal thrombosis, or suprahepatic vein thrombosis may occur in all myeloproliferative disorders, but the pathogenesis is not fully understood. Pulmonary hypertension may be the consequence of local thrombosis in the pulmonary vasculature or may be due to the high blood flow in the right heart cavities. Microvascular circulatory disturbance includes erythromelalgia, Raynaud's phenomenon, digital ischemia, acrocyanosis, blue toe syndrome, livedo reticularis, cutaneous ulcers or necrotic purpura. All these manifestations may precede the myeloproliferative disorder by several months.

Adult↗
PubMed1999

Temporal artery biopsy: a diagnostic tool for systemic necrotizing vasculitis. French Vasculitis Study Group.

OBJECTIVE: To describe the clinical, biologic, and histologic features of temporal artery biopsy (TAB)-localized systemic necrotizing vasculitides (SNV), and to assess their frequency among elderly patients undergoing TAB for suspected giant cell (temporal) arteritis (GCA). METHODS: The frequency of a TAB localization of SNV was prospectively assessed in a multicenter study of elderly patients undergoing TAB for suspected GCA. All patients with SNV fulfilling the American College of Rheumatology criteria for a specific vasculitic syndrome and with evidence of vasculitis on TAB were included in a retrospective, descriptive study. RESULTS: SNV was diagnosed based on the TAB in 1.4% of the patients with suspected GCA and in 4.5% of the positive (inflamed) TAB specimens. We retrospectively selected 27 patients (18 female, 9 male; mean +/- SD age 62+/-15 years, range 22-79 years) with SNV and TAB-localized vasculitis. Only 2 of these patients were known to have SNV before TAB localization. Twenty-two patients (81%) had cephalic symptoms, including jaw claudication in 33%, clinically abnormal temporal arteries in 33%, and neuro-ophthalmologic symptoms in 11%. All patients had systemic symptoms suggestive of SNV and histologically proven NV in the TAB specimens (70%) or elsewhere in other biopsy sites (74%). Abnormal biologic results suggestive of SNV were present in 17 patients (63%). For 4 patients, the TAB-documented involvement led to initial misdiagnoses of GCA, and systemic manifestations that developed under steroid therapy revealed the correct diagnosis. The final diagnoses of the patients were polyarteritis nodosa (PAN) (n = 11), Churg-Strauss syndrome (n = 6), micropoly-angiitis (n = 3), Wegener's granulomatosis (n = 3), hepatitis B virus-related PAN (n = 2), hepatitis C virus-related cryoglobulinemic vasculitis (n = 1), and rheumatoid vasculitis (n = 1). CONCLUSION: TAB-localized SNV presents a major diagnostic dilemma because it can mimic GCA. Careful analysis of clinical, biologic, and histologic data should lead to the correct diagnosis and help guide the clinician's choice of appropriate therapy.

Adrenal Cortex Hormones↗
PubMed1999

Serial measurements of antineutrophil cytoplasmic autoantibodies in patients with systemic vasculitis.

PURPOSE: To assess the value of serial determinations of antineutrophil cytoplasmic autoantibodies (ANCA) for monitoring disease activity in patients with systemic vasculitis. PATIENTS AND METHODS: Forty-three patients with histologically proven vasculitis (21 with Wegener's granulomatosis, 17 with microscopic polyangiitis, and 5 with renal-limited vasculitis) were studied for a median follow-up of 22 months. Disease activity was prospectively assessed and quantified by the Birmingham Vasculitis Activity Score. A total of 347 sera were analyzed for ANCA determination. RESULTS: Relapses occurred in 23 (54%) of 43 patients. Diagnostic category (Wegener's granulomatosis vs micropolyangiitis and renal-limited vasculitis), severity of initial symptoms (mean vasculitis activity score, mean number of organs involved), and ANCA pattern [cytoplasmic-ANCA (c-ANCA) vs perinuclear-ANCA (p-ANCA)] did not significantly differ between relapsers and nonrelapsers. Lung involvement was more frequent at onset among relapsers [16 of 23 (70%) vs 6 of 20 (30%); P = 0.02]. Relapses were slightly, but not significantly, more frequent in patients with Wegener's granulomatosis or a c-ANCA pattern. The percentage of relapsers was greater in patients with persistently positive ANCA than in patients with negative or decreasing ANCA titers (86% vs 20%, P = 0.0001). However, the predictive value of an increase in ANCA titers for the occurrence of a subsequent relapse was only 28% (4 of 14) for c-ANCA, 12% (2 of 17) for anti-proteinase 3-ANCA, and 43% (6 of 14) for anti-myeloperoxidase-ANCA. An increase in ANCA occurred before or during relapse in 33% (10 of 30) of cases for c-ANCA/anti-proteinase 3 antibodies, and 73% (11 of 15) of cases for anti-myeloperoxidase antibodies. CONCLUSION: The persistence of ANCA positivity is strongly associated with relapses. However, an increase in ANCA titers has a poor value for the early prediction of a subsequent relapse and should not be used as a sole parameter for therapeutic intervention. In addition, our results suggest that serial anti-myeloperoxidase determination may be useful as a prognostic marker in patients who are p-ANCA positive.

Aged↗
PubMed1999

[Influence of age on the clinical and biological characteristics of systemic scleroderma].

PURPOSE: The present study was aimed at assessing the influence of age on clinical and biological features of systemic sclerosis. METHODS: This retrospective study included 151 consecutive patients with systemic sclerosis. The median age at diagnosis was 50.0 years (range: 10-84 years). Patients were divided into two groups according to their age (lower than 50.0 years of age: 73 patients, equal to or above 50 years of age: 78 patients). The following features were compared between the two groups: gender, disease duration, extent of skin sclerosis, Crest syndrome, lung fibrosis, secondary Sjögren's syndrome, antinuclear, anticentromere, and anti-Scl70 antibodies. RESULTS: The disease duration was significantly higher in patients over 50 years of age (7.1 +/- 6.8 years vs 5.5 +/- 5.0 years, P < 0.05). Crest syndrome, secondary Sjögren's syndrome and anticentromere antibodies were significantly more common in patients over 50 years of age (17/73 vs 30/78, P < 10(-2); 9/73 vs 20/78, P < 10(-2), and 19/73 vs 31/78, P < 0.05; respectively). Anti-Scl70 antibodies were significantly more common in patients under 50 years of age (17/73 vs 10/78, P < 10(-2)). No significant difference was found in regard to the other features. CONCLUSION: The clinical and biological patterns of systemic sclerosis are different according to the age at disease onset. Crest syndrome including anticentromere antibodies and Sjögren's syndrome is more common in elderly patients, while anti- Scl-70 antibodies are more common in younger patients. This suggests the involvement of various mechanisms in the pathogenesis of systemic sclerosis, and that these mechanisms may depend on the age.

Adolescent↗
PubMed1999

[Low prevalence of anti-annexin V antibodies in antiphospholipid syndrome with fetal loss].

PURPOSE: Annexin V, a protein with potent anticoagulant activity has a calcium-dependent binding affinity for phospholipids. Annexin V is distributed in many organs, especially in the placenta and endothelium. Various studies have shown that placental annexin V is decreased in women with anti-phospholipid syndrome. It has been suggested that annexin V might be a target of anti-phospholipid antibodies and that the subsequent decrease in annexin V might be associated with obstetrical complications. We investigated the presence of anti-annexin V antibodies in the plasma of women with anti-phospholipid syndrome and obstetrical complications. METHODS: Twenty-three patients with at least one spontaneous abortion were included in the study. Anti-cardiolipin antibodies and lupus anticoagulant were present in 87% and 30% of the patients, respectively. A group of 40 healthy women were included in the control group. Anti-annexin V IgG and IgM antibodies were measured by ELISA. RESULTS: The IgG mean OD was 0.07 +/- 0.013 in patients and 0.042 +/- 0.06 in the control group. There was no significant difference between the two groups (P = NS). Only two out of the 23 patients and two out of the 40 healthy women were positive for IgG (OD > 0.25). The sensitivity of the assay was poor (8.7%). Even when the threshold was adjusted according to the mean OD in control subjects +2 SD, the sensitivity was still poor, reaching only 13%. CONCLUSION: The prevalence of anti-annexin V was low in patients with anti-phospholipid syndrome and repetitive spontaneous abortions. Anti-annexin V assay does not appear to be sensitive enough for the identification of anti-phospholipid antibodies that might be involved in the decrease in annexin V leading subsequently to thrombosis risk.

Abortion, Spontaneous↗
PubMed1999

[Prevalence of antiphospholipid antibodies in Horton's disease and in polymyalgia rheumatica].

PURPOSE: Antiphospholipid antibodies (APL) are frequently observed in the course of giant cell arteritis and polymyalgia rheumatica. However, their role and relationships with potential ischemic events are still debated. METHODS: To determine the prevalence of APL in relation with ischemic events, 62 patients with giant cell arteritis and/or polymyalgia rheumatica were retrospectively studied. RESULTS: Before corticosteroid treatment 41% of the 51 patients with giant cell arteritis and 64% of the 11 patients with isolated polymyalgic rheumatica had high IgG ACL levels, with a frequency significantly higher than that (15.6%) of the control group which was composed of healthy elderly. IgM ACL were found in only two patients with giant cell arteritis. There was no correlation between the occurrence of an ischemic event (22 ischemic events in the 51 patients with giant cell arteritis) and the presence of ACL, even though the latter were more frequently observed in the giant cell arteritis group (52% versus 41% in non ischemic patients). The prevalence of ACL increased, reaching 59% if lupus anticoagulant was associated, but the difference was not significant. ACL disappeared soon after corticosteroid therapy had been initiated. CONCLUSION: Though ACL are frequently seen in giant cell arteritis and/or polymyalgia rheumatica, they are not related to ischemic events and disappear rapidly after corticosteroid treatment.

Adrenal Cortex Hormones↗
PubMed1999

Influence of age on characteristics of polymyositis and dermatomyositis in adults.

In a retrospective study of 79 consecutive patients, we evaluated characteristics of polymyositis (PM) and dermatomyositis (DM) and compared clinical presentation, biochemical findings, histologic changes, evolution, complications, and mortality rate of elderly patients (aged > or = 65 yr) and younger patients (aged < or = 64 yr) at the onset of PM/DM. We found a high prevalence of PM/DM in elderly patients: 23 patients (29%) were aged 65 years or over. We also found that esophageal involvement (34.8% versus 16.1%, respectively) and bacterial pneumonia related to both ventilatory insufficiency and esophageal impairment (21.7% versus 5.4%, respectively) were more common in elderly patients compared with younger patients, resulting in increased morbidity and mortality rates. Moreover, malignancy frequency was higher in elderly patients compared with younger patients (47.8% versus 9.1%, respectively, p = 0.0001), particularly patients with DM (10/11). Fifty percent of malignancies were colon malignancies in elderly patients. Erythrocyte sedimentation rate, C-reactive protein, fibrinogen, and ferritin levels were also higher in the elderly patient group compared with the younger group, and the presence of serum hypoprotidemia, hypoalbuminemia, and anemia was more frequent. Finally, PM/DM complete remission was less frequent (13.6% versus 41.1%) and the mortality rate (47.8% versus 7.3%) was higher in elderly patients than in younger patients. The main causes of death in elderly patients were bacterial pneumonia, due to ventilatory insufficiency and esophageal impairment, and malignancies. Our findings therefore indicate that PM/DM-related esophageal and lung involvement should be systematically searched for in elderly patients. Esophageal manometry and pulmonary function tests should become an integral part of initial evaluation in elderly patients for early detection of impairment. Moreover, as we observed a marked overrepresentation of colon cancer in elderly patients with DM, we suggest that the search for malignancies in elderly patients with DM should include lower gastrointestinal tract investigations.

Activities of Daily Living↗
PubMed1999

Evaluation of the efficacy of etidronate therapy in preventing glucocorticoid-induced bone loss in patients with inflammatory rheumatic diseases. A randomized study.

The prevention and treatment of glucocorticoid-induced osteoporosis is a major concern for rheumatologists since inflammatory joint disease is among the most common reasons for long-term glucocorticoid therapy. We used a randomized placebo-controlled design to evaluate the efficacy of one-year cyclical etidronate therapy in preventing bone loss in 83 glucocorticoid-treated patients with rheumatoid arthritis, polymyalgia rheumatica, or giant cell arteritis. Glucocorticoid treatment duration was shorter than three months, and the starting dose was greater than 7.5 mg of prednisone-equivalent per day. Etidronate was given according to the standard cyclical schedule, i.e. 400 mg/d for periods of 14 days separated by 76-day intervals during which patients took 500 mg of supplemental calcium per day. The primary evaluation criterion was the change in lumbar spine bone mineral density after one year of etidronate therapy. Bone mineral density decreased by 1.94 +/- 0.61% in the placebo group and increased by 0.86 +/- 0.6% in the etidronate group, yielding a between-group difference of 2.8 +/- 0.86% (P = 0.002). The difference was largest in postmenopausal women (3.38 +/- 1.11%; P = 0.004). At the femoral neck, there was a smaller bone mineral density decrease in the etidronate than in the placebo group, but the difference (1.11 +/- 1.13%) was not statistically significant. The most common side effects were gastrointestinal symptoms and showed no difference between the two groups. Four fractures (including one vertebral fracture) occurred in the placebo group versus two (including one vertebral) in the etidronate group. Etidronate prevents glucocorticoid-induced lumbar spine bone loss in patients with rheumatoid arthritis, polymyalgia rheumatica, or giant cell arteritis.

Absorptiometry, Photon↗