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E Hachulla

Publications and source records attributed to E Hachulla.

At least 127 records · Page 7Linked to original sources
PubMed1999

Intravenous immunoglobulins as treatment of life threatening esophageal involvement in polymyositis and dermatomyositis.

Esophageal involvement is considered a major cause of morbidity and an indicator of poor prognosis in polymyositis (PM) and dermatomyositis (DM). We describe 3 patients with steroid resistant PM/DM with life threatening esophageal involvement, resulting in impossible oral feeding and enteral nutrition with a gastric tube. All patients had both dramatic and rapid improvement of all clinical manifestations after initiation of intravenous immunoglobulin (IVIG) therapy. Swallowing disorders completely disappeared after the second infusion of IVIG, which permitted normal oral feeding and ablation of the gastric tube. Our findings suggest IVIG should be considered the treatment of choice in such cases.

Adult↗
PubMed1999

Longitudinal determination of antiphospholipid antibodies in lupus patients without previous manifestations of antiphospholipid syndrome. A prospective study.

OBJECTIVE: To investigate whether serial anticardiolipin determination contributes to the clinical management of patients with systemic lupus erythematosus (SLE) with no previous sign of the antiphospholipid syndrome (APS). METHODS: In 90 patients with SLE with no previous clinical manifestations of the APS, repeated clinical evaluations were performed, and serial blood samples (obtained over a 30 month period, range 13-53 mo) were screened for antiphospholipid antibodies (aPL). Anticardiolipin antibodies (aCL) were detected using an ELISA and considered positive if the result was >25 GPL on 2 separate occasions. Patients were not required to be on a specific treatment regimen during the study. RESULTS: Thirty-four patients (37%) had at least one positive ELISA and 11 (11/90, 12%) 2 positive ELISA for IgG aCL during the study. Lupus anticoagulant (LAC) was found in 16 patients, and a false positive VDRL in 5. At study completion, the total number of clinical or laboratory events associated with APS was 30. In univariate analysis, aCL was significantly associated only with LAC (p<0.012). Presence of aCL also correlated with hemoglobin level, anti-DNA antibody, leukocyte count, and the SLE Disease Activity Index (SLEDAI). LAC and aCL were significantly associated with each other (OR 5.17; 95% CI 1.5-17.7), but LAC had a better positive predictive value than aCL for arterial thrombosis and neurological events. CONCLUSION: Among our patients with SLE without previous clinical manifestations of APS, positive aCL did not predict the occurrence of APS within the next 3 years, but was statistically related to the clinical disease activity (SLEDAI).

Adolescent↗
PubMed1998

[Raynaud's phenomena of occupational origin].

Raynaud's phenomenon must systematically lead to the research of a professional cause, mainly if the patient is a manual worker. The most frequent professional cause is vibrations disease which involves different occupations, especially woodcutters. In this disease, Raynaud's phenomenon may be associated with neurological or rheumatic manifestations. Prevention relies on tool improvement and reduction of exposure-time. The more uncommon hammer's syndrome is characterized by the onset of a terminal cubital artery aneurysm, resulting from repeated trauma on the palm of the hand. Raynaud's phenomenon is severe, with frequent trophic complications related to embolic migration towards digital arteries. Improved prevention explains that acrosyndromes related to vinyl chloride exposure are now very rare. Finally, a Raynaud's phenomenon may be the first symptom of a systemic sclerosis induced by silicate exposure.

Aneurysm↗
PubMed1998

[Lymph node tuberculosis in France in non-immunosuppressed adults: current pathology].

PURPOSE: Lymph node tuberculosis is more frequent in Africa or in HIV-infected patients. METHODS: We review results that were obtained in 18 patients with lymph node tuberculosis but without infection by HIV. Eight French native patients (mean age: 69 years) and ten African patients (mean age: 32.5 years) were included into the study. RESULTS: Of the 18 patients, 14 had exclusively hematopoietic localizations, two with medullar involvement and two with either liver or spleen involvement. Normal erythrocyte sedimentation rate was found in two out of 18 cases and normal C-reactive protein levels in seven out of 17 cases. Lymph node biopsy revealed lesions typically associated with tuberculosis in 12 out of 14 patients, two of them had no necrosis. Inflammatory syndrome was inconstant, tuberculin test sometimes negative. A favorable outcome was seen in all cases after 6, 12 or 18 months of treatment, depending on clinical appreciation. CONCLUSION: Tuberculous lymphadenopathy in non-HIV-infected patients may occur without inflammatory syndrome, with sometimes negative tuberculin test and without lymph node necrosis.

Adult↗
PubMed1998

[Primary biliary cirrhosis and systemic scleroderma (Reynolds syndrome): apropos of 8 new cases. The contribution of accessory salivary gland biopsy].

PURPOSE: We report eight new cases of the association primary biliary cirrhosis--systemic sclerosis (Reynolds' syndrome) and study the contribution of labial salivary gland biopsy to the disease diagnosis. METHODS: We retrospectively collected clinical and biological data as well as results of labial salivary gland biopsies in eight patients with Reynolds' syndrome. RESULTS: The eight patients were female. Systemic sclerosis corresponded to a CREST syndrome in three patients, while in two other patients two features of the CREST syndrome were observed. Anticentromere antibodies were detected in these five cases. The last three patients had systemic sclerosis with bilateral pulmonary fibrosis in two cases. All patients presented with Sjögren's syndrome. Labial salivary gland biopsies performed in six patients showed in five cases an inflammatory infiltrate and focal sialadenitis typically associated with Sjögren's syndrome. In four cases, organic microangiopathy and fibrosis were suggestive of systemic sclerosis, and in four other cases, the presence of a pericanalicular infiltrate of lymphocytes and necrosis of the excretory ducts epithelial cells suggested the existence of primary biliary cirrhosis. In three patients, all histological lesions were found in the same labial salivary gland biopsy. CONCLUSION: Coexistence of histological lesions on the same labial salivary gland suggestive of primary biliary cirrhosis, systemic sclerosis and Sjögren's syndrome has never been described previously. The diagnostic value of labial salivary gland biopsy has to be assessed in this context.

Adult↗
PubMed1998

[Prevention of glucocorticoid induced osteoporosis].

INTRODUCTION: It is generally accepted that moderate to high dose glucocorticoid therapy is associated with bone loss and increased fracture risk. The degree of bone loss is closely related to the cumulative corticosteroid dose and the highest rate of bone loss is observed in the first 3 to 6 months of therapy. CURRENT KNOWLEDGE AND KEY POINTS: Optimal management strategies to prevent bone loss should include the use of the lowest efficacious dose of glucocorticoid. Alternate day dosing and bolus do not provide effective protection. Prevention should basically include adequate calcium intakes and additional vitamin D. All patients should be encouraged to modify their lifestyles, including smoking cessation and limitation of alcohol consumption; physical exercise for 30 minutes to 1 hour every day should also be recommended. Sodium restriction and thiazide diuretics have been shown to improve hypercalciuria that sometimes accompanies glucocorticoid therapy. Hormone replacement therapy should be recommended in postmenopausal women, while estrogen-containing oral contraceptives should be advocated in young women with menstrual irregularities, except for patients with systemic lupus erythematosus. FUTURE PROSPECTS AND PROJECTS: Dual-energy X-ray absorptiometry may be useful in patient with high risk of glucocorticoid-induced osteoporosis, as it allows accurate measurements of bone mineral density. Etidronate recently proved to be efficacious in preventing bone loss and decreasing the number of vertebral fractures in menopausal women.

Absorptiometry, Photon↗
PubMed1998

[The correlation between salivary endothelial expression of E-selectin and clinical and biological parameters in systemic scleroderma].

OBJECTIVES: A body of evidence suggests the pivotal role of endothelial cells in the pathophysiology of systemic sclerosis. E-selectin is an adhesion molecule specifically expressed by activated endothelial cells. In previous studies we noticed that E-selectin was frequently expressed in the salivary gland tissue of patients with systemic sclerosis. Moreover, E-selectin expression was detectable very early in the course of the disease. To better define the role of E-selectin in the pathogenesis of systemic sclerosis, we conducted a study aimed at determining whether E-selectin expression was correlated to clinical and biological features in patients with systemic sclerosis. METHODS: Thirty-one patients presenting with systemic sclerosis were included in the study. The following parameters were systematically assessed: duration and cutaneous extent of the disease, presence of secondary Sjögren's syndrome, antinuclear antibodies, and pulmonary and esophagus involvement. E-selectin expression was assessed by immunocytochemistry on minor labial salivary glands. RESULTS: E-selectin expression was detected in 21 out of 31 patients (67%). The disease duration was significantly shorter in patients with E-selectin expression (mean 9.1 +/- 8.5 years versus 4.2 +/- 3.3 years, P < 0.05). No significant difference was found for other features. CONCLUSIONS: This study shows that endothelial E-selectin expression is mainly detectable early in the course of systemic sclerosis, when active and non-cicatrical sclerosis may be evidenced. No correlation was found between E-selectin expression and immunological disorders (antinuclear antibodies, secondary Sjögren's syndrome).

Adolescent↗
PubMed1998

[Multiple sclerosis and antiphospholipid antibodies: study of 62 consecutive patients].

INTRODUCTION: Although multiple sclerosis (MS) and antiphospholipid syndrome (AS) are usually defined by specific criteria that make them distinguishable, in some cases, transition between the two diseases based on clinical and brain imaging findings is not clear. METHODS: Our study included 62 patients (sex ratio F/M = 1.48; mean age 43.4 +/- 23.6 years) with diagnosis of MS according to Poser criteria and 31 control subjects (sex ratio F/M = 9.3, mean age 37 +/- 17 years). We examined the level of antibodies against phospholipids (anticardiolipid, anti beta 2-glycoprotein 1 and antiphosphatidylethanolamine antibodies), antinuclear, anti native DNA, antiprothrombinase antibodies and rheumatoid factor. RESULTS: Antiphospholipid antibodies were found with a significant level (anticardiolipid > 30 UI, anti beta 2-glycoprotein 1 positive) in only five patients (8%) with MS; two others showed an increase in antinuclear antibodies (1/320 degrees and 1/1280 degrees). CONCLUSION: In contrast with data recently reported, this study failed to find a significant level of antiphospholipid antibodies in MS. This result argues for the existence of different pathogenic mechanisms in MS and AS.

Adult↗
PubMed1998

[Bilateral spontaneous perirenal hematoma: an unusual complication of polyarteritis nodosa].

INTRODUCTION: Spontaneous perirenal hematoma is a serious and unusual complication of polyarteritis nodosa. Its treatment is not standardized. A review of the literature shows that therapeutic arterial embolization is an alternative to nephrectomy. EXEGESIS: We report a new case of spontaneous bilateral perirenal hematoma due to a rupture of a renal aneurysm in a 68-year-old patient presenting with polyarteritis nodosa. The patient had no hypertension. Renal arteriography helped guide the diagnosis, and selective embolization was performed to treat the left hematoma. The right hematoma only required symptomatic treatment. CONCLUSION: Arteriography with selective embolization appears to be a good alternative to nephrectomy in spontaneous perirenal hematoma complicating polyarteritis nodosa. However, in this case, the right hematoma required neither arteriography nor nephrectomy. This raises the questions of the necessity of selective embolization when the clinical status remains satisfying with a symptomatic treatment.

Aged↗
PubMed1998

[Takayasu's arteritis: vascular investigations and therapeutic management. Experience with 16 patients].

PURPOSE: There is no consensus in regard to vascular explorations and therapeutical management of Takayasu's arteritis. The objective of this study was therefore to establish the most appropriate vascular explorations and to analyze current treatments. METHODS: Clinical, biological and morphological findings related to either diagnosis or treatment were retrospectively evaluated in sixteen patients diagnosed with Takayasu's arteritis according to the American College of Rheumatology criteria. RESULTS: Median delay between the occurrence of the first symptoms and the diagnosis was 9 months. Aortic lesions and aortic valvular incompetence were more frequent. Statistical analysis showed the existence of a correlation between the lack of relapse and corticosteroid therapy (Fisher exact test, P = 0.021). Percutaneous transluminal angioplasty led to stabilization of vascular lesions. Surgical management led to satisfactory results, except for patients with aortic lesions, as survival was then less than 1 year. CONCLUSION: Early diagnosis is mandatory in patients with Takayasu's arteritis in order to propose appropriate therapy, particularly corticosteroid therapy. Surgery and angioplasty prove to be useful in occlusive forms. Late diagnosis is accompanied by severe aortic lesions and fatal outcome.

Adult↗
PubMed1998

Cerebral magnetic resonance imaging in patients with or without antiphospholipid antibodies.

OBJECTIVE: To determine in patients with systemic lupus erythematosus (SLE) or with primary antiphospholipid syndrome (PAPS) the prevalence of cerebral magnetic resonance imaging changes (MRI) and the relationship with antiphospholipid antibodies. METHODS: Twenty-nine consecutive SLE patients, 24 PAPS patients and 31 healthy controls were prospectively included in the study and underwent MRI Scan over a 1-year period. MRI scans were analyzed separately by a neuroradiologist for white matter changes [periventricular hyperintensity (PVH) (0-6 scale), deep white matter hyperintensity (WMH) (0-24 scale)], and one neurologist for cerebral atrophy (0-39 scale) and stroke subtypes. Statistical assessment consisted of a discriminant analysis performed with SAS-package with MRI data as dependent variables and, as independent variables, age, sex, arterial hypertension, diabetes mellitus, cardiopathy, migraine, neurological symptoms, antiphospholipid antibodies, SLE, steroid treatment. RESULTS: The prevalence of cerebral atrophy was increased in both SLE and PAPS groups relative to controls. PVH and WMH scores were significantly higher in SLE and PAPS than in controls. Focal infarct did not differ in the SLE group when compared with PAPS. PVH and WMH scores were significantly higher in patients with neurological symptoms. Using a correlation test we found a weak significant correlation between cerebral atrophy and lupus anticoagulant. The multivariate analysis found only three independent variables related to PVH and WMH: age, the diagnosis of SLE and cerebral atrophy. CONCLUSIONS: Age, presence of SLE and presence of neurological symptoms were independently related with WMH and PVH, but not antiphospholipid antibodies.

Adolescent↗
PubMed1998

Takayasu's arteritis diagnosed at the early systemic phase: diagnosis with noninvasive investigation despite normal findings on angiography.

We describe a case of Takayasu's arteritis discovered at the early systemic phase. Ultrasonography and computed tomography show thickening of the walls of the superior mesentery and common carotid arteries despite normal findings on angiography. Diagnosis was confirmed by arterial biopsy. We emphasize the importance of noninvasive vascular investigation to support the diagnosis of Takayasu's arteritis.

Adult↗
PubMed1998

Magnetic resonance imaging in focal myositis.

We describe 4 cases of focal myositis of the lower leg in which magnetic resonance imaging (MRI) was useful and sensitive in clearly revealing the detailed anatomic extent of muscle change and in guiding muscle biopsy. We also observed a correlation between MRI findings and severity of both clinical and histological manifestations, suggesting that MRI may provide prognostic criteria in focal myositis. MRI may be a helpful, noninvasive test for the followup of focal myositis, in assessing disease progression or resolution.

Adult↗