[Deficiencies of the heart and neurons...].
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Biomedical subjects
Publications and source records attributed to B Devulder.
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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.
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).
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.
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.
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.
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.
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.
OBJECTIVE: To assess prevalence, characteristics, and predictive factors of pulmonary involvement in polymyositis (PM) and dermatomyositis (DM). METHODS: The medical records of 55 consecutive patients with PM and DM between 1983 and 1996 were reviewed. The criteria for diagnosis of PM and DM were based upon Bohan and Peter criteria. RESULTS: Twenty-two patients (40%) developed lung impairment with a mean of 17 months after onset of PM and DM. The 3 main types of pulmonary disorders were interstitial lung disease (41%), ventilatory insufficiency with bronchopneumonia (22.8%), and alveolitis (with pulmonary function tests revealing restrictive pattern, low diffusing capacity, and normal computerized tomography) (36.2%). Both morbidity and mortality rates were as high as 40 and 27%, respectively, in PM and DM patients with lung involvement. Moreover, for the group with pulmonary diseases, serum GOT and ferritin levels were higher (p = 0.01 and p = 0.02) and the presence of anti-Jo-1 antibody and characteristic microangiopathy were more frequent (p < 0.05 and p = 0.0002). CONCLUSION: This series reveals both high prevalence (40%) and severity of pulmonary involvement in PM and DM. Our findings also suggest that high serum GOT and ferritin levels, presence of anti-Jo-1 antibody and characteristic microangiopathy may have predictive value and should encourage the search for pulmonary dysfunction and interstitial lung disease in patients with PM and DM.
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Several studies have suggested an increased risk of cancer in patients with systemic sclerosis, but the potential risk factors for these cancers remain unknown. The aim of this study was to identify, among patients with systemic sclerosis, factors associated with the development of cancer, with attention to clinical (age, sex, cutaneous sclerosis), immunological (antinuclear antibodies, anticentromere antibodies, anti-Scl-70 antibodies) and histological (pulmonary fibrosis) features. We retrospectively studied 123 patients with systemic sclerosis. The median follow-up period was 4 years. Fourteen cases of cancer (11.3%) were found (lung n = 3, breast n = 2, ovarian n = 2, skin n = 1, thyroid n = 1, rectum n = 1, uterine cervix n = 1, larynx n = 1, pancreas n = 1, myelodysplasia n = 1). The characteristics of systemic sclerosis were similar in patients with and patients without cancer. Yet, the three cases of lung cancers occurred in association with CREST syndromes and anticentromere antibodies.
POEMS syndrome is an acronym defined by Bardwick (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal component and Skin changes). Other various clinical and biological features are reported: edema, cachexia, microangiopathic glomerulopathy, most rarely pulmonary hypertension, cutaneous necrosis. Thrombocytosis or polycythemia may be a prominent feature. POEMS syndrome is sometimes associated with lymphoproliferative disorder. Castelman-like disease is frequently observed as pathologic findings on lymph nodes. Distinction between POEMS syndrome and osteosclerotic myeloma is delicate. The rate of the monoclonal protein is modest-always less than 30 g/L-and is almost of the lambda light chain class. In contrast to multiple myeloma this syndrome is rarely associated with hypercalcemia, skeletal fracture, renal involvement and increasing of M component during evolution. Bone marrow plasmocytosis is usually less than 15% and the kinetic phenotype and genetic characteristics of the plasma cell remain those found in monoclonal gammopathy of undetermined significance. The pathophysiology of this syndrome remains largely unknown but overproduction of pro-inflammatory cytokines are reported, especially TNF alpha, IL-6 and IL-1 beta. Some clinical manifestations seem to be cytokine related. Polyneuropathy and cachexia are the main cause of death. A part corticosteroid and cure of solitary bone lesion, treatment is disappointing and survival is 60% at five years.
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We evaluated the value of dynamic transcutaneous oxygen tension measurement in 15 patients with Leriche stage II intermittent claudication treated with vascular bypass procedures. Fifteen men, median age 60 years (range: 37-72 years), were studied during six months; 19 limbs were revascularized: eight by angioplasty, 11 by patent graft. Claudication perimeter and dynamic transcutaneous oxygen tension measurement were evaluated before and after revascularization. The TcPO2 was continuously measured with a multimodular Kontron Supermon at seven different sites simultaneously: precordium (reference probe), thighs, calves and feet, in the dorsal recumbent position after 30 minutes rest, during a standardized exercise stress test at 50 W and during the recovery phase. The results were expressed as index of surface defect (ISD). After revascularization, the duration of significant ischemia was significantly reduced (P < 0.001 thigh, calf, foot) in 14 patients. Dynamic transcutaneous oximetry therefore seems to be a useful method in assessing stage II occlusive peripheral arterial disease and the topography of tissue hypoxia. Dynamic transcutaneous oximetry is helpful in the surveillance after revascularization and guides the choice of specific treatment (angioplasty or patent graft) especially for multiple lesions.
In a retrospective study of 194 systemic sclerosis, we selected 37 Crest syndrome patients. Among these 37 patients, five presented a primary pulmonary hypertension. For three of these patients, the evolution of this primary pulmonary hypertension was rapidly fatal, one died of another cause. One died without certitude that death is imputable to HTAP. Diagnosis and management of the evolution of primary pulmonary hypertension may be easily assessed by doppler-echocardiography.
Between 1985 and 1995, 47 patients with evidence of polyarteritis nodosa were seen at the department of Internal Medicine. Thirty-nine patients fulfilled histological and/or arteriographic diagnostic criteria, and in eight patients, the diagnosis was based on clinical criteria. At the onset of the disease, 25 patients were below the age of 65 (group A) and 22 were above the age of 65 (group B). Except for increased frequency of weight loss in group B and increased frequency of cutaneous signs (purpura and nodes) in group A, no significant differences were found in clinical and biological features. The mean duration of follow-up was 46.9 +/- 36.5 months. The number of deaths was significantly higher among the elderly (ten deaths in group B versus two in group A).