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U Michon-Pasturel

Publications and source records attributed to U Michon-Pasturel.

At least 37 records · Page 2Linked to original sources

Cannabis arteritis revisited--ten new case reports.

The purpose of this paper was to revisit the old concept of cannabis arteritis first described in the 1960s and report 10 new cases. Ten male patients, with a median age of 23.7 years developed subacute distal ischemia of lower or upper limbs, leading to necrosis in the toes and/or fingers and sometimes to distal limb gangrene. Two of the patients also presented with venous thrombosis and three patients were suffering from a recent Raynaud's phenomenon. Biological test results did not show evidence of the classical vascular risk factors for thrombosis. Arteriographic evaluation in all cases revealed distal abnormalities in the arteries of feet, legs, forearms, and hands resembling those of Buerger's disease. A collateral circulation sometimes with opacification of the vasa nervorum was noted. In some cases, arterial proximal atherosclerotic lesions and venous thrombosis were observed. All patients were moderate tobacco smokers and regular cannabis users. Despite treatment with ilomedine and heparin in all cases, five amputations were necessary in four patients. The vasoconstrictor effect of cannabis on the vascular system has been known for a long time. It has been shown that delta-8- and delta-9-tetrahydrocanabinols may induce peripheral vasoconstrictor activity. Cannabis arteritis resembles Buerger's disease, but patients were moderate tobacco smokers and regular cannabis users. These cases show that prolonged use of cannabis could be an additive risk factor for juvenile and young adult arteritis. Cannabis arteritis is a forgotten and severe occlusive vascular disease occurring in young adults. Search for cannabis use may be an important tool for a better knowledge of arteritis in young smokers.

Adolescent↗

Relationship between parity and clinical and biological features in patients with systemic sclerosis.

OBJECTIVE: To assess the influence of parity on the clinical and biological features of systemic sclerosis (SSc). METHODS: We recorded the following clinical and biological data of 100 consecutive women with SSc: age, disease duration before diagnosis, cutaneous extension of sclerosis according to LeRoy's classification, pulmonary involvement, and antinuclear antibodies. We compared these features to the number and sex of children who were born before SSc onset. Date of birth of the first children was systematically recorded. RESULTS: Patients with limited SSc had more children before SSc onset than patients with diffuse SSc (2.4 +/- 1.8 vs 1.7 +/- 1.5; p < 0.05). The interval between first birth and SSc onset was shorter for patients with limited SSc than for patients with diffuse SSc (11.0 +/- 9.9 vs 23.5 +/- 14.5 yrs; p < 0.01). Patients with pulmonary fibrosis had more children than patients without pulmonary fibrosis (2.5 +/- 1.9 vs 2.0 +/- 1.6; p < 0.05). Age at first birth was significantly higher when the child was a girl than a boy (26.8 +/- 7.5 vs 22.9 +/- 5.3 yrs; p < 0.05). The interval between the first birth and SSc onset was shorter when the child was a girl than a boy (16.2 +/- 9.6 vs 25.4 +/- 13.4 yrs; p < 0.05). CONCLUSION: Pregnancy related microchimerism could be preferentially associated with limited SSc and pulmonary fibrosis. Microchimerism may be facilitated in cases in which the fetus is female.

Adult↗

[Myelopathy - Sjogren's syndrome association: analysis of clinical and radiological findings and clinical course].

Myelopathies associated with Sjögren's syndrome has been rarely described especially concerning magnetic resonance imaging (MRI) and treatment aspects. The aim of this study was to determine the clinical, laboratory and radiological features of myelopathies occurring in Sjögren's syndrome. Eleven patients were studied, 7 with an acute myelopathy and 4 with a chronic form. Acute myelopathy were clinically severe with a feature of transverse myelitis necessitating immunosuppressive drugs. On the other hand, chronic forms were closely similar to progressive multiple sclerosis (MS), for clinical and laboratory data. In 7 cases optic neuritis was found associated with myelopathy and fulfilled the diagnostic criteria of Devic's syndrome in 4 cases. The diagnosis of myelopathy associated with Sjögren's syndrome may be difficult especially compared with MS, HTLV1 or HIV myelopathy and sarcoidosis, in the chronic form but also with other vasculitis, MS or viral infection in the acute forms. However, in this last form, magnetic resonance imaging and cerebrospinal fluid data should bring to the diagnosis of Sjögren syndrome and confirmed by appropriate tests. This diagnosis will have direct consequences for an early treatment by immunosuppressive drugs.

Adult↗

Aerosolized iloprost in CREST syndrome related pulmonary hypertension.

OBJECTIVE: To assess the outcome of patients with CREST syndrome associated severe pulmonary hypertension treated by aerosolized iloprost in a noncomparative study. METHODS: Five patients with CREST syndrome associated severe pulmonary hypertension were treated with 100 microg/day of aerosolized iloprost. New York Heart Association functional class and exercise tolerance (6 min walk test) were assessed at baseline, after one month, and then every 6 months. A right heart catheterization was performed at baseline in all but one patient. Systolic pulmonary artery pressure (PAP) was measured with Doppler echocardiography after one month and every 6 months. RESULTS: The mean followup was 13.2 +/- 8.8 months (median 6, range 6-24). Subjective quality of life improved in all patients. NYHA functional class decreased from Class III to II in 3 patients, from Class III to I in one patient, and from Class IV to III in one patient. At 6 months, the distance walked in 6 min had increased from 352 +/- 48 to 437 +/- 56 m (p = 0.06). At one month the mean systolic PAP was 58 +/- 13 vs 81 +/- 9 mm Hg at baseline (p = 0.04). At 6 months the mean systolic PAP was 57 +/- 13 mm Hg (p = 0.06). The improvement of both clinical and hemodynamic status was maintained in the 2 patients treated for 2 years. Neither adverse effects nor need to increase the daily dose of iloprost were observed. One patient died of right heart failure and one patient did not experience any improvement of exercise tolerance and hemodynamics. CONCLUSION: Aerosolized iloprost might be potentially useful as treatment for CREST syndrome associated pulmonary hypertension. However, patients who could benefit from this treatment will probably have to undergo careful criteria selection.

Administration, Inhalation↗

[Digital necrosis in a patient with anorexia nervosa. Association of vasculopathy and radial artery injury].

BACKGROUND: Patients with anorexia nervosa can develop distal vasculopathy sometimes leading to severe Raynaud's phenomenon or acrocyanosis. We report a cas of anorexia nervosa-related vasculopathy associated with iatrogenic injury to the radial artery that led to digital necrosis. CASE REPORT: An 18-year-old woman, with a history of severe anorexia nervosa of 5 years duration and who acknowledged regular use of tobacco and cannabis, was hospitalized for necrosis of the left index and thumb that had occurred shortly after left radial artery puncture for blood gas analysis. Acrocyanosis of the 4 limbs had been present since the onset of anorexia nervosa. Arteriography of the upper limbs showed major spasm of the left radial and cubital arteries and thromboses in the left interdigital arteries of the left index and thumb. The distal portions of the arteries were then on the left and on the right. The nectrotic lesions healed after intravenous administration of ilomedine and interruption of tobacco and cannabis. Acrocyanosis of the four limbs persisted. DISCUSSION: We report a case of digital necrosis occurring after arterial puncture for blood gas analysis in a patient with a vascular bed weakened by tobacco and cannabis intoxication but also by anorexia nervosa-related vasculopathy. This observation underlines the potentially dangerous nature of invasive intravascular procedures in this context. Indications for vessel puncture must be assessed with prudence.

Adolescent↗

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↗

[Pulmonary involvement in systemic scleroderma. Part I. Chronic fibrosing interstitial lung disease].

INTRODUCTION: Chronic pulmonary interstitial fibrosis is the most frequent respiratory manifestation in systemic sclerosis, occurring in 80% of cases. It remains a severe complication of the disease and is the primary cause of mortality related to respiratory insufficiency in 20 to 60% of cases. CURRENT KNOWLEDGE AND KEY POINTS: The date of onset of interstitial lung disease remains undetermined, and only in rare cases does it reveal the presence of systemic sclerosis. The clinical signs are only observable at a later stage, when at least 50% of the lung parenchyma is affected. The methods of choice adopted for early diagnosis of this disease are high resolution computed tomography and pulmonary functional investigations; they should be carried out during the preliminary investigation and at follow-up once a year. Moreover, high resolution computed tomography also provides prognostic data, for there is a correlation between the type of lesion and its severity as determined by high resolution computed tomography and by histological findings. The value of other methods of investigation, in particular bronchoalveolar lavage, has not yet been clearly established. The association of cyclophosphamide and corticoids is currently being evaluated (indications, administration modalities, duration), and this combination may be the most effective treatment. FUTURE PROSPECTS AND PROJECTS: Interstitial lung disease is one of the major causes of morbidity and mortality in systemic sclerosis. Early diagnosis and management of this disease is therefore of utmost importance.

Adrenal Cortex Hormones↗

[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↗

[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↗

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↗

[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↗

[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↗