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Biomedical subjects

E Hachulla

Publications and source records attributed to E Hachulla.

At least 91 records · Page 5Linked to original sources
PubMed2001

Polymyositis and dermatomyositis: short term and longterm outcome, and predictive factors of prognosis.

OBJECTIVE: To assess short term and longterm outcome of polymyositis (PM) and dermatomyositis (DM), and predictive variables of PM/DM course. METHODS: The medical records of 77 consecutive patients with PM/DM were reviewed. The criteria for PM/DM diagnosis were based upon Bohan and Peter criteria. RESULTS: Thirty-one patients (40%) achieved remission of PM/DM, whereas 33 (43%) improved and 13 (17%) worsened their clinical status. Short term recurrences of PM/DM (during tapering of therapy) occurred in 36 patients and longterm recurrences (after discontinuation of therapy) in 9 patients. PM/DM were associated with both decreased functional status and quality of life at longterm followup: (1) only 52% of patients considered to be in remission experienced a return to previous normal activities; and (2) 45% of the other patients with nonremitting PM/DM still had a marked reduction of activities (as shown by the disability scale of the Health Assessment Questionnaire). Overall mortality was as high as 22%, and the main causes of death were cancer and lung complications. Factors associated with PM/DM remission were younger age and shorter duration of clinical manifestations prior to therapy initiation. Variables associated with poor outcome of PM/DM were older age, pulmonary and esophageal involvement, and cancer. CONCLUSION: Our series shows both high morbidity and mortality related to PM/DM, emphasizing that management of PM/DM patients at an early stage is required. Lung complications (i.e., aspiration pneumonia due to PM/DM related esophageal dysfunction and ventilatory insufficiency) were one of the main causes of death in our series, indicating that investigating for subclinical esophageal and lung impairment should become an integral part of initial PM/DM evaluation. The presence of poor prognostic factors should prompt both close followup and aggressive therapy in patients with PM/DM.

Adolescent↗
PubMed2001

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↗
PubMed2001

[Diagnosis of aortitis].

Aortitis can be a component of a variety of diseases, such as Takayasu arteritis, giant cell arteritis, Behçet's syndrome, Cogan's syndrome, spondylarthropathies, rheumatoid arthritis, systemic lupus erythematosus, sarcoidosis, Erdheim-Chester's disease and a variety of infectious processes like syphilis, Salmonella and others. The presentation is variable: aortic valve regurgitation, aneurysm, dissection, stenosis of the aorta or its initial branches. Sometimes systemic manifestations are at first presentation like fever or inflammatory syndrome. The differential diagnosis may be difficult in some situation like inflammatory aortic atherosclerotic aneurism, or retroperitoneal fibrosis. Some aortitis remain idiopathic. Corticosteroid and sometimes surgery are mostly required to avoid life-threatening complications.

Aortitis↗
PubMed2001

[Dermatomyositis and polymyositis: clinical aspects and treatment].

Dermatomyositis and polymyositis are the two major idiopathic inflammatory myopathies. The Bohan and Peter's criteria are still useful despite the probably different pathogenesis of the two myopathies. Cutaneous manifestations of dermatomyositis include heliotrope rash and Gottron's papules. The heliotrope rash, with or without edema, in a distribution involving periorbital skin is very suggestive of the diagnosis. Papules may be found overlying the "kneedle" of the hand or the elbows, knees, feet. Periungueal erythema with telangiectasis were characteristic but not pathognomonic. Scalp involvement is common. Skin lesions of dermatomyositis may precede the development of the myopathy and may persist after the control of the myositis. Some patients have an amyopathic dermatomyositis with normal muscle-enzyme, magnetic resonance scan and muscle biopsy. Muscle disease affects the proximal muscles, is generally symmetrical and symptoms are fatigue, weakness and sometimes myalgia. Proximal dysphagia reflects an involvement of striated muscle of the pharynx or proximal esophagus. Camptocormia reflects a severe involvement of paravertebral muscle. Other systemic features may be seen: pulmonary involvement (mostly interstitial pneumonitis and hypoventilation), arthralgias or arthritis, cardiac involvement, vasculatis and calcinosis particularly in children or adolescents with dermatomyositis. Malignant disease is associated with idiopathic inflammatory myopathies with a frequency of approximatively 10 to 15% in dermatomyositis and 5 to 10% in polymyositis and is strongly correlated with age, more than 50% of the patient over 65 years old were found to have a cancer. In the absence of malignant disease, the mainstay therapy for dermatomyositis and polymyositis is systemic corticosteroids (mostly 1mg/kg). In the lake of response or high dose dependance, intravenous immunoglobulins or immunosuppressive drugs like methotrexate or azathioprine may be discuss. Cyclophosphamide show some effectiveness in interstitial pneumonitis. Cyclosporin might be effective in children, less in adults. The efficacy of tacrolimus, mycophenolate mofetil, leflunomide and anti-TNF therapy need some prospective studies to determine if there are of value in idiopathic inflammatory myositis.

Anti-Inflammatory Agents↗
PubMed2000

[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↗
PubMed2000

[Periarteritis nodosa].

Periarteritis nodosa is a systemic vasculitis involving arteries with a diameter greater than 70-150 microns. Large vaccination against hepatitis B virus explains the decreased frequency of its association with hepatitis B virus observed in less than 10% of cases. Weight loss, fever, livedo, myalgias or arthralgias, mononeuropathy, gastrointestinal tract involvement, renal insufficiency, and cardiac failure are the most frequent clinical manifestations. Inflammatory syndrome and increased white blood cell count are common. Renal or digestive microaneurysms are present in more than 60% of cases. Prognosis is dependent of a five factors score corticosteroids with or without immunosuppressive drugs, result in 5-year survival rate over 60%. In hepatitis B virus-related periarteritis nodosa, corticosteroids are rapidly stopped; plasma exchanges in combination with antiviral agents (interferon alpha or lamivudine) give excellent results.

Humans↗
PubMed2000

Increased concentrations of the circulating angiogenesis inhibitor endostatin in patients with systemic sclerosis.

OBJECTIVE: Endostatin is an angiogenesis inhibitor derived from type XVIII collagen. The aim of this study was to determine the concentrations of circulating endostatin in patients with systemic sclerosis (SSc), and to assess the relationship between these concentrations, extension of tissular sclerosis, and presence of cutaneous scars or ulcers. METHODS: The study involved 50 patients with SSc and 30 healthy subjects. Cutaneous extension of sclerosis was graded according to Barnett's classification system: 33 patients had grade I SSc and 17 patients had grades II or III SSc. The results of pulmonary function tests were abnormal in 31 of 50 patients, 8 of whom also had abnormalities on chest radiograms. Cutaneous scars or ulcers were found in 22 of 50 patients. Endostatin concentrations were determined using a competitive enzyme immunoassay method. RESULTS: The mean circulating endostatin concentration was significantly higher in the SSc group than in the healthy subjects group (mean +/- SD 53.2 +/- 22.4 ng/ml versus 9.9 +/- 9.7 ng/ml; P < 10(-4)), in patients with grade II or grade III SSc than in patients with grade I SSc (63.2 +/- 20.2 ng/ml versus 45.1 +/- 15.6 ng/ml; P < 10(-2)), in patients with abnormal findings on chest radiograms than in patients with normal findings on chest radiograms (67.6 +/- 22.4 ng/ml versus 50.4 +/- 21.6 ng/ml; P < 0.05), and in patients with cutaneous scars or ulcers than in patients without these manifestations (60.9 +/- 25.9 ng/ml versus 47.2 +/- 13.3 ng/ml; P < 10(-2)). CONCLUSION: Circulating endostatin concentrations are significantly increased in patients with SSc. Production of endostatin may result from tissular sclerosis and could contribute to the development of ischemic manifestations.

Adult↗
PubMed2000

[Are there criteria for the diagnosis of paraneoplastic fever?].

INTRODUCTION: This study was aimed at reviewing useful criteria for the diagnosis of fever of paraneoplastic origin in patients with cancer. CURRENT KNOWLEDGE AND KEY POINTS: Apart from episodes of neutropenia, hyperthermia is observed in approximately 60% of patients with cancer. Fever is often due to infection and, as a contraindication, may hamper treatment. The differential diagnosis, i.e., fever of paraneoplastic or infectious origin, may be difficult. Paraneoplastic fever is mostly observed in patients with advanced cancer. It is related to the production of inflammatory cytokines, especially interleukin 6, and is not specific. Neither current markers of inflammation, nor X-ray, nor naproxen confirm diagnosis. The diagnosis of fever of paraneoplastic origin in patients with cancer should rely on a network of arguments to exclude other causes of fever, especially infectious causes. FUTURE AND PROJECTS: Further evaluation of the specificity of procalcitonin will determine whether or not it is a useful tool for the diagnosis of fever of paraneoplastic or infectious origin.

Diagnosis, Differential↗
PubMed2000

[Treatments for amyloidosis beyond symptomatic care].

INTRODUCTION: Amyloid syndromes are pathogenetically different, each of the various amyloid diseases requiring specific treatment. Unfortunately, those treatments are often preventive and symptomatic, some efficient therapies being limited to particular types of amyloidosis. CURRENT KNOWLEDGE AND KEY POINTS: Colchicine is effective in the prevention of amyloidosis due to familial Mediterranean fever but is less or not effective in other situations. Cytotoxic agents are useful in the treatment of AL amyloidosis with or without hemopoietic stem cell transplantation. Liver transplantation is indicated for familial polyneuropathy and kidney transplantation for dialysis-related beta 2 microglobulin amyloidosis. FUTURE PROSPECTS AND PROJECTS: In vivo binding of serum amyloid P (SAP) (component shared by all amyloid deposits) to amyloid fibril, is a new avenue in the therapeutic approach. Development of radiolabeled SAP scintigraphy allows assessment of the disease outcome and evaluation of treatment-related effects. The various treatments that were assessed until now with the objective of curing the disease are reviewed.

Amyloidosis↗
PubMed2000

[Mechanisms of amyloidosis and the proteins involved].

INTRODUCTION: Recent data in amyloid research have shed light on the amyloid substance and have broadened our knowledge on the mechanism of amyloid deposition. CURRENT KNOWLEDGE AND KEY POINTS: Despite uniform physical properties relating to the presence of beta-pleates, amyloid deposits are chemically heterogeneous and have different origins; additional types will probably be described in the future. Immunohistochemical techniques using specific antisera for each of the major protein present in fibrils could help greatly to subclassify these disorders. In most circumstances, a circulating precursor protein may result from overproduction of either intact or aberrant molecule, a reduction in its degradation or excretion, or genetic abnormalities associated with variant proteins. The cleavage of protein precursor molecules of the protein component of amyloid fibrils characterizes amyloidogenesis, though it is not necessary for some amyloidosis forms. This review summarizes advances in the understanding of the nature of amyloid substances, the mechanism of amyloid deposition and the principal pathogenic hypothesis. FUTURE PROSPECTS AND PROJECTS: SAP component is common in all amyloidosis and may be the target for future therapy.

Amyloid beta-Peptides↗
PubMed2000

[Bronchiolitis obliterans with organized pneumonia: a rare complication of primary Gougerot-Sjögren syndrome].

INTRODUCTION: Bronchiolitis obliterans organizing pneumonia (BOOP) is characterized by plugs of granulation tissue in bronchioles, alveolar ducts and alveoli. This pulmonary disorder has been reported in some cases in relation to drug consumption (D-penicillamine, amiodarone), with bacterial or viral infections (Mycoplasma pneumoniae, HIV), and with systemic diseases, such as rheumatoid arthritis. To our knowledge, only three cases of association BOOP-Sjögren's syndrome have been reported. EXEGESIS: We report three new cases of BOOP. These patients presented a primary Sjögren's syndrome without clinical or biological abnormalities suggestive of other autoimmune diseases. Initial presentation was an acute pulmonary disorder mimicking a bacterial pneumonia. Two patients had cutaneous vasculitis and the third vasculitic neuropathy. Corticosteroid therapy was begun and was quickly successful. None of the patients presented a relapse of BOOP. CONCLUSION: The incidence of BOOP is probably underestimated in patients with primary Sjögren's syndrome without cutaneous vasculitis. In case of pneumonia with antibiotic resistance, an immunological mechanism should be considered.

Adrenal Cortex Hormones↗
PubMed2000

[Aspirin and antiphospholipid syndrome].

INTRODUCTION: Antiphospholipid syndrome is the most frequent cause of acquired thrombophilia. Aspirin may have some indications. CURRENT KONWLEDGE AND KEY POINTS: The usefulness of low doses of aspirin is now well demonstrated in the prevention of obstetric complications associated with antiphospholipid antibodies (especially pregnancy loss). When heparin is combined with low-dose aspirin, the recurrent rate of fetal loss is lower than 30%. In patients with arterial or venous thrombosis, there is a high rate of recurrence during the two first years except if high-dose warfarin was used (i.e., INR > or = 3). The association warfarin-aspirin in secondary prevention of thrombosis may be evaluated in prospective studies. It is not so clear in the literature and in our experience that warfarin is superior to aspirin in stroke recurrence prevention in patients with antiphospholipid antibodies, except in Sneddon's syndrome. There are no guidelines in primary thrombosis prevention in patients with antiphospholipid antibodies. In lupus patients, aspirin may not be sufficient after many years of follow-up in preventing a first episode of thrombosis. Prospective studies may be undertaken. Atherosclerotic patients with antiphospholipid antibodies are particularly exposed to the risk of thrombosis after revascularisation or angioplasty and stent implantation. Aspirin may have a place in those patients but these must be evaluated. FUTUR PROSPECTS AND PROJECTS: Except in prevention of obstetric complications, the usefulness of aspirin in patients with antiphospholipid antibodies must be evaluated in prospective studies.

Adult↗
PubMed2000

[Diagnostic strategy in amyloidosis].

INTRODUCTION: The diagnosis of amyloidosis is a four-step strategy requiring: 1) a high degree of suspicion, as the clinical presentation of the disease is very polymorphous; 2) the biochemical nature of the disease; 3) the evaluation of the disease spread and 4) how it evolves. EXEGESIS: Simple and noninvasive biopsies usually make it possible to diagnose amyloidosis. Available treatments of generalized amyloidosis require an exact characterization of the nature of amyloid deposits, which is based on the clinical context, immunohistochemical analysis of amyloid deposits, and genetic testing. CONCLUSION: Management of amyloidosis should be improved by better characterization of amyloid deposits. This would result in epidemiological data which are currently lacking.

Amyloid↗