Churg-Strauss syndrome revealed by granulomatous acute pericarditis: two case reports and a review of the literature.

BACKGROUND: Churg-Strauss syndrome (CSS) is a necrotizing systemic vasculitis with extravascular granulomas and eosinophilic infiltrates of small vessels. CSS is usually revealed by nonspecific signs of necrotizing vasculitis in a context of late-onset asthma and blood eosinophilia. It is considered a systemic vasculitis with the highest prevalence of cardiac involvement and can lead to rapid-onset heart failure due to specific cardiomyopathy. Pericardial effusion may also occur during CSS and is usually well tolerated. OBJECTIVE: The objective of these case reports was to indicate that CSS may present as tamponade, with or without other visceral involvement. METHODS: Among CSS patients treated during the past 10 years at 2 French university hospitals, we have identified and described 2 cases revealed by tamponade with pericardial biopsy-proven granulomatous vasculitis. We have also reviewed the international medical literature in PubMed on cardiac involvement in CSS. RESULTS: The first case report describes a 66-year-old man who had an isolated cardiac tamponade with both inflammatory syndrome and eosinophilia. Long-term remission was obtained with corticosteroids. The second case report describes a 46-year-old woman whose CSS presented with tamponade and associated central nervous system and myocardial involvement. Remission was obtained with corticosteroids and cyclophosphamide. In both cases, CSS was assessed by histological analysis of a pericardial sample. CONCLUSIONS: CSS may present as isolated cardiac tamponade. Whereas pericarditis with myocardial injury warrants immunosuppressive therapy, isolated pericarditis without other visceral involvement of poor prognosis only requires corticosteroid therapy.

[Screening of abdominal aortic involvement using Doppler sonography in active giant cell (temporal) arteritis at the time of diagnosis. A prospective study of 30 patients]. / Dépistage de l'atteinte aortique abdominale par échographie-doppler au diagnostic de maladie de Horton. Etude prospective de 30 patients.

BACKGROUND: Inflammatory involvement of extracranial large-sized arteries occurs in 10-20% of patients with giant cell (temporal) arteritis. Aortic involvement may reveal giant cell arteritis or occur as a late-onset complication, and represents one of the most serious manifestation of the disease with the risk of aortic dissection and/or aneurysm rupture. The thoracic aorta is more frequently involved but abdominal aortitis may also occur in giant cell arteritis. To date, few data are available about abdominal aorta changes at the initial stage of giant cell arteritis. PATIENTS AND METHODS: This prospective monocentric study was conducted between May 1998 and May 2002, and included 30 consecutive patients with biopsy-proven giant cell arteritis. Standard clinical and biological data were collected. Each patient underwent an abdominal aortic Doppler-sonography that looked for aneurysm, ectasia, thickening of the vascular wall, and hypoechoic halo around the aorta. RESULTS: Among the 30 patients of this study (25 women, 5 men, mean age 68.5 years), 4 (13%) had an abdominal aortic aneurysm, with a low diameter (23 to 27 mm), measuring 2 to 5.5 cm in length. A vascular wall thickening superior or equal to 3 mm was noted in 17 patients (68%). A 4 to 8 mm periaortic hypoechoic halo was found in 10 patients (33%). This halo was present in 3 out of the 4 patients with aneurysm. CONCLUSION: Aortic involvement is a potentially serious complication of giant cell arteritis. The question of a systematic screening of this complication remains open to discussion. Our study shows that Doppler sonography may detect morphological abnormalities on the abdominal aorta at the initial stage of giant cell arteritis. These abnormalities comprise mild aneurysms, thickening of the vascular wall and periaortic halo, which could correspond to inflammatory locations of the disease. Complementary studies are needed to assess their specificity and their seriousness.

Giant cell arteritis presenting with aortic dissection: two cases and review of the literature.

Aortitis is the most serious location of the disease giant cell (temporal) arteritis (GCA). Aortic dissection or the rupture of an aortic aneurysm can be responsible for sudden death among patients with GCA. This report discusses two cases of GCA presenting with aortic dissection. One case had histologically proven giant cell aortitis. The second case was a fatal aortic dissection preceded by a stroke. We describe the main features of aortic dissection and aortitis during GCA, reviewing the existing literature on this subject, and focusing on the requirement of prospective aortic imaging studies to screen patients with this kind of location.

[Is training on basic skills and management of critical events responsible of ethical considerations in anaesthesia and intensive care?]. / L'apprentissage des gestes invasifs et de la gestion d'une situation critique pose-t-il des problèmes éthiques en anesthésie-réanimation?

OBJECTIVE: To provide information on morbidity and ethical questions associated with learning of invasive techniques (tracheal intubation, positioning of central venous or epidural catheters) and management of anaphylactic shock. STUDY DESIGN: Retrospective survey. METHODS: Written questionnaire to 54 anaesthesiologists and 55 residents. RESULTS: Training was primarily performed by residents having a 6 months-experience for general anaesthesia and by more experienced residents for epidural analgesia. Residents observed first two or three procedures performed by seniors, but did not have theoretical lectures in 30 to 50% of cases. Dead bodies or manikins were rarely used. Despite the presence of experienced anaesthesiologists during the first attempts, there was a high morbidity rate which was considered by 22 to 37% of the interviewed anaesthesiologists a loss of benefit for the patients. Despite a high level of coaching, a high morbidity rate was associated with the first attempts. However, only few residents explicitly stated to be concerned by ethical questions. Among anaesthesiologists, who had yet to manage anaphylactic shock, 21 and 35% of them reported that diagnostic and treatment could have been performed faster. Virtual learning was misunderstood but 46% of anaesthesiologist described numerous advantages in using simulator of anaesthesia. CONCLUSION: Despite an apparent morbidity with a loss of benefit, informed consent of the patients were rarely obtained.

[Empirical treatment of granulomatous hepatitis of unknown origin: practice investigation in the French National Society of Internal Medicine]. / Traitement d'épreuve d'une hépatite granulomateuse symptomatique de cause indéterminée: enquête de pratique au sein de la Société nationale française de médecine interne.

PURPOSES: Ten to fifteen percent of granulomatous hepatitis are idiopathic. If symptoms like prolonged fever are present, empirical treatment is discussed. The goal of this study is to describe the empirical treatment proposed in this situation by French specialists of internal medicine. METHODS: We conducted a practice investigation among the French national society of internal medicine (SNFMI), using an anonymous questionnaire that related a case of idiopathic granulomatous hepatitis. This questionnaire was proposed to all French internists present at the SNFMI congress in June and December 2004. French specialists of internal medicine had to answer if they would prescribe an empirical treatment and if so, to specify this treatment. RESULTS: Thirty-six French specialists of internal medicine answered to the questionnaire. In the proposed situation, 89% of them initiate an empirical treatment. In 18/36 cases (50%), a first-line anti-tuberculosis empirical treatment is proposed (quadritherapy in 11 cases). In 7 cases (19%), an empirical treatment with prednisone, 0.4 mg/kg/d (N=1) and 1 mg/kg/d (N=6), would be prescribed. Seven internists (19%) would prescribe an empirical treatment with cyclins at the dose of 100 to 400 mg/d. Median duration of the empirical treatment would be 28 days (range: 8-252d). The evaluation parameters mentionned are: fever (69%), weight (59%), seric level of C-reactive protein (59%), and liver biology (53%). In case of failure of first-line empirical treatments, 69% of all questionned internists prescribe a second-line treatments: prednisone at the dose of 0.4 to 2 mg/kg/d (72%), anti-tuberculosis treatments (16%), cyclins 200 mg/d (12%), with a median duration of 28 days. Seven internists (19%) propose to combine two empirical treatments. DISCUSSION: Faced with a problem of idiopathic granulomatous hepatitis, French internists questionned propose four therapeutics options: no treatment, anti-tuberculosis treatment, cyclins or steroids treatment. First-line anti-tuberculosis treatment is a coherent proposition regarding to the high prevalence of tuberculosis. There are only few data available concerning empirical treatment with steroids or cyclins. Specific proposition of such empirical treatments should be defined. CONCLUSIONS: The management of idiopathic granulomatous hepatitis is difficult. Our study shows that therapeutics practices of French internists are heterogenous. The main proposition consists in a first-line anti-tuberculosis empirical treatment, that has to be evaluated after four weeks, and switched with steroids (prednisone, 1 mg/Kg/d) in case of failure. This study is not an expert proposition but contributes to suggest clinical practice guidelines for a rare, complex, heterogenous, and typically internist situation.

[Comparison of medical ethical competencies of pregraduate third and sixth year students during their internal medical hospital rotations]. / Comparaison des compétences médicales éthiques des étudiants de 3e et de 6e année en stage hospitalier en médecine interne.

PURPOSE: The study looks for medical students ethical understanding during their internal medicine hospital rotations. As a secondary purpose, the study screens if internal medicine ward provides an environment appropriate for pregraduate medical students ethical training. METHOD: Retrospective analysis and comparison of logbook writings from 2 different pregraduate groups of students : Group A = 29, third year medical students and Group B = 28, sixth year medical students, (that is last year pre-graduation). The task instructions were the same for the two groups : to identify and to analyse an ethical problem personally witnessed on the ward and to propose a solution either local or personal. Cognitive semantic units (CSU) were first drawn from the writings then subsequently analysed through two grids 1* according to fundamental principles (P) and 2* according to professional responsibilities (R). RESULTS: Students from group A produced a mean of 13,7 CSU per writing (total of 396 CSU) and those from Group B, 7,3. (total of 205 CSU) ; significant difference with P <0,004 between the 2 groups. Both identification of ethical dilemma itself and outbreaking it in (P) and (R) items and articulating solutions were significantly different between the 2 groups(P <0,004 Wilcoxon). Patient-physician relationship was predominantly present in both groups. While end-of-life and therapeutic issues were more prominent among group B, students of group A disagreed more with staff. Students of group B focused on external causes to excuse their demotivation (final exams'preparation), being more fatalist than cynical towards ethical issues. CONCLUSION: There is an erosion of clinical ethical sensitivity during pregraduate training. This study shows that internal medicine hospital rotation provides enough opportunities (both in number and in variety) to promote keen clinical ethical learning.

[Medical practice analysis in internal medicine: a national descriptive study]. / Analyse des pratiques des spécialistes de médecine interne: une enquête transversale descriptive nationale.

PURPOSE: This descriptive and epidemiological study was conducted in Mars 2002 in Internal Medicine in order to (1) participate in elaborating a White Book about the speciality, (2) analyse the post-university formation needs of the specialists in Internal Medicine. METHODS: A questionnaire was sent to all specialists in Internal Medicine listed on the ADELI file (n = 2155). For the first three patients seen in consultation and during hospital stay, questioned specialists had to mention the age, sex, origin, motive of the visit, nature of symptoms, complexity of the problem and the nature of the required abilities. They also had to precise the main diagnosis of all patients seen in the same day. RESULTS: Three hundred and sixty answers have been received. Three hundred and thirty two were exploitable. Five thousand six hundred and eleven main diagnosis were listed. Fifteen percent of the questioned specialists did practise in other specialities than Internal Medicine. Orphaned diseases were the most common pathologies carried out in consultation (17%). Patients seen during their hospital stay suffered more frequently from infectious, haematological and malignant diseases. In 55% of the cases, patients were seen in second or third line after a visit to a general practitioner or another specialist. The abilities of the Internal Medicine specialist alone were sufficient in 70% of the cases to solve the problem. Complexity of the problem was evaluated by the specialists themselves at about 45/100 on an analogical scale. CONCLUSIONS: This study inform the medical community about the type of patients treated by the specialists in Internal Medicine, precise the exact nature of their professional exercise and their real need in medical post-university formation.

[Assessment of teaching methods of anaesthesiology and intensive care medicine in France: opinions of teacher and residents]. / Modalités de l'évaluation de l'enseignement de l'anesthésie-réanimation en France: point de vue des enseignants et des internes.

OBJECTIVE: The objective of this study was to provide informations about French practice of anaesthesiology and intensive care graduation. STUDY DESIGN: Prospective study. MATERIAL AND METHODS: [corrected] Surveys relative to evaluation tools were sent to professors, and students in anaesthesiology and intensive care in France. RESULT: About 95% of French students in anaesthesiology and intensive care want to be evaluated all over their cursus, by using "Carnet de stage", tutor-student agreement or written examinations. Pedagogical evaluation is hardly wished by students and professors despite that "Carnet de stage" and written examinations are frequently used before professional certification. In order to improve the professional competence, the patronage and use of simulator are frequently quoted by professors. CONCLUSION: Despite the fact that "Carnet de stage" and written examinations are frequently used, pedagogic dimension of evaluation need to be improved.

[Stroke management in a general internal medicine department: results of a survey regarding practice]. / Prise en charge des accidents vasculaires cérébraux en service de médecine interne générale. Résultats d'une enquête de pratiques.

PURPOSE: The incidence of strokes in the general population and the insufficient numbers of centers specialized in their management has led to the increased hospitalization of patients in general, internal medicine departments. The prognosis for patients is related to the relevance of the initial diagnostic and therapeutic measures. To optimize management of stroke patients outside of specialized units, a survey concerning practice was conducted in internal medicine departments in western France, and a meeting was held to define guidelines. METHODS: Data sheets were mailed to practitioners in internal medicine departments prior to the survey. The following data were recorded for each patient hospitalized during the two-week study: age, sex, clinical characteristics of stroke, risk factors, investigations performed, and initial and subsequent medical management. On the basis of the results, management guidelines were considered and defined. RESULTS: Patients hospitalized for a stroke in internal medicine departments have a similar profile to those hospitalized in neurology departments. CT-scan and EKG recordings were performed in most patients whereas other investigations (cervical ultrasound, echocardiography, MRI) were used less frequently. The use of rehabilitation and therapeutic strategies for secondary stroke prevention were not always performed according to current guidelines. CONCLUSION: Internal medicine departments are frequently involved in the care of stroke patients. The present study of clinical practice may help to improve stroke management in these non-specialized departments.

[Horton's disease in elderly patients aged over 75: clinical course, complications of corticotherapy. Comparative study of 164 patients. Towards a reduced initial dose]. / Maladie de Horton du sujet âgé de plus de 75 ans: particularites évolutives, complications de la corticothérapie. Etude comparative sur une série de 164 patients. Vers une dose d'attaque réduite.

PURPOSE: Our study compares clinical and therapeutic courses (corticosteroid response, corticosteroid amount, complications) in people with giant cell arteritis before and over 75 years, during the first year of treatment. METHODS: A series of 164 patients was retrospectively analysed (mean age: 73.3 years) among the two subgroups: before 75 and over 75 years. Patient received (monitoring of reduction in the corticosteroid dosage) a 240 mg intravenous bolus of methylprednisolone followed by 0.5 or 0.7 mg/kg/d of prednisone, or 0.7 mg/kg/d of prednisone without the bolus. RESULTS: Corticosteroid response was identical for the two groups, before and over 75 (patients with corticoresistance: 15% vs 11.4%; NS) and giant cell arteritis-related complications were equivalent (n = 2 vs n = 2; NS). Corticosteroid load was slightly lower in the elderly group (cumulative dose of corticosteroids during the first year of treatment 5.2 g vs 5.8 g; P = 0.03). Patients with rheumatic side effects (collapses of vertebral bodies, mainly) were more frequent in the elderly group (15.5% vs 4.3%; P = 0.01), in spite of a limited mean follow-up period (10.7 months). CONCLUSION: Even if steroid response was identical in the therapeutic course of giant cell arteritis, rheumatic side effects appeared more frequent in the elderly group (over 75 years). In order to obtain a corticosteroid-sparing effect, new studies are necessary to evaluate a reduced initial dosage of corticosteroids.

A randomized, multicenter, controlled trial using intravenous pulses of methylprednisolone in the initial treatment of simple forms of giant cell arteritis: a one year followup study of 164 patients.

OBJECTIVE: (1) To evaluate the corticosteroid sparing effect of an initial intravenous (i.v.) pulse of methylprednisolone (MP) in the treatment of simple forms of giant cell arteritis (GCA). (2) To analyze corticosteroid response, steroid related side effects, and GCA complications. METHODS: Patients received a 240 mg i.v. pulse of MP followed by 0.7 mg/kg/day oral prednisone (Group 1) or 0.7 mg/kg/day prednisone without an i.v. pulse (Group 2, controls), or a 240 mg i.v. pulse of MP followed by 0.5 mg/kg/day prednisone (Group 3). Corticosteroid dosage was reduced after normalization of 2 biological inflammatory variables to obtain half-dosage after 4 weeks in Groups 1 and 2 and 20 mg/day after 2 weeks in Group 3. Tapering was systematically attempted from the 6th month of treatment. RESULTS: One hundred sixty-four patients were included in the trial (1992-96). Cumulative doses of corticosteroids after one year were identical for all groups (p = 0.39). No significant differences were observed in the time required for normalization of C-reactive protein, corticosteroid resistance (13.5%), and corticosteroid related side effects (39% of patients; p = 0.37). Corticosteroid resistant patients received larger doses and showed a high risk of GCA related complications (p = 0.02). CONCLUSION: MP pulses have no significant longterm, corticosteroid sparing effects in the treatment of simple forms of GCA and should be limited to complicated forms. Moreover, corticosteroid resistance is a real risk factor for GCA complications.

[Temporal artery biopsy]. / Biopsie d'artère temporale.

Temporal artery biopsy is performed to confirm the diagnosis of giant cell arteritis. This proof is quite absolute and allows using corticosteroid treatment associated with considerable morbidity in elderly patients. Biopsy is necessary in patient supposed to suffer from giant cell arteritis. Treatment test, which is often difficult to interpret, is done only when temporal artery biopsy is impossible. The biopsy is easy, bilateralisation improves its efficiency. Sometimes the temporal artery biopsy allows to diagnose other vasculitides. Although this biopsy is easy, a strict clinical reflexion must precede its realisation.