[Illnesses unrelated to systemic sclerosis (IUSS) diagnosed throughout the patient's follow-up in a referral center: A cohort of 200 patients]. / Affections non-directement attribuables à la sclérodermie systémique (ANDAS) diagnostiquées au cours du suivi dans un centre de compétence: à propos d'une cohorte de 200 patients.

INTRODUCTION: Our work aimed to investigate the illnesses unrelated to systemic sclerosis (IUSS), diagnosed among patients with systemic sclerosis (SSc) throughout their follow-up in a referral tertiary care center. METHODS: All the patients with SSc followed in the Internal Medicine Department of the University Hospital between October, 2014 and December, 2015, were included. We specifically reviewed the medical records of the patients who exhibited IUSS, defined as an illness that could not be considered as a typical clinical manifestation or as a usual complication of the disease. RESULTS: Two hundred patients were included, and 38 IUSS were diagnosed among 31 SSc patients, over a 4 years median follow-up period. These diagnoses included vascular diseases (26%), heart diseases (21%), neoplasia (8%), infectious diseases (6%), autoimmune diseases (5%), endocrinopathies (5%), and others (24%). The median follow-up time before IUSS diagnosis was two years. Seventeen (45%) of these diagnoses were considered in patients showing suggestive clinical signs. A specific therapy was delivered in 25 cases (66%). Group comparisons revealed that dyslipidemia was more frequent in patients with IUSS (OR = 2.6 [1.1-1.5]; p = 0.014), while no differences were found for the other characteristics. Especially, no association between auto-antibodies specificity and the occurrence of IUSS was found. CONCLUSION: This study focused on IUSS in SSc patients and highlights the need for a polyvalent clinical approach all along the follow up of SSc patients.

Combined pulmonary fibrosis and emphysema in systemic sclerosis: A syndrome associated with heavy morbidity and mortality.

BACKGROUND: The syndrome of combined pulmonary fibrosis and emphysema (CPFE) primarily due to tobacco smoking has been reported in connective tissue disease, but little is known about its characteristics in systemic sclerosis (SSc). METHODS: In this retrospective multi-center case-control study, we identified 36 SSc patients with CPFE, and compared them with 72 SSc controls with interstitial lung disease (ILD) without emphysema. RESULTS: Rate of CPFE in SSc patients with CT scan was 3.6%, and 7.6% among SSc patients with ILD. CPFE-SSc patients were more likely to be male (75 % vs 18%, p < 0.0001), smokers (83 % vs 33%, p < 0.0001), and to have limited cutaneous SSc (53 % vs 24% p < 0.01) than ILD-SSc controls. No specific autoantibody was significantly associated with CPFE. At diagnosis, CPFE-SSc patients had a greater decrease in carbon monoxide diffusing capacity (DLCO 39 ± 13 % vs 51 ± 12% of predicted value, p < 0.0001) when compared to SSc-ILD controls, whereas lung volumes (total lung capacity and forced vital capacity) were similar. During follow-up, CPFE-SSc patients more frequently developed precapillary pulmonary hypertension (PH) (44 % vs 11%, p < 10-4), experienced more frequent unscheduled hospitalizations (50 % vs 25%, p < 0.01), and had decreased survival (p < 0.02 by Kaplan-Meier survival analysis) as compared to ILD-SSc controls. CONCLUSIONS: The CPFE syndrome is a distinct pulmonary manifestation in SSc, with higher morbidity and mortality. Early diagnosis of CPFE by chest CT in SSc patients (especially smokers) may result in earlier smoking cessation, screening for PH, and appropriate management.

[Screening for pulmonary arterial hypertension in patients with systemic sclerosis: Comparison of DETECT algorithm to decisions of a multidisciplinary team, in a competence centre]. / Dépistage de l'hypertension artérielle pulmonaire au cours de la sclérodermie systémique : comparaison de l'algorithme DETECT à une discussion pluridisciplinaire en centre de compétence.

INTRODUCTION: Pulmonary arterial hypertension (PAH) is a severe complication of systemic sclerosis and detecting PAH efficiently remains challenging. The DETECT study has offered in 2013 a composite screening tool for PAH. The objective of our study was to compare the indication of right heart catheterisation (RHC) as suggested by the DETECT algorithm with the decisions of a multidisciplinary team. METHODS: This prospective monocentric non-interventional study consecutively included systemic sclerosis patients when data required to apply DETECT algorithm were available. We evaluate the number of RHC as requested by this algorithm and confronted it with the indications of RHC suggested by a multidisciplinary group blinded for the result of DETECT algorithm. RESULTS: In total, 117 systemic sclerosis patients were included. When DETECT algorithm was applied to all patients, RHC was suggested by this algorithm for 70 patients, whereas only 15 indications were required by the multidisciplinary group; among those patients only 7 had PAH. When DETECT algorithm was applied only to the 42 patients with DLCO<60% and disease duration of more than 3 years, RHC was suggested for 31 patients whereas only 13 were indicated by the multidisciplinary group; among those patients only 7 had PAH. CONCLUSION: The DETECT algorithm is able to efficiently detect all PAH patients finally diagnosed by our multidisciplinary team. However, it increases by 3 the number of RHC that should be performed.

[Hereditary thrombophilia testing and its therapeutic impact on venous thromboembolism disease: Results from a retrospective single-center study of 162 patients]. / La recherche de thrombophilie héréditaire et son impact thérapeutique dans la maladie thromboembolique veineuse : résultats d'une étude monocentrique rétrospective sur 162 patients.

INTRODUCTION: Venous thromboembolic disease is a multifactorial, frequently recurrent pathology, whose treatment is based on anticoagulation. As part of the etiological investigation, screening for an inherited thrombophilia is framed by French guidelines published in 2009. The aim of the study is to assess the contribution of inherited thrombophilias testing in common practice. METHOD: This is a retrospective single-center observational study. Over a period of a year, all records of patients who were screened for a hereditary thrombophilia were analyzed. The conformity of the indication of hereditary thrombophilia workup in balance with the guidelines, its completeness and therapeutic impact were studied. RESULTS: Of the 494 records analyzed, 225 were related to venous thromboembolism. Among them, there were 162 pulmonary embolisms or deep vein thrombosis of the lower limbs. In this subgroup, 57 % of records complied with guidelines and 69 % were complete. Thirty-four thrombophilias were highlighted: 4 protein S deficiencies, 1 protein C deficiency, 4 combined deficiencies, 17 factor V Leiden mutations and 8 factor II G20210A mutations. For one patient, hereditary thrombophilia diagnosis had profoundly changed the curative therapeutic approach. CONCLUSION: Adherence to French guidelines remains limited. In clinical practice, diagnosis of hereditary thrombophilia has little impact on the curative therapeutic approach in venous thromboembolic disease.

[Quick diagnostic and therapeutic assistance unit: activity and impact on relationship between internal medicine and general practitioners]. / Unité d'assistance diagnostique et thérapeutique rapide : bilan d'activité et impact sur les relations médecine générale et médecine interne hospitalière.

INTRODUCTION: General practitioners report difficulties to work efficiently with hospital physicians. We created a phone number dedicated to general practitioners to contact directly a hospital physician in the general internal medicine department entitled "quick diagnostic and therapeutic assistance". The aim of this study was to assess the first year activity of this professional support and its impact on general practitioners. RESULTS: We received 663 phone calls from February 2005 to February 2006. This led to a simple medical advice (41%), and immediate (26%) or delayed (32%) consultation or admission. Results of the mail survey showed that this "quick diagnostic and therapeutic assistance" was helpful for the general practioners. CONCLUSION: Quick diagnostic and therapeutic assistance improve the quality of clinical care through better continuity of care between the public hospital and the general practitioners.

[Efficiency of rituximab in acquired hemophilia: report of two cases and review of literature]. / Efficacité du rituximab dans les hémophilies acquises: à propos de deux cas et revue de la littérature.

INTRODUCTION: Acquired hemophilia due to an inhibitor of factor VIII is a rare clinical situation. EXEGESIS: Rituximab is now used in the treatment of acquired hemophilia. We report two cases of acquired hemophilia treated by rituximab with efficiency. CONCLUSION: Rituximab appears to be a first line immunosuppressive therapy in acquired hemophilia, especially in post-partum hemophilia.

[Diagnosis of solid malignancies in an internal medicine unit: a series of 114 consecutive cases]. / Le diagnostic des cancers solides dans un service de médecine interne: étude descriptive de 114 cas consécutifs.

PURPOSE: A deterioration of the general condition, a prolonged fever or an unexplained inflammatory syndrome are frequent reasons for hospitalization in a internal medicine unit. In these situations, it is not rare to make a diagnosis of cancer. PATIENTS AND METHODS: A descriptive study was carried out over a three years period (1st October 1999 to 30th September 2002) in an internal medicine unit. Every week, all patients in whom a cancer was diagnosed were enrolled in the study. RESULTS: During this period, 165 patients were identified (3.8% of the in-patients). A histological proof was obtained in 114 patients. Digestive and bronchopulmonary cancers were the most frequent. The first signs were very varied but digestive disorders and ferriprive anaemia were the most frequent. The number of investigations necessary to diagnosis were weak (1.56 procedures) when a sign was identified but were high (5.12 procedures) when no information was provided by interview, clinical examination or usual biological tests. CONCLUSION: Diagnosis of cancer is an usual situation in an internal medicine unit. Interview and clinical examination are essential in the diagnostic step. It could decrease the number of procedures. Internal unit services are fully concerned by the announcement of cancer.

[Thoracic infectious aortitis: microbiology, pathophysiology and treatment]. / Aortites thoraciques infectieuses: implications microbiologiques, physiopathologiques et thérapeutiques.

BACKGROUND: Thoracic infectious aortitis are currently rare. They are always lethal without any treatment. The microorganisms involved are numerous with particular pathophysiological characteristics for each bacterium. Treatment is difficult and must associate medical and surgical care. RECENT FINDINGS: Bacterial epidemiology of infectious aortitis has been profoundly modified with the large use of antibiotics. Syphilitic aortitis were frequent in the beginning of the twentieth century but its incidence has dramatically fallen. It still exists and its clinical presentation must be known to begin an adequate treatment. Other bacterial aetiologies of these aortitis are more classical with high frequencies of Staphylococcus aureus and Streptococcus, which are often associated with infective endocarditis. Among Gram-negative bacteria, Salmonella spp are the most frequently met microorganisms. Atherosclerosis represents the principal risk factor of these infectious aortitis. It provokes arterial parietal damage useful for bacterial attach. A saccular aneurysm of infective origin can then appear. Treatment must consist on antibiotics before surgery; Tuberculous aortitis are also possible but are much more rare. CONCLUSION: Thoracic infectious aortitis are very rare but must be known because of their poor prognosis. Treatment is difficult and prevention of atherosclerosis which is the most important risk factor of these diseases is therefore of greatest importance.

[Cryptococcal infections in non-HIV infected patients. Study of four cases and review of literature]. / Infections à cryptocoque chez des patients non VIH. A propos de quatre cas et revue de la littérature.

BACKGROUND: Cryptococcal infections are frequent in HIV-infected patients and are regularly looked after. This infection may occur in others immunosuppressives situations and, in those cases, diagnosis is often delayed. METHODS: We report four cases of cryptococcal infections in patients whose immunosuppression isn't related with HIV infection but due to chronic lymphocytic leukemia, giant cell temporal arteritis, gastric neoplasm and lupus. Diagnosis, prognostic and treatment are detailed. RESULTS: Four patients aged from 25 to 76 presented a cryptococcal infection (three meningitis). A woman died at the admission. Another died seven years later. The two others are still alive under treatment. When infected, all patients were immunodeficiency. CONCLUSION: Cryptococcal infection may occur in patients non-HIV-infected patients. Early detection is needed to improve prognostic.

[Syphilitic aortitis. Experience of an internal medicine unit]. / Les aortites syphilitiques. Expérience d'un service de médecine interne.

INTRODUCTION: Infectious aortitis remains a rare disease. It is characterized by an endarteritis of infectious origin generally followed by the development of a so called mycotic aneurysm. Those infectious aneurysms account for 0.5 to 1.3% of all aortic aneurysms. Of the infectious agents, Treponema pallidum has a particular place. Cardiovascular syphilitic infection was very common at the beginning of the XX(th) century with a prevalence of 6.9% of all autopsies. In 1950-1960, the prevalence had decreased to less than 1%. Since 1990, syphilis was considered as disappeared. EXEGESIS: we report syphilitic aortitis in four patients. Diagnosis, treatment, and prognosis are detailed. CONCLUSION: A syphilitic infection of the aorta should be looked for in every patient suffering from an inflammatory or infectious disease of aorta.

[Factors of immunodepression in patients with tuberculosis]. / Facteurs d'immunodépression chez des patients tuberculeux.

OBJECTIVES: To study the frequency of a factor of immunodepression in patients with tuberculosis, the differences in presentation, and the diagnosis and therapeutic management according to the immune status. METHODS: Retrospective study of the files of patients hospitalised in the University Hospital Centre of Rennes in 1998 for a Mycobacterium tuberculosis infection. Comparison of two populations, immunodepressed versus non immunodepressed. RESULTS: 75 patients aged 20 to 91 were included, 41 patients were considered immunodepressed and 34 non immunodepressed. The causes of immunodepression were: HIV infection (n = 2), diabetes (n = 4), chronic alcoholism (n = 12), chronic respiratory diseases treated with corticosteroids (n = 6), neoplasia (n = 9), and inflammatory diseases (n = 7). Comparison between the 2 populations revealed more a frequent history of tuberculosis in the immunodepressed (p = 0.04), shorter delay before diagnosis (p = 0.04), greater frequency of disseminated forms (p = 0.02) and enhanced mortality (p = 0.01). There was no difference in the 2 groups with regard to the clinical signs having evoked tuberculosis, the diagnostic method, the bacteriological results or the modalities of treatment. CONCLUSION: The frequent reactivation of tuberculosis in immunodepressed patients and the severity of the infection in these patients should evoke tuberculosis and the rapid initiation of an efficient treatment in such patients. In the case of alteration in immune defences, prophylactic treatment should help to reduce the number of such reactivations.

[Digital flow exploration by color Doppler ultrasound in patients with Raynaud's disease or systemic sclerosis]. / Exploration de la microcirculation pulpaire par écho-Doppler digital chez des patients présentant une maladie de Raynaud ou une sclérodermie.

BACKGROUND: Raynaud's phenomenon (RP) is a frequent vascular paroxysmal syndrome of the extremities. Generally benign, the condition is called Raynaud's disease (RD), which may reveal a connective tIssue disease, particularly systemic sclerosis (SS). We evaluated digital blood flow in patients with RD and SS using color Doppler ultrasound. PATIENTS AND METHODS: Ultrasound examination was performed with a newly developed multi-D linear array transducer (VFX 13-5 Siemens), which allows better resolution. We first measured the diameters of the digital arteries (appendix 1) then epidermal, dermal, and hypodermal thickness for each patient (appendix 3) and performed a qualitative and quantitative analysis of pulpar microcirculation (appendix 4 & 5). All measures were made at 25 degrees C, 45 degrees C, 11 degrees C and after recovery. Thirty-three patients were included: 14 with primary RD and 19 with SS as assessed by American College of Rheumatology criteria. RESULTS: The diameters of the digital arteries showed significant discrepancies allowed to distinguish primary RP from SS. At 11 degrees C, diameters were 0.6 +/- 0.2 mm for primary RP versus 0.2 +/- 0.3 mm for SS on lateral digital arteries (LDA) [p=0.005]; 0.7 +/- 0.2 mm for primary RP versus 0.4 +/- 0.3 mm for SS on medial digital arteries (MDA) [p=0.004]. After recovery, these diameters were respectively 1 +/- 0.2 mm versus 0.5 +/- 0.4 mm for LDA [p=0.000] and 1 +/- 0.2 mm versus 0.6 +/- 0.3 mm for MDA [p=0.000] (tables 1, 2, 3, 4). Similarly, pulpar vascularization was significantly higher in primary RP than in SS (tables 6, 7, 8). No difference was found in skin thickness nor in the epidermal aspect between the two groups (table 5). CONCLUSION: Color Doppler ultrasound shows morphological and dynamic differences between RD and SS.

[Epidemiologic description of amyloidosis diagnosed at the University Hospital of Rennes from 1995 to 1999]. / Epidémiologie descriptive des amyloses diagnostiquées au CHU de Rennes de 1995 à 1999.

INTRODUCTION: The frequency of amyloidosis is not well known in France. We compiled a register of amyloidosis diagnosed from 1995 to 1999 in the University Hospital of Rennes. PATIENTS AND METHODS: This retrospective study was performed between 01 January 1995 and 31 december 1999. Diagnosis was assessed on positivity of red Congo by anatomopathology. Immunohistochemistry allowed the definition the type of amyloidosis. Clinical data, staging and outcome of patients were analysed. RESULTS: Forty-three amyloidosis were diagnosed (27 women, 16 men) with an incidence of 8,6 new cases per year. Mean age was 63.7 years. Five diagnosis were realised in 1995, six in 1996, six in 1997, 12 in 1998, 14 in 1999. Twenty amyloidosis were AL type (46.5%), seven AA (16.3%), 1 beta2 microglobulin type, 15 (35%) remained of undetermined type. Thirty-three amyloidosis (77%) were systemic, 10 were localized to one organ (23%). When diagnosis was made, biopsies concerned affected organs in 86% of the cases, accessory sites (labial salivary glands, bone marrow) in only 14% of the cases. Twenty-five patients died (58%). Two deaths were treatment-related, 16 to amyloidois, seven patients died of another complaint. CONCLUSION: Increased incidence of amyloidosis needs to be confirmed. We emphasize the importance of immunohistochemical typing on frozen samples, the value of accessory biopsies and the need for complete extension staging.

Hyperferritinemia revealing Gaucher's disease.

Hyperferritinemia is frequently observed during Gaucher's disease but has never been described as an initial manifestation. We report the case of a 57-year-old woman without a previous medical history who presented with hyperferritinemia and IgG monoclonal gammopathy. The diagnostic procedure was negative except for the bone marrow biopsy, which revealed Gaucher's cells. Low beta-glucocerebrosidase activity in leukocytes confirmed the diagnosis of adult Gaucher's disease. We discuss the differential diagnosis and the mechanisms of hyperferritinemia in this disease.

[Cannabis arteritis: four new cases]. / Artériopathies du cannabis: quatre nouveaux cas.

INTRODUCTION: The responsibility of cannabis in juvenile thromboangeitis has been suggested for few years. We describe four new cases. EXEGESIS: Young men presented with distal arteriopathy of the lower limbs in 3 cases, and of the left upper limb in the remaining patient. Symptoms occurred progressively, distal pulses had disappeared, and distal necrosis was constant. Three patients suffered from Raynaud phenomenon, none of them presented with venous thrombosis. Radiologic evaluation revealed distal abnormalities in all cases, and proximal arterial thrombosis in one case. The four patients were cannabis smokers for at least four years. With cannabis interruption and symptomatic treatment, lesions improved for three patients. For one of them, recurrence of arteriopathy occurred when he resumed to smoke cannabis. For the fourth one who never stopped cannabis, an amputation was necessary. CONCLUSION: Search for cannabis use is important because interruption may improve prognosis.

[Nonsurgical treatment of chronic subdural hematoma with steroids: two case reports]. / Traitement des hématomes sous-duraux chroniques par les corticoïdes: à propos de deux observations.

INTRODUCTION: Hemorrhages beneath the dura define a subdural hematoma. EXEGESIS: The course of chronic subdural hematoma may be asymptomatic for many months before diagnosis. Recovery is observed in 80% of patients after surgery. We report two cases of chronic subdural hematoma successfully treated with steroids. CONCLUSION: Steroids can be an alternative treatment of subdural hematoma, specially in elderly or alcoholic patients.

[Tracheobronchial amyloidosis: apropos of 2 cases]. / Amylose trachéobronchique: à propos de deux cas.

INTRODUCTION: Tracheo-bronchial amyloidosis is an uncommon localized form of amyloidosis. We report two new cases. EXEGESIS: Two patients had developed expiratory dyspnea for several months. CT-scan and flexible bronchoscopy confirmed tracheal narrowing and a diagnosis of tracheo-bronchial amyloidosis was made by tissue biopsies. The immunohistochemical type was AL in one case, undetermined in the other case. There was no argument for systemic involvement. The two patients benefited from bronchoscopic dilatation. This treatment improved clinical symptoms and pulmonary function tests with a follow up of 12 and 18 months respectively. CONCLUSION: Tracheo-bronchial amyloidosis is a localised form of amyloidosis with various respiratory symptoms. Diagnosis is made by CT-scan and flexible bronchoscopy that allows biopsies. Immunohistochemical type is more often AL. Recurrence, respiratory insufficiency and tracheo-bronchial metaplasia are the most important complications. Treatment consists of bronchoscopic dilatation or excision, and bronchoscopic laser-YAG. Pulmonary function testing allows precise follow-up.