[Pronostic value of C-reactive protein measure in elderly patient with acquired pneumonia: correlation with Fine's score]. / Valeur pronostique du dosage de la protéine C réactive (CRP) dans les pneumopathies aiguës infectieuses du sujet âgé: corrélation avec le score de Fine 97.

UNLABELLED: The Fine's score, a predictive score of infectious pneumonia gravity, does not integrate inflammatory parameters, which are routinely used in the management of infectious pneumonia. The aim of our study was to establish a correlation between the Fine's score and C reactive protein. PATIENTS AND METHODS: One hundred patients hospitalized for infectious pneumonia, 57 men and 43 female, with an average age of 85 years were retrospectively recorded. RESULTS: The median level of C reactive protein was 157 mg/L. Global mortality rate was 26% and respectively 5, 16 and 45% in the grades III, IV, V of the Fine's score. Beyond 75,5 mg/L C reactive protein concentration, the mortality rate was contained between 28 and 32%. The comparison of the ROC curves of the Fine's score and C reactive protein did not showed any difference. CONCLUSION: C-Reactive protein is less precise than the Fine's score to assess infectious pneumonia gravity but seems to be an indicator of the potential gravity of the pneumonia.

[Aspergillosis in systemic diseases treated with steroids and/or immunosuppressive drugs: report of 9 cases and review of the literature]. / Aspergilloses au cours de maladies systémiques traitées par corticoïdes et/ou immunosuppresseurs: analyse de neuf cas et revue de la littérature.

This is a multicentric retrospective study of aspergillosis in patients treated by corticosteroids and/or immunosuppressive drugs for systemic diseases and a review of the literature. Nine patients, 5 men and 4 women, mean age of 62.8 years old were included among which Horton's diseases (3 cases), systemic lupus erythematosus (2), polymyositis (1), microscopic polyangiitis (1), idiopathic thrombocytopenic purpura (1), rheumatoid polyarthritis (1). Aspergillosis occurred in average 28.4 month after the diagnosis of systemic disease, and 28 months after the beginning of its treatment: corticosteroids in all cases, at a dose of 50.8 mg/day (equivalent prednisone) in average, cyclophosphamide (2 cases), methotrexate (1), intravenous immunoglobulins (1), leflunomide (1). All cases were invasive or chronic pulmonary aspergillosis located in the lungs (6 cases), or in the brain (3). Revealing symptoms were mild and non specific. Lymphopenia was severe in most cases, in average 472 lymphocytes/mm3 and 283 CD4+/mm3. The diagnosis was confirmed 20.75 days after the first symptoms in invasive aspergillosis, and 18.5 months in the chronic pulmonary cases, by cultures in 7 cases (broncho-alveolar lavage: 4; cerebral biopsy: 3), and direct microscopy examination of broncho-alveolar lavage in 2 cases. Specific serology was positive in 4 cases. Patients were treated by voriconazole (4 cases), itraconazole (2), amphotericin B (1), association of caspofungin and voriconazole (1), successive voriconazole and itraconazole (1). Six patients recovered from aspergillosis with 10.8 months of following time, 3 patients died a few days after confirmation of the diagnosis. Fifty-four cases of the literature are analysed.

[Influenza vaccination among health-care workers. Vaccination rates in universitary hospital of Besançon, winter 2003-2004]. / La vaccination antigrippale du personnel hospitalier. Enquête de prévalence au CHU de Besançon, hiver 2003-2004.

BACKGROUND: Influenza vaccination reduces the mortality of the patients when the vaccination rates of healthcare workers is important. PURPOSE AND METHOD OF THE STUDY: To investigate the vaccination rates at the Universitary Hospital of Besançon by anonymous questionnaire. RESULTS: Three thousand hundred seventy-seven answers were analyzed (228 men and 1145 women). Two hundred seventy-seven persons declared receiving the vaccine (20.1%) corresponding to sixty-three men (27.6%) and two hundred thirteen women (18.6%) (P = 0.001). The average age of the healthcare workers vaccined was of 38.9+/-11 years. Among most than 50 years, 34% was vaccined. Among the doctors, 40.5% were vaccined against 20.6% of the nurses. In the services of geriatrics, 78.5% of the staff was vaccined. CONCLUSIONS: Our results indicate a weak rate of influenza vaccination in our establishment and a misunderstanding of the character nosocomial of the influenza among the nurse.

[Protein Z: a new factor of thrombophilia?]. / La protéine Z: un nouvel élément du bilan de thrombophilie?

INTRODUCTION: Sometimes, in front of a clinical setting of thrombophilia, the biological findings are helpless. Therefore we suggest to test a protein Z deficiency. EXEGESIS: Protein Z is a vitamin-K dependent protein forming a complex with the Z protein-dependent protease inhibitor for inhibiting the activated factor X; so protein Z acts as a "natural low molecular weight heparin". The prothrombotic phenotype associated with protein Z deficiency includes early fetal losses (before the 20th week of gestation), early and relapsing venous thrombosis in patients with factor V Leiden mutation and somehow ischaemic stroke in young people. CONCLUSION: The protein Z deficiency seems to be associated with a particular prothrombotic phenotype including early fetal losses as well as early and relapsing venous thromboses in patients carrying the factor V Leiden mutation. It is unclear whether or not it plays a role as a thrombophilic factor especially in the arterial vascular field.

[Oncogenic osteomalacia: the role of the phosphatonins. Diagnostic usefulness of the Fibroblast Growth Factor 23 measurement in one patient]. / L'ostéomalacie oncogène : rôle des phosphatonines. Intérêt du dosage du fibroblast growth factor 23 démontré chez un malade.

INTRODUCTION: Oncogenic osteomalacia (OO) is a rare paraneoplastic syndrome characterized by severe hypophosphatemia induced by phosphaturic factors which are secreted by some tumors of mesenchymal origin. Fibroblast Growth Factor 23 (FGF-23) belongs to this family. Measurement of FGF-23 might improve the diagnosis of OO. EXEGESIS: We report the case of 71-year-old Caucasian man who had a history of severe osteomalacia with multiples fractures and extreme hypophosphatemia with hyperphosphaturia and normal serum calcium level. Serum FGF-23 was 199 RU/ml (N < 100 RU/ml). The tumor, detected by F-18 FDG PET/CT SCAN was localized in the mandible. Surgical removal of the tumor relieved all symptoms with normalization of serum phosphate levels within 3 days after surgery. CONCLUSION: We conclude that FGF-23 measurement is likely to be of considerable importance for facilitating early diagnosis of OO.

Acute dysautonomia secondary to autoimmune diseases: efficacy of intravenous immunoglobulin and correlation with a stimulation of plasma norepinephrine levels.

Acute dysautonomia is a disorder characterized by severe sympathetic and parasympathetic failure with relative preservation of motor and sensory function. The disease is considered to be idiopathic in most cases, but there is now a trend towards considering the disorder as an uncommon variant of Guillain Barré syndrome. We report two cases of acute dysautonomia which did not fulfill the criteria of the idiopathic form. The first case was associated with Sjögren's syndrome and the second with thyroiditis and antiganglioside antibodies which were correlated with the severity of the disease. Intravenous gammaglobulin (IVGG) was effective in both cases, as has been reported for the idiopathic form, and in one case the treatment was associated with an increase in the supine and standing plasma norepinephrine levels, thus substantiating the positive effects of IVGG on the orthostatic blood pressure and heart rate. We conclude that the spectrum of acute dysautonomia is superimposable on that of the inflammatory peripheric neuropathies and should include both the idiopathic form and dysautonomia with autoimmune associated disorders. IVGG are effective and seems to act by increasing plasma norepinephrine levels.

Amyloid arthropathy revealed by RS3PE syndrome.

Amyloid arthropathy is a form of primary AL amyloidosis with a monoclonal component in the blood and/or urine, and RS3PE syndrome is acute edematous polysynovitis in subjects older than 60 years. A 74-year-old man was diagnosed with both disorders. He was admitted for benign acute polyarthritis of the hands and feet and reported carpal tunnel symptoms predominating on the right. A synovial biopsy at the right wrist disclosed deposits that stained with Congo red even after potassium permanganate treatment (positive Wright's test). Articular AL amyloidosis was diagnosed. The symptoms resolved under glucocorticoid therapy alone, casting some doubt on their relationship with the amyloidosis. Roentgenograms showed geodes, a feature not present in RS3PE. Whether RS3PE may be among the possible presentations of articular amyloidosis is discussed.

[AA Amyloidosis: recent knowledges on pathophysiology]. / Amylose AA : données actuelles sur la physiopathologie.

PURPOSE: Amyloidosis is a rare disease associated with an underestimated frequency because of the need of a pathological diagnosis identifying extracellular deposits with affinity for Congo red. There are moreover 20 proteins that can form extracellular fibril deposits. Some amyloidosis forms are more common than others, especially AA amyloidosis and AL amyloidosis. Among genetic amyloidosis, the transthyretin related amyloidosis is the most prevalent. The amyloid frequency could also be increased if amyloidosis related to Alzheimer's disease or prion's disease is included. In the absence of specific treatment for amyloidosis, researches are focused on amyloidosis pathophysiology especially, on AA amyloid pathophysiology. CURRENT KNOWLEDGE AND KEY POINTS: Amyloid is not only composed of fibrils but also of proteoglycanes, P component and amyloid-enhancing factor. A new research aim is focused on the cells involved in amyloid formation and on the relationship between amyloid, proteoglycanes and P component. FUTURE PROSPECTS AND PROJECTS: It was demonstrated that, in the absence of macrophages, an extracellular amyloid formation was possible with amyloid-enhancing factor as starting point. Some inhibitors of intra or extracellular amyloid formation are still to be discovered. Anti-P component has been recently developed; it was successful in the treatment of murin AA amyloidosis and gave some hope concerning the treatment of human amyloidosis.

[Relative value of different antiphospholipid antibodies detected in a department of internal medicine: retrospective study of 124 patients]. / Valeur relative des différents anticorps antiphospholipides détectés dans un service de médecine interne: étude rétrospective chez 124 patients.

PURPOSE: The value of antiphospholipid antibodies (aPL) detected in the sera of the patients of an Internal Medicine department is not univocal and is still much debated. To test the contribution of such new markers, we reviewed the records of patients having antiphospholipid antibodies detected between 1996 and 1997. METHODS: One hundred and twenty four patients, having at least one of these two aPL: lupus anticoagulant (LA), anticardiolipin antibodies (aCL), or one of these two anti-proteins: anti-beta 2glycoprotéin I antibodies (anti-beta 2GPI) or anti-prothrombin antibodies (aPT), were studied. LA was detected by a PTT-LA technique and aCL, anti-beta 2GPI and aPT by ELISA-sandwich techniques. For each patient we recorded sex, age, personal and familial history of thrombosis, fetal losses and systemic disease, the reason of aPL detection, the final diagnosis, activated partial thromboplastin time (aPTT), platelets count and type of aPL. RESULTS: The population was composed of 77 women (62%) and 47 men (38%) with a mean age of 54 years [12-92 years]. A thrombocytopenia was strongly correlated to aCL presence (OR = 6.15 et p = 0.03). The reason of aPL detection was venous thrombosis, recurrent fetal losses, systemic disease, infectious disease or fortuitous discovery of a prolonged aPTT. The final diagnosis was a systemic disease in 57% of cases, an infectious disease in 14.5%, a thrombosis in 4.5% and a neoplasia in 3%. LA was detected in 54% of patients, aCL in 39.5%, anti-beta 2GPI in 23% and aPT in 31%. No relationship between the aPTT value and the type of aPL could be established. CONCLUSION: Our study shows that familial histories of venous thrombosis or systemic disease are useful to enhance antiphospholipid antibodies detection; that LA is mostly associated to systemic and infectious diseases; that aCL and anti-beta 2GPI are predominant in case of venous thrombosis and that thrombocytopenia has to enhance aCL detection and the discussion about a possible APS.

[Value of eosinopenia in inflammatory disorders: an "old" marker revisited]. / Valeur de l'éosinopénie dans le diagnostic des syndromes inflammatoires: un " vieux" marqueur revisité.

PURPOSE: The value of eosinopenia as a test in favour of an infectious disease was suggested by Schilling since 1929. We tried to verify this hypothesis with a prospective and multicentric study. PATIENTS AND METHODS: One thousand and thirty-eight patients were included (82 females and 56 males, means age: 71.8 years). Diagnoses were: 83 infectious diseases, 38 systemic diseases, 10 neoplasia and 7 miscellaneous. RESULTS: The mean value of eosinophils was 72/mm3 in bacterial infectious diseases and 214/mm3 in non infectious diseases (p < 0.01). When leukocytes were higher than 10,000/mm3 and eosinophils counts less than 40/mm3, predictive value for an infectious bacterial disease was 100% as well as specificity. Under same conditions, when protein C reactive was higher than 100mg x l(-1), the predictive value was 85% and the specificity was 57%. CONCLUSION: Our study shows that an inflammatory syndrome associated with hyperleucocytosis above 10,000/mm3 and eosinophils counts under 40/mm3 seems strongly related to bacterial infectious diseases.

[Liveoid vasculopathy with combined thrombophilia: efficacy of iloprost]. / Vasculopathie livédoïde et thrombophilie combinée: efficacité de l'iloprost.

INTRODUCTION: Livedoid vasculopathy is characterized by early, focal painful purpuric lesions of the lower skin extremities without histologic finding of small vessel vasculitis. EXEGESIS: A 38-year-old man was seen in our unit for painful purpuric lesions of both feet localized on toes and external sides. Skin biopsy showed dermic vessel thrombosis and endothelial cell proliferation. Lupus anticogulant antibody was positive in association with a heterozygous factor V (Leiden) gene mutation (G1691A). Anticoagulation failed to relieve pain and cutaneous lesions. Intravenous iloprost, a prostacylcin analogous (Ilomedine) was dramatically and rapidly effective in our patient. CONCLUSION: Livedoid vasculopathy is a cutaneous affection related to vascular thrombotic events in which thrombophilia plays a central role. Iloprost might be an interesting alternative treatment of painful purpuric lesions when anticoagulant treatments are ineffective.

[Involvement of thoracic aorta revealing temporal arteritis. Three cases]. / Atteinte de l'aorte thoracique inaugurale de la maladie de Horton. A propos de trois cas.

INTRODUCTION: Temporal arteritis involves large vessels in 15% of cases. Their discovery is usually late, commonly several years after the diagnosis. EXEGESIS: We describe three cases of temporal arteritis with thoracic aorta involvement: two patients presented with aorta aneurysm which revealed temporal arteritis, one patient had aortic insufficiency. Clinical features of temporal arteritis were absent in two patients. Temporal artery biopsy, performed in two cases, was positive. Two patients were treated with corticosteroids, associated in one case with immunosuppressive agent. Surgery was necessary in all patients. CONCLUSION: Thoracic aorta involvement in Horton's disease is most often discovered when corticotherapy is decreased. Aneurysm rupture or aortic dissection in aortic affection are the major complication. Patient with thoracic aorta involvement need a clinical and radiological follow up over long period.

[Treatment of recurrent ulceration with low doses of thalidomide. Pilot study in 17 patients]. / Traitement de l'aphtose récurrente par thalidomide à faible dose. Etude pilote chez 17 patients.

PURPOSE: Though thalidomide in a dosage of 100 mg/day is the standard treatment for recurrent oral and genital ulcers (OGU), its toxicity would be less important with lower dosage, while its efficacy would be identical. Furthermore, duration of treatment might be a major risk factor for the development of subsequent polyneuropathy. To determine the dosage of thalidomide leading to the best efficacy/toxicity ratio, a pilot study was conducted from 1993 to 1996. METHODS: Seventeen patients with OGU (mean age: 43 years, sex-ratio: 12:5) were included in the study and presented either recurrent oral ulcerations (8 patients), oro-genital ulcerations (3 patients), Behçet disease (4 patients), or recurrent OGU associated with leukemia (2 patients). The initial dosage of thalidomide was 50 mg/day (1 tablet) for 1 month. If the patient's condition improved, the dosage was reduced to one tablet every other day for 1 month and one tablet every 3 days thereafter. Nerve conduction studies (EMG) were performed at inclusion in the study and every 6 months thereafter. RESULTS: Among the 17 patients, remission was observed in ten patients within the first month of treatment and the condition of seven patients improved. Complete remission was observed in six patients after a 2-month treatment and in one patient after 4 months. A 200-mg/8 days dosage induced prolonged remission in 12 patients. Among them, ten patients received a 150-mg dosage over 8 days thereafter and disease relapsed in four of them. Among the six patients who received a 100-mg dosage over 8 days, only one relapse was observed. EMG showed a decrease in sensory nerve action potentials in six patients after 8 months and a half on average. Only three patients had to discontinue their treatment due to the occurrence of either paresthesia (2 patients) or areflexia (1 patient). Our study shows that initially a 50-mg/day dose is efficacious in the treatment of OGU and that administration of one tablet every 2 or 3 days is efficacious in more than 60% of the patients to maintain remission. CONCLUSION: A dosage of 50 mg/day is initially efficacious in most cases, provided that the patient is carefully followed up to allow early detection of potential peripheral neuropathy.

[Tick-born encephalitis]. / L'encéphalite virale à tiques.

INTRODUCTION: Tick-borne encephalitis (TBE), a disease contracted through tick bites, is caused by a Flavivirus. Its geographical distribution comes from the geographical distribution of the reservoir of infection--i.e., mainly the tiny mammals living in the forests and bushes. The endemic area spreads from the Rhine to the Urals, from Scandinavia to Italy and Greece. CURRENT KNOWLEDGE AND KEY POINTS: Symptoms usually evolve in three phases: at first a nonspecific phase with fever and myalgia, then an afebrile phase, and finally a phase with neurological manifestations, such as meningitis, meningoencephalitis and/or myelitis, and fever. Motor neurological sequelae are possible. The cases occurring in the East are characterized by their greater severity compared to those occurring in the West. The diagnosis, easily established given a history of a tick bite in an endemic area, is confirmed by the presence of specific IgM in the blood and/or cerebral spinal fluid. FUTURE PROSPECT AND PROJECTS: There is no specific treatment. Prevention consists of individual prophylactic measures (self-examination and systematic extraction of ticks after exposure, use of repellents), and in immunization. The vaccine, prepared from inactivated viruses, should be used for target populations, that is, for people exposed to tick bites during their professional or leisure outdoor activities.

[Myelodysplasias and systemic diseases. A non-fortuitous association]. / Myélodysplasies et maladies systémiques. Une association non fortuite.

PURPOSE: Myelodysplastic syndromes are clonal hematologic disorders, expanded from myeloid stem cells. A primitive immunologic disorder is discussed. This hypothesis could explain a non-casual association with systemic diseases. The aim of our study is to test this hypothesis. METHODS: We retrospectively investigated the data of 60 patients with myelodysplastic syndromes (group I) hospitalized in our unit from 1990 to 1999. The frequency of systemic disorders was screened and compared to controls (group II). Group II consisted of 120 patients matched for age and sex and hospitalized in the same hospital during the same period. RESULTS: Sixty patients were included (mean age: 83 years old). Myelodysplastic syndrome subtypes were refractory anemia with excessive blasts (52%), refractory anemia (43%) and sideroblastic anemia (5%). Fourteen cases of systemic manifestations were reported in group I (23%) and five in the controls (4%) (P < 0.0001). Systemic manifestations in group I included vasculitis in six cases (42%), polyarthritis in three cases (21%), systemic amyloidosis AA in two cases (14%), relapsing polychondritis in one case, pyoderma gangrenosum in one case and celiac disease associated with a systemic granulomatosis in one case. In the controls, vasculitis was present in four cases and polyarthritis in one. Median age at onset of myelodysplastic syndrome was not influenced by the association with systemic disorders which, in return, have not influenced the myelodysplastic syndromes' subtypes. Myelodysplastic syndromes succeeded to systemic manifestations in 71.4% of cases and could not be attributed to immunosuppressive therapy. CONCLUSIONS: The association of myelodysplastic syndromes with systemic manifestations seems not to be casual. It raises the hypothesis of a primitive immunological disorder in both diseases. Moreover, the description of two cases of systemic amyloidosis and one case of pyoderma gangrenosum might suggest an additional disorder of macrophages or granular cells.

[Wegener's disease and central diabetes insipidus]. / Maladie de Wegener et diabète insipide central.

Vasculitis with central diabetes insipidus is a rare condition which must be recognized for proper management. We report a case of Wegener's granulomatosis with diabetes insipidus and review the literature concerning the clinical and radiological features of this association. We examined the data supporting the efficacy of cyclophosphamide in diabetes insipidus. Diabetes insipidus is inaugural in 46% of cases. Wegener's granulomatosis has no distinctive features when associated with diabetes insipidus. MR imaging reveals an enlargement of the posterior pituitary in 100% of cases. Cyclophosphamide is effective in 80% of cases. In Wegener's granulomatosis, pituitary vasculitis is a rare condition. MR imaging is the most accurate method for pituitary exploration. Cyclophosphamide is effective in the treatment of vasculitis lesions in Wegener's granulomatosis.

[Withdrawing corticosteroid therapy: the stress of the last milligrams...]. / L'arrêt de la corticothérapie: le stress des derniers milligrammes...

Suppression of the hypothalamo-pituitary-adrenal axis (HPA) is a classic complication of corticosteroid therapy. There is some controversy over the conditions necessary to recover normal HPA activity. Most agree that the total duration of treatment, the maximal dose, the total cumulative dose and the the rate of withdrawal are all important factors in predicting HPA suppression. Recent studies provide evidence contradicting this opinion. It is now demonstrated that HPA activity is completely recovered as soon as the prednisone dose falls below 5 mg per day, that adrenal function is totally blocked for doses above 7.5 mg per day, and that an intermediary response to synactene is obtained for daily doses of 7.5 to 5 mg. It is thus reasonable to conclude that corticosteroid therapy can be safely withdrawn when the daily dose is less than 5 mg and, if a higher dose is given, a temporary increment would be indicated in case of stress.

[Nail manifestations of systemic diseases]. / Manifestations unguéales des maladies générales.

General diseases-associated nail disorders may be classified into 5 types according to their clinical impact. Type I refers to nail disorders considered as peculiar features among the various symptoms of general disease such as peri-ungueal vascular dilatation, onycholysis, Beau lines, opaque nails or melanonychia. Type II refers to nail disorders with a strong clinical value such as Terry's nails, Muerhrcke's lines or koilonychia. Type III refers to nails disorders presenting as significant syndromes of several diseases such as yellow-nail syndrome of subungueal splinter haemorrhages, and type IV to specific disorders considered as true keys for diagnosis such as digital hippocratism, Bazex's acrokeratosis, Koenen's tumor, or nail hypoplasia. In type V are included drug-induced nails disorders especially after chemiotherapy or antiretroviral therapy.