Exposure to silica and primary Sjögren's syndrome in a dental technician.
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Biomedical subjects
Publications and source records attributed to L Sailler.
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Symptomatic macroglossia occurs in some rare congenital muscle diseases, such as Becker's and Duchenne's dystrophies or Pompe's disease. Herein we describe a case of symptomatic macroglossia with myositis of the tongue occurring in a patient with polymyositis. Tongue myositis was evidenced by dysarthria, frequent biting during mastication, swallowing difficulties without aspiration, and noisy breathing. Magnetic resonance imaging showed homogeneous hypertrophy of the tongue, especially the mouth's floor muscles. The diagnosis of tongue myositis was established by electromyography and biopsy. No other cause for the macroglossia was found. Symptoms resolved quickly with corticosteroid and intravenous immunoglobulin treatment. To our knowledge, this is the first reported case of symptomatic tongue myositis occurring in the course of polymyositis.
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PURPOSE: To assess the efficacy and tolerance of three methylprednisolone boluses (500 mg/d) followed by a standard dose of prednisolone, 20 mg/d, as the initial treatment of non-complicated giant-cell arteritis. METHOD: A retrospective study of 15 cases. RESULTS: Six men and nine women with a mean age of 70.9 years were treated and followed for 41.5 months. Initial mean ESR was 83 mm; mean C-reactive protein level was 94.6 mg/L. The boluses were well tolerated, excepted in one patient who developed acute psychosis. After initiating the oral treatment, two patients presented signs of clinical relapse during the first month, and were given higher doses of corticosteroids. At 1 month, 12 patients were asymptomatic, nine of whom had normalized ESR and CRP. Mean ESR was 23; mean CRP was 13 mg/L. At 3 months, the mean prednisone dose delivered was 18.2 mg/d. Mean ESR was 12 mm. The cumulative prednisone dose given during the first year was 5,349 (+/- 2,512) mg. In the 13 patients who necessitated no more than 20 mg/d prednisone, no sequelae of giant-cell arteritis, no fractures nor major treatment intolerance occurred-during the first 2 years of treatment. Treatment was stopped in eight patients after a mean duration of 48.6 months. CONCLUSION: Treatment with pulse methylprednisolone 500 mg/d for 3 days followed by 20 mg/d oral prednisone could be a valuable corticosteroid-sparing strategy in many patients with uncomplicated temporal arteritis.
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Non-complicated giant-cell arteritis is defined by the absence of ischemic complications, a remarkable response to corticosteroid treatment, and the absence of corticodependence. It is the most common type of giant-cell arteritis. Treatment relies on corticosteroids (prednisone or prednisolone) at an initial dosage of 0.7 mg/kg/j. The median duration of treatment is 2 years, but many patients need a more prolonged course. Side effects are frequent, especially atherosclerosis and osteoporosis. There is no demonstrated efficiency of corticosteroid sparing agents such as hydroxychloroquine or methotrexate. Dapsone may be efficient, but is not indicated because of its serious side-effects.
Trichinellosis is an uncommon condition that is nevertheless a continuing problem as shown by two recent outbreaks in our region. The clinical features are reviewed briefly. The physical and laboratory test findings in the 117 patients affected during the first outbreak (February 1998) are reported, as well as the methods used to treat these patients.
The development of mutations conferring drug resistance was investigated in 49 antiretroviral-naive asymptomatic HIV-1 subjects with CD4+ cell counts of 250-500/mm3 given intermittent (6-week courses, 6 weeks apart) or continuous treatment with zidovudine (AZT) plus zalcitabine (ddC) over 54 weeks. The concentration of human immunodeficiency virus type 1 RNA in the plasma and the CD4 cell counts were measured every 6 weeks. The rate of decrease of HIV-1 RNA concentration in plasma after a 6-week course of AZT + ddC was similar for each treatment cycle (approximately 1-log reduction). The plasma HIV-1 RNA concentration returned to its initial level at each treatment interruption. The mean CD4 cell counts after 54 weeks in the two treatment groups were similar. Genotype analysis by sequencing the reverse transcriptase coding region from plasma viral RNA on treatment showed a lower frequency of AZT resistance mutations after 54 weeks in patients given intermittent treatment (18%) than in those treated continuously (79 %, P < 0.001). No mutations conferring ddC resistance or multidideoxynucleoside resistance were observed in either group. These findings may have clinical implications for long-term treatment strategies.
We describe the causes of reactive hemophagocytic process in a retrospective study including 99 patients. The main diagnosis were: lymphomas (18 cases), pyogenic bacteria infections (15 cases), herpes virus infections (12 cases), other infections (multiple, parasitic, fungal, mycobacterial, unidentified) (11 cases), acute hepatitis (five cases), systemic lupus erythematosus (three cases). We also found numerous other diseases involving the reticuloendothelial system. The cause remained undetermined in 16 cases. Lymphoma accounted for 64% of the cases in previously healthy patients who had been febrile for more than 10 days at the time of the diagnosis of reactive hemophagocytic process, and for 31% in HIV-positive patients. Lymphomas were rare (5%) in non HIV-positive, immunosuppressed patients. In this setting and in previously healthy patients who had been febrile for less than 10 days, infectious diseases were widely dominant (respectively 60% and 86% of the cases). Those were mainly due to pyogenic bacteria and to herpes virus. A rapidly fatal evolution occurred in some cases of lymphomas-related hemophagocytic process. These data support the choice of aggressive investigations in order to diagnose lymphoma in previously healthy patients presenting with reactive hemophagocytic process who have been febrile for more than 10 days, and in selected HIV-patients. Such a procedure is not recommended in the other cases.
Acquired von Willebrand's disease associated with a monoclonal gammopathy and angiodysplasia of the gut is a rare disorder. It is sometimes complicated by chronic intestinal bleeding and severe anemia, that is poorly responsive to usual treatments. We report such a new case that has been revealed by anemia, and characterised by the absence of the high-molecular weight multimers. The correction of the hemostasis defect and of anemia were related to the reappearance of the high-molecular weight multimers, that was achieved only after high-dose intravenous immunoglobulin courses. The perfusions were performed every 3 weeks for 2 years without loss of efficiency, that could be explained by the dissociation of immunoglobulin-von Willebrand's factor complex.
Licorice abuse is a wellknown cause of high blood pressure, myopathy, and cardiac rhythm trouble. It should be considered as a cause of diffuse acute edema, as shown in the three following case-reports.
We report an acute respiratory distress by diaphragmatic involvement due to dermatomyositis. A fifty year-old patient with typical dermatomyositis presented an acute respiratory insufficiency with hypercapnic coma due to diaphragmatic muscle involvement. Respiratory state required mechanical ventilation initially and improved secondarily gradually upon corticosteroids and intravenous immunoglobulins. Only few cases of acute respiratory distress in dermatomyositis due to respiratory muscle involvement are reported in literature.