Systemic nocardiosis mimicking an ocular relapse of giant-cell arteritis.
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Publications and source records attributed to T Généreau.
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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.
Severe strongyloidiasis, caused by Strongyloides stercoralis, is a preventable life-threatening disease that can occur in any corticosteroid-treated patient who has travelled to a country with infested soil, even if the contact occurred up to 30 years previously. This diagnosis should be considered in corticosteroid-treated patients who experience either unusual gastrointestinal or pulmonary symptoms, or who suffer from unexplained sepsis caused by Gram-negative bacilli. Peripheral eosinophilia is not observed systematically and, even if present, is moderate in most cases. Ivermectine is the best prophylactic and therapeutic option, and thiabendazole should no longer be used. However, guidelines for the prevention and management of S. stercoralis infection in such patients have not yet been established.
PURPOSE: Mooren's ulcer (MU) is a chronic peripheral corneal ulceration featuring conjunctival immunoglobulin deposits. It is considered as the result of a limbic immune process with hyperactivation of T and B lymphocytes. The etiology remains unknown. The response to topical steroid therapy and surgical procedures usually poor and the visual outcome can be devastating. METHODS: Clinical follow-up of 3 patients who had rebel MU to conventional therapy, and were treated with 1g monthly intravenous cyclophosphamide. RESULTS: First patient was a 24-years-old man who had MU in his left eye. The response to surgical procedure and intravenous steroid treatment was poor and corneal perforation occurred. The affected cornea healed after 9 months of Cy treatment. The second patient was a 50-years-old man who had MU in his left eye, which did not improved with lamellar keratoplasty and topical steroid therapy. Corneal healing was obtained after 20 months of Cy treatment. The third patient was a 70-years-old man who presented with a furrowed MU in his right eye which healed with conjunctival resection and 4 months of Cy perfusion. No adverse effects of Cy was noted as opposed to Cy given orally. CONCLUSION: We report the effectiveness of 1g monthly intravenous cyclophosphamide (Cy) treatment in rebel MU. We suggest that immunosuppressive therapy using IV monthly Cy may be proposed in severe rebel MU.
Langerhans cell histiocytosis (LCH), characterised by the infiltration of one or more organs by large mononuclear cells, can develop in persons of any age. Although the features of this disease are well described in children, they remain poorly defined in adults. From January 2000 to June 2001, 274 adults from 13 countries, with biopsy-proven adult LCH, were registered with the International Histiocyte Society Registry. Information was collected about clinical presentation, family history, associated conditions, cigarette smoking and treatment, to assist in future management decisions in patients aged 18 years and older. There were slightly more males than females (143:126), and the mean ages at the onset and diagnosis of disease were 33 years (standard deviation (S.D.) 15 years) and 35 years (S.D. 14 years), respectively. 2 patients had consanguineous parents, and 1 had a family history of LCH; 129 reported smoking (47.1%); 17 (6.2%) had been diagnosed with different types of cancer. Single-system LCH, found in 86 patients (31.4%), included isolated pulmonary involvement in 44 cases; 188 patients (68.6%) had multisystem disease; 81 (29.6%) had diabetes insipidus. Initial treatment consisted of vinblastine administered with or without steroids, to 82 patients (29.9%), including 9 who had received it with etoposide, which was the sole agent given to 19 patients. 236 patients were considered evaluable for survival. At a median follow-up of 28 months from diagnosis, 15 patients (6.4%) had died (death rate, 1.5/100 person years, 95% Confidence Interval (95% CI) 0.9-2.4). The probability of survival at 5 years postdiagnosis was 92.3% (95% CI 85.6-95.9) overall, 100% for patients with single-system disease (n=37), 87.8% (95% CI 54.9-97.2) for isolated pulmonary disease (n=34), and 91.7% (95% CI 83.6-95.9) for multisystem disease (n=163). Survival did not differ significantly among patients with multisystem disease, with or without liver or lung involvement) 5-year survival 93.6% (95% CI 84.7-97.4) versus 87.5% (95% CI 65.5-95.9), respectively; P value 0.1). LCH in adults is most often a multisystem disease with the highest mortality seen in patients with isolated pulmonary involvement. It should be included in the differential diagnosis of disseminated or localised disease of the bone, skin and mucosa, as well as the lung and the endocrine and central nervous system, regardless of the age of the patient. A prospective international therapeutic study is warranted.
OBJECTIVE: Multiple sclerosis (MS) is by far the most popular diagnosis for patients with multifocal neurological disease. Owing to demyelinating inflammatory non-necrotic plaques of the white matter, MS can give remitting symptoms of virtually every part of the central nervous system. Corticosteroids are usually helpful. Devic's neuromyelitis optica (DNMO) is a neurological disease involving only the optic nerves and the spinal cord, where demyelination evolves towards necrosis and atrophy; the prognosis is poor and no satisfactory treatment is known. The objectives of this study are to describe clinical, biological, pathological and radiological data of patients with DNMO and to differentiate DNMO from MS. MATERIAL AND METHODS: We studied the files of 14 patients diagnosed with possible DNMO in three French hospitals between 1980 and 1999 and reviewed the literature. RESULTS: Nine patients were included as definite DNMO. Five were excluded because they did not fulfil the diagnostic criteria. For the nine patients with definite DNMO, DNMO was either monophasic or multiphasic. The prognosis was generally poor: two patients died and five others developed severe disability such as blindness, para or quadriplegia or both. Cerebrospinal fluid study and neuroimaging were essential to confirm the diagnosis of DNMO. Various immunosuppressive treatments generally failed to benefit the patients. CONCLUSION: In the literature (as well as our 14 initial patients) only a few cases of patients described as suffering from DNMO fulfilled the diagnostic criteria. The others showed evidence that another disease like MS was involved. We stress that inclusion and exclusion criteria have to be kept in mind to differentiate clearly DNMO from MS and other central nervous system white matter diseases.
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Explore the source record for details and available documents.
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OBJECTIVES: To describe infectious complications and analyse their risk factors and prognostic role in adults with systemic lupus erythematosus (SLE). METHODS: A monocentric cohort of 87 adults with SLE (1960-1997) was studied to determine the risk factors for infection (disease activity evaluated by SLAM and SLEDAI scores, type of organ(s) involved or any biological abnormality, specific treatments) by comparing patients who had suffered at least one infectious episode (n=35; 40%) with non-infected patients (n=52; 60%). Prognostic indicators were assessed by comparing survivors at 10 years with non-survivors. RESULTS: Of the 57 infectious episodes, 47 (82%) were of bacterial origin, 16 (28%) were pneumonia, and 46 (81%) were community acquired. According to univariate analysis, significant risk factors for infection were: severe flares, lupus glomerulonephritis, oral or intravenous corticosteroids, pulse cyclophosphamide, and/or plasmapheresis. No predictors were identified at the time of SLE diagnosis. Multivariate analyses retained intravenous corticosteroids (p<0.001) and/or immunosuppressants (p<0.01) as independent risk factors for infection, which was the only factor for death after 10 years of evolution (p<0.001). CONCLUSION: In adults with SLE, infections are common and most often caused by community acquired bacteria. Intravenous corticosteroids and immunosuppressants are independent risk factors for infection, which is the only independent risk factor for death after 10 years of SLE evolution.
Hypereosinophilia may be seen in the blood or tissues of cancer patients. A part of lymphomas, the tumors more frequently involved in such eosinophilia, are digestive and respiratory tract carcinomas. The prevalence of cancer-associated eosinophilia is very variable among the different kind of tumors. These cancer-associated eosinophilia are the results of the secretion by tumor or inflammatory cells of interleukin-3 and -5 and GM-CSF. The anti-cancer properties of eosinophilic polymorphonuclear are poorly understood and the prognostic significance of cancer-associated blood or tissue eosinophilia has not been assessed by controlled studies.
INTRODUCTION: The larynx is a rare site of deposition for amyloidosis. Diagnosis may be delayed and evoked in patients with prolonged hoarseness. We have reported two cases of laryngeal amyloidosis. EXEGESIS: One man and one woman suffered from hoarseness during one and three years, respectively. Laryngoscopic examination showed diffuse infiltration of the larynx. Amyloidosis was confirmed by the characteristic Congo-red staining of laryngeal biopsies. The search for other localizations of amyloidosis was negative. No monoclonal plasma cell proliferation was detected. Both patients received endoscopic CO2 laser excision. In one case, chemotherapy was initially associated due to dystrophic plasma cells in bone marrow aspiration and then withdrawn because of clinical failure. With a 6-month follow-up, hoarseness remained stable. CONCLUSION: Laryngeal amyloidosis is an essentially localized disease revealed by hoarseness. Treatment is endoscopic by laser excision. In nearly half of the cases in the literature, it had to be repeated due to localized recurrent lesions. Long-term prognosis of localized laryngeal amyloidosis is better than systemic AL amyloidosis.
Although brain tuberculomas have become rare in developed countries, diagnosis should be kept in mind when confronted with brain space-occupying lesion(s), particularly in immigrants from endemic countries. Surprisingly, Human Immunodeficiency Virus (HIV) infection does not seem to have affected the incidence of this tuberculous lesion. Clinical, biological and radiological signs are only suggestive. Thus, when no other active extracranial tuberculous process is found, the diagnosis should be confirmed by a biopsy before beginning antituberculous treatment which is rapidly effective. Adjunction of steroids may be warranted owing to the common paradoxical enlargement of lesions during the first weeks of therapy, as is now well described in lymph node tuberculosis. Cure can be obtained in more than 85% of the cases, but neurological sequelae are not rare.
Treatment of giant cell (temporal) arteritis is based on steroid therapy, which is sufficient in most cases. Several steroid-sparing strategies have been evaluated to reduce steroids side effects: alternate-day steroid administration or adding a second drug (methylprednisolone, dapsone, azathioprine, methotrexate). The efficacy of these strategies has not been proved. There is no demonstration that a medication (methylprednisolone pulses, dapsone, azathioprine, methotrexate) may be more efficient than an other in resistant giant cell arteritis. Ophthalmologic manifestations have a poor prognosis and usually specific blindness cannot be improved. The localizations of the disease in large arteries necessitate a more important initial corticosteroid dosage (1 mg/kg/d). Vascular surgery is seldom necessary but should not be done in the active phase of the disease. Anticoagulant therapy may be useful to reduce excess vascular mortality but its benefit has not been demonstrated.
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