[A rare cause of febrile backache, retroperitoneal fibrosis. Apropos of a probably iatrogenic case].
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Biomedical subjects
Publications and source records attributed to C Chapelon.
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Thirty-five cases of biopsy-proven sarcoidosis with neurologic manifestations are reported. Neurosarcoidosis was the presenting symptom in 31% of cases and the only clinical manifestation in 17%. Mean follow-up time was 48 months. Central nervous system involvement was observed in 37% and meningitis in 40% of patients. Other manifestations were cranial nerve palsies (37%), peripheral neuropathy (40%), and myopathy (26%). Multiple neurologic manifestations were present in 51% of cases. All but 4 were treated with corticosteroids. Another immunosuppressive agent or cerebral irradiation was added in 6 and 2 patients, respectively. Complete recovery was observed in 46%, improvement in 46%, 4% remained stable, and 4% worsened. There were no deaths. We advocate treating neurosarcoidosis with corticosteroids as early as possible. If the patient's condition worsens, additional immunosuppressive agents or cerebral irradiation is warranted.
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Between 1974 and 1988, 7 myocardial infarctions occurred in 6 (4 men, 2 women) out of 400 systemic lupus erythematosus patients. Their ages at the onset of lupus ranged from 13 to 44 years (m = 26). Four had renal involvement. Control of lupus in all 6 patients required high-dose steroids (at least 1 mg/kg/d of prednisone). Myocardial infarction occurred 4 to 19 years after the onset of lupus (m = 13). One patient died of cardiogenic shock. When the infarction occurred, only one patient was undergoing a lupus flare, while the disease was quiescent or slightly active in the 5 others. One patient had no risk factors for atheroma but had been taking steroids for 10 years. Among the other 4, one had hypertension, another had hyperlipidemia and 3 were smokers; they had been on steroids for 2, 4, 11 and 13 years. Coronary angiogram showed occlusion in all 4, but atheroma in only 2 patients. Lupus anticoagulant was present in 3 of these 4 patients. The mechanisms responsible for coronary occlusion in lupus patients are probably complex and interwoven. In addition to "classical" factors (i.e., vasculitis or steroid-induced atheroma), other factors, such as antiphospholipid antibodies and/or smoking, may play an important thrombogenic role.
Nailfold capillary microscopy patterns in 100 patients aged more than 65 years and free from inflammatory diseases were compared to those of 100 young healthy adults. A higher prevalence of arteriovenous sludge (36% vs 7%, p less than 10(-6)), increase in capillary loop length (12% vs. 0%, p less than 10(-3)) and especially prominent subpapillary plexus (63% vs 12%, p less than 10(-9)) was found in the geriatric group. Such capillary patterns cannot be considered as abnormal in patients aged more than 65 years. Enlargement of capillary loops and loss of capillaries were never encountered. Bushy capillary formations and hemorrhages were very uncommon in both groups.
Since first individualized in Atlanta in 1981, the frequency of AIDS is constantly increasing and the risk groups (male homosexuals, heroin addicts, transfused hemophiliacs, Haitians and Africans) are no longer the only ones concerned. The progression of the disease in African heterosexuals foreshadows its probable course in the Western World. 8,000 AIDS cases were indexed in Europe as of June 1987; 45,000 are expected in 1989. Cardiologists are a priori much less concerned by this disease than infectious disease specialists, internists, dermatologists, respiratory diseases specialist or neurologists. However, under three circumstances they may have to become involved in this disease. 1) AIDS following blood transfusions: the greater the risk as the amount of blood transfused is important; patients undergoing ECC present an increased risk. As of June 30 1988, 320 cases of posttransfusion AIDS had been reported in France (7.82 p. cent of all reported AIDS cases). The mean incubation period, estimated at 54 months in 1986, would actually be much longer: distribution according to a Gauss curve with a period of 15 years +/- 5, which would lead to expect many cases in the years to come affecting patients who were transfused before August 1985, when systematic screening became mandatory. Since that time, the risk has markedly decreased but is not non-existent (pre-serology phase, contamination with HIV 2), resulting in a limitation of the indications of transfusions and restoring as often as possible to autotransfusion and normovolemic hemodilution.
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Nailfold capillary microscopy was used to study the microcirculation patterns in 26 adult patients with infective endocarditis. Abnormal patterns were found in 13 patients (50%). Enlargement of capillary loops was never observed. Significant correlations were found between the number of capillary abnormalities and both systemic involvement (cutaneous vasculitis, arthritis, splenomegaly and/or glomerulonephritis) and immunological disturbances (circulating immune complexes, rheumatoid factor and/or hypocomplementemia) (p = 0.02 and 0.003, respectively). Capillary abnormalities were significantly reduced in 14 patients studied 4 to 48 months after endocarditis was cured. However, due to the lack of specificity, nailfold capillary microscopy cannot be regarded as a useful tool for the diagnosis of infective endocarditis. Connective tissue disorders are not the sole diagnosis to be considered in patients with abnormal nailfold capillary microcirculation patterns.
Since the first case of endocardial resection I have performed in 1971, many similar cases have been operated on in the world, principally in Equatorian areas such as Africa, Northeastern Brazil and Southern India. In caucasian race, cases are less frequent but are similar to the other ones: in fact, endomyocardial fibrosis which was described by Davies in 1948 in Africa and Löffler's fibroplastie endocarditis, associated with hypereosinophylia (1936) are similar and respond to the same treatment. Our purpose in this paper is to present the long term results of the 34 cases operated at the Broussais Hospital in Paris since 1971. There were 21 males and 13 females, mean age 32 years (8 to 64), 22 were caucasians, 12 black africans. The sites of resection were 8 right ventricular, 4 left and 22 biventricular. Valve replacement was monovalvular in 11 cases and bivalvular in 23, mainly with bioprostheses. There were 7 early deaths and 6 late deaths, 2 after 30 months and 4 after 45, 60, 84, 85 months; only 2 of them were due to cardiac causes. We have not observed any case of recurrence. All surviving patients were in class I/II of N.Y.H.A. despite the fact that 4 have had valvular replacement due to alteration of the bioprosthesis.
Twenty patients with severe systemic disease were treated intermittently with bolus intravenous injections of cyclophosphamide in doses of 0.5 to 1 g. The diseases were systemic lupus erythematosus in 9 cases, systemic necrotizing angiitis in 5 cases, Behçet's disease in 4 cases, Horton's disease in 1 case and dermatomyositis in 1 case. The rationale for this treatment was resistance to previous therapies in 9 patients, initial severity of the disease in 8 and the need for prompt reduction of corticosteroid dosage owing to side-effects in 3 other patients. No death or haemorrhagic cystitis was observed over a cumulative 120 month-patients period. Bacterial infection occurred in 6 cases, including pneumonia (3), and 1 case each of staphylococcal septicaemia, purulent meningitis and urinary infection: none of these infections were life-threatening, and all were cured without sequelae. The drug was well tolerated biologically, except for a moderate fall in neutrophils, lymphocytes and platelets. The systemic disease was stabilized or improved in 16 patients. It remained active and required corticosteroids at the same dosage level in 4 cases. It is concluded that bolus injections of cyclophosphamide are well tolerated in short-term treatments but that their effectiveness must be confirmed by controlled studies.
Neoplastic and non-neoplastic retroperitoneal fibrosis share the same macroscopic and histological features and the same clinical signs which mainly consist of abdominal pain followed by signs of compression. All carry a risk of extension to adjacent structures and may therefore produce serious complications. They may also be associated with a "systemic" syndrome with fever, altered general condition and high erythrocyte sedimentation rate. The necessary laboratory and radiological examinations are detailed. Non-neoplastic retroperitoneal fibrosis may be due to a variety of causes, the most frequent of which today are medications and aortic atheroma. Surgery is usually needed when the disease is life-threatening or of poor functional prognosis. Some authors have suggested that surgery should be combined with corticosteroid therapy. In some cases, regression of the fibrosis could only be obtained by giving steroids in high doses.
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The authors report the most extensive series of malignant primitive cardiac tumours. The patients presented with pericarditis in three cases and with a ventricular rhythm disorder in one patient. The diagnosis was made by non-invasive imaging techniques in three of the four. Angiography was carried out in three cases and was the determining diagnostic procedure in one. Diagnosis of the cardiac tumour was made prior to death in all cases. A histological diagnosis was obtained surgically in two cases. From the therapeutic viewpoint, surgical excision followed by chemotherapy and/or radiotherapy may prolong life. The average survival period of our patients was 11 months. The prognosis appears to be better after early surgical intervention. In view of the variability of the clinical features of cardiac sarcomas, cardiac computed tomography scanning is a valuable complement to cross-sectional echocardiography and, in our opinion, essential for early diagnosis.
Digital subtraction angiography (DSA) as a method of evaluating lower limb arteries required examination of one limb at a time up to now. Moreover repeated injections of contrast material were necessary. This problem was mainly due to the relative small image intensifier field available and to the difficulty of repositioning the patient between mask and image acquisition. The authors report the preliminary results obtained with 24 patients studied on angiographic unit equipped with: A DSA system (DG 300-CGR) using 512 X 512 matrix size, an angiographic table (Angix M 200) allowing an automatic sequential translation, a removable image intensifier using a 16 inch field below the table. Thus, all those characteristics allow DSA of lower limb arteries. Only one injection of contrast material (Ioxaglate: 80 ml at 8-10 ml/second) was used per procedure. This, usually, allows good quality examination of lower limb arteries.
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