Biomedical subjects
Publications and source records attributed to B Wechsler.
Etiologic research into caval syndromes enables distinction to be made between superior caval thrombosis, when there is generally a local cause, and inferior caval lesions that are usually of a general origin. Acute disseminated lupus erythematosus and Behçet's disease should be investigated for, particularly in young patients, when an inflammatory syndrome is detected, and when the thrombus lies in the inferior cava territory.
The long-term outcome of eosinophilic fasciitis (EF) described for the first time by Shulman in 1974 remains unclear. Its exact pathological classification is still contested. We reviewed 11 cases selected exclusively on their initial symptoms being compatible with EF. The outcome of the cutaneous lesions, the investigations of possible visceral involvement were analysed with a follow-up of 1 to 9.5 years (average 4.8 years). Regression of the cutaneous infiltration was complete in 5 cases and partial in 3 cases. No regression was observed in 3 cases. Steroid therapy was given in 10 patients: objective improvement was observed in 5 patients. There was no improvement in the other 5 cases. Ungual capillaroscopy was performed in 9 patients and showed none of the characteristic capillary changes of scleroderma. These results suggest that the prognosis and outcome of EF justify its distinction from systemic scleroderma. The effects of steroid therapy on the cutaneous lesions are very variable.
Splenic involvement is a classical complication of infective endocarditis (IE). Clinical manifestations are rare, 5 out of 100 IE: unexpected rupture (1 case), abscess causing reinfection (2 cases), pseudo-tumour (1 case) and terminal infarction (1 case). In addition to a review of the literature, a post mortem histological study of the spleen of 78 cases of IE was undertaken. Splenic involvement did not seem to be the direct cause of death. Three types of lesions which may or may not be associated were observed: congestive inflammatory lesions, infarction (48 p. 100), abscess (6 p. 100). Splenic infarction usually results in scarring but may progress to abscess formation. Rupture was not observed in this autopsy series. Although splenic involvement is common at post mortem it gives rise to few symptoms. Persistant pyrexia and the appearance of local signs should lead to investigation of splenic complications and eventually, to surgical ablation.
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An unusual case of Whipple's disease is reported. The diagnosis was difficult as the characteristic digestive sign and symptoms (malabsorption, diarrhea, mucosal infiltration by PAS-positive macrophages) were absent. After a ten-year history of seronegative arthritis, myocardiopathy, with aortic insufficiency, basilar pulmonary infiltrates, enlarged lymph nodes, the patient, a 56 years old man, was referred to us for a severe vegetative and neurological dysfunction: stupor, dysarthria, akinesia, hypertonia, hypothermia and abnormal thirst. A CT-scan showed a low-density area of the right hypothalamus, and PAS-positive macrophages were found in a lymph node, in the CSF and in a cerebral biopsy. The patient then received a classical antibiotic treatment, yet the neurologic dysfunction remained severe. Finally, a trial with rifampicin brought a striking improvement of the patient's condition, which has now lasted for three years.
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The authors report on a new case of leiomyosarcoma of the inferior vena cava, to add to the sixty five cases already mentioned in the literature. The patient had been suffering from subcostal pains for 12 years, and an adenomyoma of the gall bladder was diagnosed. The tumor was discovered in the course of a cholecystectomy. The vena cava was resected and ligated below the renal veins. After describing the case, the authors go on to review the literature and stress the fact that the prognosis, which is often severe, could be improved by earlier diagnosis.
Schwartz-Bartter syndrome often results from lung cancer. With reference to two observations, attention is drawn to the value of opacifying the superior vena cava for demonstrating the tumor. This investigation also allows guided biopsy sampling as exemplified in the second observation. Vascular investigations seem all the more useful as small-cell carcinoma often develops outside the bronchi and are often inaccessible by endoscopy.
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Two cases of bullous dermatosis with dysglobulinemia are described. The first one was associated with renal and neuromuscular amyloidosis and production of a monoclonal lambda IgG. Optical and electron microscopy showed amyloid deposits beneath basal lamina of the dermis. Results for direct and indirect immunofluorescence (IF) were negative. This bullous dermatosis is not an epidermolysis bullosa acquisita (EBA), in the strict sense, because the amorphous material deposited is amyloid. In the second case, associated with Waldenström's disease, there was no cutaneous or systemic amyloidosis. Direct IF was positive; linear IgM deposits were seen along the basal membrane of the bulla and the healthy skin. Indirect IF showed the presence of circulating antibodies against basal membrane zone. This bullous dermatosis is probably an EBA, despite the absence of IgG deposits. The absence of electron microscopy does not permit the confirmation of this diagnosis.
Dominant symptoms in 24 patients with neurological manifestations of Behçet's disease were meningo-encephalitis (13 cases), febrile meningeal syndrome (3 cases), intracranial hypertension (6 cases), polyneuritis of the lower limbs (1 case), and isolated retrobulbar ocular neuritis (1 case). Several classical notions concerning the "Neuro-Behçet" meningo-encephalitis were confirmed: the frequency of central motor lesions (33 p. cent), cerebrospinal fluid alterations (anomalies in 100 p. 100 of cases in the initial stage), and the often unfavorable course (40 p. cent). An unusual finding in this series was the frequency of cerebral veins thrombosis (4 certain and 2 probable). These could be differentiated from meningo-encephalitis by their symptomatology, predominantly that of intracranial hypertension, and by their course: often the first disorder to appear in the course of Behçet's disease, they followed a favorable neurological course but were constantly complicated by other vascular manifestations, usually phlebitis. It is suggested that a long-term anticoagulant treatment should be associated with corticoid therapy in these forms.
The authors report their experience of Horton's disease in 47 histologically confirmed cases treated between 1966 and 1979. The symptomatology is recalled with the incidence of the various clinical and biochemical signs. The actuarial survival curve shows a 71.2 p. cent three year and a 61 p. cent five year survival rate. There was no statistically significant difference at three years with a control population. The duration of maintenance therapy was, on average, of 24.8 months, never less than 15 months and sometimes reaching 60 months. The average maintenance dose was 12 mg. Specific and non-specific complications are discussed. The incidence of refractory and cortico-dependent forms shows that the optimal treatment for this disease has yet to be found; the often brilliant initial results of corticotherapy do not reflect its long-term efficacy.
One hundred and three cases of systemic lupus erythematosus (SLE) were reviewed after a period of up to 10 years. The pericardium was involved in 27%, the endocardium in 23% and the myocardium in 14.5% of the cases. Arrhythmia was rare and usually accompanied pericardial or myocardial lesions. Conduction disorders were present in 14.5% of the patients. Atrioventricular block often occurred long after the acute phase of SLE, and synthetic antimalarial drugs appear to be responsible for their development. Whether or not SLE affects the heart, the prognosis is the same during the first ten years of the disease.
Four patients with Behçet's disease (a 42-year-old woman and 3 men aged 24, 32 and 39 respectively), who had received high doses of prednisone (0.5 to 1 mg/kg/day) during a mean period of 21 months developed aseptic osteonecrosis of the femoral heads and condyles which obliged to reduce steroid dosage and to introduce additional drugs. Corticosteroids are useful in Behçet's disease, as in other systemic diseases, but they probably act also on the underlying immune vascularitis. The occurrence of pain in weight-bearing joints during treatment should suggest osteonecrosis and in such cases, beside radiography, scintigraphy with technetium and/or phlebography with pressure measurement might provide an early diagnosis.
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HBs antigen was detected by immunodiffusion or radioimmune assay in the blood of 19 out of 42 patients with periarteritis nodosa examined between 1970 and 1978. Histological or biochemical signs of hepatitis were present in 6% of HBs--positive patients and 1% of HBs-negative patients. The incidence of clinical symptoms was similar in both groups, except for multiple neuritis, which was much more frequent in HBs-positive patients (89%) than in HBs-negative patients (52%; p less than 0.001), suggesting a close relationship between neuritis and HBs and HBs antigen. There was a negative correlation between myalgias and skin lesions and the presence of the antigen. The prognosis of periarteritis nodosa was similar in both groups. The course of the disease seemed to be more prolonged in HBs-positive patients, but there was no significant difference in mortality rate.