The multiple clinical aspects of lupus.

New insights about the following aspects of lupus erythematosus are developed: (1) thrombotic and neurological manifestations associated with the presence of antiphospholipid antibodies; (2) cardiac complications, especially coronary ischemia, conduction abnormalities and valvular involvement; (3) pregnancy, with particular attention to fetal and maternal prognoses, Soulier-Boffa syndrome and congenital atrioventricular heart blocks; (4) subacute lupus erythematosus, bullous lupus and cutaneous manifestations associated with the lupus anticoagulant.

[Myocardial infarction in Behçet's disease]. / Infarctus du myocarde au cours de la maladie de Behçet.

A case of myocardial infarction in a 23-year old male patient with Behçet's disease is reported. The infarction occurred 4 years after the onset of the disease, which had been marked by recurrent venous thrombosis. Coronary arteriography showed stenosis of the anterior interventricular artery and occlusion of the first diagonal artery; the other coronary vessels were normal. A search for vascular risk factors, including haemostasis, was undertaken, yielding only moderate cigarette-smoking. About 10 cases of myocardial infarction associated with Behçet's disease have been reported. They concerned young, usually male subjects. Infarction usually occurred late in the course of the disease, and vascular risk factors were seldom elicited. The leukocytoclastic vasculitis of Behçet's disease alone may be responsible for stenosis, thrombosis and false arterial aneurysms, as shown by anatomical studies. The physiopathological mechanisms involved (reduction of endothelial or systemic fibrinolytic activity, rise in fibrinogen and factor VIII) are still unclear; we believe that these abnormalities are inconstant. Behçet's disease may be regarded as a possible cause of myocardial infarction in young subjects.

[Digestive manifestations of periarteritis nodosa in a series of 120 cases]. / Les manifestations digestives de la périartérite noueuse dans une série de 120 cas.

In a series of 120 patients with periarteritis nodosa (PAN), 50 had gastrointestinal manifestations; 34 had transient abdominal pain which regressed spontaneously or in response to corticosteroid therapy and required no further investigation. Thirty one more serious episodes occurred in the remaining 26 patients. Eight of these were in fact the initial signs of PAN and 13 required laparotomy. There were 20 episodes of abdominal pain (peritonitis: 9, pancreatitis: 4, acute cholecystitis: 2, duodenal ulcer: 3, intestinal infarction: 1, unexplained pain without diagnosis at laparotomy: 1) and 11 of gastrointestinal hemorrhage (melaena or hematemesis: 4; hematochezia: 5). Clinical and biological features of patients with and without gastrointestinal manifestation were not significantly different except for cardiac involvement which was significantly more frequent (p less than 0.05) in the second group. Corrected survival rates were significantly lower (p less than 0.05) in patients with gastrointestinal manifestations. These results show that, in patients with PAN, digestive manifestations, particularly perforations, carried a poor prognosis. Nevertheless exploratory laparotomy and surgery unrelated to PAN (eg appendicectomy) were well tolerated.

[Medical treatment of Behçet's disease]. / Traitement médical de la maladie de Behçet.

The treatment of Behçet's disease is essentially symptomatic and depends on the severity of the manifestations. Colchicine is usefull in the minor, particularly mucocutaneous forms. It can be safely used at a dose of 1 mg/day as maintenance treatment to prevent or limit the acute episodes. Other immunomodulators have been proposed: levamisole, disulon, thalidomide, the indications for which are limited by the side effects. Non-steroidal anti-inflammatory agents are indicated in the articular forms. Steroid therapy remains essential in the severe neurological and ophthalmological forms at an initial dose of 1 mg/kg/day. It can be preceded by the intravenous bolus administration of high doses of methylprednisolone. Immunosuppressants are useful in the same indications. Although some authors propose them right from the start, we prefer to reserve them for second line treatment in the event of steroid dependence or recurrence, because of their short-term infectious and long-term oncogenic risks. Cyclosporin, which has been proven to be effective by randomized studies, is difficult to manage and responsible for complications, in particular renal. It can therefore only be used as second line treatment by experienced users. Plasmapheresis is reserved as an emergency procedure for functionally threatening forms. Venous or arterial thromboses justify long-term anticoagulation, possibly associated with platelet anti-aggregants. In this chronic disease, the quality of follow-up and the patient's cooperation are essential in order to intervene rapidly and to detect as early as possible the complications of the disease and of the treatment.

[Venous thrombosis in Behçet's disease]. / Les thromboses veineuses dans la maladie de Behçet.

Venous lesions in Behçet's disease (BD) were defined by Adamantiades and represent one of the most suggestive signs of the disease. They are occasionally the first sign of the disease and are frequently the basis for the diagnosis in a case of recurrent thrombosis in a young subject, the preferential context of BD. Involvement of superficial vessels is virtually constant. Venous vasculitis is responsible for non-specific hypersensitivity and erythema nodosa, which constitute some of the major diagnostic criteria. Ocular periphlebitis is one of the elements responsible for posterior uveitis. The originality of the venous involvement is due to the involvement of deep territories. Any vein may be affected, but the remarkable features are the size of the thrombosed vessels: superior and inferior vena cava, iliofemoral veins and the unusual site of the involvement: supra-hepatic veins, cerebral vessels, etc. Inferior vena cava thrombosis may be associated with aneurysms of the pulmonary arteries in the context of Hughes-Stovin syndrome. Cerebral phlebitis, which can now be identified more easily by means of digital angiography, is responsible for a typical picture: headaches, bilateral papilloedema and raised CSF pressure. The classical pictures of optic chiasmatic arachnoiditis and so-called benign intracranial hypertension actually correspond to unrecognised phlebitis. They may also be associated with other neurological lesions. In one half of cases, phlebitis cutaneous manifestations. However, they may precede the diagnostic signs or may occur very late in the course of the disease. They are recurrent and affect a number of different territories.(ABSTRACT TRUNCATED AT 250 WORDS)

[Aortic aneurysm with vena cava thrombosis occurring in Behcet's disease]. / Anévrisme aortique avec thrombose cave contiguë au cours d'une maladie de Behcet.

We report a case of Behcet's disease complicated by aortic aneurysm and contiguous vena cava thrombosis due to compression. Arterial aneurysms are uncommon in the course of Behcet's disease and are associated with a poor prognosis owing to the risk of rupture. Vena cava thrombosis is found in 10% of cases; pulmonary embolism is infrequent. Venous and arterial lesions usually evolve independently. In most cases they are consecutive to vasculitis. The case reported herein is uncommon because of simultaneous and contiguous venous and arterial lesions. Eighteen months after aorto bi-iliac graft and inferior vena cava ligature, there is no recurrence of thrombosis nor aneurysm with a treatment including heparin, colchicine and azathioprine.

[Popliteal venous aneurysm revealed by recurring pulmonary embolism. Echographic, phlebographic, x-ray computed tomographic and nuclear magnetic resonance aspects]. / Anévrisme veineux poplité révélé par des embolies pulmonaires récidivantes. Aspect échographique, phlébographique, tomodensitométrique et en résonance magnétique nucléaire.

We report a characteristic case of popliteal vein aneurysm which was demonstrated not only by Doppler ultrasonography and venographic examination, but also by CT scan and magnetic resonance imaging. A review of the literature underlines the rarity of these aneurysms, since less than 20 cases have been published. They are always true aneurysms, most often revealed after an episode of pulmonary embolism. Doppler ultrasonography and venography confirm the diagnosis. The place of CT scan and magnetic resonance imaging remains to be defined. Even if asymptomatic, the embolic risk necessitates surgical resection of the aneurysm and restoration of venous continuity.

[Renal and digestive amyloidosis and Behçet's disease. Apropos of a case]. / Amylose rénale et digestive et maladie de Behçet. A propos d'un cas.

The authors report the case of a 33-year old male Tunisian who, 7 years after the onset of complete Behçet's disease (diagnosed on international criteria) developed a nephrotic syndrome diagnosed as renal amyloidosis on histological evidence. Attenuation of Congo red staining by potassium permanganate pointed to AA type amyloidosis. Despite treatment with colchicine and cyclophosphamide the patient's condition deteriorated. Amyloid deposits were found in a small bowel biopsy performed for persistent diarrhoea. The patient died 3 years after amyloidosis was diagnosed. The authors have reviewed the literature concerning this exceptional association: in all cases where it was typed the amyloidosis was AA. Attention is drawn to the failure of colchicine in this patient.

[Systemic lupus erythematous and seminoma of the ovary]. / Lupus érythémateux disséminé et séminome de l'ovaire.

A new case of ovarian seminoma associated with systemic lupus erythematosus is reported. The neoplasia was discovered during a flare-up of lupus. Excision of the seminoma followed by radiotherapy resulted in a 7 1/2-year long remission of systemic lupus erythematosus. This case agrees in every detail with a previously reported case, which suggests a pathogenetic relation between the two diseases. Although this association is rare, clinicians must be aware of its possibility and cautious when confronted with an ovarian abnormality in a young woman with systemic lupus erythematosus.

[Wegener's granulomatosis disclosed by clinical symptoms of Horton's disease]. / Granulomatose de Wegener révélée par un tableau évocateur de maladie de Horton.

In a series of 60 patients with Wegener's granulomatosis, 2 had initially presented with clinical signs suggestive of temporal arteritis. One of these two patients was a 69-year old woman suffering from inflammatory pain in the shoulders, wrists and knees, myalgias in the lower limbs and intermittent jaw claudication. The other patient was a 60-year old man with febrile polyarthritis predominantly affecting the knees and shoulders, and hyperaesthesia of the scalp. In both cases biopsy of the temporal artery gave negative results. Corticosteroids provided a dramatic improvement, but a relapse corrected the diagnosis. Three similar cases have been reported, but only one had a histological lesion of the temporal artery. Cases of temporal arteritis associated with pulmonary granulomatosis raise the problem of classification with localized Wegener's disease. An initial presentation suggestive of temporal arteritis may hide other systemic diseases, notably rheumatoid arthritis, periarteritis nodosa or Chug and Strauss angitis; Wegener's granulomatosis must be added to this list.

[Long-term prognosis of arterial lesions of Behçet's disease]. / Pronostic à long terme des lésions artérielles de la maladie de Behçet (MB).

We analyzed the charts of 25 patients with arterial lesion in Behçet's disease with mean follow-up 76 months. We conclude that prognosis is poor. Corticosteroids and surgery if possible is needed, anti-aggregant or anticoagulants and prosthetic rather than venous graft are suitable. This therapy does not exclude the risk of relapse. Immunosuppressive drugs in addition to corticosteroids seem to be more efficient than corticosteroids alone but the therapeutic modalities need to be define.

[Circulating anticoagulants excluding hemophilia. Multicenter survey undertaken under the aegis of the French National Society of Internal Medicine apropos of 207 cases]. / Anticoagulants circulants en dehors de l'hémophilie. Enquête multicentrique conduite par le secrétariat à la recherche de la SNFMI à propos de 207 observations.

A retrospective multicentre study, undertaken under the aegis of the French National Society of Internal Medicine, involved 200 subjects with acquired circulating anticoagulants; 130 were female and 77 were male; mean age was 45 +/- 23 years (range: 10 months to 80 years). Mean duration of follow-up was 23 months. In 130 subjects the anticoagulants were detected as a result of a systematic screening examination. The main overt clinical manifestations were haemorrhages, venous or arterial thrombosis and spontaneous abortion. Typing of the anticoagulant, performed in 166 cases, showed the presence of an antiprothrombinase in 141; this enzyme is not responsible for severe bleeding unless it is associated with other disorders of coagulation; less frequent were an anti-factor VIIIc (n = 16) and an anti-factor V (n = 2) anticoagulants. An underlying pathology was found in 172 subjects, including systemic lupus erythematosus (n = 60), induced lupus (n = 11), discoid lupus (n = 3), infection (n = 23), blood disease (n = 19), cancer (n = 15) and vasculitis (n = 15); other factors were pregnancy (n = 5) and medicines (n = 6). The anticoagulant disappeared spontaneously in 10 cases and in 33 of the 115 subjects treated. In subjects with lupus and in children under twelve years of age, an antiprothrombinase was regularly identified at typing.

[Ocular prognosis in Behçet's disease]. / Pronostic oculaire de la maladie de Behçet.

The ocular prognosis of Behçet's disease was assessed in 31 French native patients with ocular involvement. The mean follow-up time was 6 years. Ophthalmologic manifestations revealed the disease in 61% of patients, and it was the first symptom in 29%. Two groups of patients could be identified: the minor forms (23%) which remained stable with colchicine alone and the severe forms (77%) which required steroid (32%) and/or immunosuppressive (45%) therapy. In the severe forms, 32 eyes were unchanged, 4 eyes showed improvement, and 12 eyes showed deterioration in visual acuity. Positivity of HLA B5, sex and complete form of the disease were not predictive factors of ocular involvement, or of its seriousness. At 6 years, 16% of the eyes (versus 14% at the beginning) had a visual acuity less than 1/10, and 71% (versus 74% at the beginning) had a visual acuity greater than 8/10. A close collaboration between ophthalmologists and internists allows a good visual prognosis in Behçet's disease.

[Anti-cytoplasmic antibodies of neutrophil granulocytes in systemic vasculitis]. / Anticorps anti-cytoplasme des polynucléaires neutrophiles dans les vascularites systémiques.

Autoantibodies to the cytoplasmic components of neutrophil polymorphonuclear granulocytes (ACPA) were searched for by indirect immunofluorescence on ethanol-fixed smears from normal white blood cells in 248 sera from patients with Wegener's granulomatosis, Kawasaki disease and various other vasculitides, connective tissue diseases, nephropathies and infiltrative pulmonary diseases. Apart from a weak positivity in a few Kawasaki disease sera, ACPA were found in Wegener's granulomatosis (and micropolyarteritis) only, with a correlation between ACPA titers and disease activity. These results confirm the value of ACPA in the diagnosis and evaluation of activity of Wegener's granulomatosis.

[Intra-alveolar hemorrhage in Wegener's granulomatosis. Retrospective study of 9 cases]. / Hémorragies intra-alvéolaires de la granulomatose de Wegener. Etude rétrospective de 9 cas.

Among 66 patients with Wegener's granulomatosis, 9 had an intra-alveolar haemorrhage which revealed the disease. The diagnosis was based on dyspnoea (n = 9), haemoptysis (n = 9) and anaemia (n = 9) with a mean haemoglobin level of 8 +/- 1 g/dl. Radiology showed bilateral alveolar infiltrates (n = 9), and numerous siderophages were found either in the alveolar lavage fluid (n = 7/7) or in sputum (n = 2). In every case, the alveolar haemorrhage was accompanied by a rapidly progressive extracapillary glomerulonephritis and by lesions of the upper airways which preceded it by several months or years. All patients received corticosteroids combined, in 8 cases, with cyclophosphamide. The respiratory disease improved rapidly, in contrast with the renal disease which became worse (n = 5). Two patients died in the acute phase of the vasculitis: one of acute renal failure, the other of infectious shock.

[Specificity of CA 125 tumor marker. A study of 328 cases of internal medicine]. / Spécificité du marqueur tumoral CA 125. Etude de 328 observations de médecine interne.

The sensitivity and specificity of CA 125 were evaluated retrospectively in 328 patients, some of them with several diseases, seen between January, 1985 and December, 1986. High levels of CA 125 were found in 110 assays performed in patients with solid tumour (43 cases), peritoneal, pleural or pericardial effusion (39 cases), infection (34 cases), malignant blood disease (3 cases) and various non-malignant and non-infective diseases (45 cases). High CA 125 levels were most frequently observed in patients with effusion. The frequency of cancer increased with the CA 125 titre. CA 125 levels were particularly high in ovarian, peritoneal and uterine carcinomas. It was higher in ovarian cancer than in other types of cancer and further elevated in the presence of effusion, irrespective of the primary cancer. All patients with ovarian carcinoma had ascites. The sensitivity of CA 125 was 59 per cent and its specificity 74 per cent in solid tumours and malignant blood diseases. The corresponding figures in solid tumours alone were 72 per cent and 75 per cent respectively. While there can be no doubt that CA 125 is valuable in the follow-up of patients with ovarian carcinoma, the clinical context and notably the presence of an effusion must be taken into account when asking for a CA 125 assay to evaluate the cause of an inflammatory syndrome or of an alteration of the general condition, since CA 125 levels may be raised in a wide variety of non-malignant diseases, and especially in effusions and infections. In this study, a CA 125 titre higher than 1,000 U/ml was always due to the presence of cancer; lower titres must be interpreted according to the clinical context and sometimes the course of the disease.

[Value of the cyclophosphamide bolus in severe systemic diseases. Preliminary results]. / Intérêt des bolus de cyclophosphamide dans les maladies systémiques graves. Résultats préliminaires.

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.

[Wegener's granulomatosis in elderly subjects. 37 cases]. / Granulomatose de Wegener du sujet âgé. Trente-sept cas.

The clinical, biochemical and prognostic characteristics of 37 patients with Wegener's granulomatosis were studied in relation to age. Eleven patients were older than 60 years when the first sign of the disease appeared. Except for the fact that the renal lesions seemed to be more severe in these patients, there was no significant clinical or biochemical difference between them and patients under 60. Five patients over 60 years of age died during the first 2 years of Wegener's granulomatosis. Death was due to infection in 3 cases, to acute renal failure as complication of intra-alveolar haemorrhage in 1 case and to metastatic cancer in 1 case. Age-related comparisons of survival rates showed that the probability of surviving was lower in elderly subjects: 78 per cent at 1 year and 39 per cent at 3 and 5 years, as opposed to 96 per cent at 1 and 3 years and 84 per cent at 5 years in younger subjects (P less than 0.01). This poor prognosis is probably due to the renal lesions which constitute an important cause of death in the published series. Rapidly progressive glomerulonephritis being amenable to an early and intensive treatment, elderly patients should be treated as energetically as younger patients.

[Arterial embolism of the limbs in infectious endocarditis of the heart valves]. / Embolie artérielle des membres au cours des endocardites infectieuses sur valves natives.

Nine cases of infective endocarditis (IE) on native valves, complicated by arterial embolism of the limbs (AEL), were collected between June 1974 and October 1988 (prevalence 4.3 percent). Among the 11 AEL recorded, 9 involved the lower limbs and 2 the upper limbs. The diagnosis, suspected in patients with acute ischaemia (n = 6), transient (n = 1) or pseudophlebitic (n = 1) pain, or discovered by systematic pulse examination (n = 3), was confirmed by Doppler ultrasound (n = 3), angiography (n = 2) or oscillometry (n = 4). AEL occurred 2.8 weeks on average after the onset of treatment; it appeared 6 months after the end of treatment in 1 case and preceded the diagnosis of IE by 1 to 6 weeks in 3 cases. The causative organisms isolated in 7 cases were: non-haemolytic streptococci (n = 4), Staphylococcus aureus (n = 1), Haemophilus parainfluenzae (n = 1) and enterococcus (n = 1). Vegetations were found in 6 of the 7 patients explored by echocardiography. Two cases of embolism of the femoral artery required embolectomy. Effective heparin anticoagulation was obtained in only one patient. Six patients underwent valve replacement in the acute phase of endocarditis. After a mean follow-up period of 32 months (range 3 to 120 months), only one patient has symptoms (claudication of the left upper limb); 5 patients are asymptomatic with a reduced (n = 5) or abolished (n = 2) pulse. Three embolisms have left no sequelae. Altogether, AEL are not uncommon in infective endocarditis. They rarely influence the functional prognosis and are detected by systematic palpation of the pulses. Anticoagulation in effective doses is discussed. Attempts at removing the obstruction should be made only in cases with poorly tolerated proximal embolism. In patients with multiple or recurrent embolic accidents, valve replacement may be considered.

[Prostatic location as the manifestation of lymphoma]. / Localisation prostatique révélatrice d'un lymphome.

The authors report a case of non-hodgkin's lymphoma initially presenting with pallakiuria due to a prostatic localization of the tumour. The diagnosis was confirmed 6 months later following deterioration in the general condition with lymphomatous meningitis, after repeat examination of the prostatic slides. Less than twenty cases of prostatic lesions revealing lymphoma have been reported in the literature. There are no formal diagnostic arguments apart from the histology which is often difficult to interpret. In most cases the lymphomas are disseminated and composed of large cells or cleaved small cells with a poor prognosis and a mean survival of 14 months. Treatment primarily consists of chemotherapy, as the lymphoma is always disseminated. The role of local radiotherapy is questionable.