[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.

[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.

[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.

[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.

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.

[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.

[Epidemiologic study of pregnancy complicated by lupus]. / Recueil épidémiologique des grossesses lupiques.

Pregnancy is allowed in an increasing number of women with systemic lupus erythematosus (SLE). In order to determine the influence of pregnancy on SLE and vice versa, a French epidemiological register was established on 1 January 1987, under the aegis of the Société Nationale Française de Médecine Interne. As of 1 December 1989, 55 pregnancies in 51 women coming from 24 different Departments of Medicine were recorded. Intermediate results were analyzed from 42 women (2 successive pregnancies) of which 46 pregnancies (2 twin pregnancies) have come to term. Pregnancy can be allowed in women with SLE. The disease influences its evolution in nearly two-thirds of the patients, but without maternal death. Among 4 women whose disease previously undiagnosed, 2 developed glomerulonephritis. A woman without prior renal involvement developed proliferative diffuse glomerulonephritis during pregnancy. Fetal prognosis appears to be linked to the activity of the disease at the beginning of pregnancy: early spontaneous abortions always occurred when the disease was active, even if the signs of activity were only biological. This fact underlies the importance of complement dosage because the physiological expression of the usual inflammatory parameters increases during pregnancy. In addition, it seems that SLE treatment should be adapted to the evolution of biological tests. Renal involvement regardless of its origin--SLE or not--(it is often impossible to determine the respective contribution of each cause, which leads to increasing the corticosteroid dosage and giving antihypertensive drugs simultaneously) seems to be a factor of prematurity.

[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.

[Evolutive and prognostic aspects of Wegener's granulomatosis]. / Aspects évolutifs et pronostiques de la granulomatose de Wegener.

We have followed 45 patients affected with Wegener's granulomatosis and conducted a retrospective analysis of its evolutive aspects and prognostic factors. The study concerned 25 men and 20 women, with an age-range of 50 +/- 15 years at the onset of the disease. The time between occurrence of the first manifestations and the diagnosis was 16 +/- 21 months, the follow-up was 6.3 +/- 8 years. All patients except 3 were given steroids, associated in 39 cases with an immunosuppressor and in 9 cases with plasma exchanges. In 2 localized forms in the ENT area, remission was possible with cotrimoxazole alone. 15 deaths occurred, the causes of which are infection and renal insufficiency when death occurs early, and cardiovascular disorders and neoplasms when death occurs late. The survival probability is 93 p. cent at one year, 83 p. cent at 3 years and 75 p. cent at 5 years from the time of the first manifestations and 75 p. cent at one year, 53 p. cent at 3 years and 50 p. cent at 5 years from the time of the diagnosis. Relapses have been reported in 11 patients, some of which occurred several years after discontinuation of the treatment: they emphasize the need for prolonged monitoring of these patients. Presence of one of the poor prognostic factors: age 50 years (p less than 0.05), presence of renal insufficiency (p less than 0.05) or hemoglobinemia less than or equal to 10 g/cc (p less than 0.05), should be an indication for a more intensive treatment of Wegener's granulomatosis.