Fate of circulating immune complexes in infective endocarditis.

To examine the role of circulating immune complexes (CIC) in infective endocarditis, we studied 64 patients with infective endocarditis for the presence of CIC by the polyethylene-glycol (PEG)-precipitation test and the Clq binding test. This study was repeated during the course of the disease in 23 patients. CIC were found in 84 per cent of patients (66 per cent with acute infective endocarditis, 89 per cent with subacute infective endocarditis) during the active phase of the disease. Higer PEG precipitates were associated with typical cutaneous signs, cryoglobulins and nonstreptococcic culture-positive infective endocarditis. Under appropriate antibiotic treatment, the PEG precipitate levels of 17 patients fell within 1 month to the normal range, with a concomitant drop in cryoglobulinemia and rheumatoid factor. Conversely, uncontrolled sepsis always (six of six) yielded a rising level of CIC. These findings support the hypothesis that CIC may be important in the pathogenesis of peripheral lesions in infective endocarditis.

Malignant hypertension in antiphospholipid syndrome without overt lupus nephritis.

The antiphospholipid syndrome is usually defined by the association of a clinical manifestation (recurrent venous and/or arterial thrombosis, recurrent spontaneous miscarriages) and a biological abnormality (anticardiolipin antibody, lupus anticoagulant). We retrospectively analyzed the records of 5 patients (4 females, 1 male, aged 30 +/- 12 years) with antiphospholipid syndrome, primary (n = 1) or secondary to systemic lupus erythematosus (n = 4), who developed malignant systemic hypertension with renal insufficiency, in the absence of lupus nephritis. Before the episode of malignant hypertension, all patients had normal systemic blood pressure and renal function. During malignant hypertension the systolic pressure was 206 +/- 39 mmHg and the diastolic pressure 130 +/- 25 mmHg, peak serum creatinine was 204 +/- 95 mumol/l, daily proteinuria was 1.1 +/- 0.8 gr, and complement serum levels were normal in all patients. Renal angiography found normal proximal renal arteries. Renal biopsy showed ischaemic glomeruli without proliferative lesions (n = 5), focal intimal fibrosis either isolated (n = 3) or associated with thrombosis (n = 2) of the intrarenal vessels, and the absence of vasculitis. Immunofluorescence study did not reveal typical lupus deposits. Patients were treated with antihypertensive agents, increasing doses of prednisone (n = 3), and anticoagulant (n = 2) or anti-aggregant therapy (n = 1). After a mean follow-up of 6.8 +/- 5.2 years, 4 patients were still alive with normal blood pressure and renal function, whereas 1 patient died of a probable catastrophic antiphospholipid syndrome. Patients with antiphospholipid syndrome, primary or secondary to systemic lupus erythematosus, may develop malignant hypertension with renal insufficiency and intrarenal vascular lesions, in the absence of lupus nephritis.

Behcet's syndrome and renal involvement: a histological and immunofluorescent study of eleven renal biopsies.

The finding of focal glomerulonephritis in a patient with Behcet's syndrome led us to perform systematic renal biopsies in ten other patients with the disease. None of the patients had symptoms of renal disease. Proteinuria was found in five, two of whom had associated leukocyturia. By light microscopy mesangial and extramembranous glomerular deposits were observed in eight patients. Arterioles in ten patients showed subendothelial and medial hyaline deposits. A granular pattern of fluorescent staining identified the presence of the third component of complement in these deposits. Circulating immune deposits were sought and found in six out of seven patients. The finding of circulating immune complexes and deposition of complement in glomerular and arteriolar tissues supports an immune complex mediated nephropathy and is consistent with the hypothesis of an immunological pathogenesis in Behcet's syndrome.

[False aneurysm of the left ventricle and coronary aneurysms in Behçet disease]. / Faux anévrisme du ventricule gauche et anévrismes coronaires au cours d'une maladie de Behçet.

A false left ventricular aneurysm and coronary artery aneurysm were discovered in a 29 year old patient with Behçet's syndrome. The operation under cardiopulmonary bypass consisted of closing the neck of the false aneurysm by an endo-aneurysmal approach with a Gore-Tex patch. The coronary artery aneurysms were respected. There were no postoperative complications. Cardiac involvement is rare in Behçet's syndrome (6%). The originality of this case is the association of two aneurysmal pathologies: the coronary and ventricular aneurysms due to the angiitis and the myocardial fragility induced by ischaemia.

[Bisalbuminemia disclosing primary hyperparathyroidism with fistulized pancreatic false cyst]. / Bisalbuminémie révélant une hyperparathyroïdie primaire avec faux kyste du pancréas fistulisé.

Discovery on a protein electrophoregram of a bisalbuminemia can orientate according to its migration fast or slow to an hereditary mutation of an amino acid, or an acquired form by excess of beta lactamines due to renal insufficiency or by the rupture of a pancreatic pseudocyst in the peritoneum. This is this late mechanism that we report in this case of bisalbuminemia related to an opened pancreatic pseudocyst secondary to an adenoma of the parathyroid gland.

[Osteoarticular amyloidosis and dysglobulinemia: apropos of 2 cases]. / Amylose ostéoarticulaire et dysglobulinémie: à propos de deux cas.

We report two cases of bone and joint amyloidosis involvement related to plasma cell dyscrasia. The radiographic appearances mimic numerous benign or malignant diseases. MR imaging shows a diffuse low signal in T1 and an heterogeneous low or mild low signal in T2 weighted spin-echo sequence.

[Relapsing polychondritis and mesenteric panniculitis: apropos of 2 cases]. / Polychondrite atrophiante et panniculite mésentérique: à propos de 2 cas.

We report two cases of polychondritis associated with mesenteric panniculitis. Case 1. In February 1989, a woman born in 1949 presented with 40 degrees C fever accompanied by pain in the abdomen and pelvis. Eight days later, nodular skin lesions appeared on her lower limbs. The abdomen was swollen with gas and undepressible. An abdominal CT scan revealed partitioned peritoneal collections, and a guided needle aspiration produced a chylous fluid. Direct and indirect bacteriological examinations gave negative results. Histology showed intense inflammatory reaction with giant cells and lipophages, thereby confirming the presence of mesenteric panniculitis. Six months later, the development of chondritic lesions on the nose and the helix of the ear clinched the diagnosis of polychondritis. The patient was put on corticosteroid therapy for a few months, and in January this year (1993) she is durably asymptomatic. Case 2. In October 1977, a woman born in 1937 presented with polychondritis with prolonged fever, inflammatory syndrome and chondritic lesions of the nose, larynx and helix of the ear. In December 1978, she developed signs of abdominal obstruction. Laparotomy revealed infiltration by multiple nodular formations of the entire posterior line of attachment of the mesentery. Biopsies withdrew a puriform fluid. Histology showed a partly necrotic adipose tissue with giant cells and lipophages. High-dose corticosteroid therapy partially controlled the chondritic and abdominal manifestations. The occurrence of abdominal pain in patients with polychondritis may result from several disorders, such as iatrogenic complications, digestive tract vasculitis or ulcerative colitis, but also associated mesenteric panniculitis.

[Generalized BCG infection after intravesical instillations of Calmette-Guerin bacillus]. / BCGite généralisée après instillations intravésicales de bacilles de Calmette-Guérin.

BCG has been disappointing as immunotherapy of numerous cancers, but it has been clinically successful in the intravesical treatment of bladder carcinomas sparing the muscle coat; it has indeed become the reference treatment for this type of cancer. However, complications are repeatedly reported, including generalized BCGitis. We report such a case with positive BCG culture. From the cases already published there emerges a homogeneous and often subacute clinical presentation suggestive of an ordinary pathogen. Bacteriology is not very helpful, even when recent techniques are used, and therefore the diagnosis rests on the context and, when samples are taken, on suggestive histological findings. To discuss the physiopathology of BCGitis--generalized immune reaction or multifocal BCG proliferation--is not useless since treatment depends on it. It is probable that these 2 mechanisms working together can be incriminated justifying the prescription of both antibiotics and corticosteroids. When this is done, the prognosis seems to be favourable in most patients. Yet a strict respect of contra-indications and a very careful subsequent radiotherapy should reduce the risks.

[Fetal death caused by myocarditis and isolated congenital auriculoventricular block]. / Mort foetale par myocardite et bloc auriculo-ventriculaire congénital isolé.

A 26-year old woman gave birth, at term, to a child with isolated complete heart block. A second pregnancy was interrupted by foetal death. Among other immunological abnormalities, this young woman had an antibody resembling the anti-SS-B antibody. At pathological examination the foetus' heart was found to be free of malformation but presented with subacute myocarditis associated with microcalcifications of the conductive tissue. Such findings suggest that an incipient myocarditis may either result in foetal death or lead to fibrosis of conduction pathways with isolated complete heart block.

[Psychic disorders in systemic lupus erythematosus. Prospective study of 35 cases]. / Les troubles psychiques au cours de l'évolution du lupus érythémateux disséminé. Etude prospective sur 35 cas.

In the prospective study reported, 35 patients with systemic lupus erythematosus underwent thorough psychiatric examination. They were divided into three groups according to whether their psychiatric symptoms were acute (10 patients), subacute (9 patients) or non-existent (16 patients). Particular attention was paid to such factors as obstetrical events (pregnancy, spontaneous or induced abortion), history of psychiatric disorders, emotional traumas and treatment with corticosteroids. The overall incidence of psychiatric symptoms was high (54%), mainly due to isolated depressive states (37%). The only significant contributing factors were a personal history of psychiatric disorders (p = 0.001) and emotional traumas (p = 0.0004). There was no difference between the three groups as regards the distribution, dosage and duration of corticosteroid therapy.

[Relapse of Wegener's granulomatosis. Retrospective study of 18 cases]. / Rechutes de la granulomatose de Wegener. Etude rétrospective de 18 cas.

During a mean therapeutic follow-up of 4 years, 25 out of 61 patients with Wegener's granulomatosis had one or several relapses. As in 7 of them the initial diagnosis had proved erroneous, this study concerns the remaining 18 patients who together totalled 31 relapses after the correct diagnosis was made. Twenty relapses occurred early on, within 4 months of a change in treatment, and they corresponded to recurrent activity of the disease. Ten relapses occurred later on, after treatment had been discontinued or kept at maintenance dose level. The relapses appeared as early as the first year in 41 percent of the cases. The ENT region was affected in 61 percent of the patients. Nine relapses occurred in the absence of treatment, including 6 which took place 1 to 8 years after treatment. Three relapses occurred less than 3 months after a prednisone and oral cyclophosphamide therapy was initiated. Compared with the group of patients without relapse, those in the relapse group were younger, more often treated with azathioprine and cyclophosphamide administered intravenously, and followed up for a longer period; their probability of survival was also higher. In case of early relapse after withdrawal of treatment the mean duration of that treatment had been shorter than in the absence of relapse. None of the patients whose treatment had been stopped more than 20 months after its onset had an early relapse. These data prompted us to make the following proposals: initially, cyclophosphamide should be administered as bolus injections (the time required to obtain results is then shorter than with the oral route), and maintenance therapy should last at least 20 months. Azathioprine should be used only when cyclophosphamide is contra-indicated. The relative indications for intravenous and oral cyclophosphamide remain to be determined. Discontinuing all treatments is a difficult decision to make, but even then the patients should be under close supervision in view of the possibility of late relapses.

[Necrotizing angiitis with or without asthma. Clinical, biological and etiological differences. Role of hepatitis B virus]. / Angéites nécrosantes avec et sans asthme. Différences cliniques, biologiques, étiologiques. Rôle du virus B de l'hépatite.

Sixty-five cases of necrotizing angiitis, including 20 with asthma (group A) and 45 without asthma (group B) were studied retrospectively. All patients were investigated for markers of hepatitis B, at least the HBs antigen. Skin lesions and arthralgias were more common in group B patients. Eosinophilia during angiitis was almost constant in group A and was observed in only 6% of group B patients. Renal involvement, hepatic lesions and arterial hypertension seemed to be more frequent in group B but the difference was not significant. The HBs antigen was present in 1/20 patients of group A and in 17/45 patients of group B (p less than 0.01). The anti-HBs antibody was found with equal frequency in both groups. The clinical and biological differences observed between these two groups of patients strongly suggest that necrotizing vasculitis with and without asthma are two separate nosological entities and in particular, that they have different causes.

[Cardiovascular involvement in Behçet's disease. (author's transl)]. / Manifestations cardio-vasculaires de la maladie de Behçet.

35 cases of Behçet disease are presented including 16 vascular manifestations: 14 veinous thrombosis with 9 vena cava obstruction; 1 femoral aneurysm; 2 pulmonar arteries thrombosis; 3 pericarditis; 2 myocardial infarction. In all these cases corticosteroids and anti-coagulation therapy were instituted. With a follow-up from 5 to 36 months, only 5 relapsed in the five first months of therapy. The authors suggest systematic anticoagulation or anti-agregant therapy in Behçet disease.

[Pseudotumor of the ureter manifesting periarteritis nodosa]. / Pseudo-tumeur de l'uretère révélant une péri-artérite noueuse.

The authors report a case of anuria. The existence of an isolated pseudotumor of the pelvic ureter revealed a case of periarteritis nodosa. A segmental resection and a psoic bladder provided satisfactory functional recovery.