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We evaluated temporal 67gallium (Ga) uptake in temporal arteritis (TA) and the contribution of Ga scans to the diagnosis of TA.Ga scans were performed prospectively in 19 patients with biopsy-proven TA and five TA patients with negative temporal artery biopsy. Controls were 18 elderly patients undergoing Ga scans for various inflammatory diseases. The temporal region of interest on head profiles was defined for comparison of uptake with a control parietal region of the same area. The Ga uptake ratio (GaUR) [(temporal region - parietal region)/parietal region] was evaluated for each temple by a computer and intra- and intergroup comparisons were made.GaUR was significantly higher in biopsy-proven TA patients (0.35+/-0.19) and biopsy-negative TA patients (0.31+/-0.03) than in controls (0.18+/-0.12) (P0.001), independently of recent temporal artery biopsy or short-duration steroid therapy. High GaUR (0.4) had a 94% specificity and a 90% positive predictive value for TA diagnosis. After 6 months of steroid therapy, when patients were in remission, GaUR returned to baseline.Ga is specifically incorporated into the temporal area in TA patients which may be due to the granulomatous vasculitic process. Ga uptake ceases during remission. A high GaUR may contribute to TA diagnosis in temporal artery biopsy-negative patients and its role in the diagnosis of other localizations of the disease requires further evaluation.

Resume La mise en evidence lors de Telectrophorese des proteines d'une bisalbuminemie peut orienter selon son type lent ou rapide vers une cause hereditaire par mutation d'un acide amine, ou vers une cause acquise: par surdosage en betalactamines lors d'une insuffisance renale ou lors de la rupture d'un faux kyste du pancreas dans une sereuse. C'est cette derniere eventualite que nous rapportons ou le faux kyste rompu dans le peritoine fait suite a une pancreatite calcifiante par adenome parathyroidien.

Resume Les auteurs rapportent deux cas d'amylose osteoarticulaire associee a une dysglobulinemie. Il s'agit d'une pathologie rare, d'expression radiologique tres polymorphe; les diagnostics differentiels sont nombreux. L'imagerie par resonance magnetique trouve un hyposignal en Tl et un signal heterogene, bas a intermediaire en T2 des lesions amyloides permettant d'orienter le diagnostic.

Resume Nous rapportons 2 observations associant polychrondrite atrophiante et panniculite mesenterique. Observation 1: Une femme nee en 1949 presente en fevrier 1989 une fievre a 40°C associee a de violentes douleurs abdomino-pelviennes. Huit jours plus tard apparaissent des lesions cutanees nodulaires des deux membres inferieurs. L'abdomen est meteorise et non depressible. Le scanner abdominal montre des collections peritoneales cloisonnees dont la ponction guidee ramene un liquide chyleux. La laparotomie revele une masse mesenterique de 20 cm de diametre. Les recherches bacteriologiques directes et indirectes sont negatives. L'histologie confirme l'existence d'une panniculite mesenterique en montrant une intense reaction inflammatoire avec presence de cellules geantes et de lipophages. Six mois plus tard, le diagnostic de polychondrite est pose devant des chondrites touchant le nez puis le pavillon de l'oreille. Une corticotherapie est administree pendant quelques mois. En janvier 1993, la patiente est durablement asymptomatique. Observation 2: Une femme nee en 1937 presente en octobre 1977 une polychondrite associant fievre prolongee, syndrome inflammatoire, chondrites du nez, du larynx et du pavillon de l'oreille. En decembre 1978 survient un syndrome occlusif. La laparotomie revele une infiltration de toute la racine du mesentere avec des formations nodulaires multiples. Les biopsies entrainent l'ecoulement d'un liquide puriforme. L'histologie montre un tissu adipeux partiellement necrose comportant la presence de cellules geantes et de lipophages. Un traitement corticoide a fortes doses controlera partiellement les manifestations douloureuses chondritiques et abdominales. La survenue de douleurs abdominales au cours d'une polychondrite peut relever de mecanismes multiples: complications du traitement, vascularite digestive, colite ulcereuse mais aussi panniculite mesenterique associee.

Resume Deux manifestations apparaissent comme la sanction de l'efficacite reconnue de l'association des corticoides et du cyclophosphamide dans la maladie de Wegener, autrefois mortelle: les recidives et les stenoses de l'arbre tracheobronchique. A propos de ces dernieres, les auteurs rapportent une observation eloquente d'un patient apparemment gueri des manifestations systemiques de sa maladie de Wegener et developpant des stenoses etagees tracheales et bronchiques. Le controle de celles-ci est conjointement assure par un traitement endoscopique au laser et une radiotherapie anti-inflammatoire.

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.

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.

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.

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.

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.

We report the case of a 67-year-old man who developed pericarditis as the initial manifestation of myelomonocytic leukemia. Leukemic pneumonitis developed 4 months later and the patient later died.

Congenital heart block is rare; it is acquired in utero, definitive and, more often than not, complete. It can be diagnosed by the appearance of fetal bradycardia around the 23rd week of gestation, during ultrasonographic monitoring of pregnancy. Heart block is usually associated with the presence of anti-Ro and/or anti-La antibodies in the mother's serum. These maternal immunological abnormalities can be isolated or associated with an autoimmune disease, usually systemic lupus erythematosus, but also Sjögren's syndrome, or more rarely still, an as yet unclassified connective tissue disease. Anti-Ro and anti-La antibodies cross the placental barrier and react with a fetal heart, leading to acute fetal myocarditis by the 17th week of gestation. When severe, it is lethal, otherwise it can result in degeneration and endocardial fibroelastosis, disrupting conduction and leading to congenital heart block. The ideal treatment would be prevention with corticosteroids. When the mother is Ro or La antibody-positive before pregnancy, elimination of these circulating antibodies can be attempted by treatment with 0.5 mg/kg body wt/d of prednisolone for 3 months. If the treatment is successful, corticotherapy can be prescribed early in the pregnancy to try to protect the fetus. However, there is not always a relationship between maternal anti-Ro antibodies and fetal heart block. If the Ro/La antibody-positive woman is already pregnant, but before her 17th week, it is possible to prescribe dexamethasone, which crosses the placenta and remains active, sometimes in association with plasmapheresis.

We retrospectively analyzed the records of 5 patients with antiphospholipid syndrome, primary (n = 1) or secondary to systemic lupus erythematotus (n = 4), who developped a malignant systemic hypertension with renal insufficiency and biopsy-proven intra-renal vascular lesions, in the absence of lupus nephritis.

Dermatological manifestations were present in 48 % of 69 patients with Wegener's granulomatosis. Vasculitis was always observed in skin biopsies from purpuric lesions and granulomata were the most frequent pathological findings in other lesions.

From May 1983 to December 1990, we observed 19 cases of systemic lupus erythematous diagnosed during childhood. Main clinical and biological signs were similar compared with adults excepts for adenopathy. The probability of survival at 26 years was 92 %.

19 of 52 patients with Wegener's granulomatosis relapsed, totalling 40 episodes. The relapses involved the upper airways in 35 % of the cases and were provoked by reduction of treatment in 52 %. Patients who relapsed were older. In this series azathioprine was the most frequent treatment, the follow-up was longer and the probability of survival was higher. All patients who were treated with corticosteroids and cyclophosphamide for less than 14 months relapsed. These data incite us to suggest some therapeutic modalities.

The authors report 2 cases of relapsing polychondritis associated with mesenteric panniculitis confirmed at histology after laparotomy and biopsy. Mesenteric panniculitis is one of several causes of abdominal pain in patients with polychondritis.

The finding of a focal and segmental glomerulonephritis (GN) in a patient with a Behcet's syndrome led us to perform systematic renal biopsies in ten other patients with the disease. Renal biopsy specimens of 11 patients with Behcet's syndrome (followed for 6 months to 15 years) have been studied by light, electron and immunofluorescence microscopy. In all cases, blood pressure and renal function were normal. Proteinuria was present in five patients. By light and electron microscopy, amyloidosis could not be demonstrated in any case. In one patient, the focal and segmental GN was associated with fibrinoid, electron dense, mesangial and irregular subepithelial deposits. These deposits were also detected in seven other patients but to a lesser degree. Arteriolosclerosis was present in all cases. By immuno fluorescence, small scattered granules of C3 were observed in 10 patients in the mesangium and along the capillary basement membrane. They were diffuse in six cases and focal in four. Small focal deposits containing IgA and/or IgG, Clq were also observed in four cases. Circulating immune complexes found in six out of seven patients in whom they were sought. Rare cases of focal and segmental GN and amyloidosis have been reported in Behcet's syndrome. To our knowledge, glomerular C3 deposits have not been yet reported. These findings with the presence of circulating immune complexes suggest that renal symptoms occasionnally observed in Behcet's syndrome could be related to immune complex deposition.

A retrospective study was conducted in fifteen patients with the hypereosinophilia syndrome to assess the cardiac manifestations of the affection. Clinical, electrical, and radiological findings, together with the results of ultrasonography (8 cases), hemodynamic tests (6 cases), and pathological examinations (7 cases) were used to classify the fifteen patients into four groups: 7 cases with endomyocardial fibrosis (EMF), 5 with non-obstructive cardiomyopathy (NOCM), 2 with pre-existing cardiopathies, and 1 with a normal heart. The manifestations in the 7 EMF cases varied according to the extent of the fibrosis present: 2 adiastolies, 3 valve incompetences, and 2 asymptomatic forms. Cardiac insufficiency can occur in the absence of fibrosis, pathological examination in one case demonstrating that it was due to a myocarditis, with infiltration of eosinophils and necrotic foci. The results of this study underline the close relationship between Loëffler's fibroplastic endocarditis and Davies' fibrous endomyocarditis. The presence of vascular lesions in the hypereosinophilia syndrome leads to difficulties in nosological distinction with some forms of necrotizing angitis especially Churg and Strauss' syndrome.

Highly selective angiography was used to study eleven cases of Horton's arteritis. The results showed the presence of segmental vascular abnormalities which, though mainly affecting the temporal artery region, were also present in that of the internal maxillary artery. This examination procedure can be of great value by assisting the surgeon in his choice of biopsy sample, which is essential for diagnostic purposes.

A hundred and twenty-six patients with systemic polyarteritis nodosa (PN) were studied over a 23 year period. The clinical symptoms and paraclinical data of PN are reviewed, and the results obtained by the different therapeutic approaches used during this period are presented: steroids alone and then associated with immunosuppressors and, in the most recent cases, associated with plasma exchange. The actuarial survival of patients in this retrospective and unrandomised series was comparable whether steroids were used alone or with immunosuppressors. The different therapeutic approaches in PAN are discussed especially with respect to the role of immunosuppressor therapy and plasma exchange.

The authors report 8 cases of scleroderma with oedema in the initial stages which could be divided amongst three groups on the basis of their clinical, biological, histological characteristics and course: normal systemic scleroderma, mixed connective tissue disease, Shulmans syndrome. The relationship between one of these cases and Buschke's scleroderma is discussed. These cases of scleroderma are characterized by diagnostic difficulties in the initial phase and by their relatively favourable vital prognosis (one death only). Corticosteroids may be effective to some extent in the presence of mixed connective tissue disease of Shulman's syndrome.

It is important to study coagulation during lupus disease, not only to detect circulating anticoagulants, which may be the cause of hemorrhage, but also in order to seek chronic hypercoagulability, which is more dangerous as it may favour the development of thrombosis on inflammatory arteritis or aggravate the development of glomerular lesions. We have thus explored in this way 25 patients with systemic lupus of which 17 had histologically confirmed renal disease. 25% had hypocoagulability, but 50% had hypercoagulability which justitied treatment with heparin.

The authors have collected 19 cases of ankylosing spondylitis with an alteration of intracardiac conduction. The lesions are usually situated high in the bundle of His, as shown by successive electrocardiograms and endocavitary studies. Progression by regressive acute episodes and the response to anti-inflammatory agents suggests that the disorder of conduction is inflammatory in origin. Syncopal attacks are rare (1 case out of 19) and a pacemaker is rarely indicated. Almost half the patients had aortic insufficiency, one patient had tricuspid stenosis and two patients had heart failure in the absence of any valvular lesion. The associated ankylosing spondylitis is characterised by the severity of the inflammatory signs (average sedimentation rate 50 mm in the first hour) and by the extent of peripheral articular involvement and extra-rheumatological manifestations. Almost one in two patients had iritis, with the same proportion applying to a past history of Reiters syndrome.

Full renal investigation were conducted in 11 patients with Behçet's syndrome. Biological tests revealed the presence of mild proteinuria in 5 of them. Needle biopsy showed extra-membranous or mesangial fibrinoid deposits in 6 cases, associated in 1 case with segmental and focal glomerulitis lesions. Immunofluorescence studies demonstrated glomerular deposits of mesangial and extra-membranous C3, sometimes with immunoglobulins (IgA-AgG). Ultrastructural examination was carried out in 7 cases and showed "hump" deposits attached to the external face of the basal membrane and rounded nodules of 7 to 15 mu included in the mesangium. The presence of immune complexes in the renal glomeruli, therefore, is not all unusual in this disease, which is probably auto-immune in nature, and in fact appears to be a quite frequent occurrence. These deposits, which were probably well-tolerated by most of the patients in this series, might be involved in the production of progressive glomerulopathies in chronic cases, as observed in one of the cases studied.

A 20-year-old patient with biologically-confirmed lupus developed a perirenal abscess following puncture biopsy of the kidney. Postoperative treatment included metronidazole at a mean dose of 2.5 g daily for 68 days (total dose : 165 g). Generalised convulsive seizures occurred on four occasions, associated with paresthesia of the four limbs, but without loss of motor or reflex activity, though some distal hypoesthesia was detected. An acute lupus attack was eliminated, the convulsive seizures not recurring after discontinuation of treatment, and the paresthesias diminishing progressively over a period of three months. Electrical investigations showed lack of motor anomalies but a marked reduction in sensory conduction. This is the 13th reported case of polyneuritis due to metronidazole, the 4th case of convulsive seizures, and the first case in which both manifestations occurred. The plasma concentration curve after oral administration of 1 g of the product to this patient demonstrated that the product was not being metabolised in a pathological manner.