[Membranous glomerulonephritis during primary Gougerot-Sjögren syndrome]. / Glomérulonéphrite extra-membraneuse au cours d'un syndrome de Gougerot-Sjögren primitif.

INTRODUCTION: Glomerulonephritis, mainly membranoproliferative or membranous (MG), is observed much less often than interstitial involvement in Sjögren's syndrome (SS). CASE: We report a case of MG revealed by thrombosis of the inferior vena cava and of a renal vein in a 40-year-old woman with primary SS, which began with polyarthritis, immune-type lymphadenopathy, and Hashimoto thyroiditis and did not include obvious sicca syndrome. After failure of moderate-dose steroids and then azathioprine, each over separate 9-month periods, the MG responded well within a few weeks to monthly alternation of methylprednisolone and oral cyclophosphamide for 6 months. DISCUSSION: SS may be an underestimated cause of glomerulonephritis, especially membranoproliferative and membranous glomerulonephritis. They should be considered even in the absence of obvious sicca syndrome. Although the prognosis is usually good, renal insufficiency can occur. In cases of MG, if moderate-dose steroids fail, monthly alternation of methylprednisolone and cyclophosphamide for 6 months appears effective and well tolerated, with a low risk of carcinogenicity.

[Juvenile dermatomyositis and panniculitis-type subcutaneous T-cell lymphoma. A case report]. / Dermatomyosite juvénile et lymphome T sous-cutané à type de panniculite. A propos d'un cas.

INTRODUCTION: Juvenile dermatomyositis does not have, unlike the adult form, a paraneoplastic character. EXEGESIS: We report the case of a 12-year-old girl who has a typical dermatomyositis complicated by the appearance of very aggressive lesions of lobular panniculitis predominating in the lower limbs. Their refractory character to different treatments (in particular corticosteroids, chloroquine, dapsone, methotrexate, azathioprine, intravenous immunoglobulins and ciclosporin A) will finally lead to the diagnosis of subcutaneous T-cell lymphoma. This will be complicated by a macrophagic activation syndrome leading to death despite chemotherapy. CONCLUSIONS: The occurrence of panniculitis during a dermatomyositis should lead to the search for a subcutaneous T-cell lymphoma, especially if the lesions are locally aggressive and refractory to usual treatments, which permits an early chemotherapy.

[Pulmonary hyalinising granuloma: report of 2 original cases with cervicofacial and orbital involvement]. / Le granulome pulmonaire hyalinisant: à propos de deux observations originales avec localisations cervicofaciale et orbitaire.

INTRODUCTION: Pulmonary hyalinizing granuloma is a rare fibrosing nodular disease of the lung characterized by its histological appearance, which includes at the center of the lesion a dense network of concentric hyalinized collagen lamella surrounded by perivascular lymphoplasmacytic infiltrate that rarefies in the center of the nodule. EXEGESIS: We report two new cases: the first with laryngeal (endoluminal tumor-like), orbital (subeyelid nodule) and mesenteric (9 x 6 cm mass) location of hyalinizing granuloma; the second with cervical, facial (trismus), orbital (pseudotumor) and limb (ankylosing elbow) fibrosis. CONCLUSION: The extrapulmonary diffusion of the disease is extremely rare. In one of the cases, with corticosteroids and after a follow-up of 12 months, the pulmonary tumors vanished but the fibrosis resolved only partially.

[Aneurysm of the thoracic aorta and spondylodiscitis disclosing brucellosis]. / Anévrysme de l'aorte thoracique et spondylodiscite révélant une brucellose.

Brucellosis, which decreased during the eighties in France, remains a public health problem in many Mediterranean countries. We report the case of a 65-year old patient native of Morocco, of thoracic aorta aneurysm and lumbar spondylodiscitis due to Brucella melitensis, revealed by haemoptysis and lumbar pains, with a favourable outcome after aortic graft resection, spinal plaster immobilization and specific lengthy antibiotic treatment. This case report is characterized by the absence of endocarditis or infectious focus near the aneurysm. Diagnosis of aneurysm and spondilitis due to Brucella melitensis is based on imaging and bacteriological and serological examination. Because of a clinical and biological intolerance for rifampicin and cotrimoxazole, this patient received ofloxacin-doxycyclin-streptomycin. We discuss antibiotic recommendations and stress the interest of the early diagnosis of complicated forms of brucellosis for a better prognosis.

[The heart and antiphospholipid antibodies. Personal experience and review of the literature]. / Coeur et anticorps antiphospholipides. Expérience personnelle et revue de la littérature.

Since the recognition of the antiphospholipid syndrome, a great number of cardiac manifestations have been reported in association with these antibodies: valvular disease, coronary artery disease, cardiomyopathy and intracardiac thrombosis. However this association raises numerous questions related to the pathogenic role of antiphospholipids, their prognostic significance and their frequency in a non-selected population with a definite cardiac manifestation. In view of the literature and our personal experience, it seems necessary to distinguish two kinds of situations. During systemic lupus and primary antiphospholipid syndrome (which must be systematically looked for in patients with history of thrombo-embolic disease), antiphospholipids antibodies certainly play a role in the occurrence of cardiac manifestations, but the precise place of thrombosis has to be best defined along with immunologic/inflammatory mechanisms. On the other hand, in a non-selected population, antiphospholipids antibodies may just be the consequence of the cardiac lesion and do not seem to have prognostic implications. This distinction, actually hypothetical, should be supported on the basis of distinct specificities of antiphospholipids antibodies and especially their dependence on beta 2-glycoprotein I, which would help to distinguish the harmful antibodies from those which probably just appear as an epiphenomenon.

Intravenous gamma globulin as first line therapy in polymyositis and dermatomyositis: an open study in 11 adult patients.

OBJECTIVE: Polymyositis (PM) and dermatomyositis (DM) are inflammatory muscle diseases of presumed autoimmune origin. Many possible interventions are available to treat these patients: corticosteroids, immunosuppressive drugs, plasmapheresis, and total body irradiation. But these therapies are not always effective and may be responsible for certain serious side effects. Polyvalent intravenous immunoglobulin (IVIG) has been tried with success in inflammatory myopathies after failure of traditional treatment. An attempt was made to evaluate the efficacy of IVIG as first line therapy in patients with PM or DM. METHODS: Eleven Caucasian patients [6 women, 5 men, mean age 55.6 (SD 10.1) years], with active recent inflammatory myopathy, were treated by high doses of IVIG as first choice. The average duration of inflammatory myopathy before IVIG was 9.6 months (SD 10.2 months, with a range of 1 month to 3 years). Five patients had PM and 6 had DM. None had myositis associated with connective tissue disease. Two patients had a history of malignant disease: 1 lymphoma and 1 breast tumor with relapse of the malignancy during the study. One patient had a probable lung carcinoma and in another patient, ovarian carcinoma was diagnosed a few months after the onset of IVIG. We used preparations of polyvalent human i.v. gamma globulins with intact IgG. All patients received 1 g/kg daily for 2 days each month. The mean course of treatment was 4 months. RESULTS: Clinical assessment, evaluated by proximal muscle power and biochemical tests, was carried out before each treatment period. Significant clinical improvement was noted in only 3 of the 11 patients (one with acute coxsackie virus B infection, and one with possible drug induced myopathy). Mean muscle power estimated for the 11 patients before and after IVIG therapy was not significantly improved. Eight patients showed significant biochemical improvement (more than 50%). Mean CK levels for the 11 patients showed a statistically significant decrease during IVIG therapy (p < 0.01). Minor IVIG side effects were noted in one patient. CONCLUSION: IVIG therapy seems effective rarely as first therapy in patients with inflammatory myopathy but may be considered especially in viral or drug induced myopathy. IVIG therapy as the first treatment may also be tried in patients with contraindication for steroids, and in mild myopathy, especially in the elderly, to avoid steroid induced side effects.

[Cardiac amyloidosis. General review]. / L'amylose cardiaque. Revue générale.

Cardiac amyloidosis, most often of AL type, is a non-exceptional disease as it represents 5 to 10% of non-ischemic cardiomyopathies. It realizes typically a restrictive cardiomyopathy. Nevertheless the wide diversity of possible presentation makes it a "big shammer" which must be evoked in front of every unexplained cardiopathy after the age of forty. If some associated manifestations can rapidly suggest the diagnosis, as a peripheric neuropathy especially a carpal tunnel syndrome or palpebral ecchymosis, cardiac involvement can also evolve in an apparently isolated way. The most suggestive paraclinic elements for the diagnosis are, in one hand, the increased myocardial echogenicity with a "granular sparkling" appearance seen throughout all walls of the left ventricle and, in the other hand, the association of a thickened left ventricle and a low voltage (electrocardiogram could also show pseudo-infarct Q waves). In front of such aspects, the proof of amyloidosis is brought by an extra-cardiac biopsy or by scintigraphy with labelled serum amyloid P component, so that the indications of endomyocardial biopsy are very limited today. The identification of the amyloid nature of a cardiopathy has an direct therapeutic implication: it contra-indicates the use of digitalis, calcium channel blockers and beta-blockers. The treatment of AL amyloidosis (chemotherapy with alkylant agents) remains very unsatisfactory especially in the cardiac involvement which is the most frequent cause of death (in AL amyloidosis). Last, cardiac amyloidosis is a bad indication for transplantation which results are burden by rapid progression of deposits especially in the gastro-intestinal tract and the nervous system.

[Pancreatitis in systemic lupus erythematosus. Review of the literature apropos of 5 cases]. / Pancréatite au cours du lupus érythémateux disséminé. Revue de la littérature à propos de cinq observation.

We report five cases of pancreatitis in systemic lupus erythematosus. Three patients died. The cause of death was directly related to pancreatitis in two cases. Several causes of pancreatitis may be suspected: vasculitis, thrombosis when associated to antiphospholipid syndrome, biliary calculi, infection, metabolic abnormalities or adverse effects of therapy. About seventy cases of pancreatitis have been reported in systemic lupus erythematosus in the literature Pancreatitis was the inaugural sign of lupus in six cases. In 12 cases, besides lupus, no other cause of pancreatitis was found. However, pancreatic vasculitis or thrombosis was rarely demonstrated even in post-mortem examinations. The role of corticosteroids in pancreatitis in lupus is controversial since the evolution of pancreatitis in lupus is generally good with corticosteroids. We think that diagnosis of pancreatitis in lupus should not implicate discontinuation or decrease of corticosteroids dosage, unless their responsibility is absolutely demonstrated.

[Do neuromyosites exist?]. / Les neuromyosites existent-elles?

Neuromyositis defined as the association of dermatomyositis or polymyositis and a neuropathy without any found cause is a very controversial entity because of the possibility of, in one hand, muscular modifications caused by neurological involvement and, on the other hand, neurogenic type manifestations caused by polymyositis. The study of 4 cases seen in an Internal Medicine department and the review of the literature allowed us to show that the concept of neuromyositis corresponds to a clinico-pathological reality when the diagnosis is based on the association of definite criteria of both primary muscle and nerve involvement excluding muscular abnormalities that could be the consequence of nerve involvement and vice versa. The criteria, most relevant when associated are: a) for muscular involvement: high increase of muscular enzyme over 6 times the superior limit of the normal values, pseudomyotonic electrical discharges, perifascicular atrophy, intense inflammatory infiltrates and massive necrosis, b) for neurological involvement: early abolition of tendinous reflexes in a patient without notable muscular atrophy and with little or no myalgia, sensitive abnormalities in areas other than those of muscular involvement, especially when they are intense, early weakness of distal muscles, decrease of nerve conduction speed, target fibers and lesions of nerve trunks (and albuminocytological dissociation in the particular case of polyradiculoneuritis). Once the diagnosis of neuropathy settled, it is necessary to exclude an usual cause (alcoholism, diabetes...) before concluding to neuromyositis. When we apply these restrictive (but nevertheless necessary for the validity of diagnosis) criteria, only 6 cases of the literature respond to this entity. It is a peripheral neuropathy in 5 cases (like two of ours) and a polyradiculoneuritis in one case (like our two others). Among these 6 cases, there is a vasculitis in two, frequency much higher to what is observed in adult polymyositis, which suggest a possible causative role of vascular involvement in neuropathy arising. In the other cases we can just give pathogenic hypothesis making the neuropathy and the polymyositis the result of the same process (immunological disturbance, paraneoplastic origin, viral disease). In one of our four patients, who have shown an HTLV-I infection by polymerase chain reaction in situ hybridization was positive in muscle which suggest a direct pathogenic role of the virus. HTLV-I infection should be considered as a possible cause of neuromyositis especially in endemic areas.

[4 new cases of neuromyositis, one of them associated with HTLV-I infection]. / Quatre nouvelles observations de neuromyosites, dont un cas associé à une infection par HTLV-I.

Neuromyositis is a very rare type of polymyositis where, beside the usual muscular manifestations, there are signs of peripheral neuropathy which can be found at clinical, electromyographic and/or pathological examination. We have seen between 1983 and 1990 four cases of neuromyositis. The neurological disorder was an axonopathy in two cases which is usual in neuromyositis; in the other two cases, there was a polyradiculoneuritis which seems to be very rare in this syndrome. In the four patients the disease was particularly severe and unresponsive to treatment. One of our patients had HTLV-I infection diagnosed by polymerase chain reaction amplification and in situ hybridization, while the serological test was negative. To our knowledge only three cases of HTLV-I associated neuromyositis have been reported in the literature.

[Multiple arterial dissections and inflammatory syndrome: apropos of 4 cases]. / Dissections artérielles multiples et syndrome inflammatoire: à propos de 4 observations.

We report four cases of multiple arterial dissection associated with an inflammatory syndrome. The features of lesions and the absence of histological inflammatory arterial disease suggest an arterial dysplasia complicated by an infectious disease.

[Changes in the diastolic left ventricular function in primary antiphospholipid antibody syndrome]. / Altération de la fonction ventriculaire gauche diastolique au cours du syndrome primitif des anticorps antiphospholipides.

Previous studies have shown that left ventricular (LV) diastolic function is frequently impaired in patients with systemic lupus erythematosus. We prospectively studied echo-Doppler indices of LV diastolic function in 18 patients with primary antiphospholipid syndrome (PAPS), who where compared to a group of 18 healthy controls. Heretofore undescribed LV relaxation abnormalities were found in the PAPS group: this finding suggests the existence of a causal link.

[Long-term results of the treatment of diffuse systemic scleroderma with interferon-gamma]. / Résultats à long terme du traitement des sclérodermies systémiques diffuses par l'interféron gamma.

There are very few drugs effective in the treatment of diffuse scleroderma and therefore we conducted an open study with gamma interferon. Among 20 diffuse scleroderma, evolving since less than 3 years, an improvement was observed in 8 patients. There was no serious side effect. Survival of these 20 patients was 85% at 5 years.