[Hemorrhagic syndrome due to a heparin-like anticoagulant in a patient with systemic lupus erythematosus]. / Syndrome hémorragique dû à un anticoagulant héparine-like chez un patient lupique.

INTRODUCTION: In systemic lupus erythematosus, hemostasis disorders are mainly thrombotic, but more rarely hemorrhagic. CASE REPORT: A 25-year-old man presented with a macrophagic activation syndrome revealing a systemic lupus erythematosus, secondarily complicated by a hemorrhagic syndrome ; biological investigations revealed an increase thrombin time and an activated partial thromboplastin time, normalized by protamin neutralization in vitro, thus confirming the presence of a heparin-like anticoagulant. The hemostasis balance normalized after the specific treatment of lupus. CONCLUSION: This rare anomaly of hemostasis balance has been described in blood cancers and solid cancers. This is the first description of a case associated with an autoimmune connective tissue disorder such as lupus. After one year of follow-up, no diagnosis of blood or solid cancer was made.

[Horton's disease revealed by brachial C5 plexopathy]. / Maladie de Horton et plexite brachiale C5-C6: une observation avec revue de la littérature.

BACKGROUND: Shoulder palsy due to brachial plexopathy (or lower cervical radiculopathy) is a rare occurrence in patients with giant cell temporal arteritis (TA). We report a patient with C5 plexopathy revealing TA, with review of the literature. EXEGESIS: A 67-year-old woman presented with a complex neurological syndrome of rapid onset, including a left trigeminal neuralgia, painless masticatory palsy, impaired swallowing, and severe palsy of the left shoulder, corresponding to a C5 plexitis. Giant cell arteritis was suspected on clinical and laboratory grounds and was documented on temporal artery biopsy. Treatment with pulse methylprednisolone (100 mg every 8 hours for three days), followed by prednisone (1 mg/kg daily), resulted in progressive improvement of the neurological deficits, full shoulder strength being recovered within 6 months. An extensive world literature allowed us to review 23 other cases of TA complicated by either lower cervical radiculopathy or C5 brachial plexopathy. These peripheral neuropathic manifestations occurred sometimes bilaterally and were isolated or, less often, part of a mononeuritis multiplex. Involved patients were relatively young (mean age 67 years) and 54% were men. The pathophysiology of TA-associated C5 radiculopathy or brachial plexitis is still poorly understood. Fortunately, functional prognosis was consistently good with corticosteroid treatment. CONCLUSION: Giant cell arteritis should always be considered in elderly or middle-aged patients presenting with C5 radiculopathy or plexopathy and elevated inflammatory markers.

[Disease associations in 250 patients with temporal (giant cell) arteritis]. / Maladie de Horton: associations morbides chez 250 malades.

INTRODUCTION: Miscellaneous disorders have been described in association with temporal (giant cell) arteritis (TA), most often anecdotally, except with arteriosclerosis. METHOD: In a retrospective study, we reported our personal experience of disease associations in a series of 250 patients diagnosed with TA and followed-up in the department between 1976 and 2003. RESULTS: Disease associations were found in 43 patients, i.e. 17% of cases: concurrent malignancy (23 patients: 17 cancers and 6 blood diseases), primary Gougerot-Sjögren's syndrome (6 cases), endocrine disease other than Hashimoto's thyroiditis (7 cases: 3 hyperparathyroidism [HPP], 3 hyperthyroidism, 1 association HPP + hyperthyroidism), polyneuropathy (3 cases), essential thrombocythaemia (2 cases), anti-neutrophilic cytoplasmic (anti-myeloperoxidase) antibodies (2 cases), and miscellaneous associations (1 case of RS3PE syndrome, nephrotic syndrome, myasthenia, sarcoidosis, and macro-creatine kinase type 2). More than one disease associated was present 5 patients. In 77% of the patients, there was a strong temporal association between TA and the alternate illness. No systemic necrotizing vasculitis or rheumatoid arthritis was observed in any patient. CONCLUSION: In our experience, there was a frequent, non-fortuitous, association between TA and malignancy. Auto-immune conditions were rare, but the prevalence of Gougerot-Sjögren's syndrome might have been underestimated. Hyperthyroidism and HPP are not exceptional and must be recognised in order to avoid severe bone loss induced by corticosteroids.

[Dysmetabolic hepatosiderosis, characteristics in 51 patients]. / Hépatosidéroses dysmétaboliques, caractéristiques chez 51 patients.

OBJECTIVE: Dysmetabolic hepatosiderosis is a recently described entity, which reunites iron overloads associated with polymetabolic syndromes, steatosis and steato-hepatitis. The aim of this study was to specify the relationship between iron overload, polymetabolic syndrome, HFE mutations of primary hemochromatosis and steatosis. METHOD: This was a 5-year retrospective study of 51 patients hospitalised and/or seen in consultation, non-alcoholic, presenting with hyperferritinemia associated with a polymetabolic syndrome and/or hepatic steatosis. RESULTS: Patients mean age was of 53 +/- 12 years with a sex ratio M/F of 5.4. Metabolic disorders were found in 97% of the non-steatosis patients (overweight: 40%, perturbed sugar metabolism: 47%, dyslipidemia: 79.5%). Hyperferritinemia was constant but moderate (513.4 +/- 280.3 ng/ml). Transferrin saturation was predominantly normal (0.44 +/- 0.17). The hepatic enzymes were normal or only slightly perturbed. Steatosis was revealed by sonography in 62% of cases. The hepatic iron overload, documented in 6 patients by hepatic needle biopsy, was discreet (concentration/age ratio of 1.64 +/- 0.19). Among the 59% patients screened for the HFE gene, 40% were positive for C282Y and/or H63D. There was no correlation between ferritin levels and iron parameters, polymetabolic syndrome parameters and hepatic enzymes. The HFE mutations had no influence on the iron parameters. CONCLUSION: Dysmetabolic hepatosiderosis must be know by hospital practitioners because of their prevalence in cases of hyperferritinemia and their therapeutic incidence.

[Diabetes insipidus revealing primary malignant non-Hodgkin's lymphoma of bone]. / Diabète insipide révélateur d'un lymphome malin non Hodgkinien primitivement osseux.

INTRODUCTION: Diabetes insipidus is a very rare, occasionally misleading, revealing symptom of non Hodgkin's malignant lymphoma. We report a case of this uncommon association and a review of the literature is done. EXEGESIS: Soon after a transient attack of diplopia due to a 6th cranial nerve palsy, idiopathic cerebral diabetes insipidus was diagnosed in a 32-year-old immunocompetent man. As anterior hypopituitarism was absent, the patient was discharged and treated with desmopressin acetate. Six months later, recurrence of diploplia together with raised erythrocyte sedimentation rate was observed and B cell lymphoma was observed in the iliac bone and skull, with extension into the right cavernous sinus. Both tumoral lesions led to important uptake of gallium-67. After a 26-month follow-up following intensive chemotherapy including autologous peripheral stem cell transplantation and local radiotherapy, lymphoma symptoms had disappeared, but the patient's condition had only partially improved with regard to diabetes insipidus. The association of lymphoma and diabetes insipidus which has only been reported in 14 cases is discussed. CONCLUSION: Since endocrine symptoms are often preceding hematological diagnosis, malignant lymphomas should systematically be viewed as potential etiology of diabetes insipidus. Magnetic resonance imaging which is more sensitive than computerized tomography in revealing brain lymphomas is mandatory. Gallium scan imaging might help guide the diagnosis in some instances.

[Frequency of autoimmune diseases in 218 patients with autoimmune thyroid pathologies]. / Fréquence des maladies auto-immunes chez 218 patients atteints de pathologies thyroïdiennes auto-immunes.

PURPOSE: The aim of our study was to investigate the frequency of auto-immune diseases in patients suffering from autoimmune thyroid diseases. METHOD: We realised a retrospective study from 1981 to 1993 including 218 patients suffering from thyroiditis who were followed in the same hospital service. There were 202 women and 16 men with a mean age 49 at the moment their thyroid pathology was discovered. RESULTS: Thirty patients had one or more autoimmune disease associated to their thyroid disorders, representing 13.7% of total patients. The two most frequent autoimmune diseases were lupus and Sjögren's syndrome. In 17 cases the diagnosis of the associated autoimmune disease was made simultaneously. The systemic disease preceded with an 8-year delay the thyroid disease in five cases, and the thyroid disease was annunciatory in eight cases with a delay of 5 years. The frequency of autoimmune diseases seems to be higher in patients suffering from thyroid disorders than in the general population. They are probably common physiopathological mechanisms. CONCLUSION: The frequency of these associations suggests the need for a long-lasting survey of those patients having thyroid disorders. Initial evaluation and a regular checking in patients suffering from an autoimmune disease is recommenced.

[Treatment of prolactinoma]. / Traitement des adénomes à prolactine.

Prolactinomas rank first in frequency among hormone-secreting pituitary adenomas, but their management remains controversial. The authors present a review of the literature concerning the various therapeutic methods used and their results. As regards microadenomas, opinions are divided since the results obtained with bromocriptine and with selective adenomectomy are about the same. As regards macroadenomas, surgery exposes to more frequent complications and above all to recurrences. The majority of authors is in favour of bromocriptine first followed, if necessary, by surgical excision. Pregnancy may accelerate the development of prolactinomas. This risk is minimal with microadenomas and more real with macroadenomas, requiring more radical treatment before pregnancy and close monitoring.

Autoimmune insulin syndrome.

Initially described in Japan, the autoimmune insulin syndrome is caused by the presence of anti-insulin antibodies in patients who have never received insulin. This syndrome accounts for spontaneous or reactive hypoglycaemia with very high levels of total immuno-reactive insulin. Discordance between the levels of immunoreactive insulin and C peptide indicate the possible presence of anti-insulin antibodies; this can avoid an incorrect diagnosis of insulinoma. These autoimmune hypoglycaemias often present a difficult diagnostic problem in distinguishing them from factitious hypoglycaemia. The course of the autoimmune insulin syndrome is usually favourable, with a spontaneous rapid diminution of the levels of anti-insulin antibodies. The reasons for the appearance of anti-insulin antibodies and the exact mechanisms of the hypoglycaemia remain uncertain. However, the frequent association of the autoimmune insulin syndrome with certain autoimmune diseases suggest a common immune dysfunction. Drugs containing a sulphydryl group have been implicated in the aetiology of this syndrome.

[Antithyrotropin receptor antibodies in a series of Basedow's disease]. / Les anticorps anti-récepteurs de la TSH dans une série de maladie de Basedow.

Thyrotrophin binding inhibiting immunoglobulins (TBII) were tested in 40 Graves' disease patients with hyperthyreosis, by an isotopic receptor assay (TRAK-Assay Behring). The samples were taken before, during and at the end of the hyperthyroid treatment. TRAb were present in serum of 86.67% of the patients before the treatment and in none of the 20 healthy control volunteers. There was no correlation between TRAb values and either the clinical signes of the disease neither the other thyroidal antibodies. After discontinuation of the antithyroid therapy (18 to 24 months after the onset) TRAb were negative in 71.4% of the patients; among this group 33% had a late relapse of the disease. In the positive TRAb group after discontinuation of the antithyroid therapy, 83.3% had a relapse of the disease and this relapse occurred shortly. There is a significant correlation between the TRAb values and the occurrence of relapse. In conclusion, TRAb values at the end of hyperthyroid treatment in Graves' disease patients seems to be a useful predictor value of the clinical course of the disease. This may be also a good help in the decision to continue or discontinue antithyroid therapy in Graves' disease patients with hyperthyreosis.