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Resume Les auteurs etudient les causes des deces et les facteurs pronostiques dans la maladie de Horton. Dans une serie de maladies de Horton dont le diagnostic a ete confirme par biopsie, 14 patients sont decedes au cours du traitement ; 36 patients en arret therapeutique sont consideres comme gueris apres un recul superieur a 6 mois. Les principales causes des deces sont cardiovasculaires (n = 7) et digestives (n = 4) ; les deces sont survenus en moyenne apres 195 jours de traitement, la moitie precocement, au cours des 3 premiers mois ; un deces est imputable a l'arterite a cellules geantes (autopsie), cinq sont directement imputables a la corticotherapie qui apparait comme la principale cause. Les facteurs de pronostic ont ete recherches en comparant dans les 2 series de malades l'âge, le sexe, les antecedents, le tableau clinique, les signes biologiques avant traitement, la dose initiale de corticoides et celle recue apres 6 mois de traitement. Seuls apparaissent comme facteurs de mauvais pronostic : le plus grand âge (p

In a retrospective study of 115 patients with giant cell arteritis, 5 clinically patent autoimmune diseases were recorded; including 2 patients (1.8%) with Grave's disease and 1 with hypothyroidism. Among these patients, 46 had systematic assays of blood thyroid hormones and 39 were systematically investigated for anti-thyroid antibodies (ATAb):3 (6.5%) had biological hypothyroidism and 4 (10.3%) had ATAb. These findings were not significantly different from those of a control group of 39 age and sex matched patients.

Resume Les auteurs rapportent le cas d'un syndrome auto-immun multiple associant thyroidite auto-immune, syndrome de Gougerot-Sjogren et cirrhose biliaire primitive.

Resume Les auteurs rapportent un cas de pneumopathie rapidement evolutive, survenant lors d'un episode de granulopenie febrile apres polychimiotherapie oncostatique pour une tumeur metastatique, derivee des cellules du systeme APUD. Cette infection, a la faveur d'abondantes colonies de Candida tropicalis dans les secretions bronchiques, et malgre la negativite de l'histologie ou des serologies specifiques, sera attribuee a ce type de levure, et traitee par fluconazole en monotherapie avec une excellente efficacite clinique et radiologique.

Resume Les auteurs rapportent le cas d'une septicemie a Eikenella corrodesn survenant lors d'un episode d'aplasie medullaire febrile compliquant une chimiotherapie pour cancer ovarien de stade avance. Eikenella corrodens , germe a Gram negatif anaerobie facultatif, est un bacille commensal des voies aero-digestives superieures. Il peut devenir pathogene de maniere localisee, plus rarement generalisee. Dans ce cas une immunodepression est souvent retrouvee. Nous rapportons ici un cas de septicemie, a point de depart digestif probable, dans un tableau de granulopenie profonde apres chimiotherapie.

The authors report a case of eosinophilic fasciitis associated with several autoimmune diseases, such a Graves' disease, pernicious anaemia and vitilgo. These associations suggest an autoimmune etiology for the eosinophilic fasciitis.

We report the case of a 77-year-old woman who presented an acute form of dermatopolymyositis. Six months later, during oral corticotherapy with high efficiency we found a mediastinal mass with suspicion of liver metastases. A neuroendocrine tumor was proven by mediastinoscopy and a chemotherapy was given in outpatient with complete thoracic and hepatic response and without related toxicity.

The authors report two unusual cases of primary Sjogren's syndrome which occured in young north africans patients. The two patients showed a multisystemic disorder, with, in the first case, renal tubular acidosis (associated with nephrocalcinosis), pulmonary involvement and haemotologic features, and in the second case, severe pulmonary fibrosis and cutaneous vasculitis.

The authors report 12 cases of medullary thyroid carcinoma (MTC). Seven patients suffered from sporadic form of MTC and 5 from familial MCT: 2 isolated MTC, 1 multiple endocrine neoplasia (MEN) type IIa, and 1 MEN type IIb. The calcitonine assay and the pentagastrin test allow an early diagnostic, and must be performed in the family members at risk for hereditary MTC and in the cases of suggestive clinical signs and syndroms, and perhaps in all cold nodules.

Fourteen patients with neurolic manifestations were documented in a series of forty-six patients with primary Sjogren's syndrome. They included three psychiatric disorders (2 depressions and 1 chronic psychosis), 7 peripheral neuropathies, 4 ocular features, and 3 central manifestations (1 cerebral vascular accident, 1 myelitis, and 1 cerebellous involvement). The other systemic manifestations were registered: Raynaud's phenomenon and lung manifestations were found to be statistically significantly more frequent in patients with neurologic manifestations compared to the series as a whole.

The authors report two cases of chronic pancreatic ascites with a pancreatic fistula treated with success, one surgically and the other with medical processes. They suggest that somatostatin prescribed at first could reduce surgical indications.

We describe 10 cases of multiple autoimmune syndromes which group together 12 autoimmune diseases. Autoimmune thyroid disorders are the most frequent involvement. The classification according to the prevalence of the different autoimmune diseases appears to be difficult. Such associations, probably underestimated, underline the need for a prolonged survey of patients with autoimmune disorders.

A 64 year-old man was found to have a vascular purpura with histologic features of cutaneous necrotizing vasculitis, fifteen days after influenza vaccination. No other cause (neoplasic, autoimmune, infectious or drug-induced) was apparent, and the reponsability of vaccination is retained while it is a very rare complication.

The authors report their own experience about the possible role of hepatitis C virus (HCV) in Sjogren's syndrome, studying two population: 15 HCV chronic liver diseases and 26 primary Sjogren's syndrome.

The authors report a retrospective study of 218 cases of thyroiditis with a 11 years follow up. The association with other autoimmune diseases are reported. 30 patients (13,7 %) had association with other auto-immune disorder. Systemic lupus, Sjogren disease, and hematological disease are the most frequently found.

Eleven cases of vasculitis associated with neoplasia are reported. The vasculitides were : leucocytoclastic in 6 cases, cutaneous or systemic necrotizing vasculitis in 3 cases and temporal giant cell arteritis in 2. The associated neoplasms were : solid tumors in 5 cases and malignant blood diseases in 6. The clinical features, laboratory blood tests, pathological findings and evolution of the two diseases are presented.

Thirty eight patients with primary Sjogren syndrome are reported. Systemic features were registered in 26/38 patients : hematologic diseases (14), pulmonary (17), neuropsychiatric (12), dermatologic (11), rheumatologic (11), gastroenterologic (3). Eleven patients had only one manifestation, 5 had 2 disorders, and 10 had 3 or more. These features often revealed Sjogren syndrome.

Cutaneous infections due to Mycobacterium marinum seems to be frequent. We report two cases with different clinical forms : nodular and sporotrichoid. Treatment is based upon tetracyclines or rifampicin plus ethambutol.

A 39 years old woman was admitted with fever, arthritis and pleural effusion. She presented pleuro-pericarditis and she rapidely developed an acute abdominal syndrome. Pleural and pericardial effusion were sero-hemorragic while massive hemoperitoneum, with peritoneal lesions of vasculitis with micro-aneurysms was found after an urgent laparotomy. Diffuse serous hemorragic lesions at the time of diagnosis of SLE seems to be extremely rare.

Among 115 patients with Giant Cell Arteritis (GCA), incidence of thyroid disease was evaluated. Eleven cases were encountered. These included hyperthyroidism in 5 cases, hypothyroidism in 4 cases (2 of them with serum thyroid antibodies) and autoimmune thyroid disease in 2 without hypothyroidism. It is concluded that there is not significant association between GCA and autoimmune thyroid disease.

Twenty three autoimmune thyroid diseases associated with one or more other autoimmune disease are reported. Thyroid antibodies are detectable in 13 of them; 13 had overt orsubclinal hypothyroidism and 6 had Graves' disease. The other autoimmune disease included: 18 connective tissue disease, 4 thrombocytopenic purpura, 3 pernicious aneamia, 2 diabetes mellitus, 1 primary biliary cirrosis, 1 celiac disease. The long delay between the two disease strongly suggest to detect thyroid disease by screening autoimmune disease with determination of serum TSH and antithyroid antibodies.

In contrast to the known associations of leucocytoclastic vasculitis with malignancy, Polyarteritis Nodosa (PAN) were unfrequently reported with cancer. We report two patients in whom PAN and malignancy simultaneously appeared. In first case, a noisy PAN, involving arteries of peripheral nerves, gallbladder, gut, mesentery presents a cancer of colon. In the second case, cutaneous polyarteritis nodosa revealed myelodysplasia.

We report three cases of adrenal hemorrhage that were confirmed by hormone measurements and computed tomography. Precipitating factors included in two cases heparin therapy and surgery. Despite the availability of computed tomography adrenal hemorrhage mimics many other diseases, and an early diagnosis remains difficult.

The authors report a case of primary Sjogren's syndrome which rapidly developed a lymphoma increasing in malignancy. Cryoglobulin consisting of a monoclonal IgM Kappa immunoglobulin was a good marker of a monoclonal B-Cell clone which developped a low grade followed by a high grade lymphoma.