Acute Q Fever in an Ankylosing Spondyloarthritis Patient Treated with Etanercept.

Q fever is a rare zoonotic infection caused by Coxiella burnetii. Tumor necrosis factor-alpha (TNF-α) has an important role in the early control of this infection. However, TNF-α blockers increase the risk of infectious diseases. We present herein a patient who developed acute Q fever under anti-TNF-α who had a good evolution after anti-TNF stoppage and treatment with doxycycline.

[Seizures in patients with systemic lupus erythematosus]. / Lupus érythémateux systémique et épilepsies.

Seizures are one of the most serious neuropsychiatric manifestations of systemic lupus erythematous (SLE). This descriptive and retrospective study aims at describing clinical and paraclinical features and therapeutic approach of seizures in patients with SLE. The characteristics of the seizure group was compared to those of a control group (patients with LES who had not presented seizures). A total of 177 patients were included in these analyses. Among them, 14 (8 %) developed seizures before, at or after the SLE diagnosis. The age of occurrence of seizures was younger than for other complications of the disease. There was no significant association with the antiphospholipid syndrome. Disease activity in these patients was significantly higher than in the control group. During the follow up, the subjects being under anticonvulsants and/or corticosteroids and/or immunosuppressive therapy, we observed good outcomes (n=5), re-occurence of seizures (n=4), cognitive impairment (n=3 ) and death (n=2). Our study shows that seizures tend to occur early in the course of SLE, in the context of important disease activity and other serious clinical manifestations and in younger individuals. Seizures portend a negative impact on the overall long-term prognosis and quality of life in patients with SLE.

La comitialité est une des manifestations neurologiques les plus sévères du lupus érythémateux systémique (LES). Notre objectif est de décrire les caractéristiques des épilepsies chez les patients lupiques. Dans une étude rétrospective descriptive, les données cliniques et paracliniques des patients lupiques présentant une épilepsie ont été comparées à celles des patients lupiques n'ayant pas présenté de crises convulsives. Nous avons recensé 177 patients lupiques dont 14 (8 %) avaient présenté une épilepsie avant, au moment ou après le diagnostic de LES. L'âge de survenue des épilepsies était plus jeune que celui des autres manifestations. L'association à un syndrome des antiphospholipides n'était pas significative. Les patients lupiques présentant une épilepsie avaient un score d'activité de la maladie lupique (SLEDAI) significativement plus élevé que celui du groupe contrôle. L'évolution était marquée par la disparition des crises convulsives (n=5), la récidive (n=4), l'installation de troubles cognitifs (n=3 ) et le décès (n=2). Cette étude montre que la comitialité tend à survenir de façon précoce au cours du LES, aggravant alors le pronostic fonctionnel et vital. Elle associe un SLEDAI assez élevé ainsi qu'un âge de survenue plus jeune.

[Cardiac involvement in polymyositis]. / Manifestations cardiaques au cours de la polymyosite.

Cardiovascular involvement in polymyositis constitutes a major cause of death. However, the cardiac location is rarely symptomatic and does not usually represent the principle clinical feature at the time of the initial presentation. We present here an unusual case of polymyositis with severe and polymorph cardiac disturbances that predominant the muscular signs.

Primary bilateral adrenal lymphoma revealed by hemophagocytic syndrome.

Primary adrenal lymphoma is rare. It is often bilateral and in most of the cases of B-cell type. The clinical features are various and not specific. We report a case of a 69-year-old woman who had a diffuse large B-cell lymphoma associated with hemophagocytic syndrome. The abdominal imaging reveals the existence of bilateral adrenal hypertrophy. A CT scan-guided biopsy concluded to a diffuse large B-cell lymphoma CD 20-positive associated EBV. The treatment consisted on "CHOP like" chemotherapy associated with rituximab. Primary adrenal lymphoma has a poor prognosis, even more poorly if associated with hemophagocytic syndrome.

[Lupus hepatitis: a case series of 12 patients]. / Hépatite lupique: une série de 12 patients.

PURPOSE: Several liver manifestations have been reported in systemic lupus erythematosus (SLE) and are usually non specific. We report on our experience of lupus hepatitis. METHODS: A retrospective monocenter study of 73 patients with SLE. The diagnosis of lupus hepatitis was established after exclusion of other causes of hepatitis and hepatic vein thrombosis. RESULTS: Liver involvement was noted in 12 patients (16.4%). There were nine female and three male patients; the mean age of these patients was 29 years. In seven patients liver involvement was concurrent with the diagnosis of SLE and it occurred later during an exacerbation of the disease in the five remaining patients. In all patients, liver manifestations were associated with other organ involvement. Clinical manifestations were: hepatomegaly (n=4), jaundice (n=4), abdominal pain (n=3), ascites (n=2), portal hypertension (n=1) and hepatic failure with encephalopathy (n=1). Elevated liver enzyme was noted in 11 cases and liver cholestasis in eight cases. Presence of anti-ribosomal P antibodies was noted in one case. Liver biopsy was performed in five patients, and revealed chronic active hepatitis in three cases, chronic hepatic granulomas in one case and nonspecific inflammation in one case. The outcome was favorable in 11 patients without relapse, and one patient died of encephalopathy and liver failure. CONCLUSION: Liver involvement associated with SLE is not uncommon. It is frequently asymptomatic and limited to liver test abnormalities. The role of anti-ribosomal P autoantibodies remains uncertain.

Primary antiphospholipid syndrome and Evan's syndrome: 2 case reports.

The main clinical features of primary antiphospholipid syndrome are recurrent foetal loss, arterial or venous thrombosis and thrombocytopaenia. Evan's syndrome is characterized by simultaneous or sequential association of autoimmune anaemia and thombocytopaenia, rarely reported in primary antiphospholipid syndrome. We describe 2 cases of this association. Treatment with steroids was started in both cases, the follow-up was marked by the normalization of haemoglobin value and platelet count, without relapse.

[Thyroid involvement in Wegener's granulomatosis: a case report]. / Atteinte thyroïdienne au cours de la granulomatose de Wegener : à propos d'un cas.

Wegener's granulomatosis is a systemic necrotizing vasculitis affecting small to medium-sized vessels, characterized by involvement of the upper airways, lungs and kidneys. Endocrine involvement, particularly the thyroid gland is extremely rare. We report a 56-year-old woman presenting a limited form of Wegener's granulomatosis with the following clinical manifestations: sinusitis, pulmonary nodules, diabetes insipidus and a cold thyroid nodule. A right hemithyroidectomy was performed and the histopathological examination revealed a necrotizing vasculitis surrounded by granulomatous lesions. She was treated by corticosteroids without immunosuppressive therapy with a favourable outcome.

Eosinophilic fasciitis and toxic thyroid adenoma--a novel association.

Eosinophilic fasciitis is characterized by skin induration, peripheral blood eosinophilia, hypergammaglobulinaemia and thickening, fibrosis and inflammatory cell infiltration of the fascia. There have been several reports of illness particularly haematological disorders in patients with eosinophilic fasciitis. However, their association with thyroid disorders has rarely been described and includes autoimmune thyroiditis rather than toxic thyroid adenoma. We describe a case of a 53-year-old woman with simultaneous association of eosinophilic fasciitis and subclinical hyperthyroidism caused by toxic thyroid adenoma. Corticosteroids, antithyroid drugs and radioiodine therapy were started, with a good follow-up