[Pulmonary damage during polymyositis and dermatomyositis: interstitial lung disease].

Interstitial lung disease is a serious complication of polymyositis/dermatomyositis and leads to death from chronic respiratory insufficiency in 30 to 66% of cases. It is a criterion of poor prognosis in these disorders. Its onset occurs at variable points in the course of polymyositis/dermatomyositis, and precedes them in half of all cases.

High-risk pregnancies in Diamond-Blackfan anemia: a survey of 64 pregnancies from the French and German registries.

We reviewed 64 pregnancies in 26 women with Diamond-Blackfan anemia (DBA) included in the French and German DBA registries. Complications were seen in 42 pregnancies (66%) and included abortion, pre-eclampsia, in utero fetal death, intrauterine growth retardation, retroplacental hematoma, pre-term delivery and fetal malformations. Of the 34 children (53%) born alive, 13 had DBA.

[Aortic involvement in giant cell arteritis. A prospective follow-up of 11 patients using computed tomography].

Purpose: To determine clinical and radiological features, using computed tomography (CT-scan) in patients with aortic involvement related to giant cell arteritis (GCA), and to assess both clinical and CT-scan outcome after therapy institution.

Methods: Aortic involvement due to GCA was investigated in all patients, using CT-scan at diagnosis, and at 3, 6 and 12 months follow-up after therapy institution.

Results: The 11 consecutive patients consisted of 4 men and 7 women with mean age of 64.

[Spontaneous splenic hematoma: trauma injury or drug reaction or both?].

Introduction: Spontaneous splenic hematomas are uncommon and frequently associated with infectious, hematologic, or neoplastic diseases. Presentation is typically acute but progressive forms have been described.

Case: We report the case of a 45-year-old man consulting for abdominal pain that was found to be due to a spontaneous splenic hematoma.

Diamond-blackfan anemia and growth status: the French registry.

Objectives: To study the frequency and risk factors of growth retardation (GR) in patients with Diamond-Blackfan anemia.

Study Design: A cross-sectional survey including the 95 patients followed by hematologists affiliated with the French Society of Pediatric Hematology and Immunology for whom growth data were available; 43 patients were transfusion dependent, 32 were steroid dependent, and 20 patients were off treatment. GR was defined as height below 2 SD.

Inflammatory pseudotumor of the liver revealing gynecological Corynebacterium infection.

Inflammatory pseudotumor (IPL) of the liver is a rare benign clinicopathologic entity, which has been associated with infectious conditions, including alimentary, digestive tract and mouth infections. We report a new case, where a patient developed hepatic IPL that revealed endometritis related to Corynebacterium infection; the patient had a favorable outcome of hepatic IPL after appropriate antibiotic therapy initiation. Our findings therefore confirm that antibiotic therapy may be effective in patients with hepatic IPL associated with underlying infections, avoiding unnecessary surgery.

[Association of sarcoidosis and Takayasu's arteritis: an additional case report].

Introduction: Takayasu arteritis has been described in association with various auto-immune disorders (mainly inflammatory digestive tract diseases). However, only few cases of Takayasu arteritis associated with sarcoidosis have been reported, raising the question of an association by chance.

Exegesis: We report the case of a 26-year old woman with a 1-year history of sarcoidosis, who presented with a right painful upper limb, revealing inflammatory humeral, axillary and subclavian arteritis related to Takayasu arteritis.

[Tuberculosis peritonitis: an always present disease. About 4 new cases].

Introduction: Tuberculous peritonitis, a major problem in developing country, occurs preferentially in immigrant population and in patients with acquired immune deficiency syndrome (AIDS). Although rare in France, it did not disappear and epidemiological, clinical and therapeutic approach deserve to be reminded.

Exegesis: We reported 4 patients (immigrants in two cases), occurred in caucasian and African persons (one with AIDS).

[The script concordance test: a new evaluation method of both clinical reasoning and skills in internal medicine].

Objective: The script concordance test is designed to evaluate knowledge organization, which constitutes a crucial parameter of clinical skills. The objective of the present study was to assess the value of a new written evaluation tool to measure clinical skills in Internal Medicine.

Materials And Methods: A 95-item examination was completed by a group of medical students (N =17), a group of residents in Family practice (N =9), a group of residents in Internal Medicine (N =5), and a group of experienced physicians in Internal Medicine (N =7).

[Cardiopulmonary function before and after cyclophosphamide treatment in severe systemic sclerosis: comparison of monthly intravenous bolus and autologous haematopoietic stem cell transplantation].

Purpose: Cyclophosphamide in monthly intravenous bolus is used to treat severe forms of systemic sclerosis with pulmonary involvement. Since 1996, cyclophosphamide therapeutic intensification with autologous haematopoietic stem cells transplantation allowed significant improvement in skin and functional scores in severe systemic sclerosis. Cyclophosphamide potential cardiotoxicity in this setting has been questioned.

Opportunistic infections in polymyositis and dermatomyositis.

Objective: To assess prevalence and characteristics of opportunistic infections in patients with polymyositis/dermatomyositis (PM/DM). To determine the predictive values for opportunistic infections on clinical presentation, biochemical findings, and paraclinical features of PM/DM to detect patients at risk of opportunistic infections.

Methods: The medical records of 156 consecutive PM/DM patients in 3 medical centers were reviewed.

Regulatory serine residues mediate phosphorylation-dependent and phosphorylation-independent activation of interferon regulatory factor 7.

Interferon regulatory factor (IRF)7 is a key transcription factor required for establishment of antiviral resistance. In response to infection, IRF7 is activated by phosphorylation through the action of the non-canonical IkappaB kinases, IkappaB kinase-epsilon and TANK-binding kinase 1. Activation leads to nuclear retention, DNA binding, and derepression of transactivation ability.