Radouane Niamane

Targeting Acquired Hemophilia A with Rheumatoid Arthritis by a Rituximab Shot: A Case Report and Review of the Literature

Patient: Male, 66 Final Diagnosis: Acquired hemophilia A Symptoms: Polyarticular flare Medication: — Clinical Procedure: — Specialty: Rheumatology Objective: Challenging differential diagnosis Background: Acquired hemophilia A (AH) is a rare hemorrhagic diathesis, characterized by the presence of autoantibodies directed against the pro-coagulant activity of factor VIII. It is associated with rheumatoid arthritis (RA) in 4% to 8% of cases and its prognosis remains severe. Case Report: A 66-year-old patient has been followed up for 20 years for deforming and severe RA, which was in low-disease activity. However, the patient presented a polyarticular flare involving the metacarpophalangeal and the proximal interphalangeal joints, the left elbow, and the right knee, which was warm and swollen. Articular puncture of this knee yielded a hematic fluid that did not coagulate. Its cytological analysis showed significant presence of red blood cells, which were also abundantly present in the other cell lines. Activated partial thromboplastin time was lengthened and not corrected by the addition of control plasma. Prothrombin time (Quick’s test), fibrinogen level, and vitamin K-dependent factors were without abnormalities. In contrast, factor VIII was collapsed at 7% and the anti-factor VIII antibody was positive. The diagnosis of AH with anti-factor VIII inhibitor was thus retained. With regard to RA, the Disease Activity Score was 6.32 and exhibited a very active RA. Rituximab with methotrexate was begun and the evolution was favorable. After 6 months, the reappearance of the anti-factor VIII inhibitor was found, thus justifying a second cycle of rituximab. Conclusions: AH is not exceptional in RA. Rituximab remains a relevant alternative for managing simultaneous AH with inhibitor and RA.

FRI0548 Influence of homocysteine and vertebral fractures on prevalent abdominal aortic calcification in postmenopausal women: a multicentric cross-sectional study

Background Osteoporosis and cardiovascular diseases are two major public health problems. Both are associated with high morbidity, long-term hospitalization, mortality and loss of independence leading to institutionalization. Vertebral morphometry using dual-energy X-ray absorptiometry (DXA) also known as vertebral fracture assessment (VFA) is a fast, low-radiation technique which produces images that are of sufficient quality to be used to diagnose the presence of vertebral deformity consistent with fracture. VFA has demonstrated utility for vertebral visualization and thus is an important tool for fracture detection in women and men. It has been shown also in many populations that this technique can simultaneously identify abdominal aortic calcification (AAC). Hyperhomocysteinemia, a condition that recent epidemiological studies have shown to be associated with increased risk of vascular disease. A potential role of homocysteine in bone fragility has been considered from the observation of a high prevalence of osteoporosis in subjects with homocystinuria. Objectives The main of this study was to examine the relationship between plasma homocysteine (Hcy), asymptomatic osteoporotic vertebral fractures (VFs) using vertebral fracture assessment (VFA) and prevalent abdominal aortic calcification (AAC) in Moroccan postmenopausal women. Methods The study cohort consisted of 188 consecutive postmenopausal women with no prior known diagnosis of osteoporosis or taking medication interfering with bone metabolism. Mean age, weight, height, body mass index and plasma homocysteine were determined. Lateral VFA images and scans of the lumbar spine and proximal femur were obtained using a Lunar Prodigy Vision densitometer (GE Healthcare Inc., Waukesha, WI). VFs were defined using a combination of Genants semiquantitative approach and morphometry. VFA images were also scored for prevalent AAC using a validated 24 point scale. Results Fifty-eight (30.9%) patients had densitometric osteoporosis. VFs were identified using VFA in 76 (40.4%) patients: 61 women had grade 1 VFs and 15 had grade 2 or 3 VFs. One hundred twenty nine women (68.6%) did not have any detectable AAC, whereas the prevalence of significant atherosclerotic burden defined as AAC score of 5 or higher, was 13.8%. A significant positive correlation between AAC score and homocysteine was observed. Women with extended AAC, were older, had a lower weight, BMI and BMD, higher homocysteine levels and more prevalent VFs than women without extended AAC. Multiple regression analysis showed that the presence of extended AAC was significantly associated with Age and grade 2/3 VFs and not independently associated with homocysteine levels. Conclusions This study did not confirm that homocysteine is important determinant of extended AAC in postmenopausal women. However, this significant atherosclerotic marker is independently associated with VFs regardless of age. Disclosure of Interest None declared

AB0836 Performance of quantitative ultrasound and six osteoporosis risk indexes in menopausal women: validation and comparative evaluation study

Background A number of questionnaire-based systems and the use of portable quantitative ultrasound scanners (QUS) have been devised in an attempt to produce a cost-effective method of screening for osteoporosis. Objectives to assess the sensitivity and specificity of different techniques and their ability to act as screening tools in relation to dual energy X-ray absorptiometry (DXA). Methods 295 white postmenopausal women aged over 60 were enrolled. Each subject completed a standardized questionnaire which permits the measure of six osteoporosis indexes and had bone mineral density (BMD) measured using QUS and DXA. Sensitivity and specificity of the different techniques in relation to DXA were plotted as receiver-operating characteristic (ROC) curves at DXA T-score total hip ≤ -2.5 (osteoporosis). Results BUA sensitivity and specificity values were respectively 76.8% and 51.2% at the total hip. The optimal cut-off T-score for QUS was -2 at the total hip. The osteoporosis self-assessment tool (OST) provided consistently the highest AUC (0.80) among the clinical tools and had the best sensitivity and specificity balance (90.2%>44.5%). OST negative likelihood ratio was 0.22. Conclusions OST (based only on the weight and the age) performed slightly better than QUS and other risk questionnaires in predicting low BMD at the total hip. Disclosure of Interest None declared

Anomalie osseuse rare: brachydactylie non syndromique de type C

La brachydactylie est un raccourcissement congenital des mains ou des pieds en rapport avec l’absence ou la petite taille des phalanges, des os metacarpiens ou metatarsiens. Il s’agit d’une anomalie peu frequente, decelable aisement sur le plan clinique, mais pour laquelle un complement radiologique est indispensable. Nous presentons le cas d’un patient de 40 ans qui a consulte pour une douleur mecanique de la base du pouce droit en rapport avec une arthrose Trapezo-metacarpienne dite rhizarthrose et chez qui l’examen a trouve fortuitement un raccourcissement des 2eme, 3eme et 5eme doigts des deux mains (A). Les pieds etaient epargnes. Il n’y avait pas d’autres atteintes dysmorphiques (oculaire, nasale, auriculaire ou digestive). L’interrogatoire a permis de reveler la meme atteinte uniquement chez la mere sans notion de consanguinite. La radiographie standard de face des deux mains a montre une brachymesophalangie des deuxiemes, troisiemes et cinquiemes rayons (B). Ce type de brachydactylie est plus complexe. Le quatrieme rayon est epargne, apparaissant ainsi comme le doigt le plus long. La brachydactylie peut etre classee en cinq types differents de A a E. La radiographie standard est l’examen cle pour la caracterisation des brachydactylies, precisant la topographie et le type de l’atteinte. Plusieurs formes de brachydactylie sont causees par des mutations des genes codant les composants de la Proteine Osseuse Morphogenetique. Les brachydactylies peuvent egalement s’integrer dans un grand nombre de syndrome poly malformatifs. Notre patient presente une brachydactylie isolee non syndromique de type C se transmettant selon un mode autosomique dominant.