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A 48-year-old woman presented with isolated sixth cranial nerve palsy. She subsequently developed systemic lupus erythematosus (SLE) based on clinical and laboratory parameters. Three years later, she presented again with sixth cranial nerve palsy affecting the contralateral eye. Within 2 weeks of steroid initiation, complete recovery occurred. The unusual rare presentation of SLE in the current patient, as well as the pathogenesis and treatment of cranial neuropathy in SLE are discussed. [ABSTRACT FROM AUTHOR]

Longitudinal myelitis is an uncommon complication of systemic lupus erythematosus (SLE). We describe an unusual case of longitudinal myelitis and ischemic stroke in the presence of homozygous prothrombin G20210A, heterozygous MTHFR 677T mutations and the absence of antiphospholipid antibodies in a young woman with SLE. [ABSTRACT FROM AUTHOR]

We report a 39‐year‐old primigravida, a case of systemic lupus erythematosus (SLE) and secondary anti‐phospholipid syndrome (APS) with a smooth antenatal course who delivered by caesarean for non‐reassuring foetal heart rate. On day 2 postoperatively, she developed a sudden severe colicky upper abdominal pain with tachypnoea, dyspnoea, and tachycardia, and blood pressure (BP) reaching 150/95 mmHg. Computed tomography of the abdomen revealed lesions consistent with liver infarction. She developed haemolytic anaemia, elevated liver enzymes, and low platelets (HELLP syndrome); heparin and methylprednisolone were started. On day 3, BP normalized, respiratory symptoms improved but abdominal symptoms persisted. Methylprednisolone was increased to 80 mg/day on day 8 when she had significant clinical response and was discharged on day 16. This case emphasizes that a morbid clinical course including liver infarction should be anticipated in patients with SLE and APS complicated with HELLP syndrome. [ABSTRACT FROM AUTHOR]

The objective of the study was to describe the practice patterns of the antiphospholipid syndrome (APS) as compared with consensus guidelines for diagnosis and to determine whether practice patterns correlate with patient demographics and physician specialty. A retrospective medical chart review was conducted at the American University Hospital, in Beirut, Lebanon. All adult and pediatric patients admitted to the hospital between 1 January and 31 December 1998 who underwent either anticardiolipin antibodies (aCL) or lupus anticoagulant (LA) testing were included in the study. Work-up of APS syndrome was compared with: (a) the consensus guidelines for clinical diagnosis; (b) physician specialty; and (c) patient demographics (age, gender, ethnicity, health insurance status). Eighty-seven patients fulfilled at least one clinical criterion for APS; 92% were for work-up of thrombosis and 8% for pregnancy morbidities. Fifty-one percent underwent both aCL and LA. Overall 38% (33) of patients had an abnormal test result, however only 18% (6) underwent retesting, of whom only two satisfied a minimum of 6 weeks between test and retest. The APS diagnostic work-up was requested by 11 different specialties. Rheumatologists were the most consistent in asking for both tests. APS is seen and diagnosed by a variety of medical specialties. Practice patterns as compared with the latest consensus are sub-optimal, and need to be improved. Interventions to help improve this have been discussed and are being implemented. [ABSTRACT FROM AUTHOR]

The biological, physical and functional status change was measured in 104 patients with recent (< 1 year) onset synovitis. Measurements were taken initially and at 1 year. The purpose was to determine which biological and physical measures correlated with poor performance. Patients completed questionnaires quantifying activity, (the Human Activity Profile [HAP] and Sickness Impact Profile [SIP]), pain (Wisconsin Brief Pain Inventory), fatigue (Multidimensional Assessment of Fatigue), sleep, and mood (POMS and CESD). They were evaluated for articular involvement (Ritchie Index) and biological markers of disease activity (platelets, sedimentation rate, C-reactive protein and rheumatoid factor) (RF). In all, 30 men and 74 women participated. A total 445 patients met the American College of Rheumatology criteria for rheumatoid arthritis (RA); of these, 24 were rheumatoid factor (RF) positive, 18 had spondylitis, and 41 had unclassified arthritis. Low activity level at 1 year (HAP and SIP) correlated with high numbers of inflamed joints initially (p < 0.002); with more than 10 involved joints regardless of diagnostic group (p = 0.001); and joint symmetry (p < 0.009) A high platelet count was associated with low activity level at 1 year (p = 0.01); and high sedimentation rate was associated with more pain (p < 0.05). Low activity level (p < 0.09), fatigue (p = 0.03) and sleeplessness (p = 0.02) were correlated with RF + RA. [ABSTRACT FROM AUTHOR]

Familial clustering of elevated antiphospholipid antibody levels has been described, but the reports are heterogeneous with regard to the characterization of the antiphospholipid syndrome (APS), coexisting autoimmune diseases and clinical implications. We report a familial occurrence of APS in two patients, in the presence of SLE in the mother and absence of autoimmune diseases in the daughter along with acquired circulating inhibitors in both cases: platelet inhibitor and factor IX inhibitor, respectively. [ABSTRACT FROM AUTHOR]

La spondyloarthrite axiale (SpAax) est une pathologie relativement fréquente et potentiellement handicapante. Cependant, le diagnostic reste souvent retardé, en raison des symptômes peu spécifiques au début de la maladie et, en particulier, de la faible sensibilité du HLA-B27 dans certaines régions du monde. Les objectifs de l'étude étaient d'évaluer la valeur des stratégies de référence pour la SpAax chez les patients ayant une lombalgie chronique suspecte, et d'estimer la valeur de la lombalgie inflammatoire (LI) comme élément-clé de référence. L'étude est multicentrique et prospective, se déroulant dans les cliniques traitant la lombalgie en première ligne (rhumatologie, médicine interne, médicine de famille, chirurgie orthopédique, neurochirurgie et neurologie). Les critères d'inclusion étaient les patients adultes de moins de 45 ans et ayant une lombalgie chronique « suspecte » de maladie inflammatoire selon le jugement du médecin de première ligne, et nécessitant une référence au rhumatologue. Les propriétés diagnostiques (sensibilité, spécificité, rapport de vraisemblance positif (RV)) des différentes stratégies de référence pour la SpAax (Brandt, Herman, RADAR, MASTER, Braun et ASAS [1]) et celles de la LI (items individuels et critères de classification de Calin, Berlin et ASAS [2]) ont été calculées en prenant comme référence (gold standard) le diagnostic final du rhumatologue de référence. L'étude a inclus 335 patients de 7 pays du Moyen-Orient. L'âge moyen était de 34 ans, 56 % étaient des femmes, avec une durée moyenne des symptômes de 6 ans. Le diagnostic de SpAax a été retenu chez 138 patients (41 %, dont 53 % avec HLA-B27 positif et 94 % remplissant les critères de classification de l'ASAS) et rejeté chez 100 patients (30 %). La stratégie de référence optimale (RV le plus élevé, 4,6 (Tableau 1)) était la stratégie RADAR 2/3 comportant une LI, une bonne réponse aux AINS et une manifestation extra-musculosquelettique (MEM (psoriasis, uvéite ou maladie inflammatoire des intestins)), suivie de la stratégie BRANDT III (LI, HLA-B27). Parmi les critères de LI, ceux de l'ASAS avaient le meilleur RV pour le diagnostic de SpAax (2,5) et la question la plus rentable était l'amélioration de la lombalgie avec l'exercice et non pas avec le repos. La stratégie de référence optimale pour la SpAax chez les patients ayant une lombalgie chronique suspecte était RADAR 2/3, une stratégie simple basée sur la LI (selon les critères de l'ASAS), la bonne réponse aux AINS et la présence d'une MEM, et ne nécessitant pas de test HLA-B27. [ABSTRACT FROM AUTHOR]