Cessation of oral anticoagulants in antiphospholipid syndrome.

Objective To study the outcome of patients with antiphospholipid syndrome (APS) after oral anticoagulant treatment cessation. Methods We performed a retrospective study of patients with APS experiencing cessation of oral anticoagulant and enrolled in a French multicentre observational cohort between January 2014 and January 2016. The main outcome was the occurrence of recurrent thrombotic event after oral anticoagulation cessation. Results Forty four APS patients interrupted oral anticoagulation. The median age was 43 (27-56) years. The median duration of anticoagulation was 21 (9-118) months. Main causes of oral anticoagulant treatment cessation were switch from vitamin K antagonists to aspirin in 15 patients, prolonged disappearance of antiphospholipid antibodies in ten, bleeding complications in nine and a poor therapeutic adherence in six. Eleven (25%) patients developed a recurrent thrombotic event after oral anticoagulation cessation, including three catastrophic APS and one death due to lower limb ischemia. Antihypertensive treatment required at time of oral anticoagulants cessation seems to be an important factor associated with recurrent thrombosis after oral anticoagulant cessation (15.2% in patients with no relapse versus 45.5% in patients with recurrent thrombosis, p = 0.038). Oral anticoagulant treatment was re-started in 18 (40.9%) patients. Conclusion The risk of a new thrombotic event in APS patients who stopped their anticoagulation is high, even in those who showed a long lasting disappearance of antiphospholipid antibodies. Except for the presence of treated hypertension, this study did not find a particular clinical or biological phenotype for APS patients who relapsed after anticoagulation cessation. Any stopping of anticoagulant in such patients should be done with caution.

[Vitamin B12 deficiency due to nitrous oxide use: unrecognized cause of combined spinal cord degeneration]. / Carence en vitamine B12 par toxicité du protoxyde d'azote : une cause méconnue de sclérose combinée de la moelle.

INTRODUCTION: Nitrous oxide is widely used in anesthesia. It is usually safe but may be associated with severe side effects when it is used repeatedly or on a prolonged time. Here, we report a case of drug-induced subacute combined spinal cord degeneration related to cobalamin deficiency. CASE REPORT: A 20-year-old man with sickle-cell disease (SS) who was followed for severe recurrent vaso-occlusive crisis with repeated hospital admissions presented with ascending motor and sensitive neurological deficits that were later associated with bladder dysfunction. He was first considered to develop Guillain-Barre syndrome. However, persisting neurological signs despite adequate treatment lately led to diagnose subacute combined medullar degeneration due to abnormal cobalamin (vitamin B12) metabolism induced by repeated use of nitrous oxide during painful episodes of sickle-cell disease. CONCLUSION: Inhaled nitric oxide is widely used in the treatment of vaso-occlusive crisis. Clinicians should be aware of possible severe neurologic side effects.

[Eosinophilic fasciitis and metastatic choroïdal melanoma: a paraneoplastic syndrome?]. / Fasciite à éosinophiles révélant l'extension métastatique d'un mélanome de la choroïde : un syndrome paranéoplasique ?

INTRODUCTION: Eosinophilic fasciitis (EF) is a rare inflammatory and fibrosing disorder with clinical, biological and histological characteristics. It has sometimes been reported with hematologic malignancy, but has never been associated with malignant uveal melanoma. CASE REPORT: A 67-year-old woman with choroidal melanoma was found to have metastatic skeletal extension of her melanoma while she developed swelling and pain in the lower limbs. Histologic examination confirmed an EF and oral corticosteroid treatment was started. A treatment for the ophthalmologic and skeletal extension of her melanoma was initiated. The paraneoplastic character of this EF was entertained. CONCLUSION: The simultaneous occurrence of EF and metastatic skeletal melanoma is suggestive of the possibility of EF constituting a paraneoplastic syndrome.

[Temporal artery biopsy may remain positive even after long-term corticosteroid treatment: report of two cases]. / Maladie de Horton: biopsie temporale positive après corticothérapie prolongée. A propos de deux observations.

INTRODUCTION: Temporal arteritis is the most common systemic vasculitis of the elderly. It is diagnosed with the combination of a clinico-biological syndrome and typical histologic features recognized on temporal artery biopsy (TAB). Cortisteroid therapy is quickly recommended, before the TAB is performed or before the histologic results confirm the diagnosis. It is recommended to perform TAB as soon as possible after the treatment has begun in order to avoid a presumed improvement or normalisation of the histological features. EXEGESIS: We report the cases of two patients, a 76-year-old woman and a 78-year-old man who had persistent clinical and histological features of temporal arteritis 5 years and one year respectively after corticosteroid therapy was initiated. CONCLUSION: Histological changes in the temporal artery biopsy may persist for as long as five years in a patient receiving a corticosteroid treatment for temporal arteritis. Even when largely delayed after the beginning of the treatment, temporal artery biopsy may prove to be important in diagnosing persistent temporal arteritis.

[Pravastatin-induced dermatomyositis]. / Dermatomyosite induite par la pravastatine.

INTRODUCTION: The toxic myopathy caused by statins (HMG-CoA reductase inhibitors) is well established. Recent reports add to these effects systemic immune diseases including systemic lupus erythematosus, vasculitis, polymyositis or dermatomyositis. EXEGESIS: We report a case of dermatomyositis in a 69-year-old patient treated with pravastatin [Elisor]. She presented with typical features of dermatomyositis 2 years after she started a treatment with pravastatin. The treatment was discontinued and she slowly improved, with a transient dermocorticosteroid treatment. Eight other patients with dermatomyositis and chronic treatment with HMG-CoA reductase inhibitors are reported in the literature. All of them presented with classical features of dermatomyositis. The discontinuation of the treatment was followed by spontaneous clinical and biological improvement in 3/9 patients. The other patients received high doses of corticosteroids and improved, except one patient who died of respiratory failure (pulmonary fibrosis) despite the adjunction of oral cyclophosphamide [Endoxan]. In these patients, dermatomyositis can be considered as a severe adverse reaction to HMG-CoA reductase inhibitors although a distinct casual link cannot be definitely established. CONCLUSION: The increasing prescription of statins has led to the parallel increment of reported side-effects, where autoimmune diseases are now described. Among them, our case of dermatomyositis in a patient receiving pravastatin adds to the eight reported cases in the literature and highlights the potential role of statins as triggers of immune systemic diseases.

Recurrent thromboembolism in a patient with beta-thalassemia major associated with double heterozygosity for factor V R506Q and prothrombin G20210A mutations.

Double heterozygosity for factor V R506Q and prothrombin G20210A mutations was identified in a 24-year-old man with beta-thalassemia major. The patient experienced a first thrombotic event at the age of 19 years and three recurrent thromboses in a short time interval, the third occurring while the patient was receiving long-term anticoagulant treatment. This case suggests that patients with major thalassemia and congenital thrombophilic mutations need intensive and long-lasting anticoagulant treatment. Thus, even if thrombotic events could be explained by a hypercoagulable state observed in patients with major thalassemia, after a first thrombotic event has occurred these patients should be screened for acquired and congenital thrombophilia.

[Acquired haemophilia in a patient with systemic lupus erythematosus]. / Hémophilie acquise au cours d'un lupus érythémateux systémique.

INTRODUCTION: In patients with lupus, the most common acquired circulating anticoagulant is antiprothrombinase which is responsible for thrombosis. The presence of antibodies directed against factor VIII is rarely found in systemic lupus erythematosus. A case of acquired haemophilia in a patient with lupus is reported. CASE REPORT: A 30 year-old woman with systemic lupus erythematosus developed a right coxalgia and ecchymotic skin lesions which were prominent on the right arm and forearm. Laboratory values were as follows: positive antinuclear antibodies > 1: 2 560, anti-DNA antibodies (300 IU/ml), prolonged activated partial thromboplastin time, reduced factor VIII activity (1 p. 100) and the presence of antibodies against factor VIII. Magnetic nuclear resonance of the right hip confirmed the presence of an intramuscular hematoma. The patient was initially treated with intravenous pulse and oral corticosteroids, intravenous immunoglobulins and intravenous cyclophosphamide. Clinical and biological improvement was promptly obtained. DISCUSSION: In our patient with systemic lupus erythematosus, bleeding revealed acquired haemophilia with antibodies against factor VIII. It should be pointed out that the association between lupus and haemophilia is uncommon and that at present no standardized treatment can be recommended.