Granulomatosis with polyangiitis and pregnancy: A case report and review of the literature.

The association of granulomatosis with polyangiitis and pregnancy is rare and therapeutic options are limited by the risk of teratogenicity and fetotoxicity. There is a paucity of published literature to guide clinical decision-making in these cases. We report the case of a 26-year-old woman with no medical history who presented at 21 weeks of gestation with a bilateral sudden loss of hearing and erosive rhinitis. The diagnosis of granulomatosis with polyangiitis was confirmed radiologically and biologically. Corticosteroids were not enough to stabilize the disease and she received intravenous immunoglobulins with remission. A successful delivery of a healthy male newborn was done at 36 weeks. A review of all published literature on granulomatosis with polyangiitis in pregnancy between 1970 and 2017 is presented. Trial registration: Not applicable.

Tocilizumab for the treatment of birdshot uveitis that failed interferon alpha and anti-tumor necrosis factor-alpha therapy: two cases report and literature review.

Biotherapies appear as potential drugs for the treatment of inflammatory noninfectious uveitis. In this report, we show that tocilizumab, an anti-IL-6 agent, greatly improved two patients with birdshot chorioretinopathy refractory to conventional immunosuppressive drugs, interferon α2a, and anti-TNFα agents. After a follow-up of 22 months, patients exhibited an improvement of both visual acuity and macular edema. A corticosteroid-sparing effect was achieved in both cases.

[Which place for direct oral anticoagulants in routine hospital medical practice?]. / Quelle place pour les anticoagulants oraux directs en pratique hospitalière quotidienne?

PURPOSE: More than one million patients received an oral anticoagulant treatment in France. This medication is the first aetiology of iatrogenic events. Recently, direct oral anticoagulant (DOA) provided efficacy and safety in the treatment of atrial fibrillation and venous thromboembolic events. Given the growing increase in the prescription of these molecules, with many advantages but also disadvantages, it seemed interesting to assess in routine hospital medical practice, the proportion of patients for which the initiation of AOD could be safe. METHODS: This prospective, observational study was conducted in the department of internal medicine from October 2012 and September 2013. All inpatients receiving oral anticoagulant treatment have been included. Demographic data, indication of anticoagulant treatment, contraindications and interactions have been reported. From these information, we have established the percentage of patients who could benefit from DOA safely and securely. RESULTS: Two hundred and ninety inpatients were included with a mean age of 76.3±15.2 years old. Atrial fibrillation and thromboembolic venous disease were the most prevalent indications of anticoagulant treatments (67.2% and 22.4% of cases respectively). Among all patients, 260 had an indication of DOA (89.7%), authorized by the French National health agency. Eighty percent had both indication and no contraindication for DOA. However, only 53.1% of patients neither had drug-drug interaction. Main contraindications were severe renal failure (clearance<30mL/min) in 10.7% of cases, and recent history of gastric ulcer in 15.3% of cases. The most frequent interactions with DOA were antiplatelet agent (14.5%) and amiodarone (11.6%). Almost two thirds of inpatients (65.1%) had at least one drug-drug interaction with VKA. CONCLUSION: These results, coming from "real life", provide that only 53.1% of inpatients under anticoagulants could receive DOA safely. Caution is warranted, and VKA still have a preponderant role among anticoagulant drugs.

[Psoriatic arthritis during rituximab treatment of granulomatosis with polyangiitis: a new paradoxical side effect?]. / Rhumatisme psoriasique au cours d'un traitement par rituximab pour une polyangéïte avec granulomatose : une nouvelle réaction paradoxale ?

INTRODUCTION: Rituximab is a monoclonal antibody targeting the CD20 molecule of the B lymphocyte. Its efficacy has been recently reported in ANCA-associated vasculitis. We report a case of psoriatic arthritis that occurs during a treatment with rituximab in granulomatosis with polyangiitis. CASE REPORT: A 66-year-old woman, without past history of psoriasis, presented with a relapsing granulomatosis with polyangiitis in July 2010 with sinus and lung involvement. Treatment with rituximab was started, allowing a complete remission in 6 months. Two months after the first two infusions of rituximab she developed asymmetric arthritis of 3 distal interphalangeal joints. A few months later, the clinical presentation showed asymmetrical arthritis of the hands and wrists and dactylitis. Standard radiographs and MRI showed an inflammatory impairment according with psoriatic arthritis. CONCLUSION: Accountability of rituximab was retained in the development of the disease given the chronology of psoriatic arthritis development. It may be a paradoxical reaction, by analogy to those observed in anti-TNFα.

Intravenous immunoglobulins as treatment of severe cutaneous polyarteritis nodosa.

We describe three patients with severe refractory cutaneous polyarteritis nodosa, resulting in painful ulcers involving the lower limbs and causing toe necrosis. Due to the severity of the cutaneous manifestations, the three patients received intravenous immunoglobulins at a dose of 1 g/kg/day for 2 days monthly. After the second intravenous immunoglobulin infusion, skin signs dramatically improved and completely healed after the third intravenous immunoglobulin infusion. Our findings indicate that intravenous immunoglobulins can be included in a therapeutic strategy to treat refractory cutaneous polyarteritis nodosa.

[Usefulness of magnetic resonance imaging in Churg-Strauss syndrome related cardiac involvement. A case series of three patients and literature review]. / Intérêt de l'imagerie par résonance magnétique nucléaire au cours de l'atteinte cardiaque du syndrome de Churg-Strauss. Trois observations et revue de la littérature.

PURPOSE: The reported prevalence of cardiac complications is variable in patients with Churg-Strauss syndrome (15-92%) and depends on diagnostic tools. Diagnosis at early stage of heart involvement is crucial, resulting in appropriate management. METHODS: We report three patients who developed cardiac manifestations, revealing Churg-Strauss syndrome. The diagnosis of cardiac involvement was obtained using cardiac magnetic resonance imaging (MRI). RESULTS: Two patients were males and the remaining one was a female. Presenting clinical manifestations were: cardiac failure (n=1) and retrosternal pain (n=2). Laboratory findings disclosed: high blood count of eosinophils (range: 6000-11,000/mm(3)); antineutrophil cytoplasmic antibodies were positive in a single patient. Cardiac MRI demonstrated: (1) late gadolinium enhancement (n=3), involving mainly the apical and mid-cavity left ventricular segments; (2) impaired left ventricular function (n=2), mean left ventricular ejection fraction being: 51%; and (3) pericardial effusion (n=3). Outcome was favourable after institution of combined therapy with prednisone and cyclophosphamide (n=2); one patient also underwent plasma exchanges. CONCLUSION: Our case series underlines that MRI is a helpful tool in the diagnosis of Churg-Strauss syndrome-related cardiac complications. We further suggest that clinical assessment of patients with Churg-Strauss syndrome should include cardiac MRI, in order to detect cardiac involvement at an early stage; indeed, because cardiac manifestations are predictive factors of poor prognosis, diagnosis at early stages of cardiac involvement may result in improvement of patients management.

[Muscle lymphoma: a case report]. / Un lymphome musculaire: à propos d'une observation.

Skeletal muscle involvement is uncommon in lymphoma, occurring in less than 1.5% of patients. We report the original case of a 61-year-old man who presented with pseudotumoral muscle lesions of the lower limbs, revealing non-Hodgkin T-cell lymphoma. In our patient, magnetic resonance imaging (MRI) was useful in clearly revealing the detailed anatomic extent of muscle change; indeed, MRI showed muscle enhancement after intravenous administration of gadolinium on T1-weighted images as well as high-signal intensity on T2-weighted images. Moreover, MRI was helpful in guiding the optimal site for muscle biopsy.

[Liver involvement in hereditary haemorrhagic telangiectasia (Rendu-Osler-Weber disease). Report of one case and review of the literature]. / Atteinte hépatique au cours de la maladie de Rendu-Osler. A propos d'un cas et revue de la littérature.

INTRODUCTION: Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular dysplasia; mutations in at least three genes (ENG, ACVRL1 et MADH4), which are components of transforming growth factor (TGF)-beta, may lead to the clinical picture of HHT. HHT is a multisystemic angiodysplasia, resulting in multiple vascular malformations, involving notably the liver. EXEGESIS: We report the case of a patient with anicteric cholestasis, revealing HHT. Abdominal CT-scan revealed hepatic artery dilation and multiple arteriovenous fistula. At 2-year follow-up, the patient exhibited liver involvement-associated high output cardiac insufficiency. CONCLUSION: Liver involvement is frequent in HHT, occurring in 8-31% of patients; it may lead to life-threatening complications, such as high output cardiac failure, portal hypertension or severe cholangitis. Abdominal Doppler ultrasonography is a non-invasive accurate method, suitable for first-line imaging of the liver in patients with HHT; it should be done in all patients, in order to detect HHT-related hepatic vascular malformations.

[Usefulness of combined positron emission tomography and computed tomography imaging in Erdheim-Chester disease]. / Intérêt de la tomographie par émission de positons couplée au scanner dans la maladie d'Erdheim-Chester.

INTRODUCTION: Erdheim-Chester disease is a rare non-Langerhans form of histiocytosis. We report the use of combined fluorodeoxyglucose positron emission tomography and computed tomography (18F-FDG PET-CT) in this disease. EXEGESIS: Three men, aged from 55 to 74 years with confirmed Erdheim-Chester disease were included. 18F-FDG PET-CT allowed to detect visceral and vascular involvement of the disease which were overlooked with CT-scan or magnetic resonance imaging: left common carotid and ilio-femoral artery in one patient, coronary, femoral and tibia in the second, aortic, common carotid, femoral and mandibula in the remaining patient. Also, sequential 18F-FDG PET-CT was useful to appreciate treatment efficiency (decrease hyperfixation) and decide treatment modification (interferon alpha). CONCLUSION: 18F-FDG PET-CT combined imaging allows to assess the extent of involvement in Erdheim-Chester disease. 18F-FDG PET-CT may be also a useful tool in the management of Erdheim-Chester disease.