U. Warzocha

Transient elastography as a screening tool for liver fibrosis and cirrhosis in a community-based population aged over 45 years

Background Liver stiffness measurement (LSM) has been used to measure fibrosis in patients with various types of chronic liver diseases. However, its usefulness as a screening procedure in apparently healthy people had not been evaluated to date. Methods 1358 subjects >45 years old from a general population attending for a medical check-up were consecutively enrolled in the study. All subjects were submitted to medical examination and laboratory tests in addition to LSM, performed on the same day by a single operator. Subjects with LSM values >8 kPa were referred to a liver unit for further investigations. Results 168 subjects were not considered for analysis due to missing data (n=23), LSM failure (n=51) or unreliable LSM values (n=94). Among the 1190 remaining subjects, 89 (7.5%) had LSM >8 kPa including nine patients with LSM >13 kPa. Despite the fact that normal liver tests were observed in 43% of them (38 out of 89), a specific cause of chronic liver disease was found in all cases. Non-alcoholic fatty liver disease (NAFLD) was the likely cause of chronic liver disease in 52 patients, alcoholic liver disease (ALD) in 20, and both causes were associated in seven additional patients. Hepatitis C virus and hepatitis B virus chronic hepatitis was documented in five and four cases, respectively, and primary biliary cirrhosis in one. Liver biopsy was obtained for 27 patients, including the nine patients with LSM >13 kPa, who were diagnosed with liver cirrhosis due to ALD (n=5), chronic hepatitis C (n=3) or chronic hepatitis B (n=1). The 18 remaining biopsies showed liver fibrosis in all cases except one (isolated steatosis), with ALD and NAFLD being present in six and eight cases, respectively. Conclusion LSM proved to be a useful and specific procedure to screen for cirrhosis in the general population and to detect undiagnosed chronic liver disease in apparently healthy subjects.

Pregnancies in systemic necrotizing vasculitides: report on 12 women and their 20 pregnancies

OBJECTIVES To describe pregnancies of women with systemic necrotizing vasculitides (SNVs), i.e. PAN, WG, Churg-Strauss syndrome (CSS) or microscopic polyangiitis (MPA), followed over the past 15 years at four French centres. METHODS Retrospective analysis of women whose SNV appeared during pregnancy or who became pregnant after SNV diagnosis. RESULTS Among the 12 women identified, one experienced rupture of pancreatic artery microaneurysms at 27 weeks revealing PAN, leading to surgical haemostasis and caesarean delivery. Eleven others started 19 pregnancies after SNV diagnosis (8 in four WG, 6 in three CSS, 1 each in three PAN and 2 in one MPA); 14 conceived during vasculitis remission. Two ended in first-trimester abortions, four miscarried; the remaining 13 pregnancies yielded 14 live newborns (1 twin pregnancy), with 7 pre-term births. Life-threatening complications occurred during 3 of these latter 13 pregnancies and required caesarean delivery. The twin pregnancy (in a CSS patient with initial vasculitis-related cardiac involvement, but in remission at conception) was complicated by transient maternal cardiac failure at 32 weeks. One WG patient with vasculitis-related renal damage developed thrombotic microangiopathy-associated renal impairment at 32 weeks, and another WG patient had severe pneumonia at 37 weeks. Other pregnancies were uneventful or with minor vasculitis manifestations. CONCLUSION Pregnant SNV patients should be monitored closely, because miscarriages and pre-term births are not uncommon. Pregnancy does not seem to have a major impact on vasculitis activity. However, life-threatening manifestations can occur, especially in patients with vasculitis-related cardiac or renal damage.

Successful treatment of idiopathic orbital inflammation with infliximab: an alternative to conventional steroid-sparing agents.

Therapy for orbital pseudotumor, also called idiopathic orbital inflammation, is nonspecific and mainly based on the use of corticosteroids. Two patients with steroid-dependent idiopathic orbital inflammation, one with myositis and the other with dacryoadenitis, and intolerant of standard steroid sparing agents (methotrexate or azathioprine), were treated with infliximab, a monoclonal antitumor necrosis factor alpha antibody. In both patients, orbital manifestations disappeared following treatment. After follow-up for at least 20 months, the 2 patients still receiving infliximab remained event free. Infliximab enabled steroid tapering to less than 5 mg per day, suggesting that infliximab could constitute an alternative to conventional steroid sparing agents.

Adult-onset asthma associated with periocular xanthogranuloma: new diagnostic and therapeutic approaches in a very rare systemic disease.

Purpose: To report some novel findings concerning the systemic manifestations and treatment of adult-onset asthma and periocular xanthogranuloma, a rare type of non-Langerhans histiocytosis that can lead to important visual dysfunction. Methods: A retrospective case series of 2 patients was evaluated for orbital and systemic manifestations using fluorodeoxyglucose positron emission tomography/CT and/or orbital MRI. Histological specimens were reviewed in all patients. Oral prednisone was initiated at 1 mg/kg daily and gradually tapered to a minimum effective dose. Efficacy was assessed on the basis of an objective observation of decreased swelling. Results: One patient displayed original uptake foci involving intrathoracic lymphadenopathies, the trajectory of the paraumbilical vein and perirectal fat. Low-dose prednisone was able to induce a durable response in the authors’ patients. Conclusions: Fluorodeoxyglucose positron emission tomography/CT may be useful for the diagnostic workup and follow-up assessment of patients with adult-onset asthma and periocular xanthogranuloma. Oral corticosteroids can be used successfully as first-line treatment in such patients.

Adult Onset Asthma and Periocular Xanthogranuloma (AAPOX), a Rare Entity With a Strong Link to IgG4-Related Disease: An Observational Case Report Study.

AbstractAdult onset asthma and periocular xanthogranuloma (AAPOX) is a rare non-Langerhans histiocytosis characterized histopathologically by a periocular infiltration of foamy histiocytes and Touton giant cells. Benign hyperplasia with plasma cell infiltration is classically described in eyelids or lymph nodes of AAPOX patients. It is also a characteristic feature of IgG4-related disease (IgG4-RD), a new entity defined by an IgG4-bearing plasma cell infiltration of organs.To determine if AAPOX syndrome shares clinical, biological, and histopathological characteristics with IgG4-RD, we used the comprehensive clinical diagnostic criteria for IgG4-RD in a retrospective case series of three consecutive patients with histologically-proven AAPOX. Patients who were diagnosed with AAPOX at a French academic referral center for orbital inflammation between November 1996 and March 2013 were enrolled. Biopsies from ocular adnexa or other organs were systematically reexamined. For each patient, clinical and serological data, radiologic findings, and treatment were retrospectively analyzed.Two AAPOX patients fulfilled all of the diagnostic criteria for a definite IgG4-RD. One patient who lacked the serological criteria fulfilled the criteria of a probable IgG4-RD.These 3 cases of AAPOX patients fulfilled the IgG4-RD comprehensive clinical diagnostic criteria. To our knowledge, this is the first observational case report study to clearly show a strong relationship between IgG4-RD and AAPOX syndrome.

Efficacy of tocilizumab highlighted by FDG-PET/CT in a patient with relapsing polychondritis-associated aortitis

Relapsing polychondritis (RP) is a rare systemic inflammatory disease primarily affecting the ears, nose and tracheobronchial tree cartilage, but also the cardiovascular system. Cardiovascular complications are the second cause of mortality in RP. We report the case of a woman with a corticosteroid-resistant RP-associated aortitis, who was successfully treated with tocilizumab (TCZ). The FDG-PET/CT was a useful tool for diagnosing aortitis and assessing the effect of biotherapy. We conducted a systematic literature review confirming this is the first case of rapid and sustained remission in a patient with corticosteroid-resistant RP-associated aortitis after TCZ treatment administered as a first-line immunotherapy. However, further studies are needed to confirm the beneficial effect of TCZ used in this life-threatening condition.

Macroanévrysmes multiples, une complication rare d’une vascularite de type périartérite noueuse

u cours d’une maladie de Wegener [1]. Cette complication rare a évolué favoralement sous corticoïdes et immunosuppresseurs et n’a pas nécessité de drainage algré les signes initiaux d’hypertension intracrânienne. Certaines observations omparables sont rapportées dans d’autres vascularites, notamment dans le cadre ’un neuro-Behcet [2], ou d’un syndrome de Kawasaki [3]. La présentation cliique, souvent dominée par des céphalées et de la fièvre, amène au diagnostic ’épanchements sous duraux uniou bilatéraux, avec une excellent réponse linique au traitement de la maladie de fond. La physiopathologie de ces épanhements n’est pas clairement établie. Les hypothèses d’une hyperhémie causée ar l’inflammation méningée, de troubles de résorption liés à la pachyméningite u de la participation d’une vascularite des vaisseaux sous duraux peuvent être oulevées. onclusion.– L’épanchement sous dural bilatéral spontané est une complication xceptionnelle de la pachyméningite de la maladie de Wegener. Le traitement mmunosuppresseur peut permettre de traiter cette complication sans avoir ecours à la chirurgie. éférences 1] Shiotani A, et al. Intern Med 1997;36:514–8. 2] Suzuki N, et al. Ann Rheum Dis 2003;62:374–5. 3] Bailie NM, et al. Eur J Paediatr Neurol 2001;5:79–81.

Takayasu disease revealed by bilateral loss of vision.

PURPOSE Takayasu arteritis is a nonspecific granulomatous inflammatory arteriopathy of unknown cause, most frequently diagnosed in young Asian women. The authors present an atypical initial presentation of Takayasu disease with bilateral loss of vision in a young Haitian man. METHODS Case report. RESULTS The presenting feature was bilateral loss of vision, a result of malignant hypertensive retinopathy and choroidopathy. Systemic evaluation disclosed inflammatory signs and renal artery obstruction which caused the malignant hypertension. CONCLUSION This case, involving an unusual presentation of Takayasu disease, illustrates how a malignant hypertensive retinopathy and choroidopathy led to the ultimate diagnosis of Takayasu disease.

P.369 Le Fibroscan : une méthode fiable de dépistage de la cirrhose dans la population générale

Introduction La mesure de l’elasticite du foie (MEF) par le Fibroscan (Echosens, Paris) a ete utilisee pour le depistage de la cirrhose dans des groupes a risque mais jamais dans la population generale. L’objectif de cette etude prospective etait de determiner chez des sujets apparemment sains : 1) la specificite de la MEF pour le diagnostic de cirrhose ; 2) la prevalence de la cirrhose ; 3) les consequences pratiques de son depistage. Patients et Methodes 1 358 sujets de plus de 45 ans tires au sort et convoques dans un Centre de Prevention Sanitaire et Sociale pour un bilan de sante gratuit ont ete inclus. Tous ont eu le meme jour un examen clinique, des tests biologiques et une MEF toujours effectuee par le meme operateur. Les criteres de validite de la MEF etaient : un minimum de 10 acquisitions, un taux de reussite > 60 % et un IQR Resultats 85 sujets (6,2 %) ont ete exclus pour echec de la technique ou mesures non valides. L’obesite etait la cause principale d’echec ou de non validation. Parmi les 1 273 sujets inclus, 93 (7,3 %) avaient une elasticite superieure a 8kPa. Huit de ces sujets (0,63 %) avaient une elasticite superieure a 14kPa et tous avaient une cirrhose confirmee histologiquement. La cirrhose etait d’origine alcoolique dans 4 cas, virales C dans 2 cas, virale B dans 1 cas et metabolique dans 1 cas. Dans tous les cas, la cirrhose etait meconnue avant la MEF. Tous les patients ont secondairement accepte une prise en charge therapeutique. Conclusion Dans cette etude du depistage de la cirrhose par MEF dans une population generale agee de plus de 45 ans : 1) au seuil de 14kPa, la specificite et la valeur predictive positive pour le diagnostic de cirrhose etait de 100 % ; 2) la prevalence de la cirrhose etait au minimum de 0,63 % ; 3) le depistage a abouti au diagnostic et a la prise en charge therapeutique de cirrhoses jusque-la meconnues.