K.H. Ly

Interleukin-1 blockade in refractory giant cell arteritis.

Giant cell arteritis is a primary large-vessel vasculitis characterized by an arterial wall inflammation associated with intimal hyperplasia leading to arterial occlusion. Glucocorticoids remain the mainstay of giant cell arteritis treatment. However, relapses and glucocorticoid-related complications are frequent and therapeutic options for refractory giant cell arteritis are quite limited. Like tumor necrosis factor-α and interleukin-6, interleukin-1β is also highly expressed in inflamed arterial walls of patients with giant cell arteritis and may contribute in the pathogenesis of this disease. We report treatment of three cases of refractory giant cell arteritis successfully treated with anakinra, an interleukin-1 blockade therapy. Anakinra was effective for all patients, yielding improvement in their inflammation biomarkers and/or in their symptoms, as well as a disappearance of arterial inflammation in PET/CT for two of them.

Risk Factors for Permanent Visual Loss in Biopsy-proven Giant Cell Arteritis: A Study of 339 Patients.

Objective. To determine the risk factors for permanent visual loss (PVL) in patients with biopsy-proven giant cell arteritis (GCA) and the usefulness of the factors in clinical practice. Methods. From 1976 through 2015, the clinical charts and laboratory results of 339 patients with biopsy-proven GCA were recorded prospectively at the time of diagnosis. We used multivariable logistic regression analysis to determine which of 24 pretreatment characteristics were associated with PVL. Results. Visual ischemic manifestations occurred in 108 patients, including PVL in 53 (16%), bilaterally in 15 patients (28%). The independent predictors associated with an increased risk of PVL were age (OR 1.06, 95% CI 1.01–1.12, p = 0.01), a history of transient visual ischemic symptoms (OR 2.62, 95% CI 1.29–5.29, p < 0.01), and jaw claudication (OR 2.11, 95% CI 1.09–4.10, p = 0.03). The presence of fever (OR 0.30, 95% CI 0.14–0.64, p < 0.01) and rheumatic symptoms (OR 0.23, 95% CI 0.10–0.57, p = 0.001) were associated with a markedly reduced risk of developing visual loss (3.7% if features were both present). No laboratory variables were independently associated with PVL. Conclusion. The visual ischemic risk of untreated GCA can be readily estimated upon simple clinical findings, but not laboratory variables. However, we did not identify a subgroup of patients carrying no risk of developing visual loss. Glucocorticoid treatment remains, therefore, urgent for any patient with a high clinical suspicion index.

Physiopathologie de l’artérite à cellules géantes

Giant cell arteritis is a large-vessel vasculitis affecting all three layers of the arterial wall. Histopathology of this vasculitis consists of an inflammatory infiltrate with CD4(+) T cells, macrophages, multinucleated giant cells, forming granulomas in the media. This infiltrate is associated with a destruction of the arterial wall, a fragmentation of the internal elastic lamina and a vascular remodeling leading to intimal hyperplasia. Recent studies have clarified the role of Th17 cells in the initial phase of the disease, pro-inflammatory cytokines and vascular smooth muscle cells in vascular remodeling. This review aims to update data on giant cell arteritis pathogenesis and to propose clues of investigation for a better understanding of this condition.

Mise au pointL’ascite non liée à la cirrhose : physiopathologie, diagnostic et étiologiesNon-cirrhotic ascites: Pathophysiology, diagnosis and etiology

Ascites, in 20% of cases, is not linked to liver cirrhosis. The pathophysiology is most often different. The understanding of these pathophysiological mechanisms can lead to etiologic diagnosis. The diagnostic approach is mainly based on the biological study of ascites, especially protein concentration and albumin gradient between serum and ascites. In Western countries, tumors and heart diseases are the predominant causes, while developing countries are mainly concerned by infectious diseases, among which tuberculosis is the leading cause. Other uncommon causes must be recognized, as ascites may be the presenting feature of the disease. Their knowledge will facilitate the therapeutic approach.

L’ascite non liée à la cirrhose : physiopathologie, diagnostic et étiologies

Ascites, in 20% of cases, is not linked to liver cirrhosis. The pathophysiology is most often different. The understanding of these pathophysiological mechanisms can lead to etiologic diagnosis. The diagnostic approach is mainly based on the biological study of ascites, especially protein concentration and albumin gradient between serum and ascites. In Western countries, tumors and heart diseases are the predominant causes, while developing countries are mainly concerned by infectious diseases, among which tuberculosis is the leading cause. Other uncommon causes must be recognized, as ascites may be the presenting feature of the disease. Their knowledge will facilitate the therapeutic approach.

Development of Giant Cell Arteritis after Treating Polymyalgia or Peripheral Arthritis: A Retrospective Case-control Study

Objective. We investigated the development of giant cell arteritis (GCA) in patients with prior diagnoses of isolated polymyalgia rheumatica and/or peripheral arthritis (PMR/PA), and the potentially relevant characteristics of both illnesses in such patients. Methods. We retrospectively compared the features of 67 patients at the onset of PMR/PA, and their outcomes, to those of a random group of 65 patients with PMR/PA who did not develop late GCA. We also compared the features and outcomes of patients with late GCA to those of a random sample of patients with more usual GCA (65 with concurrent PMR/PA and 65 without). Results. Patients with late GCA represented 7.4% of all patients with GCA included in a large hospital-based inception cohort. PMR/PA preceded overt GCA by 27 months on average. Permanent visual loss developed in 10 patients, including 8 of 48 (17%) patients featuring cranial arteritis. A questionable female predominance was the only distinguishing feature of PMR/PA evolving into GCA; late GCA more often featured subclinical aortitis (OR 6.42, 95% CI 2.39–17.23; p < 0.001), headache (OR 0.44, 95% CI 0.19–1.03; p = 0.06), and fever (OR 0.29, 95% CI 0.13–0.64; p = 0.002) less often compared to the more usual form of GCA. Patients with either form of GCA experienced similar outcomes. Conclusion. A cranial arteritis pattern of late GCA is associated with a significant risk for ischemic blindness. However, compared to the usual form of GCA, late GCA is often less typical, with a higher frequency of silent aortitis. Patients with relapsing/refractory PMR may not be at increased risk for late GCA.

Endocardite infectieuse à Bartonella henselae : un risque majeur de destruction valvulaire

ion de lymphome oculaire. Pour trois autres patients le diagnostic e syphilis secondaire cutanéo-muqueuse était réalisé avec un à ix mois de retard en raison de l’importance des signes généraux t d’adénopathies suspectes de lymphome B, ou d’une éruption rythémato-squameuse avec un infiltrat lympho-plasmocytaire uspect de pseudolymphome cutané. Tout patients confondus, 5 % des patients étaient asymptomatiques, 8 % présentaient une yphilis primaire, 33 % une syphilis secondaire cutanéo-muqueuse ouvent accompagnée de signes généraux et 40 % une neurosyphiis précoce sous la forme d’une uvéite antérieure (6 %), postérieure 43 %), d’une panuvéite (19 %) d’un œdème papillaire (6 %), d’une éningite (6 %), d’une paralysie faciale périphérique (6 %) et d’une urdité (12 %). Un seul patient présentait une neurosyphilis tardive, ous la forme d’une syphilis méningo-vasculaire (AVC ischémique). uatorze patients avaient une méningite lymphocytaire. Quand lle était réalisée, l’IRM cérébrale était normale hormis le cas de eurosyphilis tardive. Les patients présentant une syphilis priaire ou secondaire isolée étaient traités par une à trois injections ’extencilline. Les patients présentant une neurosyphilis étaient raités par pénicilline G à fortes doses pendant au moins 14 jours 94 %) ou ceftriaxone (6 %). Six patients atteints de neurosyphiis gardaient des séquelles ophtalmologiques minimes (25 %) ou ajeures (12 %). onclusion.– Après une diminution drastique du nombre de cas e syphilis, on observe une augmentation des cas qui semble orrespondre à un relâchement des mesures de protection des apports sexuels. Les patients hospitalisés pour syphilis entre 000 et 2010 présentaient majoritairement une atteinte neuroloique justifiant un traitement parentéral par pénicilline G ou une tteinte cutanéo-muqueuse accompagnée de signes systémiques t/ou d’adénopathies faisant égarer le diagnostic, en particulier ans le groupe des patients non-VIH chez qui la sérologie devrait tre systématique devant de tels symptômes.