Prognostic value of 18 FDG-PET at diagnosis and follow-up in giant cell arteritis: An observational restrospective study.

OBJECTIVES: To evaluate the ability of 18FDG PET/CT, at diagnosis of giant cell arteritis (GCA) and during follow-up, to predict occurrence of relapse in large-vessel GCA (LV-GCA). METHODS: We conducted a retrospective study using the French Study Group for Large-Vessel Vasculitis (GEFA) network. Data from patients with LV-GCA diagnosed by PET/CT and who had PET/CT in the following year were collected. For each PET/CT, PET vascular activity score (PETVAS) and total vascular score (TVS) were assessed, and their ability to predict the occurrence of subsequent relapse was assessed. RESULTS: A total of 65 LV-GCA patients were included, of whom 55 had undergone a follow-up PET/CT 3 to 12 months after the diagnosis of GCA. Patients for whom the second PET/CT (PET2) was performed during active GCA were excluded. PETVAS and TVS decreased between PET1 and PET2 in all patients (p < 0.001). There was no correlation between vascular activity scores in PET2 and time to prednisone taper. For relapse prediction, at PET1, the AUC of the TVS and PETVAS were respectively 51.9 and 41.9 at 6 months, 55.3 and 49.7 at 1 year, 55 and 55.7 at 2 years. For PET2, the AUC were respectively 46.1 and 46.7 at 6 months, 52.1 and 48.9 at 1 year, 58.4 and 52.3 at 2 years. CONCLUSION: PET vascular activity scores at diagnosis and at follow-up PET/CT performed outside a period of GCA activity do not display high performance to predict the occurrence of subsequent relapse in LV-GCA patients.

BOB-ACG study: Pulse methylprednisolone to prevent bilateral ophthalmologic damage in giant cell arteritis. A multicentre retrospective study with propensity score analysis.

INTRODUCTION: Giant cell arteritis (GCA) is complicated in 10 to 20% of cases by permanent visual ischemia (PVI). International guidelines advocate the use of intravenous pulse of methylprednisolone from 250 to 1000mg per day, for three days, followed by oral prednisone at 1mg/kg per day. The aim of this study is to assess whether this strategy significantly reduces the risk of early PVI of the second eye, compared with direct prednisone at 1mg/kg per day. METHODS: We conducted a multicentre retrospective observational study over the past 15 years in 13 French hospital centres. Inclusion criteria included: new case of GCA; strictly unilateral PVI, prednisone at dose greater than or equal to 0.9mg/kg per day; for the intravenous methylprednisolone (IV-MP) group, total dose between 900 and 5000mg, close follow-up and knowledge of visual status at 1 month of treatment, or earlier, in case of contralateral PVI. The groups were compared on demographic, clinical, biological, iconographic, and therapeutic parameters. Statistical analysis was optimised using propensity scores. RESULTS: One hundred and sixteen patients were included, 86 in the IV-MP group and 30 in the direct prednisone group. One patient in the direct prednisone group and 13 in the IV-MP group bilateralised, without significant difference between the two strategies (3.3% vs 15.1%). Investigation of the association between IV-MP patients and contralateral PVI through classical logistic regression, matching or stratification on propensity score did not show a significant association. Weighting on propensity score shows a significant association between IV-MP patients and contralateral PVI (OR=12.9 [3.4; 94.3]; P<0.001). Improvement in visual acuity of the initially affected eye was not significantly associated with IV-MP (visual acuity difference 0.02 vs -0.28 LogMar), even in the case of early management, i.e., within the first 48hours after the onset of PVI (n=61; visual acuity difference -0.11 vs 0.25 LogMar). Complications attributable to corticosteroid therapy in the first month were significantly more frequent in the IV-MP group (31.8 vs 10.7%; P<0.05). DISCUSSION: Our data do not support the routine use of pulse IV-MP for GCA complicated by unilateral PVI to avoid bilateral ophthalmologic damage. It might be safer to not give pulse IV-MP to selected patients with high risks of glucocorticoids pulse side effects. A prospective randomised multicentre study comparing pulse IV-MP and prednisone at 1mg/kg per day is desirable.

Intravenous versus subcutaneous tocilizumab in Takayasu arteritis: multicentre retrospective study.

OBJECTIVES: In this large multicentre study, we compared the effectiveness and safety of tocilizumab intravenous versus subcutaneous (SC) in 109 Takayasu arteritis (TAK) patients. METHODS: We conducted a retrospective multicentre study in referral centres from France, Italy, Spain, Armenia, Israel, Japan, Tunisia and Russia regarding biological-targeted therapies in TAK, since January 2017 to September 2019. RESULTS: A total of 109 TAK patients received at least 3 months tocilizumab therapy and were included in this study. Among them, 91 and 18 patients received intravenous and SC tocilizumab, respectively. A complete response (NIH <2 with less than 7.5 mg/day of prednisone) at 6 months was evidenced in 69% of TAK patients, of whom 57 (70%) and 11 (69%) patients were on intravenous and SC tocilizumab, respectively (p=0.95). The factors associated with complete response to tocilizumab at 6 months in multivariate analysis, only age <30 years (OR 2.85, 95% CI 1.14 to 7.12; p=0.027) and time between TAK diagnosis and tocilizumab initiation (OR 1.18, 95% CI 1.02 to 1.36; p=0.034). During the median follow-up of 30.1 months (0.4; 105.8) and 10.8 (0.1; 46.4) (p<0.0001) in patients who received tocilizumab in intravenous and SC forms, respectively, the risk of relapse was significantly higher in TAK patients on SC tocilizumab (HR=2.55, 95% CI 1.08 to 6.02; p=0.033). The overall cumulative incidence of relapse at 12 months in TAK patients was at 13.7% (95% CI 7.6% to 21.5%), with 10.3% (95% CI 4.8% to 18.4%) for those on intravenous tocilizumab vs 30.9% (95% CI 10.5% to 54.2%) for patients receiving SC tocilizumab. Adverse events occurred in 14 (15%) patients on intravenous route and in 2 (11%) on SC tocilizumab. CONCLUSION: In this study, we confirm that tocilizumab is effective in TAK, with complete remission being achieving by 70% of disease-modifying antirheumatic drugs-refractory TAK patients at 6 months.

Clinical, biological, and ophthalmological characteristics differentiating arteritic from non-arteritic anterior ischaemic optic neuropathy.

BACKGROUND/AIMS: To identify characteristics that can distinguish AAION from NAAION in emergency practice. METHODS: This is a multicentre retrospective case-control study. Ninety-four patients with AAION were compared to ninety-four consecutive patients with NAAION. We compared the clinical, biological, and ophthalmological characteristics at baseline of patients with AAION and those with NAAION. RESULTS: Patients with AAION were older and more likely to have arterial hypertension. Cephalic symptoms and acute-phase reactants were more frequent in AAION. Profound vision loss and bilateral involvement were more frequent in AAION at baseline. Central retinal and cilioretinal artery occlusions was only observed in AAION, and delayed choroidal perfusion was more frequently observed in AAION than in NAAION. Using logistic regression, an age >70 years (OR = 3.4, IC95% = 0.8-16.1, p = 0.105), absence of splinter haemorrhage (OR = 4.9, IC95% = 1.4-20.5, p = 0.019), delayed choroidal perfusion (OR = 7.2, IC95% = 2.0-28.0, p = 0.003), CRP > 7 mg/L (OR = 43.6, IC95% = 11.6-229.1, p < 0.001) and platelets >400 × G/L (OR = 27.5, IC95% = 4.6-270.9, p = 0.001) were independently associated with a diagnosis of AAION. An easy-to-use score based on these variables accurately distinguished AAION from NAAION with a sensitivity of 93.3% and specificity of 92.4%. CONCLUSION: In patients presenting with AION, a set of ophthalmological and laboratory criteria can efficiently discriminate patients with AAION and NAAION and can identify which patients would benefit from high-dose glucocorticoids. External validation of our results is required.

Ethical Management of COVID-19 Pandemic at a Neurological Hospital: The Ethicovid Report.

BACKGROUND: During the first wave of the coronavirus disease-2019 (COVID-19) pandemic, it was necessary to prepare for the possibility of triaging patients who could benefit from access to an intensive care unit (ICU). In our neuroscience institution, the challenge was to continue to manage usual neurological emergencies as well as the influx of COVID-19 patients. METHODS: We report the experience of an ethical consulting unit to support care clinical decisions during the first wave of the pandemic (March 16 to April 30, 2020). Three objective evaluation criteria were defined: 2 of these criteria, patient's factors and general disease severity (Simplified Acute Physiology Score II), were common to all patients, and the third was the specific severity of the disease (neurological for brain injury, respiratory for COVID-19). Given our scarce resources, we used a high probability of a 3-month modified Rankin Scale ≤3 as the criterion for further resuscitation and management. RESULTS: A total of 295 patients were admitted during the first pandemic wave; 111 with COVID-19 and 184 with neurological emergencies. The ethical unit's expertise was sought for 75 clinical situations in 56 patients (35 COVID-19 and 21 neurological). Decisions were as follows: 11% no limitation on care, 5% expectant care with reassessment (maximum therapy to assess possible progress pending decision), 67% partial limitation (no intensification of care or no transfer to ICU), and 17% limitation of curative care. At no time did a lack of availability of ICU beds require the ethical unit to advise against admission to the ICU. CONCLUSIONS: Our ethical consulting unit allowed for collegial ethical decision-making in line with international recommendations. This model could be easily transferred to other triage situations, provided it is adapted to the local context.

Incidence of giant cell arteritis in six districts of Paris, France (2015-2017).

This prospective population-based study estimated the incidence of giant cell arteritis (GCA) in northeastern Paris. GCA cases diagnosed between 2015 and 2017 were obtained from local hospital and community-based physicians and the national health insurance system database. Criteria for inclusion were living in the study area at that time and fulfilling the 1990 American College of Rheumatology classification criteria and/or its expanded version. Cranial and large-vessel GCA cases were defined by the presence or absence of cranial signs and/or symptoms, respectively. Annual incidence was calculated by dividing the number of incident cases by the size of the study population ≥ 50 years old. Completeness of case ascertainment was assessed by a three-source capture-recapture analysis. Among the 62 included cases, 42 (68%) were women, mean (± SD) age 77.3 ± 9.1 years. The annual incidence of GCA in northeastern Paris and completeness of case ascertainment were estimated at 7.6 (95% CI 5.9-9.8) per 100,000 inhabitants ≥ 50 years old and 66% (95% CI 52-92%), respectively. Incidence increased with age, peaked at age 80-89 years, and was almost twice as high in women versus men. Large-vessel GCA cases, mean (± SD) age 68.6 ± 11.5 years, accounted for 8% of all GCA cases. In this study, GCA epidemiology was mainly driven by cases with cranial GCA signs or symptoms and incidence results were consistent with recent European and past French studies.

Lower Relapses Rate With Infliximab Versus Adalimumab in Sight-Threatening Uveitis: A Multicenter Study of 330 Patients.

PURPOSE: To compare the relapse rate of sight-threatening noninfectious uveitis (NIU) in patients treated with infliximab (IFX) or adalimumab (ADA). DESIGN: Observational retrospective multicenter study. METHODS: A total of 330 patients (median age, 36 years; interquartile range, 27-54), 45.2% men) with sight-threatening NIU (ie, retinal vasculitis and/or macular edema) treated with anti-tumor necrosis factor [TNF]-α agents (IFX intravenously at 5 mg/kg at weeks 0, 2, 6, and every 4 to 6 weeks or ADA subcutaneously at 80 mg, then 40 mg every 2 weeks). Data were obtained retrospectively from patients' medical records. Main outcome measures were relapse rate, complete response of NIU, corticosteroid sparing effect, and safety. RESULTS: Main etiologies of uveitis included Behçet disease (27%), idiopathic juvenile arthritis (5.8%), and sarcoidosis (5.5%). The estimated relapse rate at 6 months after introduction of biological agents was 13% (95% CI = 0.009-0.16). IFX was associated with less relapse risk than ADA (hazard ratio [HR] = 0.52, 95% CI = 0.36- 0.77, P = .001). ADA and IFX were comparable in terms of complete response rate of NIU as well as corticosteroid-sparing effect. Behçet disease was associated with higher odds of complete response (HR = 2.04, 95% CI = 1.16 -3.60, P = .01] and lower relapse rate (HR = 0.53, 95% CI = 0.33-0.85, P = .009) than other causes of NIU with anti-TNF-α agents. CONCLUSIONS: In sight-threatening NIU, IFX seems to be associated with a lower relapse rate than ADA.

Use of Retinal Angiography and MRI in the Diagnosis of Giant Cell Arteritis With Early Ophthalmic Manifestations.

BACKGROUND: Giant cell arteritis (GCA) is a vasculitis often revealed by visual signs. Diagnosis is challenging and urgent. Retinal angiography (RA) and MRI allow effective diagnosis. We compared those and proposed an imaging-based approach to diagnose GCA in ophthalmological practice. METHODS: We conducted a retrospective study based on the data collected from patients suspected to have GCA on ophthalmological findings. Fluorescein (FA) and indocyanine green (ICG) RAs and MRI were performed and compared with final diagnosis. RESULTS: Among the 41 patients included, 25 were diagnosed with GCA. Sensitivities and specificities of FA and ICG were not different. MRI showed a higher sensitivity and specificity. The approach consisting in performing RA followed by MRI provided a better accuracy. CONCLUSION: Our study shows that RA can be supplemented by MRI in a specialized center to provide the most accurate diagnosis in GCA revealed by visual signs.

Visual Recovery with Iloprost Added to Corticosteroids in a Case of Giant Cell Arteritis.

INTRODUCTION: To date, corticosteroids remain the cornerstone treatment of ocular involvement of GCA, and no other treatment has proven to be effective in this setting. We herein report on a unique case of GCA with ocular involvement worsening despite high dose corticosteroids and recovering with intravenous iloprost. CASE REPORT: A 70-year-old man presented with acute vision loss in his left eye related to anterior ischemic optic neuropathy. The diagnosis of giant-cell arteritis was confirmed by a temporal artery biopsy. Despite intravenous pulse methylprednisolone for 3 days then oral prednisone at 60 mg/day, the patient developed from day 5 fluctuating vision loss in the right eye, related to ocular ischemia by occlusion of the ophthalmic artery, and responsive to hyperhydration. Iloprost, an analog of prostacyclin PGI2, was then administered intravenously for 5 days and resulted in a stable improvement in visual acuity in the right eye. CONCLUSION: This case highlights the potential role of vasodilatator agents in giant cell arteritis with ocular involvement.

PARACENTRAL ACUTE MIDDLE MACULOPATHY IN GIANT CELL ARTERITIS.

PURPOSE: To report the occurrence of paracentral acute middle maculopathy (PAMM) in giant cell arteritis (GCA), describe its features and outcomes, and identify risk factors associated with PAMM in patients with GCA. METHODS: Review of medical records of patients with GCA who were examined in the Rothschild Foundation Hospital. Patients were divided into three groups: GCA with PAMM (Group 1), GCA with ophthalmic involvement but without PAMM (Group 2), and GCA without ophthalmic involvement (Group 3). We analyzed the data for age, sex, medical history, laboratory testing, visual acuity, and posterior segment vascular involvement. RESULTS: Among the 96 patients who met the inclusion criteria, 52 had ophthalmic involvement, and 16 patients were included in Group 1 (GCA with PAMM). In this subgroup, the mean age was 81.6 years and was found to be older than other groups. The visual prognosis was similar between Groups 1 and 2. Of the 20 eyes with PAMM, 35% were also associated with homolateral anterior ischemic optic neuropathy. No statistical difference was found in initial symptoms, signs, and laboratory testing. CONCLUSION: Paracentral acute middle maculopathy is frequently observed lesions in ocular GCA. Patients can present with isolated findings of PAMM as the only indication of GCA. Optical coherence tomography of the macula should be routinely performed in patients with suspected GCA, specifically if they complain of visual changes, to look for signs of ischemia in the middle layers of the retina. Isolated PAMM should raise suspicion for GCA in patients at risk.

Efficacy and safety of TNF-α antagonists and tocilizumab in Takayasu arteritis: multicentre retrospective study of 209 patients.

OBJECTIVE: To assess the safety and the efficacy of TNF-α antagonists and tocilizumab in patients with Takayasu arteritis (TAK). METHODS: A total of 209 patients with TAK [median age 29 years (interquartile range 7-62)], 186 (89%) females] were included. They received either TNF-α antagonists [n = 132 (63%) with 172 lines; infliximab (n = 109), adalimumab (n = 45), golimumab (n = 8), certolizumab (n = 6) and etanercept (n = 5)] or tocilizumab [n = 77 (37%) with 121 lines; i.v. and s.c. in 95 and 26 cases, respectively]. RESULTS: A complete response at 6 months was evidenced in 101/152 (66%) patients on TNF-α antagonists and 75/107 (70%) patients on tocilizumab. Age ≥30 years [odds ratio 2.09 (95% CI 1.09, 3.99)] was associated with complete response, whereas vascular signs [OR 0.26 (95% CI 0.1, 0.65)], baseline prednisone ≥20 mg/day [OR 0.51 (95% CI 0.28, 0.93)] were negatively associated with the complete response to TNF-α antagonists or tocilizumab. During a median follow-up of 36 months, 103 relapses were noted. Supra-aortic branches and thoracic aorta involvement [HR 2.44 (95% CI 1.06, 5.65) and 3.66 (1.18, 11.4), respectively] and systemic signs at baseline [HR 2.01 (95% CI 1.30, 3.11)] were significantly associated with relapse. The cumulative incidence of treatment discontinuation and relapse were similar in TNF-α antagonists and tocilizumab. Fifty-eight (20%) adverse effects occurred on biologic targeted therapies [37 (21%) on TNF-α antagonists and 21 (17%) on tocilizumab (P = 0.4), respectively]. CONCLUSION: This large multicentre study shows high efficacy of biologic targeted treatments in refractory TAK. Efficacy, relapse and drug retention rate were equivalent with TNF-α antagonists and tocilizumab.

Myeloproliferative neoplasms and clonal haematopoiesis in patients with giant cell arteritis: a case-control and exploratory study.

OBJECTIVES: GCA is a large vessel vasculitis for which triggering factors remain unknown. Clonal haematopoiesis (CH) was associated with atherosclerosis through the induction of inflammation in myeloid cells, and data suggest that CH expansion and inflammation may support each other to induce a pro-inflammatory loop. Our objective was to describe the impact of JAK2p.V617F-mutated myeloproliferative neoplasms (MPNs) on GCA and to screen MPN-free patients for CH mutations. METHODS: We performed a retrospective case-control study comparing the characteristics of 21 GCA patients with MPN and 42 age- and gender-matched GCA patients without MPN. Also, 18 GCA patients were screened for CH through next-generation sequencing (NGS). RESULTS: The most frequent associated MPN was essential thrombocythaemia (ET; n = 11). Compared with controls, GCA patients with MPN had less-frequent cephalic symptoms (71.4 vs 97.6%; P = 0.004) and higher platelet counts at baseline [485 × 109/l (interquartile range 346-586) vs 346 (296-418); P = 0.02]. There was no difference between groups for other clinical features. Overall survival was significantly shorter in patients with MPN compared with controls [hazard ratio 8.2 (95% CI 1.2, 56.6); P = 0.03]. Finally, screening for CH using NGS in 15 GCA patients without MPN revealed CH in 33%. CONCLUSION: GCA patients with MPN display higher platelet counts and shorter overall survival than controls. This association is not fortuitous, given the possible pathophysiological relationship between the two diseases. CH was found in one-third of GCA patients, which may be higher than the expected prevalence for a similar age, and should be confirmed in a larger cohort.

Prognosis Factors and Outcomes of Neuro-ophthalmologic Sarcoidosis.

BACKGROUND: Neuro-ophthalmologic manifestations are uncommon in sarcoidosis. We aim to assess the prognostic factors and outcome of neuro-ophthalmic sarcoidosis. METHODS: We conducted a multicenter retrospective study on patients with neuro-ophthalmic sarcoidosis. Response to therapy was based on visual acuity, visual field, and orbital MRI exam. Factors associated with remission and relapse were analyzed. RESULTS: Thirty-five patients [median (IQR) age of 37 years (26.5-53), 63% of women] were included. The diagnosis of sarcoidosis was concomitant of neuro-ophthalmologic symptoms in 63% of cases. Optic neuritis was the most common manifestation. All patients received corticosteroids and 34% had immunosuppressants. At 6 months, 61% improved, 30% were stable, and 9% worsened. Twenty percent of patients had severe visual deficiency at the end of follow-up. Nonresponders patients had significantly worse visual acuity at baseline (p = 0.01). Relapses were less frequent in patients with retro-bulbar optic neuropathy (p = 0.03). CONCLUSION: Prognosis of neuro-ophthalmic sarcoidosis is poor.

Anti-Tumor Necrosis Factor α versus Tocilizumab in the Treatment of Refractory Uveitic Macular Edema: A Multicenter Study from the French Uveitis Network.

PURPOSE: To analyze the factors associated with response (control of ocular inflammation and corticosteroid-sparing effect) to biologics (anti-tumor necrosis factor [TNF]-α agents and tocilizumab) in patients with refractory uveitic macular edema (ME). DESIGN: Multicenter, retrospective, observational study. PARTICIPANTS: Adult patients with uveitic ME refractory to systemic corticosteroids, disease-modifying antirheumatic drugs, or both. METHODS: Patients received anti-TNF-α agents (infliximab 5 mg/kg at week 0, 2, 6, and every 4-6 weeks [n = 69] and adalimumab 40 mg/2 weeks [n = 80]) and tocilizumab (8 mg/kg every 4 weeks intravenously [n = 39] and 162 mg/week subcutaneously [n = 16]). MAIN OUTCOME MEASURES: Analysis of complete and partial response rates, relapse rate, low vision (visual acuity in at least 1 eye of ≥ 1 logarithm of the minimum angle of resolution), corticosteroid-sparing effect, and adverse events at 6 months. RESULTS: Two hundred four patients (median age, 40 years [interquartile range, 28-58 years]; 42.2% men) were included. Main causes of uveitis included Behçet's disease (17.2%), birdshot chorioretinopathy (11.3%), and sarcoidosis (7.4%). The overall response rate at 6 months was 46.2% (21.8% of complete response) with anti-TNF-α agents and 58.5% (35.8% of complete response) with tocilizumab. In multivariate analysis, treatment with tocilizumab (odds ratio, 2.10; 95% confidence interval [CI], 1.06-4.06; P = 0.03) was associated independently with complete response of uveitic ME compared with anti-TNF-α agents. Anti-TNF-α agents and tocilizumab did not differ significantly in terms of relapse rate (hazard ratio, 1.00; 95% CI, 0.31-3.18; P = 0.99) or occurrence of low vision (odds ratio, 1.02; 95% CI, 0.51-2.07; P = 0.95) or corticosteroid-sparing effect (P = 0.29). Adverse events were reported in 20.6% of patients, including serious adverse events reported in 10.8% of patients. CONCLUSIONS: Tocilizumab seems to improve complete response of uveitic ME compared with anti-TNF-α agents.

Validation of a multimodal algorithm for diagnosing giant cell arteritis with imaging.

PURPOSE: The purpose of this study was to identify which combination of imaging modalities should be used to obtain the best diagnostic performance for the non-invasive diagnosis of giant cell arteritis (GCA). MATERIALS AND METHODS: This IRB-approved prospective single-center study enrolled participants presenting with a suspected diagnosis of GCA from December 2014 to October 2017. Participants underwent high-resolution 3T magnetic resonance imaging (MRI), temporal and extra-cranial arteries ultrasound and retinal angiography (RA), prior to temporal artery biopsy (TAB). Diagnostic accuracy of each imaging modality alone, then a combination of several imaging modalities, was evaluated. Several algorithms were constructed to test optimal combinations using McNemar test. RESULTS: Forty-five participants (24 women, 21 men) with mean age of 75.4 ± 16 (SD) years (range: 59-94 years) were enrolled; of these 43/45 (96%) had ophthalmological symptoms. Diagnosis of GCA was confirmed in 25/45 (56%) patients. Sensitivity and specificity of MRI, ultrasound and RA alone were 100% (25/25; 95% CI: 86-100) and 86% (19/22; 95% CI: 65-97), 88% (22/25; 95% CI: 69-97) and 84% (16/19; 95% CI: 60-97), 94% (15/16; 95% CI: 70-100) and 74% (14/19; 95% CI: 49-91), respectively. Sensitivity, specificity, positive predictive and negative predictive values ranged from 95 to 100% (95% CI: 77-100), 67 to 100% (95% CI: 38-100), 81 to 100% (95% CI: 61-100) and 91 to 100% (95% CI: 59-100) when combining several imaging tests, respectively. The diagnostic algorithm with the overall best diagnostic performance was the one starting with MRI, followed either by ultrasound or RA, yielding 100% sensitivity (22/22; 95% CI: 85-100%) 100% (15/15; 95% CI: 78-100) and 100% accuracy (37/37; 95% CI: 91-100). CONCLUSION: The use of MRI as the first imaging examination followed by either ultrasound or RA reaches high degrees of performance for the diagnosis of GCA and is recommended in daily practice.

Early diffusion-weighted MRI at 3 Tesla detects ischemic changes of the optic nerve in anterior ischemic optic neuropathy.

OBJECTIVES: To assess the impact of timing from visual symptoms' onset to diffusion-weighted (DW) 3 T MRI completion to detect ischemic changes of the optic disc and optic nerve in AION patients. METHODS: This IRB-approved retrospective single-center study included 3 T MRI data from 126 patients with AION and 111 controls with optic neuritis treated between January 2015 and May 2020. Two radiologists blinded to all data individually analyzed imaging. A senior neuroradiologist resolved any discrepancies by consensus. The primary judgment criterion was the restricted diffusion of the optic disc and/or the optic nerve assessed subjectively on the ADC maps. ADC values were also measured. Spearman rank correlations were used to examine the relationships between timing from visual symptoms' onset to MRI completion and both the restricted diffusion and the ADC values. RESULTS: One hundred twenty-six patients (47/126 [37.3%] women and 79/126 [62.7%] men, mean age 69.1 ± 13.7 years) with AION were included. Restricted diffusion of the optic disc in AION eyes was more frequent in the early MRI group than in the late MRI group: 35/49 (71.4%) eyes versus 3/83 (3.6%) eyes, p < 0.001. ADC values of the pathological optic discs and optic nerves were lower in the early MRI group than in the late MRI group: 0.61 [0.52-0.94] × 10-3 mm2/s versus 1.28 [1.01-1.44] × 10-3 mm2/s, p < 0.001, and 0.74 [0.61-0.88] × 10-3 mm2/s versus 0.89 [0.72-1.10] × 10-3 mm2/s, p < 0.001, respectively. CONCLUSIONS: DWI MRI showed good diagnostic performance to detect AION when performed early after the onset of visual symptoms. KEY POINTS: • Restricted diffusion of the optic disc in eyes affected by AION was significantly more likely to be observed in patients who had undergone MRI within 5 days after onset of visual symptoms. • ADC values of the pathological optic discs and optic nerves were significantly lower in patients who had undergone MRI within 5 days after onset of visual symptoms of AION: 0.61 × 10-3 mm2/s versus 1.28 × 10-3 mm2/s, p < 0.001, and 0.74 × 10-3 mm2/s versus 0.89 × 10-3 mm2/s, p < 0.001, respectively. • The optimal threshold for timing from visual symptoms' onset to MRI completion to detect restricted diffusion of the optic disc and/or optic nerve was 5 days, with an AUC of 0.88 (CI95%: 0.82-0.94).

Lymphoma complicating rheumatoid arthritis: results from a French case-control study.

OBJECTIVES: To study the characteristics of B-cell non-Hodgkin's lymphoma (NHL) or Hodgkin lymphoma complicating rheumatoid arthritis (RA) and to identify RA-related factors associated with their occurrence. METHODS: A multicentre case-control study was performed in France. Cases were patients with RA fulfilling ACR-EULAR 2010 criteria in whom B-cell NHL or Hodgkin lymphoma developed after the diagnosis of RA. For each case, 2 controls were assigned at random from the ESPOIR cohort and were matched on age at lymphoma diagnosis (cases)/age at the 10-year follow-up visit in the cohort (controls). Case and control characteristics were compared to identify parameters associated with the occurrence of lymphoma. RESULTS: 54 cases were included and matched to 108 controls. Lymphomas were mostly diffuse large B-cell lymphoma (DLBCL, n=27, 50.0%). On immunochemistry, 4 of 27 (14.8%) lymphoma cases were positive for Epstein-Barr virus. On univariate analysis, factors associated with the occurrence of lymphoma were male sex (OR 3.3, 95% CI 1.7 to 6.7), positivity for ACPA (OR 5.1, 95% CI 2.0 to 15.7) and rheumatoid factor (OR 3.9, 95% CI 1.6 to 12.2), and erosions on radiographs (OR 3.8, 95% CI 1.7 to 8.3) and DAS28 (OR 2.0, 95% CI 1.5 to 2.7), both at the time of matching. Methotrexate, TNF blockers and a number of previous biologics were not associated with the occurrence of lymphoma. On multivariable analysis, erosions and DAS28 remained significantly associated with increased risk of lymphoma. CONCLUSION: Lymphomas complicating RA are mostly DLBCL. Risk of lymphoma in patients with RA was increased with markers of disease activity and severity, which supports the paradigm of a continuum between autoimmunity and lymphomagenesis in RA.

Discriminating between IgG4-related orbital disease and other causes of orbital inflammation with intra voxel incoherent motion (IVIM) MR imaging at 3T.

PURPOSE: The purpose of this prospective study was to determine the capabilities of intravoxel incoherent motion (IVIM) MRI at 3 Tesla in discriminating between IgG4-related orbital disease (IgG4-ROD) and other causes of orbital inflammation. MATERIALS AND METHODS: Main selection criteria for the patients enrolled in this prospective study were age over 18 years and histopathologicaly proven orbital inflammatory lesion. MRI examinations were performed prior to surgery and treatment in all patients with suspected orbital inflammation. Two neuroradiologists, blinded to clinical data, independently analyzed structural MRI examinations and IVIM sequences obtained with 15 b values ranging from 0 to 2000 s/mm². Apparent diffusion coefficient (ADC), "true" diffusion coefficient (D), perfusion fraction (f) and pseudodiffusion coefficient (D*) values were calculated from all orbital lesions. Diagnostic capabilities of IVIM parameters were assessed using receiver operating-characteristic (ROC) curves and area under the curve (AUC). Sensitivity, specificity, and accuracy of IVIM parameters were calculated for the best threshold values and reported with their corresponding 95% confidence intervals (CI). RESULTS: Thirty-five patients (21 women and 14 men; mean age, 49.2 ± 13.75 [SD] years; age range: 23-77 years) with 48 orbital lesions were enrolled in the study. Fifteen patients (15/35; 43%) had IgG4-ROD and 20 (20/35; 57%) had other causes of orbital inflammation. Median D value was significantly greater in patients with IgG4-ROD (1 × 10-3 mm2/s; interquartile range [IQR]: 0.9 × 10-3; 1.2 × 10-3) as compared to patients with non IgG4-ROD (0.80 × 10-3 mm2/s; IQR: 0.7 × 10-3; 1 × 10-3) (P = 0.04). There was no significant difference for ADC, f or D*. Area under the curve were of 0.54, 0.73, 0.63 and 0.56 for ADC, D, f and D*, respectively. Optimal threshold derived from ROC curves for D was 0.87 × 10-3 mm2/s, yielding 92% sensitivity (95% CI: 62-100%) and 71% specificity (95% CI: 44-90%) for the diagnosis of IgG4-ROD. No differences in standard morphological MRI criteria were found between IgG4-ROD and non IgG4-ROD. CONCLUSION: Our study shows that IVIM MRI is a useful imaging technique to distinguish IgG4-ROD from other causes of orbital inflammation.