The functional disabilities of the dominant and opposite hands in patients with systemic sclerosis.

OBJECTIVES: Hand involvement in patients with systemic sclerosis (SSc) is responsible for 75% of the overall disability but varies greatly among individuals. No study has yet compared the functionalities between the two hands of SSc patients. We thus evaluated the joint limitations and extent of skin involvement in the dominant and contralateral hands. METHODS: This prospective, descriptive, comparative single-centre study enrolled SSc patients diagnosed using the ACR/EULAR criteria. We assessed limitations in the joint range of motion during active and passive mobilisation; the first commissure opening angles; the Kapandji scale and Rodnan hand scores; the digital pressures; the finger brachial pressure indices; and the number of telangiectasias, calcinosis, digital ulcerations, and painful joints on each hand. RESULTS: Thirty patients were included. Spontaneous flexion joint limitations were significantly greater in the dominant hand (p<0.0001). The Kapandji score was lower (p<0.001) and the Rodnan hand score significantly higher, for the dominant hand (p<0.001). The digital pressure was similar between the hands. CONCLUSIONS: The dominant hand exhibited significantly more skin sclerosis and mean flexion deterioration, a lower Kapandji score, and a tendency toward reduced mean extension, compared with the other hand. No vascular pathology was noted in either hand. Larger studies are needed to confirm these results and to draw therapeutic conclusions.

Use of complementary and alternative medicine by patients treated for systemic lupus erythematosus, primary Sjögren's syndrome or systemic sclerosis in a french rural region.

BACKGROUND: Complementary and alternative medicine (CAM) is composed of a wide range of interventions and frequently used in parallel with conventional medicine. The aim of this study was to assess the prevalence, modalities, and association factors of CAM utilization in patients treated for systemic lupus erythematosus, primary Sjögren's syndrome or systemic sclerosis. PATIENTS AND METHODS: This was a prospective single-center observational study conducted in a french university hospital center. Inclusion criteria were patients followed for systemic lupus erythematosus, primary Sjögren's syndrome or systemic sclerosis. Data were collected with a survey which assessed socio-demographic, disease characteristics, CAM use details, life quality and anxiety score. RESULTS: A total of 121 patients were included, mostly women (87%), with an average age of 56 years. Proportion of patients seeking CAM was 55%. A total of 186 CAM interventions were recorded: most common was osteopathy, homeopathy and acupuncture. Patients were looking for well-being (22%), reducing their fatigue (18%) and pain (33%). Concerning physical and mental feeling after CAM use, a subjective improvement was reported in 89% of cases. In multivariate analysis, CAM use by patient was associated with these 3 variables : coming from a Western culture, being professionally active, having a poor quality of life and anxiety scores. CONCLUSION AND OUTLOOK: This is the first study to focus on CAM use in patients followed for three AID in a french rural region. The current challenge is to enrich conventional medicine with CAM that are effective and safe through supervised programs to move towards an integrative medicine.

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.

Frequency and Significance of Hepatic Involvement in New-Onset Giant Cell Arteritis: A Study of 514 Patients.

Abnormalities of liver function in giant cell arteritis (GCA) have long been described1 and are present at the acute phase of the disease in 30% to 60% of cases.2-4 Hepatic involvement is mostly anicteric cholestasis (eg, elevated alkaline phosphatase [ALP] and gamma-glutamyl transferase [GGT]), and, more rarely, cytolytic hepatitis (eg, elevated aspartate aminotransferase [AST] and/or alanine aminotransferase [ALT]).

Use of high-plex data provides novel insights into the temporal artery processes of giant cell arteritis.

Objective: To identify the key coding genes underlying the biomarkers and pathways associated with giant cell arteritis (GCA), we performed an in situ spatial profiling of molecules involved in the temporal arteries of GCA patients and controls. Furthermore, we performed pharmacogenomic network analysis to identify potential treatment targets. Methods: Using human formalin-fixed paraffin-embedded temporal artery biopsy samples (GCA, n = 9; controls, n = 7), we performed a whole transcriptome analysis using the NanoString GeoMx Digital Spatial Profiler. In total, 59 regions of interest were selected in the intima, media, adventitia, and perivascular adipose tissue (PVAT). Differentially expressed genes (DEGs) (fold-change > 2 or < -2, p-adjusted < 0.01) were compared across each layer to build a spatial and pharmacogenomic network and to explore the pathophysiological mechanisms of GCA. Results: Most of the transcriptome (12,076 genes) was upregulated in GCA arteries, compared to control arteries. Among the screened genes, 282, 227, 40, and 5 DEGs were identified in the intima, media, adventitia, and PVAT, respectively. Genes involved in the immune process and vascular remodeling were upregulated within GCA temporal arteries but differed across the arterial layers. The immune-related functions and vascular remodeling were limited to the intima and media. Conclusion: This study is the first to perform an in situ spatial profiling characterization of the molecules involved in GCA. The pharmacogenomic network analysis identified potential target genes for approved and novel immunotherapies.

Unexplained hypothermia is associated with bacterial infection in the Emergency Department.

BACKGROUND: Early recognition and antibiotic therapy improve the prognosis of bacterial infections. Triage temperature in the Emergency department (ED) constitutes a diagnostic and prognostic marker of infection. The objective of this study was to assess the prevalence of community-acquired bacterial infections and the diagnostic ability of conventional biological markers in patients presenting to the ED with hypothermia. METHODS: We conducted a retrospective single-center study over a 1-year period before the COVID-19 pandemic. Consecutive adult patients admitted to the ED with hypothermia (body temperature < 36.0 °C) were eligible. Patients with evident cause of hypothermia and patients with viral infections were excluded. Diagnosis of infection was based on the presence of at least two among the three following pre-defined criteria: (i) the presence of a potential source of infection, (ii) microbiology data, and (iii) patient outcome under antibiotic therapy. The association between traditional biomarkers (white blood cells, lymphocytes, C-reactive protein [CRP], Neutrophil to Lymphocyte Count Ratio [NLCR]) and underlying bacterial infections was evaluated using a univariate and a multivariate (logistic regression) analysis. Receiver operating characteristic curves were built to determine threshold values yielding the best sensitivity and specificity for each biomarker. RESULTS: Of 490 patients admitted to the ED with hypothermia during the study period, 281 were excluded for circumstantial or viral origin, and 209 were finally studied (108 men; mean age: 73 ± 17 years). A bacterial infection was diagnosed in 59 patients (28%) and was mostly related to Gram-negative microorganisms (68%). The area under the curve (AUC) for the CRP level was 0.82 with a confidence interval (CI) ranging from 0.75 to 0.89. The AUC for the leukocyte, neutrophil and lymphocyte counts were 0.54 (CI: 0.45-0.64), 0.58 (CI: 0.48-0.68) and 0.74 (CI: 0.66-0.82), respectively. The AUC of NLCR and quick Sequential Organ Failure Assessment (qSOFA) reached 0.70 (CI: 0.61-0.79) and 0.61 (CI: 0.52-0.70), respectively. In the multivariate analysis, CRP ≥ 50 mg/L (OR: 9.39; 95% CI: 3.91-24.14; p < 0.01) and a NLCR ≥10 (OR: 2.73; 95% CI: 1.20-6.12; p = 0.02) were identified as independent variables associated with the diagnosis of underlying bacterial infection. CONCLUSION: Community-acquired bacterial infections represent one third of diagnoses in an unselected population presenting to the ED with unexplained hypothermia. CRP level and NLCR appear useful for the diagnosis of causative bacterial infection.

New-onset giant cell arteritis with lower ESR and CRP level carries a similar ischemic risk to other forms of the disease but has an excellent late prognosis: a case-control study.

INTRODUCTION: Biopsy-proven giant cell arteritis (GCA) occasionally presents without acute-phase reaction. In this setting, GCA may be initially overlooked and glucocorticoid treatment unduly delayed, potentially increasing ischemic risk. PATIENTS AND METHODS: From an inception cohort of patients with newly diagnosed, biopsy-verified GCA, we retrieved all cases without elevation of erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) level before starting glucocorticoid treatment. We compared the baseline features and outcomes of these patients and two additional patients recruited after GCA diagnosis with those of 42 randomly selected patients with high baseline ESR and CRP. RESULTS: Of 396 patients, 14 (3.5%) had lower baseline values of both ESR and CRP. Lower baseline ESR and CRP were associated with fewer American College of Rheumatology criteria met (p < 0.001, 95% CI - 1.1; - 0.9), and less jaw claudication (p = 0.06, 95% CI 0.8; 44.9), but similar rates of permanent blindness (p = 1.0). Patients with lower ESR and CRP also showed obvious differences regarding mean blood cell counts and mean hemoglobin level, but also less anti-cardiolipin antibody positivity (p = 0.04, 95% CI 0.8; ∞) and hepatic cholestasis (p = 0.03, 95% CI 1.0; 422). Patients with lower ESR and CRP had fewer GCA relapses (p = 0.03, 95% CI - 1.1; - 0.1), fewer glucocorticoid-induced complications (p = 0.01, 95% CI - 2.0; - 0.1), and successfully stopped glucocorticoids sooner than the other patients (18.3 months vs 34 months in average, p = 0.02, 95% CI - 27;- 0.9). CONCLUSION: Biopsy-proven GCA presenting with lower ESR and CRP is not an exceptional occurrence. It is clinically less typical but carries similar ischemic risk to other forms of the disease. Conversely, the late GCA prognosis of these patients is excellent.

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.

Giant cell arteritis-related stroke in a large inception cohort: A comparative study.

OBJECTIVE: Stroke caused by giant cell arteritis (GCA) is a rare but devastating condition and early recognition is of critical importance. The features of GCA-related stroke were compared with those of GCA without stroke and atherosclerosis-related or embolic stroke with the aim of more readily diagnosing GCA. METHODS: The study group consisted of 19 patients who experienced GCA-related strokes within an inception cohort (1982-2021) of GCA from the internal medicine department, and the control groups each consisted of 541 GCA patients without a stroke and 40 consecutive patients > 50 years of age with usual first ever stroke from the neurology department of a French university hospital. Clinical, laboratory, and imaging findings associated with GCA related-stroke were determined using logistic regression analyses. Early survival curves were estimated using the Kaplan-Meier method and compared using the log rank test. RESULTS: Amongst 560 patients included in the inception cohort, 19 (3.4%) developed GCA-related stroke. GCA-related stroke patients had more comorbid conditions (p = 0.03) and aortitis on imaging (p = 0.02), but less headache (p < 0.01) and scalp tenderness (p = 0.01). Multivariate logistic regression analysis showed that absence of involvement of the anterior circulation (OR = 0.1 - CI: 0.01-0.5), external carotid ultrasound (ECU) abnormalities (OR = 8.1 - CI: 1.3-73.9), and C-reactive protein (CRP) levels > 3 mg/dL (OR = 15.4 - CI: 1.9-197.1) were independently associated with GCA-related stroke. Early survival of GCA-related stroke patients was significantly decreased compared with control stroke patients (p = 0.02) and GCA patients without stroke (p < 0.001). CONCLUSIONS: The location of stroke and assessment of ECU results and CRP level could help improve the prognosis of GCA-related stroke by bringing this condition to the clinician's attention more quickly, thus shortening diagnostic delay.

Features and risk factors for new (secondary) permanent visual involvement in giant cell arteritis.

OBJECTIVES: New permanent visual loss (PVL) in treated patients with giant cell arteritis (GCA) is a rare but worrisome occurrence. In this study, we aimed to describe the frequency and main features of new PVL occurring after the beginning of glucocorticoid therapy in patients with newly diagnosed GCA. METHODS: We included in an inception cohort all consecutive patients newly diagnosed with GCA in the internal medicine department of a tertiary-care hospital between 1976 and May 2020. The study population comprised all the patients without bilateral PVL before treatment who were followed for at least one year. Only well-documented visual events that set after the initiation of glucocorticoid treatment were regarded as new PVL. RESULTS: Eleven out of 502 patients (2.2%) experienced a new PVL including 6 occurrences during the initial therapeutic phase and 5 during the tapering phase. Patients with new PVL during treatment had higher mean age, more often displayed temporal artery abnormalities on physical examination, and had higher mean platelet counts at GCA onset. There was a strong excess risk of contralateral recurrence during treatment in patients with unilateral loss at GCA onset compared with patients with uncomplicated GCA (10.5% vs 1.1%, OR=10.26, p<0.001). CONCLUSIONS: New PVL in treated GCA is a rare, but significant occurrence. Older patients and patients who already had unilateral PVL at diagnosis have higher risk of new ischaemic visual loss during treatment compared to the other patients. Close clinical, laboratory, and eye monitoring of these high-risk patients is of paramount importance.

Intraoperative findings during temporal artery biopsy: keys to optimise the diagnosis of giant cell arteritis.

The objective of this study was to describe the perioperative findings during temporal artery biopsy (TAB) and the characteristics associated with a diagnosis of giant cell arteritis (GCA). Perioperative findings were prospectively described by a single operator blinded to the clinical and laboratory characteristics of the patients on 40 consecutive TABs, of which 21 were positive (53%) for GCA. Patients with a TAB positive for GCA (TAB+) more frequently had abnormalities on palpation of the temporal artery than negative TAB (TAB-) patients (mainly pulse abolition (p=0.007), indurated artery (p=0.002), and painful artery (p=0.021)). The appearance of a big artery (p<0.001), a thickened artery (p<0.001), and an indurated artery at incision (p<0.001) was significantly associated with a positive TAB. A multivariate model identified a big artery, no local bleeding, and pain during artery traction as being associated with TAB positivity (sensitivity 71.4% and specificity 89.5%). The appearance of the temporal artery during TAB is important in predicting the positivity of the biopsy. Whether this should influence the optimal length of the TAB warrants a prospective study.

Diagnostic Value of 18F-FDG PET/CT vs. Chest-Abdomen-Pelvis CT Scan in Management of Patients with Fever of Unknown Origin, Inflammation of Unknown Origin or Episodic Fever of Unknown Origin: A Comparative Multicentre Prospective Study.

Fluorodesoxyglucose Positron Emission Tomography (PET/CT) has never been compared to Chest-Abdomen-Pelvis CT (CAPCT) in patients with a fever of unknown origin (FUO), inflammation of unknown origin (IUO) and episodic fever of unknown origin (EFUO) through a prospective and multicentre study. In this study, we investigated the diagnostic value of PET/CT compared to CAPCT in these patients. The trial was performed between 1 May 2008 through 28 February 2013 with 7 French University Hospital centres. Patients who fulfilled the FUO, IUO or EFUO criteria were included. Diagnostic orientation (DO), diagnostic contribution (DC) and time for diagnosis of both imaging resources were evaluated. One hundred and three patients were included with 35 FUO, 35 IUO and 33 EFUO patients. PET/CT showed both a higher DO (28.2% vs. 7.8%, p < 0.001) and DC (19.4% vs. 5.8%, p < 0.001) than CAPCT and reduced the time for diagnosis in patients (3.8 vs. 17.6 months, p = 0.02). Arthralgia (OR 4.90, p = 0.0012), DO of PET/CT (OR 4.09, p = 0.016), CRP > 30 mg/L (OR 3.70, p = 0.033), and chills (OR 3.06, p = 0.0248) were associated with the achievement of a diagnosis (Se: 89.1%, Sp: 56.8%). PET/CT both orients and contributes to diagnoses at a higher rate than CAPCT, especially in patients with FUO and IUO, and reduces the time for diagnosis.

Characteristics and Clinical Value of 18F-FDG PET/CT in the Management of Adult-Onset Still's Disease: 35 Cases.

While the diagnosis of adult-onset Still's disease (AOSD) involves the exclusion of differential diagnoses, the characteristics and value of 18F-Fluorodeoxyglucose (18F-FDG) Positron Emission Tomography coupled with CT (PET/CT) in the management of AOSD remain poorly known. Our retrospective study included patients from four centers, fulfilling Yamaguchi or Fautrel criteria, who underwent a PET/CT during an active AOSD. Thirty-five patients were included. At the time of PET/CT, the Yamaguchi criteria were met in 23 of 29 evaluable cases. PET/CT showed bone marrow (74.3%), lymph node (74.3%), and splenic (48.6%) FDG uptake. Despite arthralgia or arthritis in most patients, joints were rarely the sites of 18F-FDG accumulation. The spatial distribution of 18F-FDG uptake was nonspecific, and its intensity could be similar to malignant disease. Lymph node or bone marrow biopsy was performed after PET/CT in 20 patients (57.1%). The intensity of bone marrow; splenic and lymph node hypermetabolism appeared to be correlated with disease activity. Abnormal PET/CT in the cervical lymph nodes and age ≥ 60 years seemed to be predictive factors for monocyclic evolution. The clinical value of PET/CT is not in direct diagnosis; but as an aid in excluding differential diagnoses by searching for their scintigraphic features and guiding biopsy.

Abdominal symptoms during Sjogren's syndrome: a pilot study.

BACKGROUND: Abdominal symptoms in patients with primary Sjögren syndrome (pSS) are poorly documented. The objective of the study was to describe the abdominal symptoms of patients with pSS and to assess their association with characteristics of the disease. METHODS: One hundred and fifty patients with pSS were evaluated using a composite global symptom score for abdominal symptoms and their severity. Data concerning the clinical and biological characteristics of pSS and abdominal disorders were also collected. RESULTS: Of the patients with pSS, 95% suffered from abdominal symptoms (median global symptom score 7.5 ± 5.5 points out of 30). More than half of the patients experienced abdominal tension (68%), upper abdominal pain (54%), abdominal discomfort (58%) and/or constipation (54%). Regarding the pSS activity, in relation to European League Against Rheumatism (EULAR) Sjögren syndrome disease activity index score items, general and central nervous system involvement wereassociated with a high global symptom score. The EULAR Sjogren Syndrome Patient Reported Index (ESSPRI) symptom score was positively correlated with the global symptom score (p < 0.01). Multivariate analysis showed a significant association between a high global symptom score and SSA seronegativity, gastroparesis, and ESSPRI score (p < 0.01 for each). CONCLUSIONS: The majority of patients with pSS suffered abdominal symptoms. There is currently no therapeutic recommendation because of the lack of information on the underlying pathophysiological mechanisms. TRIAL REGISTRATION: NCT03157011 . Date of registration: July 17, 2017.

Giant cell arteritis or polymyalgia rheumatica after influenza vaccination: A study of 12 patients and a literature review.

INTRODUCTION: Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) are inflammatory rheumatic diseases common in people over the age of 50 years. Seasonal influenza vaccination (IV) is strongly recommended in this population, among whom it is considered to be effective and well tolerated. IV-induced GCA or PMR are thought to be exceptional. PATIENTS AND METHODS: We retrieved all post-IV cases from an inception cohort of patients with newly diagnosed GCA. We also included two patients with post-IV PMR and reviewed all published reports of post-IV GCA or PMR, with selection of cases demonstrating disease onset within 1 month following IV. We compared the results of HLA-DRB1 typing, performed in seven patients with post-IV GCA or PMR, with those of 11 GCA patients with familial aggregation and 16 randomly selected GCA patients without a reported trigger. RESULTS: Of 358 GCA recruited since 2002, 10 (2.8%) qualified for post-IV GCA, of whom two also showed familial aggregation. Thirty-two patients (19 with GCA and 13 with PMR) including our patients were reviewed; their mean age was 71.8 ± 7.4 years and the M/F ratio was 0.8. Six patients (19%) had a history of PMR. Patients with post-IV GCA/PMR had the DRB1*13:01 haplotype more frequently compared to those with familial GCA (5/7 vs. 2/11, p = 0.048) or with GCA without a known trigger (3/16, p = 0.026). Post-IV PMR generally appeared self-limited, whereas post-IV GCA often displayed a more protracted course (chronic relapsing disease in one-third of the patients). CONCLUSION: Post-IV onset of GCA/PMR is not an exceptional occurrence and may be part of the spectrum of the autoimmune syndrome induced by adjuvants (ASIA). IV can trigger GCA or PMR, especially in persons at higher spontaneous risk, such as those with a personal or familial history of GCA/PMR. Whether the presence of the DRB1*13:01 allele further increases the risk of post-IV GCA/PMR through a stronger vaccine-induced immune reaction deserves further investigation. Unlike PMR, GCA can be a serious complication of IV.

Abdominal symptoms during Sjogren's syndrome: a pilot study

Abstract Background: Abdominal symptoms in patients with primary Sjögren syndrome (pSS) are poorly documented. The objective of the study was to describe the abdominal symptoms of patients with pSS and to assess their association with characteristics of the disease. Methods: One hundred and fifty patients with pSS were evaluated using a composite global symptom score for abdominal symptoms and their severity. Data concerning the clinical and biological characteristics of pSS and abdominal disorders were also collected. Results: Of the patients with pSS, 95% suffered from abdominal symptoms (median global symptom score 7.5 ±5.5 points out of 30). More than half of the patients experienced abdominal tension (68%), upper abdominal pain (54%), abdominal discomfort (58%) and/or constipation (54%). Regarding the pSS activity, in relation to European League Against Rheumatism (EULAR) Sjogren syndrome disease activity index score items, general and central nervous system involvement wereassociated with a high global symptom score. The EULAR Sjogren Syndrome Patient Reported Index (ESSPRI) symptom score was positively correlated with the global symptom score (p < 0.01). Multivariate analysis showed a significant association between a high global symptom score and SSA seronegativity, gastroparesis, and ESSPRI score (p < 0.01 for each). Conclusions: The majority of patients with pSS suffered abdominal symptoms. There is currently no therapeutic recommendation because of the lack of information on the underlying pathophysiological mechanisms. Trial registration: NCT03157011. Date of registration: July 17, 2017.(AU)

Risk profiling for a refractory course of giant cell arteritis: The importance of age and body weight: "Risk profiling for GC resistance in GCA".

BACKGROUND: Giant cell arteritis (GCA) is a disease that relapses often, and some patients run a refractory course. Although prompt recognition of resistant GCA is a major issue, there is no well-recognized, baseline risk factor for poor response to glucocorticoid (GC) treatment. METHODS: We included all patients consecutively diagnosed with GCA and homogeneously treated since 1976 in a single department and regularly followed-up for at least 18 months. Using a set of customized criteria defining response to GCs, we separated patients into highly responsive, usually responsive, dependent on GCs, and resistant to GCs. We determined which of the baseline variables were associated with GC-resistance and conducted factor analyses of mixed data and decision tree analyses. We also determined whether being GC-resistant was associated with poorer tolerance to GCs and higher death rates. RESULTS: In all, 455 patients were followed for 93.4 ± 67.6 (standard deviation) months; 41 (9%) and 21 (4.6%) patients developed GC-dependent and GC-resistant disease, respectively. Factor analyses suggested an association between clinical pattern and degree of responsiveness to GCs; The decision tree analyses, built on an age at GCA onset 〈 66 years and body weight 〉 71 kg, delineated a high risk profile (44% of the patients who featured both characteristics were GC-resistant vs. less than 3% who featured neither, p < 0.001). Infections were more prevalent in the GC-resistant or GC-dependent patients, but without decreasing their survival. CONCLUSION: Extra-cranial, large-vessel GCA may be associated with prolonged GC requirements. A simple combination of age and body weight defined a subgroup of patients at high risk for developing GC resistance. Our findings need confirmation in prospective controlled studies.

Features and prognosis of giant cell arteritis in patients over 85 years of age: A case-control study.

BACKGROUND: We examined the initial features, course, and prognosis of giant cell arteritis (GCA) in patients ≥ 85 years of age (≥85 year) and compared them to those of younger patients. METHODS: The present retrospective study included all patients who were newly diagnosed with GCA in the Internal Departments of two French University Hospitals from 1976 or 1998 to 2017 and who were followed up for at least 6 months. Logistic regression analyses were conducted to identify baseline and prognostic characteristics associated with being ≥85 year. RESULTS: Of the 865 patients assessed in this study, 87 were ≥85 year. Compared to younger patients, patients ≥ 85 year had more comorbid conditions (odds ratio [OR] = 1.11-1.74, p < 0.01), less often exhibited polymyalgia rheumatica (PMR; OR = 0.33-0.96, p = 0.04), and more often developed permanent visual loss (OR = 1.29-3.81, p < 0.01). The older patients also showed less dependence on glucocorticoid (GC) medications (OR = 0.23-0.94, p = 0.04), had fewer relapses (OR = 0.31-0.87, p = 0.015), less often recovered from GCA (OR = 0.22-0.69, p < 0.01), and more often died during treatment (OR = 1.45-4.65, p = 0.001) compared to younger patients. Being ≥85 year was the only factor associated with an increased 1-year mortality (hazard ratio = 1.77-5.81, p = 0.0001) for the whole cohort. CONCLUSIONS: GCA in very elderly patients was characterized by a higher rate of severe ischemic complications and an increased risk for early death compared to younger patients. Thus, there is a need for the early diagnosis of GCA and close clinical monitoring in this unique population.

Serum and lymphocytic neurotrophins profiles in systemic lupus erythematosus: a case-control study.

BACKGROUND: Neurotrophins play a central role in the development and maintenance of the nervous system. However, neurotrophins can also modulate B and T cell proliferation and activation, especially via autocrine loops. We hypothesized that both serum and lymphocytic neurotrophin levels may be deregulated in systemic Lupus erythematosus (SLE) and may reflect clinical symptoms of the disease. METHODS: Neurotrophins in the serum (ELISA tests) and lymphocytes (flow cytometry) were measured in 26 SLE patients and 26 control subjects. Th1 (interferon-γ) and Th2 (IL-10) profiles and serum concentration of BAFF were assessed by ELISA in the SLE and control subjects. FINDINGS: We have demonstrated that both NGF and BDNF serum levels are higher in SLE patients than healthy controls (p=0.003 and p<0.001), independently of Th1 or Th2 profiles. Enhanced serum NT-3 levels (p=0.003) were only found in severe lupus flares (i.e. SLEDAI ≥ 10) and significantly correlated with complement activation (decreased CH 50, Γ=-0.28, p=0.03). Furthermore, there was a negative correlation between serum NGF levels and the number of circulating T regulatory cells (Γ=0.48, p=0.01). In circulating B cells, production of both NGF and BDNF was greater in SLE patients than in healthy controls. In particular, the number of NGF-secreting B cells correlated with decreased complement levels (p=0.05). One month after SLE flare treatment, BDNF levels decreased; in contrast, NGF and NT-3 levels remained unchanged. CONCLUSION: This study demonstrates that serum and B cell levels of both NGF and BDNF are increased in SLE, suggesting that the neurotrophin production pathway is deregulated in this disease. These results must be confirmed in a larger study with naive SLE patients, in order to avoid the potential confounding influence of prior immune-modulating treatments on neurotrophin levels.