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]).

Clinical features and long-term outcomes of patients with systemic polyarteritis nodosa diagnosed since 2005: Data from 196 patients.

BACKGROUND: The landscape of polyarteritis nodosa (PAN) has substantially changed during the last decades. Recent data regarding causes, characteristics, and prognosis of systemic PAN in the modern era are lacking. METHODS: This retrospective study included patients with systemic PAN referred to the French Vasculitis Study Group between 2005 and 2019. Characteristics, associated conditions and outcomes were collected, and predictors of relapse and death were analyzed. RESULTS: 196 patients were included. Main clinical symptoms were constitutional (84%), neurological (59%), skin (58%) and musculoskeletal (58%) manifestations. Secondary PAN accounted for 55 (28%) patients, including myelodysplastic syndrome (9%), solid cancer (7%), lymphoma (4%) and autoinflammatory diseases (4%). No patient had active HBV infection. All treated patients (98.5%) received glucocorticoids (GCs), alone (41%) or in combination with immunosuppressants (59%), with remission achieved in 90%. Relapses were independently associated with age >65 years (HR 1.85; 95% CI1.12-3.08), gastrointestinal involvement (1.95; 95% CI1.09-3.52) and skin necrotic lesions (HR 1.95; 95%CI 1.24-3.05). One-, 5- and 10-year overall survival rates were 93%, 87% and 81%, respectively. In multivariate analyses, age >65 years (HR 2.80; 95%CI 1.23-6.37), necrotic purpura (HR 4.16; 95%CI 1.62-10.70), acute kidney injury (HR 4.89; 95% 1.71-13.99) and secondary PAN (HR 2.98; 95%CI 1.29-6.85) were independently associated with mortality. CONCLUSION: Landscape of PAN has changed during the last decades, with the disappearance of HBV-PAN and the emergence of secondary PAN. Relapse rate remains high, especially in aged patients with gastrointestinal and cutaneous necrosis, as well as mortality.

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.

Immune-Mediated Diseases Following COVID-19 Vaccination: Report of a Teaching Hospital-Based Case-Series.

The occurrence and course of immune-mediated diseases (IMDs) following COVID-19 vaccination has been little explored so far. We retrieved, among adult patients hospitalized at the Internal Department of a French university hospital up to May 2022, all those who had developed, or relapsed to, an IMD less than 3 weeks following COVID-19 vaccination, without other triggers. Twenty-seven (24 new-onset) post-COVID-19 vaccine IMDs were recorded. They comprised giant cell arteritis or polymyalgia rheumatica (n = 16, HLA-DRB1*04 in 58% of 12 assessed GCA cases), immune-mediated necrotizing myositis or acute rhabdomyolysis, systemic vasculitis, immune thrombocytopenic purpura, rheumatoid arthritis, anti-synthetase syndrome, and adult-onset Still's disease. The causative vaccines were mRNA-based (20 cases) or viral vector-based (7 cases). The IMD typically occurred after the first vaccine dose, with an average delay of 8 (5 SD) days. The patients' mean age was 67 years, and 58% were women. The IMDs had protracted courses in all but three of the patients and typically required high-dose glucocorticoids, in combination with immunomodulators in 13 patients. One patient died of intractable rhabdomyolysis, whereas five suffered permanent damage from IMDs. Eleven patients with well-controlled IMDs completed their COVID-19 vaccination schedule, and two suffered mild IMD relapses. There is a risk of IMDs, notably GCA/PMR, and muscle disorders, following COVID-19 vaccination. Such adverse reactions typically occurred after the first dose, raising concern about subsequent COVID-19 vaccinations. However, early re-challenge in well-controlled IMDs appeared safe.

Weaning of maintenance immunosuppressive therapy in lupus nephritis (WIN-Lupus): results of a multicentre randomised controlled trial.

OBJECTIVES: Lupus nephritis (LN) is a frequent complication of systemic lupus erythematosus (SLE). Severe (proliferative) forms of LN are treated with induction immunosuppressive therapy (IST), followed by maintenance IST, to target remission and avoid relapses. The optimal duration of maintenance IST is unknown. The WIN-Lupus trial tested whether IST discontinuation after 2‒3 years was non-inferior to IST continuation for two more years in proliferative LN. METHODS: WIN-Lupus was an investigator-initiated multicentre randomised controlled trial. Patients receiving maintenance IST with azathioprine or mycophenolate mofetil for 2-3 years, and hydroxychloroquine, were randomised (1:1) into two groups: (1) IST continuation and (2) IST discontinuation. The primary endpoint was the relapse rate of proliferative LN at 24 months. Main secondary endpoints were the rate of severe SLE flares, survival without renal relapse or severe flare, adverse events. RESULTS: Between 2011 and 2016, 96 patients (out of 200 planned) were randomised in WIN-Lupus: IST continuation group (n=48), IST discontinuation group (n=48). Relapse of proliferative LN occurred in 5/40 (12.5%) patients with IST continuation and in 12/44 (27.3%) patients with IST discontinuation (difference 14.8% (95% CI -1.9 to 31.5)). Non-inferiority was not demonstrated for relapse rate; time to relapse did not differ between the groups. Severe SLE flares (renal or extrarenal) were less frequent in patients with IST continuation (5/40 vs 14/44 patients; p=0.035). Adverse events did not differ between the groups. CONCLUSIONS: Non-inferiority of maintenance IST discontinuation after 2‒3 years was not demonstrated for renal relapse. IST discontinuation was associated with a higher risk of severe SLE flares. TRIAL REGISTRATION NUMBER: NCT01284725.

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.

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.

Inflammatory myopathies associated with myelodysplastic syndromes: A French multicenter case control study and literature review.

OBJECTIVE: Patients with inflammatory myopathies (IM) are known to have an increased risk of developing malignancies. Autoimmune and inflammatory diseases occur in up to 25% of patients with myelodysplastic syndrome (MDS). This study aimed to describe the rare association between IM and MDS. METHODS: We report here the main characteristics, treatment, and outcome of 21 patients (11 national cases and 10 additional cases from a literature review) with IM associated to MDS. RESULTS: Median age of patients at IM diagnosis was 66 years (range 26 - 78). Diagnosis of the two conditions were concomitant in most patients (n=14/21) whereas MDS diagnosis preceded IM diagnosis in 5 patients. Different types of IM were observed but dermatomyositis was the most frequent (59%). Compared to IM without MDS (IM/MDS-), patients with MDS (IM/MDS+) were older (median 66 vs 55, p=0.3), more frequently male (sex ratio M/F 1.125 vs 0.41, p=0.14) and positive for anti-TIF1γ (24% vs 4%, p=0.0039). Antisynthetase syndrome was never observed among IM/MDS+ patients (0% vs 28%, p=0.01). MDS WHO type was not univocal, but the prognostic score was of low risk in almost all cases. IM was usually steroid sensitive (82% of patients) but often steroid dependent (56% of patients). Overall survival of IM patients with MDS was worse compared to patients with IM without MDS (p=0.0002). CONCLUSION: IM associated with MDS are mainly represented by dermatomyositis and/or anti-TIF1γ autoantibodies. Antisynthetase syndrome has not been described in association with MDS. Despite low-risk MDS, overall survival of IM patients with MDS is worse than IM patients without MDS.

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.

A Prospective International Study on Adherence to Treatment in 305 Patients With Flaring SLE: Assessment by Drug Levels and Self-Administered Questionnaires.

Nonadherence to treatment is a major cause of lupus flares. Hydroxychloroquine (HCQ), a major medication in systemic lupus erythematosus, has a long half-life and can be quantified by high-performance liquid chromatography. This international study evaluated nonadherence in 305 lupus patients with flares using drug levels (HCQ < 200 ng/ml or undetectable desethylchloroquine), and self-administered questionnaires (MASRI < 80%). Drug levels defined 18.4% of the patients as severely nonadherent. In multivariate analyses, younger age, nonuse of steroids, higher body mass index, and unemployment were associated with nonadherence by drug level. Questionnaires classified 23.4% of patients as nonadherent. Correlations between adherence measured by questionnaires, drug level, and physician assessment were moderate. Both methods probably measured two different patterns of nonadherence: self-administered questionnaires mostly captured relatively infrequently missed tablets, while drug levels identified severe nonadherence (i.e., interruption or erratic tablet intake). The frequency with which physicians miss nonadherence, together with underreporting by patients, suggests that therapeutic drug monitoring is useful in this setting. (Trial registration: ClinicalTrials.gov: NCT01509989.).

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.