scholarly journals CSF Findings in Acute NMDAR and LGI1 Antibody–Associated Autoimmune Encephalitis

2021 ◽  
Vol 8 (6) ◽  
pp. e1086
Author(s):  
Marc Dürr ◽  
Gunnar Nissen ◽  
Kurt-Wolfram Sühs ◽  
Philipp Schwenkenbecher ◽  
Christian Geis ◽  
...  

Background and ObjectivesCSF in antibody-defined autoimmune encephalitis (AE) subtypes shows subtype-dependent degrees of inflammation ranging from rare and often mild to frequent and often robust. AEs with NMDA receptor antibodies (NMDAR-E) and leucine-rich glioma-inactivated protein 1 antibodies (LGI1-E) represent opposite ends of this spectrum: NMDAR-E with typically frequent/robust and LGI1-E with rare/mild CSF inflammation. For a more in-depth analysis, we characterized CSF findings in acute, therapy-naive NMDAR-E and LGI1-E in a multicentric, retrospective, cross-sectional setting.MethodsEighty-two patients with NMDAR-E and 36 patients with LGI1-E from the GErman NEtwork for Research of AuToimmune Encephalitis (GENERATE) with lumbar puncture within 90 days of onset and before immunotherapy were included. CSF parameters comprised leukocytes, oligoclonal bands (OCBs), and CSF/serum ratios for albumin, immunoglobulin G (IgG), A (IgA), and M (IgM), the latter 3 converted to Z scores according to Reiber formulas. The MRZ reaction was tested in 14 patients with NMDAR-E and 6 patients with LGI1-E, respectively.ResultsCSF was abnormal in 94% of NMDAR-E but only in 36% of LGI1-E patients. Robust quantitative intrathecal immunoglobulin synthesis (IIS, IgG > IgM >> IgA) was characteristic for NMDAR-E, but absent in LGI-E. In NMDAR-E, CSF leukocytes were higher when IIS was present or more pronounced. In addition, in NMDAR-E, CSF leukocytes were lower and IIS occurred less often and if so to a lesser degree at older age. Patients with NMDAR-E with severe functional impairment more often had positive OCBs. In CSF obtained later than 3 weeks of onset, leukocytes were lower. In parallel, the correlation of leukocytes with IIS disappeared as IIS was partially independent of disease duration. The MRZ reaction was positive in 5 (36%) patients with NMDAR-E. All these associations were completely absent in LGI1-E. Here, younger patients showed more blood-CSF barrier dysfunction. In LGI1-E, but not in NMDAR-E, the blood-CSF barrier was more dysfunctional when CSF leukocytes were higher.DiscussionNMDAR-E and LGI-E differ in their typical extent of CSF inflammation. In addition, the patterns formed by the different inflammatory CSF parameters and their relationship with disease severity, age, and disease duration are subtype-characteristic. Moreover, signs for multiple sclerosis-like chronic inflammation are present in a subgroup of patients with NMDAR-E. These CSF patterns might be markers for the different immunopathogeneses of LGI1-E and NMDAR-E.

2021 ◽  
Vol 12 ◽  
Author(s):  
Tobias Zrzavy ◽  
Romana Höftberger ◽  
Isabella Wimmer ◽  
Thomas Berger ◽  
Paulus Rommer ◽  
...  

Autoimmune encephalitis (AIE) poses a diagnostic challenge due to its heterogeneous clinical presentation, which overlaps with various neurological and psychiatric diseases. During the diagnostic work-up, cerebrospinal fluid (CSF) is routinely obtained, allowing for differential diagnostics as well as for the determination of antibody subclasses and specificities. In this monocentric cohort study, we describe initial and serial CSF findings of 33 patients diagnosed with antibody-associated AIE (LGI1 (n=8), NMDA (n=7), CASPR2 (n=3), IgLON5 (n=3), AMPAR (n=1), GAD65/67 (n=4), Yo (n=3), Ma-1/2 (n=2), CV2 (n=2)). Routine CSF parameters of 12.1% of AIE patients were in normal ranges, while 60.6% showed elevated protein levels and 45.4% had intrathecal oligoclonal bands (OCBs). Repeated CSF analyses showed a trend towards normalization of initial pathological CSF findings, while relapses were more likely to be associated with increased cell counts and total protein levels. OCB status conversion in anti-NMDARE patients coincided with clinical improvement. In summary, we show that in routine CSF analysis at diagnosis, a considerable number of patients with AIE did not exhibit alteration in the CSF and therefore, diagnosis may be delayed if antibody testing is not performed. Moreover, OCB status in anti-NMDAR AIE patients could represent a potential prognostic biomarker, however further studies are necessary to validate these exploratory findings.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1684.1-1684
Author(s):  
H. H. Gezer ◽  
D. Erdem Gürsoy ◽  
S. Acer Kasman ◽  
N. Öz ◽  
M. T. Duruöz

Background:Although osteoporosis is an inherent comorbidity in inflammatory rheumatic disease and the risk of bone loss is high in patients with several rheumatic diseases, evidence is limited in psoriatic arthritis (PsA). One of the most prominent features in PsA is increased serum urate (SU) levels. Due to its antioxidant effects and protective role against osteoporosis, high SU levels are associated with increased bone mineral density (BMD) and reduced bone loss in the healthy population, and in patients with rheumatoid arthritis. However, whether this association is also present in patients with PsA has not been investigated.Objectives:The aim of this study was to evaluate PsA patients with respect to the presence of osteoporosis and its association with SU levels.Methods:This ongoing study included 86 patients (68 female, 18 male) who were diagnosed with PsA according to the CASPAR criteria and had indications for BMD testing according to the National Osteoporosis Foundation. Clinical characteristics including body mass index (BMI), pain VAS, patient global VAS, enthesitis, and tender and swollen joint counts were recorded. Evaluations included the PASI, PsAQoL, and HAQ. Disease activity was assessed using the DAPSA, BASDAI, and MDA. Osteoporosis was defined as a BMD T-score of -2.5 or less and osteopenia as a BMD T-score between -1 and -2.5 (WHO osteoporosis).Results:The mean age of the study group was 55.4 (SD:9.2) years and the mean disease duration was 84.5 (SD:91.6) months. Indicators of secondary osteoporosis were type-1 diabetes mellitus (1%), hyperthyroidism (2.3%), early menopause (<age 40) (8.1%), and chronic liver disease (9.3%). As for the steroid use, the rates of never, previous and current users were 33.7%, 20.9% and 22.1%, respectively. Osteoporosis was found in 9.3% and osteopenia in 33.7% of the patients. A history of vertebral compression fractures or any fracture was present in 20.9% of the patients, half of whom were in postmenopausal. BMD L1-L4T- and Z-scores were lower in female patients (p<0.05). DAPSA remission and MDA rates were 6% and 15%, respectively. Bone mineral density was similar across DAPSA disease activity categories (remission-low-moderate-high) (p>0.05). The frequency of osteoporosis did not differ significantly between patients with DAPSA remission and non-remission (p>0.05). The mean L1-L4T- and Z-scores, and BMD g/cm2were significantly higher in patients with MDA than those without MDA (p<0.05). The mean SU level was 5 (SD:1.3) mg/dl, and 18.6% of the patients had a SU level of 6 mg/dl or higher. There was no significant correlation between SU and BMD (p>0.05). BMI showed a weak correlation with femur total T-score (r=0.244). PASI showed weak inverse correlations with femur neck T-score (r=-0.286) and total femur T-score (r=-0.245). BMD variables showed no correlations with disease duration, acute phase reactants, BASDAI, PsAQoL, and cumulative steroid dose.Conclusion:Patients with PsA did not have an increased prevalence of low BMD despite fractures. Osteoporosis was associated with MDA and the severity of psoriasis, but not with DAPSA, SU level, functional impairment, and quality of life.References:[1]Gulati AM, Michelsen B, Diamantopoulos A, et al. Osteoporosis in psoriatic arthritis: a cross-sectional study of an outpatient clinic population. RMD Open 2018;4: e000631. DOI: 10.1136/rmdopen-2017-000631[2]Han W, Bai X, Wang N, Han L, Sun X, Chen X. Association between lumbar bone mineral density and serum uric acid in postmenopausal women: a cross-sectional study of healthy Chinese population. Arch Osteoporosis 2017; 12:50. DOI: 10.1007/s11657-017-0345-0.Disclosure of Interests:None declared


2021 ◽  
Vol 8 (5) ◽  
pp. e1030
Author(s):  
Miriam Schlüter ◽  
Eva Oswald ◽  
Stephan Winklmeier ◽  
Ingrid Meinl ◽  
Joachim Havla ◽  
...  

ObjectivesTo evaluate the long-term effects of natalizumab (NTZ) on different features of intrathecal immunoglobulin (Ig) synthesis in patients with multiple sclerosis (MS) and to quantify the expression of α4-integrin in stages of B-cell maturation.MethodsWe combined a cross-sectional (49 NTZ-treated MS patients, mean treatment duration 5.1 years, and 47 untreated MS patients) and a longitudinal study (33 patients with MS before and during NTZ, mean treatment duration: 4.8 years), analyzing paired serum and CSF samples for IgG, IgA, and IgM levels, reactivity against selected viruses (measles virus, rubella virus, and varicella zoster virus [MRZ] reaction), and oligoclonal bands (OCBs). Banding patterns before and after therapy were directly compared by isoelectric focusing in 1 patient. In addition, we determined the expression of α4-integrin by FACS analysis on blood-derived B-cell subsets (plasmablasts, memory B cells, and naive B cells) of healthy controls.ResultsIn serum, NTZ decreased IgM and IgG, but not IgA, levels. IgM hypogammaglobulinemia occurred in 28% of NTZ-treated patients. In CSF, NTZ treatment resulted in a strong reduction of intrathecally produced IgG and, to a lesser extent, IgA, whereas IgM indices [(Ig CSF/Serum)/(Albumin CSF/Serum)] remained largely unchanged. Reduction of the IgG index correlated with NTZ treatment duration, as did serum IgM and IgA levels. MRZ reaction was unchanged and OCB persisted. Direct comparison of OCB pattern before and after NTZ revealed the persistence of individual bands. α4-Integrin expression was highest on plasmablasts (CD19+CD38+CD27+).ConclusionOur data indicate that NTZ reduces short-lived plasmablasts in the CNS compartment but has little effect on locally persisting long-lived plasma cells.


2014 ◽  
pp. 73-77
Author(s):  
Van Chuong Nguyen ◽  
Thi Kim Anh Nguyen

Background: A Research glomerular filtration rate (GFR) of 61 patients with type 2 diabetes mellitus with renal scanning 99mTc-DTPA glomerular filtration rate at the hospital 175. Objective: (1) To study characteristics of imaging of renal function. (2) Understanding the relationship between GFR with blood sugar, HbA1c, blood pressure and albuminuria in patients with type 2 diabetes. Methods: Descriptive, prospective, cross-sectional study. Clinical examination, Clinical tests and 99mTc-DTPA GFR gamma - camera renography for patients. Result: GFR of the study group was 75,4 ± 22,3 ml/phut/1,73m2, the left kidney was 35,0 ± 13,0 is lower than the right kidney and 39,8 ± 11,9; p <0,01. There is no correlation between GFR with blood glucose and HbA1c, the risk of reduced GFR in hypertensive group associated is OR = 6,5 with p<0,01; albuminuria (+) is OR = 4,2 with p <0,01; and disease duration > 10 years is OR = 3,5 with p <0.01. Conclusion: GFR of the left kidneys is lower than the right kidney; correlation decreased GFR associated with hypertension, albuminuria and disease duration. Keywords: GFR, diabetes, albuminuria


2020 ◽  
Author(s):  
Yaqun Huang ◽  
Sha Yan ◽  
Hongfu Xie ◽  
Ben Wang ◽  
Zhixiang Zhao ◽  
...  

BACKGROUND Rosacea is a chronic inflammatory dermatosis with facial skin involved, leading to physical and emotional problems, which greatly affected quality of life (QoL) of patients. Dermatology Life Quality Index (DLQI) and willingness to pay (WTP) are well-established instruments assessing the health-related quality of life (HRQoL), while very few studies have been focused on this topic about rosacea in China. OBJECTIVE To investigate HRQoL in Chinese rosacea patients assessed by DLQI and WTP and investigate potential predictors for patients with HRQoL severely affected. METHODS This cross-sectional study was conducted on 973 patients with rosacea. Sociodemographic data, clinical features and DLQI were collected, and WTP was assessed by three standardized items. Multivariable logistic analysis was performed to investigate independent factors influencing QoL. RESULTS 921 questionnaires were accomplished by participants. The mean DLQI score was 11.6 (median 11). Patients were willing to pay an average of $1050.2 or € 896.2 (median $431.4 or € 368.1) for complete cure. 33.3% would like to pay more than 20% of their monthly income to achieve sustainable control. There were positive correlations between WTP with DLQI (P < .05). DLQI could be independently impacted by age (21-30 and 31-40, OR = 3.242 and 3.617, respectively), the occupational requirement of appearance (high, OR = 4.410), disease duration (< 2 years, OR = 1.582), oedema (OR = 1.844) and severity of flushing, burning, stinging and pruritus (severe, OR = 2.003, 1.981, 2.491, 2.249, respectively). There were no significant associations between WTP and most of the clinical factors. CONCLUSIONS The QoL was negatively affected and should not be ignored among rosacea patients in China. Patients aged 21-40y, having occupational requirement of appearance, with the disease duration less than 2 years, and suffering severe flushing and related symptoms were more likely to have severe or very severe limitation of QoL.


2021 ◽  
pp. 238008442110021
Author(s):  
O.O. Olatosi ◽  
A.A. Alade ◽  
T. Naicker ◽  
T. Busch ◽  
A. Oyapero ◽  
...  

Introduction: Malnutrition in children is one of the most prevalent global health challenges, and malnourished children have a higher risk of death from childhood diseases. Early childhood caries (ECC) is the most common chronic disease of childhood. Complications from ECC such as pain, loss of tooth/teeth, and infection can undermine a child’s nutrition and growth. Aim: This study aims to evaluate the severity of decay, missing, and filled tooth (dmft) by nutritional status using the z scores of the anthropometric measurements: height for age (HFA), weight for age (WFA), weight for height (WFH), and body mass index for age (BMIA) among children with ECC in Nigeria. Study Design: This is a cross-sectional study conducted in 5 local government areas (LGAs) in Lagos State, Nigeria. A multistage sampling technique was used. Results: A total of 273 cases of ECC were included in the analyses (mean age 4.19 ± 0.96 y). Overall, the mean dmft was 3.04 ± 2.28, and most (96%) were accounted for by untreated decay. The distribution of dmft within the different z score categories of BMIA (<–3 = severely wasted, –2 to –3 = wasted, –2 to +2 = normal, +2 to +3 = overweight and >+3 = obese) showed the highest dmft scores among the combined severely wasted and wasted groups, lowest among children with normal z scores, and intermediate in the overweight and obese groups. There was a significant negative correlation between BMIA z score, WFH z score, and dmft ( r = −0.181, P < 0.05 and r = −0.143, P < 0.05, respectively). However, the correlations between HFA z score, WFA z score, and dmft were positive but not significant ( r = 0.048, P = 0.44 and r = 0.022, P = 0.77, respectively). Conclusion: Our study showed an increased severity of dental caries among severely wasted or wasted children with ECC compared to those of normal or overweight. Knowledge Transfer Statement: The results from this study will raise awareness among clinicians and policy makers on the need for a primary prevention program for early childhood caries in countries with high burden of malnutrition and limited resources. Also, it will help draw the attention of clinicians to the caries status of malnourished children that can be managed to improve the nutritional outcomes.


2020 ◽  
Vol 12 ◽  
pp. 1759720X2098283
Author(s):  
María Lourdes Ladehesa-Pineda ◽  
Iván Arias de la Rosa ◽  
Clementina López Medina ◽  
María del Carmen Castro-Villegas ◽  
María del Carmen Ábalos-Aguilera ◽  
...  

Aims: To evaluate the association of estimated cardiovascular (CV) risk and subclinical atherosclerosis with radiographic structural damage in patients with axial spondyloarthritis (axSpA). Methods: Cross-sectional study including 114 patients axSpA from the SpA registry of Córdoba (CASTRO) and 132 age- and sex-matched healthy controls (HCs). Disease activity and the presence of traditional CV risk factors were recorded. The presence of atherosclerotic plaques and carotid intima media thickness (cIMT) were evaluated through carotid ultrasound and the SCORE index was calculated. Radiographic damage was measured though modified Stoke Ankylosing Spondylitis Spinal Score (mSASSS). The association between mSASSS and SCORE was tested using generalized linear models (GLM), and an age-adjusted cluster analysis was performed to identify different phenotypes dependent on the subclinical CV risk. Results: Increased traditional CV risk factors, SCORE, and the presence of carotid plaques were found in axSpA patients compared with HCs. The presence of atherosclerotic plaques and SCORE were associated with radiographic structural damage. The GLM showed that the total mSASSS was associated independently with the SCORE [β coefficient 0.24; 95% confidence interval (CI) 0.10–0.38] adjusted for disease duration, age, tobacco, C-reactive protein, and non-steroidal anti-inflammatory drugs (NSAID) intake. Hard cluster analysis identified two phenotypes of patients. Patients from cluster 1, characterized by the presence of plaques and increased cIMT, had a higher prevalence of CV risk factors and SCORE, and more structural damage than cluster two patients. Conclusion: Radiographic structural damage is associated closely with increased estimated CV risk: higher SCORE levels in axSpA patients were found to be associated independently with mSASSS after adjusting for age, disease duration, CRP, tobacco and NSAID intake.


Author(s):  
Marga Decraene ◽  
Vera Verbestel ◽  
Greet Cardon ◽  
Violeta Iotova ◽  
Berthold Koletzko ◽  
...  

In 2019, the World Health Organization (WHO) published 24 h movement behavior guidelines for preschoolers with recommendations for physical activity (PA), screen time (ST), and sleep. The present study investigated the proportion of preschoolers complying with these guidelines (on a total week, weekdays and weekend days), and the associations with adiposity. This cross-sectional study included 2468 preschoolers (mean age: 4.75 years; 41.9% boys) from six European countries. The associations were investigated in the total sample and in girls and boys separately. PA was objectively assessed by step counts/day. Parent-reported questionnaires provided ST and sleep duration data. Generalized estimating equations were used to analyze the association between guideline compliance and adiposity indicators, i.e., body mass index (BMI) z-score and waist to height ratio (WHR). Only 10.1% of the preschoolers complied with the 24 h movement behavior guidelines, 69.2% with the sleep duration guideline, 39.8% with the ST guideline and 32.7% with the PA guideline. No association was found between guideline compliance with all three movement behaviors and adiposity. However, associations were found for isolated weekday screen time (BMI z-scores and WHR: p = 0.04) and weekend day sleep duration (BMI z-scores and WHR: p = 0.03) guideline compliance with both lower adiposity indicators. The latter association for sleep duration was also found in girls separately (BMI z-scores: p = 0.02; WHR: p = 0.03), but not in boys. Longitudinal studies, including intervention studies, are needed to increase preschoolers’ guideline compliance and to gain more insight into the manifestation of adiposity in children and its association with 24 h movement behaviors from a young age onwards.


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1017.2-1018
Author(s):  
N. Kelly ◽  
E. Hawkins ◽  
H. O’leary ◽  
K. Quinn ◽  
G. Murphy ◽  
...  

Background:Rheumatoid arthritis (RA) is a chronic, autoimmune inflammatory condition that affects 0.5% of the adult population worldwide (1). Sedentary behavior (SB) is any waking behavior characterized by an energy expenditure of ≤1.5 METs (metabolic equivalent) and a sitting or reclining posture, e.g. computer use (2) and has a negative impact on health in the RA population (3). Sleep is an important health behavior, but sleep quality is an issue for people living with RA (4, 5). Poor sleep quality is associated with low levels of physical activity in RA (4) however the association between SB and sleep in people who have RA has not been examined previously.Objectives:The aim of this study was to investigate the relationship between SB and sleep in people who have RA.Methods:A cross-sectional study was conducted. Patients were recruited from rheumatology clinics in a large acute public hospital serving a mix of urban and rural populations. Inclusion criteria were diagnosis of RA by a rheumatologist according to the American College of Rheumatology criteria age ≥ 18 and ≤ 80 years; ability to mobilize independently or aided by a stick; and to understand written and spoken English. Demographic data on age, gender, disease duration and medication were recorded. Pain and fatigue were measured by the Visual Analogue Scale (VAS), anxiety and depression were assessed using the Hospital Anxiety and Depression Scale (HADS), and sleep quality was assessed using the Pittsburgh Sleep Quality Index. SB was measured using the ActivPAL4™ activity monitor, over a 7-day wear period. Descriptive statistics were calculated to describe participant characteristics. Relationships between clinical characteristics and SB were examined using Pearson’s correlation coefficients and regression analyses.Results:N=76 participants enrolled in the study with valid data provided by N=72 participants. Mean age of participants was 61.5years (SD10.6) and the majority 63% (n = 47) were female. Participant mean disease duration was 17.8years (SD10.9). Mean SB time was 533.7 (SD100.1) minutes (8.9 hours per day/59.9% of waking hours). Mean sleep quality score was 7.2 (SD5.0) (Table 1). Correlation analysis and regression analysis found no significant correlation between sleep quality and SB variables. Regression analysis demonstrated positive statistical associations for SB time and body mass index (p-value=0.03846, R2 = 0.05143), SB time and pain VAS (p-value=0.009261, R2 = 0.07987), SB time and HADS (p-value = 0.009721, R2 = 0.08097) and SB time and HADSD (p-value = 0.01932, R2 = 0.0643).Conclusion:We found high levels of sedentary behavior and poor sleep quality in people who have RA, however no statistically significant relationship was found in this study. Future research should further explore the complex associations between sedentary behavior and sleep quality in people who have RA.References:[1]Carmona L, et al. Rheumatoid arthritis. Best Pract Res Clin Rheumatol 2010;24:733–745.[2]Anon. Letter to the editor: standardized use of the terms “sedentary” and “sedentary behaviours”. Appl Physiol Nutr Metab = Physiol Appl Nutr Metab 2012;37:540–542.[3]Fenton, S.A.M. et al. Sedentary behaviour is associated with increased long-term cardiovascular risk in patients with rheumatoid arthritis independently of moderate-to-vigorous physical activity. BMC Musculoskelet Disord 18, 131 (2017).[4]McKenna S, et al. Sleep and physical activity: a cross-sectional objective profile of people with rheumatoid arthritis. Rheumatol Int. 2018 May;38(5):845-853.[5]Grabovac, I., et al. 2018. Sleep quality in patients with rheumatoid arthritis and associations with pain, disability, disease duration, and activity. Journal of clinical medicine, 7(10)336.Table 1.Sleep quality in people who have RASleep variableBed Time N(%) before 10pm13(18%) 10pm-12pm43 (60%) after 12pm16 (22%)Hours Sleep mean(SD)6.56 (1.54)Fall Asleep minutes mean(SD)33.3(27.7)Night Waking N(%)45(63%)Self-Rate Sleep mean(SD)2.74 (0.90)Hours Sleep mean(SD)6.56 (1.54)Disclosure of Interests:None declared


2020 ◽  
Vol 105 (7) ◽  
pp. 2430-2441
Author(s):  
Åse Bjorvatn Sævik ◽  
Anna-Karin Åkerman ◽  
Paal Methlie ◽  
Marcus Quinkler ◽  
Anders Palmstrøm Jørgensen ◽  
...  

Abstract Context Contrary to current dogma, growing evidence suggests that some patients with autoimmune Addison disease (AAD) produce corticosteroids even years after diagnosis. Objective To determine frequencies and clinical features of residual corticosteroid production in patients with AAD. Design Two-staged, cross-sectional clinical study in 17 centers (Norway, Sweden, and Germany). Residual glucocorticoid (GC) production was defined as quantifiable serum cortisol and 11-deoxycortisol and residual mineralocorticoid (MC) production as quantifiable serum aldosterone and corticosterone after &gt; 18 hours of medication fasting. Corticosteroids were analyzed by liquid chromatography–tandem mass spectrometry. Clinical variables included frequency of adrenal crises and quality of life. Peak cortisol response was evaluated by a standard 250 µg cosyntropin test. Results Fifty-eight (30.2%) of 192 patients had residual GC production, more common in men (n = 33; P &lt; 0.002) and in shorter disease duration (median 6 [0-44] vs 13 [0-53] years; P &lt; 0.001). Residual MC production was found in 26 (13.5%) patients and associated with shorter disease duration (median 5.5 [0.5-26.0] vs 13 [0-53] years; P &lt; 0.004), lower fludrocortisone replacement dosage (median 0.075 [0.050-0.120] vs 0.100 [0.028-0.300] mg; P &lt; 0.005), and higher plasma renin concentration (median 179 [22-915] vs 47.5 [0.6-658.0] mU/L; P &lt; 0.001). There was no significant association between residual production and frequency of adrenal crises or quality of life. None had a normal cosyntropin response, but peak cortisol strongly correlated with unstimulated cortisol (r = 0.989; P &lt; 0.001) and plasma adrenocorticotropic hormone (ACTH; r = –0.487; P &lt; 0.001). Conclusion In established AAD, one-third of the patients still produce GCs even decades after diagnosis. Residual production is more common in men and in patients with shorter disease duration but is not associated with adrenal crises or quality of life.


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