scholarly journals AB0623 RATE AND PREDICTIVE FACTORS ASSOCIATED WITH SUSTAINED REMISSION IN IDIOPATHIC INFLAMMATORY MYOSITIS

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1607.1-1607
Author(s):  
T. Vojinovic ◽  
X. Gjoni ◽  
M. Fredi ◽  
F. Franceschini ◽  
I. Cavazzana

Background:Idiopathic inflammatory myositis (IIM) is a group of heterogeneous connective tissue diseases, characterised by chronic muscle inflammation, myositis-specific or myositis-associated autoantibodies and different extra-muscular features. Achieveing low disease activity or remission in patients with IIM has proven to be difficult due to the wide clinical spectrum of the different IIM types.Objectives:To retrospectively assess any predictive factors for sustained remission in IIM patientsMethods:We retrospectively analyzed data taken from medical charts, which included age at disease onset, gender, laboratory data as well as clinical features present at onset, organ involvement and treatment history. A total of 151 adult patients with IIM followed-up for > 1 year were retrospectively enrolled. Remission was defined as no clinical and laboratory evidence of disease activity persistent for more than 6 months during follow-up, while undergoing myositis therapy or under no medication. The remission of cutaneous involvement was defined as no current activity of skin rash, absence of Gottron’s papules as well as heliotrope rash and erythema, whereas the remission of pulmonary involvement was considered as no requirement for intensification of immunosuppressive therapy during follow-up. Likewise, absence of muscle weakness or hyposthenia was taken into account for evaluating muscle involvement. Moreover, the clinical features were accompanied by normalization of myogenic enzymes such as creatine kinase (CK) and lactate dehydrogenase levels.Results:Among all 151 patients, 89 (58.9%) patients achieved sustained remission. By univariate analysis, overlap myositis (7.9% vs 27.4%; p=0.003; OR:0.22), cancer-associated myositis (CAM) (7.8% vs 19.35%; p=0.046; OR:0.3), as well as the presence of anti Ku (3.37% vs 12.9%; p=0.05; OR:0.23) and anti TIF-1 gamma (1.1% vs 8%; p=0.043; OR:0.13) antibodies and polyarthritis (11.2% vs 24.19%; p=0.045; OR: 0.397) at onset were significantly associated with active IIM, not achieving remission.Out of 89 patients in remission, 79 (88.8%) achieved long-term sustained remission, lasting at least 2 years, whereas 10 patients (11.2%) showed relapse. Most of relapsed patients showed a relapse/remitting disease, with no evident trigger for the relapse. We divided our cohort of 89 patients in remission in 2 subsets: 83 patients in remission undergoing therapy (93.3%) and 6 patients in drug-free remission (6.7%). No significant differences were found between two groups, except for younger age at onset (p=0.0002) found in patients achieving drug-free remission.Conclusion:Sustained remission occurs in about one half of patients with IIM. The presence of anti Ku and anti TIF-1 gamma antibodies as well as polyarthritis at onset lowers the chance of achieving sustained remission. Younger age at diagnosis has proved to predict drug-free long-lasting remission.Disclosure of Interests:None declared

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 388.1-389
Author(s):  
P. Breillat ◽  
K. Mariampillai ◽  
P. Martins ◽  
P. Legendre ◽  
B. Dunogue ◽  
...  

Background:Autoantibodies permit to classify and subgroup connective tissue diseases (CTD) in homogeneous groups of patients in terms of phenotype and prognosis. Anti PM-Scl antibodies have been associated with different CTD categories such as: idiopathic inflammatory myositis (IIM), systemic sclerosis (SSc), Sjögren’s syndrome (SjS), systemic lupus erythematosus (SLE), mixed connective tissue disease (MCTD) or undifferentiated connective tissue disease (UCTD).Objectives:To determine clinical spectrum of anti-PM-Scl associated disease and if it an homogenous condition.Methods:This multicentric (four hospitals) observational and retrospective study included all consecutive patients with positive testing for anti PM-Scl antibodies on immunoblot assay and connective tissue disease (2011 -2020). Epidemiological, biological, clinical and radiological data were collected in standard form as well as patient’s outcome.Results:One hundred twenty height patients (female n=96;75%) were included. Median [quartiles] age at diagnosis was 50 [18;84] (IQR) and follow-up duration of 7 [3.75-12] years. Seventy-six (59.3%) patients were simple anti-Pm-Scl positive, and 40.7% were associated with other antibodies: anti-SSA/Ro52 (n=13; 10.92%), SSc associated antibodies (n=21; 16.4%), anti-dsDNA for (n=9; 7%), anti-RNP (n=6; 4.7%) and anti-CCP antibodies (n=6; 4.7%). Most patients had cutaneous involvement (n=106; 83%) with skin thickening (n=47; 36%), mechanics hands (n= 28; 22%), calcinosis (n=26; 20.3%) and subcutaneous edema (n=20; 15.62%). Vascular involvement was frequent with Raynaud phenomenon (n= 89; 69%), telangiectasia (n=36; 28%), skin ulcers (n=27; 21%), pulmonary hypertension (n=8/120; 6.7%) and scleroderma renal crisis (n=2; 1.5%). A majority of patients also displayed an interstitial lung disease (ILD) (n=83; 65.8%); nonspecific interstitial pneumonia (92.7%) and/or organizing pneumonia (25.3%). ILD was characterized by a subacute onset in 37/81 (45.7%); median [quartiles] forced vital capacity (FVC) and total lung capacity (TLC) at diagnosis of 88% [73-105] and 79.5% [68.5-101] respectively. Sixty patients (47%) had muscular sign including myalgia (47%), elevated CPK (n=51; 40%) and muscular weakness (Medical Research Council score <4) (n=19/124;15%). Finally, fifty-three (41.7%) had gastroesophageal reflux. Thirty-nine patients (30.4%) experienced at least one muscular or ILD relapse and 6 (4.84%) died during follow-up (2 breast cancer, 1 pneumonia, 3 unknown etiology). Concerning patients’ prognosis, relapses were associated with skeletal (n=29, 74.4% vs n=32, 35.96%, p < 0.001) or cardiac muscle involvement (n=7, 18.4% vs n=2, 2.5%, p=0.007), and subacute ILD (n=19, 65.5% vs n=18, 34.62%, p= 0.05) with organized pneumonia pattern (n=11, 32.3% vs n=10, 13.9%, p=0.05). Strikingly, ILD occurred mainly in men (90.6% vs 57.2%, p < 0.001) and was associated with anti-Scl-70 positivity (n=14, 16.67% vs 0%, p= 0.01). Muscle involvement was associated arthralgia (n=46, 76.67% vs n=34, 50.75%, p=0.005), respiratory signs at diagnosis: dyspnea NYHA ≥3 (n=46, 75.41% vs n=30, 44.78%, p < 0.001), sub-acute ILD (n=24, 61.54% vs n=13, 30.95%, p=0.0111) with lower FVC (73% [64;88] vs 98 [76;105], p < 0.001). Ulcers were associated with Anti-Scl70 positivity (n=9, 33.33% vs n=5, 4.95%, p < 0.001), Raynaud phenomenon (n=27, 100% vs n=62, 61.39%, p < 0.001), digestive involvement (n=20, 74.07% vs n=34, 33.66%, p < 0.001), ILD with chronic onset (n=15, 78.95% vs n=29, 46.77%, p=0.027) and increased incidence of deaths (n=4, 16% vs n=2, 2.02%, p= 0.01).Conclusion:Conducted on the largest cohort of Anti-PM-Scl patients, this study highlights two main phenotypes that determine different outcome and prognosis. One was associated with muscular disease and subacute onset ILD with more frequent relapses. The second with a vascular phenotype associated with chronic ILD, digestive involvement, chronic evolution and increased incidence of death. This could lead to a reclassification of PM-Scl associated auto immune diseases.Disclosure of Interests:None declared


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 1099.2-1099
Author(s):  
R. Fakhfakh ◽  
N. El Amri ◽  
K. Baccouche ◽  
H. Zeglaoui ◽  
E. Bouajina

Background:Sustained remission (SR) is an ultimate treatment goal in the management of patients with rheumatoid arthritis (RA) (1) and is associated with better RA prognosis, reflected by the quality of life, physical function and radiographic progression (2).Objectives:To investigate the prevalence and predictors of SR in RA patients.Methods:A longitudinal prospective study of patients with RA. At the inclusion, the patients were in remission DAS28 ESR≤ 2.6 for at least 6 months. A B-mode and power doppler (PD) ultrasound of 42 joints and 20 tendons was performed. Synovial hypertrophy (SH) and tenosynovitis in B-mode and PD were defined and scored from 0 to 3 using the OMERACT. The CDAI, SDAI, Boolean remission criteria, the health assessment questionnaire (HAQ) and the radiological Sharp score were calculated. Then, the DAS28 erythrocyte sedimentation rate (ESR) was evaluated at 6 and 12 months. SR was defined as the persistence of a DAS28 ESR≤2.6 at 6 or 12 months without any change in RA therapy during the follow-up. Unstable remission (UR) was defined either as DAS28 ESR > 2.6 at 6 or 12 months or an increase in RA therapy because of a relapse during the follow-up.Results:At baseline, thirty-seven patients were included. At 6 and 12 months, 28 and 24 patients completed follow-up, respectively. In decreasing order, Boolean remission (92.2%), DAS28ESRremission (85.7%), SDAI remission (85%) and CDAI remission (83.3%) achieved SR at 6 months. At 12 months, SR was found in 100% in Boolean remission, 87.5% in SDAI remission, 86.7% in CDAI remission and in 79.7% in DAS28 ESR remission. At 6 months, only the ESR (17mm/1h in SR versus 32 mm/1h in UR, p=0.04) was associated with SR. The disease duration, remission duration, swollen and tender joints, DAS28ESR, HAQ, rheumatoid factor, radiological Sharp score and ultrasound parameters weren’t associated with SR. At 12 months, the squeeze test (15% in SR vs 80% in UR, P=0.01), the ESR (15 mm/1h in SR versus 30 mm/1h in UR, p=0.03), the Boolean remission (61.1% in SR versus 0% in UR, p=0.04) and the DAS28ESR (mean: 1.8 in SR versus 2.5 in UR, P=0.01) were associated with SR. However, no association was found with radiological Sharp score and ultrasound parameters. On multivariate analysis, the ESR (OR=1.13, CI95%=1.01-1.2, p=0.03) and the Squeeze test (OR=21.3, CI95%=1.7-263, p=0.01) were predictors of SR, at 12 months.Conclusion:At 6 and 12 months, 79.7%-85.7% of patients in DAS28 ESR remission achieved sustained remission, respectively. Boolean and DAS28 ESR remission were associated with SR. Unlike DAS28 ESR, Boolean remission seems to reflect more the SR. The squeeze test and the ESR were predictors’ factor. However, the radiological and the ultrasound parameters didn’t show any association.References:[1]Ajeganova S, Huizinga T. Sustained remission in rheumatoid arthritis: latest evidence and clinical considerations. Ther Adv Musculoskelet Dis. 2017;9(10):249-62.[2]Xie W, Li J, Zhang X, Sun X, Zhang Z. Sustained clinical remission of rheumatoid arthritis and its predictive factors in an unselected adult Chinese population from 2009 to 2018. Int J Rheum Dis. 2019;22(9):1670-8.Disclosure of Interests:None declared


Rheumatology ◽  
2019 ◽  
Author(s):  
Eri Watanabe ◽  
Takahisa Gono ◽  
Masataka Kuwana ◽  
Chihiro Terai

Abstract Objective The aim of this study was to clarify predictive factors for sustained remission in adult patients with PM/DM, particularly focusing on stratification by myositis-specific autoantibodies (MSAs). Methods A total of 162 adult patients with PM/DM who were followed up for >1 year after diagnosis were retrospectively enrolled. MSAs were evaluated comprehensively in 102 patients whose sera were available. Sustained remission was defined as no evidence of disease activity (active skin rash, active myositis or active interstitial lung disease) for longer than a 6-month continuous period while undergoing myositis therapy or no medication. Clinical data were reviewed in patients’ medical charts Results The sustained remission rate for all patients was 58% during the median follow-up period at 4 years. With regard to MSAs, the achievement rate of sustained remission among MSA-negative patients was significantly higher than that for patients with anti-aminoacyl-tRNA synthetase (P = 0.004), anti-melanoma differentiation-associated gene 5 (P = 0.037) or anti-transcriptional intermediary factor 1-γ (P = 0.013) antibodies. MSA-negative status (odds ratio 5.84, P = 0.009) and absence of severe muscle weakness requiring assistance at diagnosis (odds ratio 43.6, P < 0.001) were independent factors associated with sustained remission in multivariate analysis. Cumulative remission rates were significantly higher (P < 0.001) in patients with both the MSA-negative status and absence of severe muscle weakness at diagnosis than the others. Conclusion MSA-negative status and the absence of severe muscle weakness requiring assistance at diagnosis are independent predictive factors for sustained remission in adult PM/DM patients.


Author(s):  
Claire T Deakin ◽  
Charalampia Papadopoulou ◽  
Liza J McCann ◽  
Neil Martin ◽  
Muthana Al-Obaidi ◽  
...  

Abstract Objectives Uncertainty around clinical heterogeneity and outcomes for patients with JDM represents a major burden of disease and a challenge for clinical management. We sought to identify novel classes of patients having similar temporal patterns in disease activity and relate them to baseline clinical features. Methods Data were obtained for n = 519 patients, including baseline demographic and clinical features, baseline and follow-up records of physician’s global assessment of disease (PGA), and skin disease activity (modified DAS). Growth mixture models (GMMs) were fitted to identify classes of patients with similar trajectories of these variables. Baseline predictors of class membership were identified using Lasso regression. Results GMM analysis of PGA identified two classes of patients. Patients in class 1 (89%) tended to improve, while patients in class 2 (11%) had more persistent disease. Lasso regression identified abnormal respiration, lipodystrophy and time since diagnosis as baseline predictors of class 2 membership, with estimated odds ratios, controlling for the other two variables, of 1.91 for presence of abnormal respiration, 1.92 for lipodystrophy and 1.32 for time since diagnosis. GMM analysis of modified DAS identified three classes of patients. Patients in classes 1 (16%) and 2 (12%) had higher levels of modified DAS at diagnosis that improved or remained high, respectively. Patients in class 3 (72%) began with lower DAS levels that improved more quickly. Higher proportions of patients in PGA class 2 were in DAS class 2 (19%, compared with 16 and 10%). Conclusion GMM analysis identified novel JDM phenotypes based on longitudinal PGA and modified DAS.


2019 ◽  
Vol 6 (2) ◽  
pp. 236
Author(s):  
Srujith C. H. ◽  
Kavitha Mohanasundaram ◽  
Jagadeesan M. ◽  
Halleys Kumar E. ◽  
Kannan R. ◽  
...  

Background: Idiopathic inflammatory myopathies (IIMs) are a group of chronic systemic autoimmune diseases characterized by proximal muscle weakness and elevated muscle enzymes. Aim and Objective was to analyze the demographic profile of patients with idiopathic inflammatory myopathies (IIM).Methods: This was a cross sectional observational study conducted over a period of two years (2016-2018). After obtaining institutional ethical committee clearance, informed consent from patients. 16 patients who fulfilled the criteria were included in the study. The demographic and the clinical data were analysed.Results: The mean age was 47.3±11.2 years. The study showed female predominance. ANA was positive in 11(68.7%) patients. Among the 16 patients, 5 (31.25%) had polymyositis and 11 (68.7%) had dermatomyositis. The median enzymes levels were creatinine kinase 1134 U/L, lactic dehydrogenase 477U/L, ALT (alanine aminotransferase) 154 IU/L, AST (aspartate aminotransferase) 236IU/L. Raynaud's phenomenon was seen in 37.5%. In our study, 31.25% had hypothyroidism and 6.25% had diabetic mellitus. On follow up 37.5% developed interstitial lung disease (ILD) and 18.75% were found to have malignancy.Conclusions: Steroids and immunomodulators are the mainstay of treatment in patients with idiopathic inflammatory myositis. All our patients improved with steroids. It is important to evaluate these patients during early stages and follow up to prevent complications.


2021 ◽  
Vol 8 (2) ◽  
pp. e960
Author(s):  
Derek McFaul ◽  
Nektar N. Hakopian ◽  
Jessica B. Smith ◽  
Allen Scott Nielsen ◽  
Annette Langer-Gould

ObjectiveTo determine whether MS disease-modifying therapies (DMTs) can be safely discontinued in patients aged 50 years or older with suspected benign/burnt-out MS and to define criteria to identify such patients.MethodsWe conducted a retrospective cohort study of 136 patients with suspected benign/burnt-out MS who discontinued DMTs from the electronic health record (EHR) at Kaiser Permanente Southern California.ResultsThe majority discontinued an injectable DMT (n = 131, 96%). At the time of DMT discontinuation, mean and SD for age was 60.6 (6.2) years, disease duration 19.5 (10.7) years, and time since last relapse 11.0 (7.2) years. After a mean duration of follow-up of 5.0 years post-DMT discontinuation, 5 (3.7%) patients had a relapse, 2 (1.5%) had mild residual deficits, and 3 (2.2%) had asymptomatic MRI disease activity. Patients with MS disease activity following DMT discontinuation were younger (median = 53.6 years) than those who remained disease activity free. Fifty patients (36.8%) had only 1 lifetime relapse, of whom 1 relapsed post-DMT discontinuation. Sixty (56.6%) of 106 patients with spinal cord MRIs before discontinuation showed demyelinating lesions.ConclusionsDMT discontinuation in older patients with suspected benign/burnt-out MS appears safe. Our findings suggest that MRI evidence of spinal cord involvement does not preclude the possibility of benign/burnt-out MS, and for those with 2 or more lifetime relapses, a benign/burn-out classification is best reserved for those aged 55 years and older. Future studies to determine whether DMT discontinuation is safe at a younger age in patients with a single lifetime relapse are needed.Classification of EvidenceThe study provides Class IV evidence that DMTs can be safely discontinued in older patients with suspected benign/burnt-out MS.


Author(s):  
Tamara Vojinovic ◽  
Ilaria Cavazzana ◽  
Paolo Ceruti ◽  
Micaela Fredi ◽  
Denise Modina ◽  
...  

Abstract Interstitial lung disease (ILD) represents one of the most severe extra-muscular features of idiopathic inflammatory myositis (IIM). We aimed to identify any clinical and serological predictors of ILD in a monocentric cohort of 165 IIM patients. ILD+ patients were defined as having restrictive impairment in lung function tests and signs of ILD at chest high-resolution computed tomography (HRCT). Available HRCT images were centralized and classified in different ILD patterns: non-specific interstitial pneumonia (NSIP), organizing pneumonia (OP), usual interstitial pneumonia-like (UIP), indeterminate for UIP, and interstitial lung abnormalities (ILA). Lung function test data were recorded at onset, at 1 and 5 years after ILD diagnosis. ILD was found in 52 IIM patients (31.5%): 46.2% was affected by anti-synthetase syndrome (ARS), 21% by polymyositis (PM), 19% by dermatomyositis (DM), and 13.5% by overlap myositis. Most of ILD+ showed NSIP (31.9%), OP (19%), indeterminate for UIP (19%), and UIP (12.8%) patterns. At multivariate analysis, ILD was predicted by anti-Ro52 (p: 0.0026) and dyspnea (p: 0.015) at IIM onset. Most of ILD onset within is 12 months after IIM. In five cases, ILD occurs after 12 months since IIM diagnosis: these patients more frequently show dry cough and anti-Ku antibodies. Anti-Ro52 + ILD patients showed a significant increase of DLCO at 1 and 5 years of follow-up, compared with anti-Ro52 negative cases. ILD occurs in about one third of IIM and was predicted by dyspnea at onset and anti-Ro52 antibodies. Anti-Ro52 defines a subgroup of ILD showing a significant improvement of DLCO during follow-up. This retrospective study has been approved by local ethic committee (ASST-Spedali Civili of Brescia, Italy); protocol number: NP3511


RMD Open ◽  
2018 ◽  
Vol 4 (2) ◽  
pp. e000738 ◽  
Author(s):  
Janet E Pope ◽  
Mohammad Movahedi ◽  
Emmanouil Rampakakis ◽  
Angela Cesta ◽  
John S Sampalis ◽  
...  

Objective(s)This study evaluated the interaction of anticitrullinated protein antibody (ACPA) and rheumatoid factor (RF) in predicting sustained clinical response in an observational registry of patients with rheumatoid arthritis (RA) followed in routine practice.MethodsPatients with RA enrolled in the Ontario Best Practices Research Initiative registry, with ≥1 swollen joint, autoantibody information and ≥1 follow-up assessment were included. Sustained clinical remission was defined as Clinical Disease Activity Index (CDAI) ≤2.8 in at least two sequential visits separated by 3–12 months. Time to sustained remission was assessed using cumulative incidence curves and multivariate cox regression.ResultsAmong 3251 patients in the registry, 970 were included, of whom 262 (27%) were ACPAneg/RFneg, 60 (6.2%) ACPApos /RFneg, 117 (12.1%) ACPAneg/RFpos and 531 (54.7%) ACPApos /RFpos at baseline. Significant between group differences were observed in age (p=0.02), CDAI (p=0.03), tender joint count (p=0.02) and Health Assessment Questionnaire (p=0.002), with ACPApos patients being youngest with lowest disease activity and disability. No difference in biologic use was found between groups (20.2% of patients).Over a mean follow-up of 3 years, sustained remission was achieved by 43.5% of ACPApos/RFpos patients, 43.3% of ACPApos /RFneg patients, 31.6 % of ACPAneg/RFpos patients and 32.4% of ACPAneg/RFneg patients (p=0.01). Significant differences were observed in CDAI improvement based on ACPA and RF status where ACPApos/RFpos had a shorter time to achieving sustained remission (HR 1.30; 95% CI 1.01 to 1.67) and experienced significantly higher improvements compared with ACPAneg/RFneg patients.Conclusion(s)Combined ACPA and RF positivity were associated with improved and faster response to antirheumatic medications in patients with RA.


2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 941.1-941
Author(s):  
L. Marelli ◽  
F. Minoia ◽  
G. Beretta ◽  
C. Mapelli ◽  
G. Leone ◽  
...  

Background:Uveitis is the main extraarticular complication of juvenile idiopathic arthritis (JIA) with still a significant impact on JIA morbidity, despite continuous improvement in systemic treatment. Although antinuclear antibody positivity and early onset of JIA have been associated with a high risk of uveitis onset, so far no clinical features have been widely recognized as predictive factors for JIA-associated uveitis (JIA-U) response to treatment.Objectives:To investigate clinical features associated with response to systemic treatment in a long-term cohort of patients with JIA-UMethods:Clinical records of patients with JIA-U were retrospectively reviewed with regard to clinical features, therapeutic choices and outcome. Clinical and laboratory variables were compared by means of Mann-Whitney U test or chi-square/Fisher exact test, as appropriate.Results:Data from 164 JIA-U patients were analysed (81.7% female), with a median follow up of 12.1 years (7.1-17.3). Median age at JIA and uveitis onset was 2.6 (1.6-4.8) and 4.8 (2.9 – 7.0) years, respectively. Monotherapy with a conventional disease-modifying antirheumatic drug (DMARD) was used in 25.0% of patients, while 111 patients (67.7%) received at least one biologic DMARD (bDMARDs). Compared to patients responsive to DMARDs, children requiring a bDMARDs for uveitis had a lower median age at both JIA (2.4 vs 4.3 years, p 0.0234) and uveitis onset (4.1 vs 6.2 years, p 0.0023). Despite no differences in ocular damage at onset and median disease duration, patients not responsive to conventional DMARDs showed a higher frequency of ocular damage at the last visit (66.2% vs 33.3%, p 0.011). Children requiring more than one bDMARD for uveitis presented a more frequent polyarticular course (87.0% vs 20.2%, p 0.0022), a longer disease duration (median follow-up: 14.2 vs 10.4 years, p 0.0397) and a higher frequency of visual loss (best corrected visual acuity < 4/10: 23.3% vs 6.3%, p 0.0069).Conclusion:JIA-U patients with a lack of response to conventional DMARDs were significantly younger both at JIA and uveitis onset. Severe JIA-U requiring more than one bDMARDs was associated with polyarticular JIA course and longer disease duration. Children resistant to conventional treatment need prompt recognition and additional strategies to improve long-term outcome.References:[1]Heiligenhaus et al. Predictive factors and biomarkers for the 2-year outcome of uveitis in juvenile idiopathic arthritis. Rheumatology 2019.Disclosure of Interests:None declared


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