Identification of prognostic factors and construction of a nomogram for patients with relapse/refractory adult-onset Still’s disease

Yin, Ruxue; Wang, Gangjian; Yang, Xiaopei; Zhang, Lei; Wang, Shuo-Lin; Li, Tianfang; Liu, Shengyun

doi:10.1007/s10067-021-05722-7

Cited by 8 publications

(7 citation statements)

References 33 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…favorable factor correlated with good prognosis, while Kim et al reported that elevated ESR and nonresponse to corticosteroids were associated with poor prognosis. 11,24 Other predictive factors for the identification of different subsets have been identified, such as high fever (>39°C), thrombocytopenia, hepatitis, elevated CRP, and hyperferritinemia, all of which have been associated with a systemic subset. 8,20,22,23,25,26 This is consistent with our findings, since this systemic subset profile is similar to clusters 1 and 3, particularly in cases of monocyclic presentations.…”

Section: Discussionmentioning

confidence: 99%

Routine biomarker profile for the prediction of clinical phenotypes of adult‐onset Still's disease using unsupervised clustering algorithm

Gallardo‐Pizarro,

Campos‐Rodríguez,

Martín‐Iglesias

et al. 2024

Int J of Rheum Dis

View full text Add to dashboard Cite

AimThis study addresses the challenge of predicting the course of Adult‐onset Still's disease (AoSD), a rare systemic autoinflammatory disorder of unknown origin. Precise prediction is crucial for effective clinical management, especially in the absence of specific laboratory indicators.MethodsWe assessed the effectiveness of combining traditional biomarkers with the k‐medoids unsupervised clustering algorithm in forecasting the various clinical courses of AoSD—monocyclic, polycyclic, or chronic articular. This approach represents an innovative strategy in predicting the disease's course.ResultsThe analysis led to the identification of distinct patient profiles based on accessible biomarkers. Specifically, patients with elevated ferritin levels at diagnosis were more likely to experience a monocyclic disease course, while those with lower erythrocyte sedimentation rate could present with any of the clinical courses, monocyclic, polycyclic, or chronic articular, during follow‐up.ConclusionThe study demonstrates the potential of integrating traditional biomarkers with unsupervised clustering algorithms in understanding the heterogeneity of AoSD. These findings suggest new avenues for developing personalized treatment strategies, though further validation in larger, prospective studies is necessary.

show abstract

Section: Discussionmentioning

confidence: 99%

Routine biomarker profile for the prediction of clinical phenotypes of adult‐onset Still's disease using unsupervised clustering algorithm

Gallardo‐Pizarro,

Campos‐Rodríguez,

Martín‐Iglesias

et al. 2024

Int J of Rheum Dis

View full text Add to dashboard Cite

show abstract

“…Different prognostic factors have been reported for AOSD, depending on study design, the treatment outcomes selected as the end-point, and heterogeneity of AOSD [18,[20][21][22][23][24][37][38][39][40][41][42][43][44][45][46][47][48][49]. A previous nationwide epidemiological survey of AOSD in Japan in 2010 showed that patients receiving GC pulse therapy were those who had had more serositis, HPS, higher neutrophil counts, and higher serum ferritin levels than those who did not receive GC pulse therapy [23].…”

Section: Discussionmentioning

confidence: 99%

Associated factors with poor treatment response to initial glucocorticoid therapy in patients with adult-onset Still’s disease

et al. 2022

View full text Add to dashboard Cite

Background High-dose glucocorticoids (GC) are first-line treatment for adult-onset Still’s disease (AOSD); however, some of the patients remain refractory to initial GC therapy, or rapidly relapse. The aim of this study was to identify prognostic factors for poor treatment response to initial GC therapy for AOSD. Methods Data on newly diagnosed AOSD patients were extracted from our database (n=71, mean age 51.6 years). The primary outcome was a poor treatment outcome at 4 weeks, which was defined as failure to achieve remission or relapse after achieving remission within 4 weeks, followed by administration of two or more rounds of GC pulse therapy or of any other immunosuppressive drugs. Results The initial mean dose ± standard deviation of prednisolone was 0.82 ± 0.23 mg/kg/day, and 34 (47.3%) patients received GC pulse therapy at week 0. Twenty-nine of 71 patients exhibited a poor treatment outcome at 4 weeks (40.8%). The second round of GC pulse therapy or immunosuppressive drugs was added in 17 or 24 of the 29 patients, respectively. These patients had higher baseline white blood cell (WBC) counts, serum ferritin levels, systemic feature score based on clinical symptoms (modified systemic feature score, mSFS), more hemophagocytic syndrome (HPS) over the 4 weeks, and the higher severity score based on modified Pouchot score or severity index of the Japanese Ministry of Health, Labour and Welfare, than the remaining 42 patients. Multivariable logistic regression model identified baseline WBC count as a prognostic factor for poor outcome (odds ratio per 1000/μl increment: 1.12, 95% CI 1.04–1.29), while thrombocytopenia, hyperferritinemia, and mSFS at baseline did not achieve statistical significance. Receiver-operating characteristic curve analysis showed that the optimal cut-off for WBC count was 13,050/μl. The Kaplan-Meier method showed the cumulative rate of poor treatment outcome to be 60.0% in patients with WBC ≥13,050/μl and 23.5% in those with WBC <13,050/μl. Conclusions A higher WBC count but not thrombocytopenia, hyperferritinemia, and mSFS at baseline was a significant prognostic factor for poor treatment outcome at week 4 in this retrospective cohort of AOSD patients. Our findings provide important information for determining the initial treatment strategy of newly-diagnosed AOSD.

show abstract

“…Different prognostic factors have been reported for AOSD, depending on study design and the treatment outcomes selected as the end-point (18, [20][21][22][23][24][37][38][39][40][41][42][43][44][45][46][47][48][49]. A previous nationwide epidemiological survey of AOSD in Japan in 2010 showed that patients receiving GC pulse therapy were those who had had more serositis, HPS, higher neutrophil counts, and higher serum ferritin levels than those who did not receive GC pulse therapy (23).…”

Section: Discussionmentioning

confidence: 99%

Associated factors with poor treatment response to initial glucocorticoid therapy in patients with adult-onset Still’s disease

Kondo

Sugihara

Umezawa

et al. 2021

Preprint

View full text Add to dashboard Cite

Background High-dose glucocorticoids (GC) are first-line treatment for adult onset Still’s disease (AOSD), however some of the patients remain refractory to initial GC therapy, or rapidly relapse. The aim of this study was to identify prognostic factors for poor treatment response to initial GC therapy for AOSD. Methods Data on newly-diagnosed AOSD patients were extracted from our database (n=71, mean age 51.6 years). The primary outcome was a poor treatment outcome at 4 weeks, which was defined as failure to achieve remission or relapse after achieving remission within 4 weeks, followed by administration of two or more rounds of GC pulse therapy or of any other immunosuppressive drugs. Results The initial mean dose ± standard deviation of prednisolone was 0.82 ± 0.23 mg/kg/day, and 34 (47.3%) patients received GC pulse therapy at week 0. Twenty-nine of 71 patients exhibited a poor treatment outcome at 4 weeks (40.8%). The second round of GC pulse therapy or immunosuppressive drugs was added in 17 or 24 of the 29 patients, respectively. These patients had higher baseline white blood cell (WBC) counts, serum ferritin levels, systemic feature score based on clinical symptoms (modified systemic feature score, mSFS), more hemophagocytic syndrome (HPS) over the 4 weeks, and the higher severity score based on modified Pouchot score or severity index of the Japanese Ministry of Health, Labour and Welfare, than the remaining 42 patients. Multivariable logistic regression model identified baseline WBC count as a prognostic factor for poor outcome (odds ratio per 1,000/µl increment: 1.12, 95% CI 1.04-1.29), while thrombocytopenia, hyperferritinemia, and mSFS at baseline did not achieve statistical significance. Receiver-Operating Characteristic curve analysis showed that the optimal cut-off for WBC count was 13,050/µl. The Kaplan-Meier method showed the cumulative rate of poor treatment outcome to be 60.0% in patients with WBC ≥13,050/µl and 23.5% in those with WBC <13,050/µl. Conclusions A higher WBC count but not thrombocytopenia, hyperferritinemia, and mSFS at baseline was a significant prognostic factor for poor treatment outcome at week 4 in this retrospective cohort of AOSD patients. Our findings provide important information for determining the initial treatment strategy of newly-diagnosed AOSD.

show abstract

Identification of prognostic factors and construction of a nomogram for patients with relapse/refractory adult-onset Still’s disease

Cited by 8 publications

References 33 publications

Routine biomarker profile for the prediction of clinical phenotypes of adult‐onset Still's disease using unsupervised clustering algorithm

Routine biomarker profile for the prediction of clinical phenotypes of adult‐onset Still's disease using unsupervised clustering algorithm

Associated factors with poor treatment response to initial glucocorticoid therapy in patients with adult-onset Still’s disease

Associated factors with poor treatment response to initial glucocorticoid therapy in patients with adult-onset Still’s disease

Contact Info

Product

Resources

About