Leflunomide and Azathioprine Combination in Refractory Adult-Onset Still's Disease

2005 ◽  
Vol 39 (4) ◽  
pp. 764-767 ◽  
Author(s):  
Ayse Cefle
Keyword(s):  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Reactive Protein ◽  
Still's Disease ◽  
Normal Erythrocyte Sedimentation Rate ◽  
One Year ◽  
The One ◽  
Adult Onset Still's Disease

OBJECTIVE: To present a case of Adult-onset Still's disease (ASD) in a patient who was successfully treated with leflunomide and azathioprine. CASE SUMMARY: A 24-year-old woman with ASD was initially treated with indomethacin, corticosteroids, and hydroxychloroquine; there was no clinical improvement. Methotrexate was added to the regimen, followed by azathioprine. The patient still experienced disease flares with this treatment, and cyclophosphamide was started. However, because of persisting disease activity, leflunomide combined with azathioprine was given. Only on this regimen was complete disease control achieved, with a normal erythrocyte sedimentation rate as well as normal C-reactive protein and ferritin levels. No recurrences or adverse effects attributable to leflunomide or azathioprine were observed at the one-year follow-up. DISCUSSION: Clinical experience concerning leflunomide and azathioprine combination in ASD is limited. This combination may be modifying the clinical expression of ASD through its effects on T lymphocyte clonal expansion and production of proinflammatory cytokines. CONCLUSIONS: Leflunomide combined with azathioprine appears to be an effective and safe treatment of ASD.

Adult-onset Still’s disease successfully treated with Chinese herbal medicine: A case report with 15-month follow-up

2020 ◽  
Vol 18 (6) ◽  
pp. 530-534
Author(s):  
Ming-sheng Lyu ◽  
De-ying Li ◽  
Shao-zhong Zhou ◽  
Cheng-jun Ban ◽  
Jun Yan
Keyword(s):  
Case Report ◽  
Herbal Medicine ◽  
Chinese Herbal Medicine ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Chinese Herbal ◽  
Still's Disease ◽  
Adult Onset Still's Disease

Long‐term follow‐up of patients with adult‐onset Still's disease

2006 ◽  
Vol 35 (5) ◽  
pp. 395-397 ◽  
Author(s):  
N. Akritidis ◽  
A. Papadopoulos ◽  
G. Pappas
Keyword(s):  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Long Term Follow Up ◽  
Adult Onset Still's Disease

scholarly journals AB0369 Adult-onset still’s disease treatment predictors at 1-year follow-up in a single rheumatologic centre experience

2018 ◽  
Author(s):  
M. Giannotta ◽  
F. Cacciapaglia ◽  
M. Giannini ◽  
M. Nivuori ◽  
R. Fanizzi ◽  
...  
Keyword(s):  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Disease Treatment ◽  
Still's Disease ◽  
Treatment Predictors ◽  
Adult Onset Still's Disease

scholarly journals Real-Life Data on the Efficacy of Canakinumab in Patients with Adult-Onset Still’s Disease

2020 ◽  
Vol 2020 ◽  
pp. 1-7
Author(s):  
Antonio Vitale ◽  
Virginia Berlengiero ◽  
Jurgen Sota ◽  
Luisa Ciarcia ◽  
Nicola Ricco ◽  
...  
Keyword(s):  
Adverse Events ◽  
Disease Activity ◽  
Real Life ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Sparing Effect ◽  
Adult Onset Still's Disease

Background. Interleukin-1 inhibition has revealed to be a successful treatment approach for patients with adult-onset Still’s disease (AOSD). However, real-life experience is focused on the use of anakinra, while data about canakinumab (CAN) are mainly based on case reports and small case series. Patients and Methods. Patients classified with AOSD according to Yamaguchi criteria and treated with CAN were consecutively enrolled. Their clinical and therapeutic data were retrospectively collected and statistically analysed to assess the role of CAN as a therapeutic opportunity in AOSD patients in terms of clinical and laboratory disease control along with corticosteroid-sparing effect. Results. Nine AOSD patients (8 females and 1 male) treated with CAN for 15.00 ± 12.3 months were enrolled. Resolution of clinical manifestations was reported in 8/9 cases at the 3-month assessment; a significant decrease in the number of tender joints ( p = 0.009 ), swollen joints ( p = 0.027 ), and disease activity score on 28 joints-C-reactive protein (DAS28-CRP) ( p = 0.044 ) was observed during the study period. The systemic score of disease activity significantly decreased at the 3-month and 6-month assessments and at the last visit compared to the start of treatment ( p = 0.028 , p = 0.028 , and p = 0.018 , respectively). The daily corticosteroid dosage was significantly reduced at the 3-month and at the last follow-up visits ( p = 0.017 and p = 0.018 , respectively). None of the patients experienced adverse events or severe adverse events during the follow-up. Conclusions. CAN has shown prompt and remarkable effectiveness in controlling AOSD activity in a real-life contest, with a significant glucocorticoid-sparing effect and an excellent safety profile.

Clinical characteristics and follow-up analysis of adult-onset Still’s disease complicated by hemophagocytic lymphohistiocytosis

2016 ◽  
Vol 35 (5) ◽  
pp. 1145-1151 ◽  
Author(s):  
Yun Zhang ◽  
Yingyun Yang ◽  
Yujia Bai ◽  
Dan Yang ◽  
Yangyang Xiong ◽  
...  
Keyword(s):  
Hemophagocytic Lymphohistiocytosis ◽  
Clinical Characteristics ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Adult Onset Still's Disease

Cerebral Haemorrhage Complicating Adult-Onset Still's Disease: A Case Report

1996 ◽  
Vol 24 (6) ◽  
pp. 492-494 ◽  
Author(s):  
H Kurabayashi ◽  
K Kubota ◽  
K Tamura ◽  
T Shirakura
Keyword(s):  
Serum Level ◽  
Cerebral Haemorrhage ◽  
Japanese Woman ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Reactive Protein ◽  
Still's Disease ◽  
Old Japanese ◽  
Adult Onset Still's Disease

The case of a 75-year-old Japanese woman with adult-onset Still's disease who presented with cerebral haemorrhage is described. She had been in clinical remission for 2 years, after induction therapy including non-steroidal anti-inflammatory drugs, prednisolone, cyclophosphamide and mizoribine followed by auranofin, until her cerebral haemorrhage occurred, although her serum level of ferritin had gradually increased. After the onset of cerebral haemorrhage, the patient's serum level of thrombomodulin was elevated although c-reactive protein and lactate dehydrogenase were not increased. Anti-cardiolipin antibody and lupus anti-coagulant were not detected. Patients with adult-onset Still's disease are rarely reported to develop cerebral vascular disease, possibly because the disease is most frequent in young adults. The cerebral haemorrhage may have been caused by the vasculitis due to Still's disease.

Serum S100A12 May Be a Useful Biomarker of Disease Activity in Adult-onset Still’s Disease

2014 ◽  
Vol 41 (12) ◽  
pp. 2403-2408 ◽  
Author(s):  
Chang-Bum Bae ◽  
Chang-Hee Suh ◽  
Jeong-Mi An ◽  
Ju-Yang Jung ◽  
Ja-Young Jeon ◽  
...  
Keyword(s):  
Disease Activity ◽  
Advanced Glycation Endproducts ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Glycation Endproducts ◽  
Reactive Protein ◽  
Still's Disease ◽  
Adult Onset Still's Disease

Objective.S100A12 and soluble receptor for advanced glycation endproducts (sRAGE) have been suggested as biomarkers of disease activity in patients with systemic juvenile idiopathic arthritis. We investigated the clinical significance of these markers in adult-onset Still’s disease (AOSD).Methods.Blood samples were collected from 37 patients with active AOSD and 38 healthy controls (HC). Of the patients with AOSD, followup samples were collected from 19 patients after resolution of disease activity.Results.Serum S100A12 (547.9 ± 148.4 ng/ml) in patients with AOSD was higher than those of HC (272.3 ± 133 ng/ml, p < 0.001). The sRAGE levels of AOSD (514.1 ± 273.6 pg/ml) were lower than those of HC (850.3 ± 405.8 pg/ml, p < 0.001). Serum S100A12 correlated with serum sRAGE (r = −0.228, p = 0.049). Serum S100A12 correlated with erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), ferritin, and systemic score, whereas sRAGE did not correlate with any disease activity markers. In addition, the level of S100A12 was decreased after disease activity was resolved in followed-up patients with AOSD (505.7 ± 161.3 ng/ml vs 361.3 ± 162.5 ng/ml, p = 0.01). Further, the change of S100A12 was well correlated with that of ESR, CRP, and systemic score.Conclusion.S100A12 levels showed strong correlations with known disease activity markers such as ESR, CRP, ferritin, and systemic score. In the followup patients with AOSD, most patients showed decreased S100A12 levels after resolution of disease activity. These results suggest that serum S100A12 can be a reliable clinical marker for monitoring disease activity and treatment response.

scholarly journals Complex presentation of adult-onset Still’s disease

2019 ◽  
Vol 12 (4) ◽  
pp. e228210
Author(s):  
Muhammad Zafran ◽  
Nancy Wassef
Keyword(s):  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Rheumatology Clinic ◽  
Still's Disease ◽  
Intravenous Antibiotics ◽  
Chest X Ray ◽  
Clinical Dilemma ◽  
Adult Onset Still's Disease

A 61-year-old woman was admitted with feeling generally unwell with influenza-like symptoms, for almost a month. This was followed by dyspnoea, productive cough and fever of >40°C. She was started on oral antibiotics in community, but due to rising inflammatory markers, she was referred for admission to our hospital. Chest X-ray showed left basal pneumonia and SE was started on intravenous antibiotics according to microbiologist’s advice. During admission she developed deranged liver functions with right upper quadrant tenderness, pleural and pericardial effusions. This was followed by multiple joint aches, mouth ulcers and a rash on her chest. Finally, after several days and clinical dilemma, she was diagnosed with adult-onset Still’s disease by the rheumatologist and was started on prednisolone, to which she showed marked improvement, and was later maintained on methotrexate and hydrotherapy. She was in remission during her follow-up in the rheumatology clinic.

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