To investigate the efficacy and safety of Tocilizumab (TCZ) in patients with refractory adult-onset Still's disease (AOSD). We enrolled 8 female patients from October 2013 to July 2014. All patients fulfilled Japan's Yamaguch AOSD classification and recognized as refractory AOSD. All Patients received TCZ treatment 4-8 mg/kg every 4 weeks. Evaluation of efficacy was conducted after 3 months and 6 months, including clinical manifestations of AOSD patients, improvement of inflammatory markers as well as glucocorticoids dosage adjustments. Treatment-related adverse events were also recorded. Patients treated with Tocilizumab with average age 41.1 years old, the average disease duration 23.6 months. Two patients drop off due to infusion side effects. Others were followed at least 6 months. After 3 months of follow-up, remission rates of fever, arthritis and rashes from 8 patients were 87.5%, 100% and 87.5%. White blood cell counts, erythrocyte sedimentation rate, C-reactive protein and ferritin levels were decreased (P < 0.01) significantly compared to treatment before. Furthermore, the average dose of prednisone was reduced from 51.7 ± 38.4 mg/d to 12.9 ± 7.7 mg/d (P < 0.01). Our findings suggest that tocilizumab could alleviate the clinical manifestations of refractory AOSD rapidly and efficiently.
Download full-text PDF |
Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5647407 | PMC |
| http://dx.doi.org/10.1038/s41598-017-13639-y | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Characteristics Associated With Early Ileocolonic Resection in Pediatric Crohn's Disease.
J Surg Res
January 2025
Department of Pediatric Surgery, Massachusetts General Hospital, Boston, Massachusetts.
Introduction: Pediatric-onset Crohn's disease (CD) has a more severe phenotype than adult-onset, and nearly one-third of pediatric CD patients will require surgical therapy. There is limited data on patient/disease characteristics that are associated with earlier surgical management.
Methods: All pediatric CD patients (<22 yrs) who underwent ileocolectomy from 2005 to 2021 were included.
Autoimmune Enteritis in an Adult With Psoriatic Arthritis, Successfully Treated With Glucocorticoids and Adalimumab.
Cureus
December 2024
Rheumatology, Rochester Regional Health, Rochester, USA.
Article Synopsis
- Autoimmune enteritis (AIE) is a rare and severe condition that mainly leads to persistent diarrhea and malnutrition, often diagnosed in infants but can occur in adults.
- A male patient in his 60s with a history of psoriasis developed intractable diarrhea despite various treatments and ultimately required total parenteral nutrition.
- After biopsies confirmed AIE, he was treated with prednisone, budesonide, and adalimumab, leading to significant improvement and normalization of his bowel habits.
Breaking the storm: ruxolitinib as a lifeline in refractory hemophagocytic lymphohistiocytosis with macrophage activation syndrome.
Proc (Bayl Univ Med Cent)
July 2024
Department of Pathology, University of New Mexico School of Medicine, Albuquerque, New Mexico, USA.
This case report presents the management of a 25-year-old woman with refractory hemophagocytic lymphohistiocytosis (HLH) secondary to adult-onset Still's disease. Despite initial treatment with corticosteroids and biologic therapies, including anakinra and tocilizumab, her condition continued to deteriorate, necessitating intensive care unit admission. Following multidisciplinary consultation, ruxolitinib therapy, a Janus kinase (JAK) inhibitor targeting hyperinflammation, was initiated.
View Article and Find Full Text PDFDrug-refractory epilepsy due to a novel CLN5 mutation: A report of three patients from an Indian family.
Seizure
January 2025
Department of Neurology, AIIMS, New Delhi.
Article Synopsis
- Neuronal Ceroid Lipofuscinosis (NCL) are lysosomal storage disorders leading to neurodegeneration, with adult-onset Kufs disease having distinct characteristics like later onset and preserved vision.
- The authors describe three siblings diagnosed with a novel pathogenic CLN5 subtype, showing symptoms like developmental regression and drug-resistant myoclonic epilepsy, typical of Kufs A.
- This case highlights the increasing recognition of genotypic-phenotypic variations in NCL, as these siblings had an unusual presentation of Kufs A with severe epilepsy.
Less frequent skin ulcers among patients with Werner syndrome treated with pioglitazone: findings from the Japanese Werner Syndrome Registry.
Aging (Albany NY)
December 2024
Department of Endocrinology, Hematology and Gerontology, Chiba University Graduate School of Medicine, Chiba, Japan.
Background And Aim: Werner syndrome (WS) is an autosomal recessive, adult-onset, progeroid syndrome caused by mutations. As refractory skin ulcers significantly affect the quality of life of patients with WS, this study identified ulcer risk factors and assessed prevention methods.
Methods: We analyzed the data of 51 patients with WS enrolled in the Japanese Werner Syndrome Registry between 2016 and 2022.
Want AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!