Association of Fcγ Receptor Polymorphisms with Adult Onset Still’s Disease in Korea

2009 ◽  
Vol 36 (2) ◽  
pp. 347-350 ◽  
Author(s):  
JIN-HYUN WOO ◽  
YOON-KYOUNG SUNG ◽  
JIN-SOOK LEE ◽  
WON TAE CHUNG ◽  
JUNG-YOON CHOE ◽  
...  
Keyword(s):  
Adult Onset Still’S Disease ◽  
High Dose ◽  
Disease Course ◽  
Adult Onset ◽  
Still’S Disease ◽  
Chi Square ◽  
Still's Disease ◽  
Specific Pcr ◽  
Chi Square Test ◽  
Adult Onset Still's Disease

Objective.Fcγreceptors (FcγR) have important functions in the regulation of immune response and clearance of immune complex. High levels of immunoglobulins have been observed in patients with the active stage of adult onset Still’s disease (AOSD), and high-dose intravenous immunoglobulin treatment has decreased the disease activity of AOSD. We investigated polymorphisms of FcγR as genetic factors influencing susceptibility or disease course of AOSD in Korea.Methods.We genotyped the FcγRIIA H/R131, IIIA F/V176, and IIIB NA1/NA2 loci in 98 patients with AOSD and 151 healthy controls. Genotyping was performed using sequence-specific PCR. Patients with AOSD were subdivided into groups according to disease course: monocyclic systemic, polycyclic systemic, or chronic articular type. Allelic, genotypic, and haplotypic associations were analyzed by chi-square test.Results.No significant skewing in any of the 3 FcγR polymorphisms was found between Korean AOSD patients and controls. FcγRIIA R/R131 and R/H131 genotype in patients with chronic articular-type disease was more frequent than in controls (p = 0.006 and pcorr = 0.018). No differences of genotypic and allelic frequencies were found between other disease course types and controls. Haplotype IIA R131-IIIA F176-IIIB NA2 was more frequent in AOSD patients than in controls (p = 0.021).Conclusion.Although FcγR polymorphisms are not associated with development of AOSD in Koreans, the haplotype IIA R131-IIIA F176-IIIB NA2 may be associated with AOSD. Also, the FcγRIIA polymorphism may be associated with chronic articular-type AOSD. We need to identify whether these polymorphisms are associated with a response to anti-tumor necrosis factor agents in patients with AOSD.

Clinical Features and Prognosis of Adult-onset Still’s Disease: 61 Cases from China

2009 ◽  
Vol 36 (5) ◽  
pp. 1026-1031 ◽  
Author(s):  
TING ZENG ◽  
YU-QIONG ZOU ◽  
MEI-FANG WU ◽  
CHENG-DE YANG
Keyword(s):  
Prognostic Factors ◽  
Clinical Features ◽  
Chinese Patients ◽  
Adult Onset Still’S Disease ◽  
Disease Course ◽  
Adult Onset ◽  
Still’S Disease ◽  
Laboratory Findings ◽  
Still's Disease ◽  
Adult Onset Still's Disease

Objective.To describe the onset, clinical features, prognostic factors, and treatment of adult-onset Still’s disease (AOSD) in cases from China.Methods.Sixty-one Chinese patients with AOSD were analyzed retrospectively.Results.Common clinical features were fever (100.0%), rash (88.5%), and arthritis (82.0%). The laboratory findings were as follows: leukocytosis (83.6%), increased erythrocyte sedimentation rate (100.0%), elevated transaminase concentrations (23.0%), elevated ferritin levels (79.6%), negative antinuclear antibody (88.5%), and negative rheumatoid factor (88.5%). Of the 61 patients, 44.3% exhibited a monocyclic disease pattern, 29.5% experienced disease relapse at least once, 16.4% exhibited chronic articular course, and 9.8% died; most deaths were due to pulmonary infection and respiratory failure. Based on the disease course, we divided the 61 patients into 2 groups: those with favorable outcome (cyclic disease course, n = 45) and unfavorable outcome (chronic disease course or death, n = 16). We analyzed the prognostic factors for the 2 groups, and found that pleuritis, interstitial pneumonia, elevated ferritin levels, and failure of fever to subside after 3 days of prednisolone at 1 mg/kg/day were unfavorable prognostic factors for patients with AOSD.Conclusion.Patients with AOSD had complex symptoms with no specific laboratory findings. Our results indicate that AOSD is not a relatively benign disease, especially in cases that are refractory to high doses of prednisone.

scholarly journals Successful Treatment with Intravenous Cyclophosphamide for Refractory Adult-Onset Still’s Disease

2015 ◽  
Vol 2015 ◽  
pp. 1-5
Author(s):  
Yoshika Tsuji ◽  
Nozomi Iwanaga ◽  
Anna Adachi ◽  
Kinuyo Tsunozaki ◽  
Yasumori Izumi ◽  
...  
Keyword(s):  
Macrophage Activation ◽  
Adult Onset Still’S Disease ◽  
High Dose ◽  
Adult Onset ◽  
Still’S Disease ◽  
Intravenous Cyclophosphamide ◽  
Still's Disease ◽  
Female Case ◽  
High Doses ◽  
Adult Onset Still's Disease

We report a 64-year-old female case of intractable adult-onset Still’s disease (AOSD). Initial high-dose steroid therapy combined with cyclosporin A was ineffective against macrophage-activation syndrome (MAS), which was accompanied by the systemic type of AOSD. Treatment for MAS with intravenous cyclophosphamide resulted in remission of AOSD and a reduction in the high doses of steroids. Efficacy of biologics against MAS in AOSD is unclear. Cyclophosphamide, a conventional cytotoxic agent, should be considered as one of the therapeutic options for refractory types of AOSD with MAS.

scholarly journals An Unusual Case of Adult-Onset Still’s Disease with Hemophagocytic Syndrome, Necrotic Leukoencephalopathy and Disseminated Intravascular Coagulation

2014 ◽  
Vol 2014 ◽  
pp. 1-7
Author(s):  
Rajaie Namas ◽  
Naveen Nannapaneni ◽  
Malini Venkatram ◽  
Gulcin Altinok ◽  
Miriam Levine ◽  
...  
Keyword(s):  
Hemophagocytic Syndrome ◽  
Adult Onset Still’S Disease ◽  
Normocytic Anemia ◽  
High Dose ◽  
African American Female ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Elevated Liver Enzymes ◽  
Adult Onset Still's Disease

Case. A 34-year-old African-American female with a history of adult-onset Still’s disease presented to an outside hospital with oligoarthritis. She experienced a generalized tonic-clonic seizureen routevia ambulance, was intubated upon arrival, and transferred to the intensive care unit for treatment of suspected pneumonia and sepsis. She subsequently developed generalized cutaneous desquamation that progressed despite the cessation of antibiotics and other potential offending drugs which required transfer to our hospital’s burn unit. She was suspected to have reactive hemophagocytic syndrome based on her clinical presentation of fever, rash, polyarthritis, elevated liver enzymes, coagulopathy, splenomegaly, normocytic anemia, thrombocytopenia, hypertriglyceridemia, hyperferritinemia, and hemophagocytosis visualized in bone marrow biopsy specimen. Magnetic resonance imaging demonstrated necrotic demyelination of the deep white matter and corona radiata. The patient developed multiorgan dysfunction and DIC without any other attributable etiology. Despite aggressive broad spectrum therapy and high dose of steroids she progressively deteriorated and eventually expired.Conclusion. Previous publications have highlighted the prevalence of necrotic leukoencephalopathy in children with familial hemophagocytic syndrome. Our patient demonstrated some uncommon features complicating her HLH including DIC and necrotic leukoencephalopathy, which are very rare entities in AOSD.

scholarly journals Efficacy of high-dose anakinra in refractory macrophage activation syndrome in adult-onset Still’s disease: when dosage matters in overcoming secondary therapy resistance

2020 ◽  
Vol 12 ◽  
pp. 1759720X2097485
Author(s):  
Sofia Ajeganova ◽  
Ann De Becker ◽  
Rik Schots
Keyword(s):  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Adult Onset Still’S Disease ◽  
High Dose ◽  
Adult Onset ◽  
Still’S Disease ◽  
Standard Dosage ◽  
Still's Disease ◽  
Adult Onset Still's Disease ◽  
Activation Syndrome

Macrophage activation syndrome (MAS) is a severe, potentially fatal complication of rheumatic diseases. This case demonstrates the significant challenges and therapeutic considerations in adult-onset Still’s disease (AOSD) complicated with MAS at initial presentation, which will be discussed. MAS in our patient was refractory to the first-line therapy with high-dose corticosteroids, early administration of anakinra at a standard dosage and subsequent add-on treatments with cyclosporine A, IVIG, etoposides and tocilizumab. At 2 months after presentation, the patient was still critically ill with clinical, laboratory and histological signs of an active uncontrolled MAS. Notably, adoption of anakinra at a high dosage finally induced remission. This case confirms that adjusted dosage of anakinra is an effective therapeutic strategy in a severe AOSD-related MAS. It is tempting to speculate that anakinra at a high dosage, if used earlier, would have significantly changed the course of the disease in our patient and could have led to earlier remission.

scholarly journals Successful treatment of macrophage activation syndrome complicating adult-onset Still’s disease with tocilizumab: a case report

2017 ◽  
Vol 17 (2) ◽  
pp. 64-68
Author(s):  
Ho So ◽  
Tak-Lung Wong ◽  
Hin Ting Pang ◽  
Weng Nga Lao ◽  
Man-Lung Yip
Keyword(s):  
Macrophage Activation Syndrome ◽  
Macrophage Activation ◽  
Adult Onset Still’S Disease ◽  
High Dose ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Initial Control ◽  
Adult Onset Still's Disease ◽  
Activation Syndrome

Abstract We report a case of 50-year-old female patient with adult-onset Still’s disease (AOSD) complicated by macrophage-activation syndrome (MAS). After initial control of the disease with high-dose parenteral corticosteroids, tocilizumab (TCZ) therapy aided in maintaining the remission with rapid tapering of steroid dose. TCZ may be useful for MAS complicating AOSD.

The Mid-Range of the Adjusted Level of Ferritin Can Predict the Chronic Course in Patients with Adult Onset Still’s Disease

2009 ◽  
Vol 36 (1) ◽  
pp. 156-162 ◽  
Author(s):  
SANG-WON LEE ◽  
YONG-BEOM PARK ◽  
JUNG-SOO SONG ◽  
SOO-KON LEE
Keyword(s):  
Chronic Disease ◽  
Ferritin Level ◽  
Adult Onset Still’S Disease ◽  
Disease Course ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Laboratory Results ◽  
Disease Pattern ◽  
Adult Onset Still's Disease

Objective.To find a measure that can predict the disease course in patients with adult onset Still’s disease (AOSD).Methods.We retrospectively investigated the medical records of 71 hospitalized patients with AOSD. Patients were divided according to chronic and nonchronic disease course. The initial laboratory results were defined as those at the time of admission, the extremely deviated laboratory results as the highest or the lowest results, and the adjusted laboratory results as area under the curve divided by the days of hospitalization. All measures were compared and the odds ratio (OR) for the chronic disease pattern was assessed.Results.The mean age was 39.7 ± 13.5 years and women accounted for 63 of the total 71 (88.7%). Thirty patients (42.3%) had self-limited disease, 9 (12.7%) intermittent disease, and 23 (32.4%) the chronic disease pattern (32.4%). Nine patients (12.7%) died. The initial levels of aspartate aminotransferase (AST), alanine aminotransferase (ALT) and ferritin, the highest levels of lactate dehydrogenase (LDH) and ferritin, and the adjusted level of ferritin in patients with chronic disease were significantly higher than those with nonchronic disease. Among them, only the middle range of the adjusted ferritin level (784.1~4120.0 ng/ml) was found to have a significant predictive value for the chronic disease pattern (OR 81.7, p = 0.007).Conclusion.A novel measure, the adjusted level of ferritin during the first hospitalization, might be useful to predict progression to chronic disease in patients with AOSD.

Adult Onset Still’s Disease with Neurological Involvement：A Single Center Report

Rheumatology ◽  
2020 ◽  
Author(s):  
Mengzhu Zhao ◽  
Di Wu ◽  
Min Shen
Keyword(s):  
Clinical Features ◽  
Aseptic Meningitis ◽  
Immunosuppressive Agents ◽  
Adult Onset Still’S Disease ◽  
Neurological Involvement ◽  
High Dose ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Adult Onset Still's Disease

Abstract Objectives Adult onset Still’s disease (AOSD) is a multifactorial systemic autoinflammatory disease. Neurological damage has been scarcely reported in AOSD. We aimed to characterize the clinical features of AOSD patients with neurological involvement. Methods Totally, 187 AOSD patients were admitted to Peking Union Medical College Hospital from January 2015 to August 2019. The complete medical records were reviewed in this retrospective study. Clinical features of 14 AOSD patients with neurological involvement were collected and compared with those without. Results The prevalence of neurological involvement in AOSD inpatients was 7.5%. Median disease duration was 4.5 months with a range of 1 to 15 months. The frequent symptoms were fever (14, 100%), rash (13, 92.9%), liver dysfunction (11, 78.6%), arthralgia/arthritis (10, 71.4%), and lymphadenopathy (10, 71.4%). Four (28.6%) patients had macrophage activation syndrome (MAS). Aseptic meningitis was the most common presentation (64.3%) when the nervous system was involved. Other rare manifestations included cranial nerve palsy, encephalitis, and cerebral infarction. Rate of MAS, serum levels of lactate dehydrogenase and ferritin were significantly higher in AOSD patients with neurological involvement than in those without. All patients received high dose corticosteroid therapy and immunosuppressive agents, and two were given tocilizumab. Clinical remission was achieved in all of the 14 AOSD patients with neurological involvement. Conclusion Neurological involvement, particularly aseptic meningitis, is not a rare complication of AOSD. It is frequently complicated by MAS. There may be a potential relationship between neurological damages of AOSD and MAS.

scholarly journals Adult-Onset Still’s Disease: Clinical Aspects and Therapeutic Approach

2021 ◽  
Vol 10 (4) ◽  
pp. 733
Author(s):  
Stylianos Tomaras ◽  
Carl Christoph Goetzke ◽  
Tilmann Kallinich ◽  
Eugen Feist
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Large Body ◽  
Adult Onset Still’S Disease ◽  
Clinical Aspects ◽  
Disease Course ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Systemic Onset ◽  
Adult Onset Still's Disease

Adult-onset Still’s disease (AoSD) is a rare systemic autoinflammatory disease characterized by arthritis, spiking fever, skin rash and elevated ferritin levels. The reason behind the nomenclature of this condition is that AoSD shares certain symptoms with Still’s disease in children, currently named systemic-onset juvenile idiopathic arthritis. Immune dysregulation plays a central role in AoSD and is characterized by pathogenic involvement of both arms of the immune system. Furthermore, the past two decades have seen a large body of immunological research on cytokines, which has attributed to both a better understanding of AoSD and revolutionary advances in treatment. Additionally, recent studies have introduced a new approach by grouping patients with AoSD into only two phenotypes: one with predominantly systemic features and one with a chronic articular disease course. Diagnosis presupposes an extensive diagnostic workup to rule out infections and malignancies. The severe end of the spectrum of this disease is secondary haemophagocytic lymphohistiocytosis, better known as macrophage activation syndrome. In this review, we discuss current research conducted on the pathogenesis, diagnosis, classification, biomarkers and complications of AoSD, as well as the treatment strategy at each stage of the disease course. We also highlight the similarities and differences between AoSD and systemic-onset juvenile idiopathic arthritis. There is a considerable need for large multicentric prospective trials.

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