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 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 Adult-onset Still’s disease, the most internistic of the rheumatic diseases: current concepts and a report of six cases

2013 ◽  
pp. 259-264
Author(s):  
Roberto Boni ◽  
Pier G. Rabitti
Keyword(s):  
Serum Ferritin ◽  
Hemophagocytic Syndrome ◽  
Unknown Origin ◽  
Essential Elements ◽  
Adult Onset Still’S Disease ◽  
Inflammatory Disorder ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Adult Onset Still's Disease

Adult-onset Still’s disease (AOSD) is a rare systemic inflammatory disorder of unknown origin. It is characterized by spiking fever, evanescent rash, arthralgia/arthritis, and leukocytosis. The differential diagnosis includes a number of other conditions, and management is complicated by the lack of course predictors and the risks associated with complications and treatments. This report examines recent advances in our understanding of adult-onset Still’s disease (pathogenesis, diagnosis, complications, treatment). Current research in this field is focused on the significance of serum ferritin in AOSD, mechanisms underlying the hemophagocytic syndrome, and use of biologic therapies in patients who are refractory to conventional treatment. Six cases of AOSD diagnosed by our staff between 2002 and 2009 are also analyzed and compared with other cases reported in the literature. This analysis showed that Still’s rash and serum ferritin levels were not essential elements for diagnosis. In addition, the course of the disease showed little relation to the severity / characteristics of the presenting picture, but the evolution worsened with the age of the patient at diagnosis.

Elevated Levels of G-CSF in Patients with Active Phase of Adult-onset Still’s Disease

2020 ◽  
pp. jrheum.200617
Author(s):  
Yudong Liu ◽  
Shulan Zhang ◽  
Changshe Xia ◽  
Jiali Chen ◽  
Chunhong Fan
Keyword(s):  
Disease Activity ◽  
Therapeutic Potential ◽  
Assessment System ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Elevated Liver Enzymes ◽  
Csf Levels ◽  
Adult Onset Still's Disease

Objective Neutrophilia is a hallmark of adult-onset Still’s disease (AoSD). We aimed to investigate the levels of granulocyte colony-stimulating factor (G-CSF), an essential regulator of neutrophil production and function, in the pathogenesis of AoSD. Methods Sera were collected from 70 patients with AoSD and 20 healthy controls (HCs). The levels of G-CSF were determined by ELISA. Low-density granulocytes (LDGs) were quantified by flow cytometry. Correlations between G-CSF levels and disease activity, laboratory parameters, or LDGs levels in patients with AoSD were analyzed by Spearman’s correlation test. Results Active AoSD patients presented significantly higher levels of G-CSF compared to inactive AoSD patients (p<0.001) and HCs (p<0.0001). The levels of G-CSF were significantly decreased after active AoSD patients achieved disease remission (p=0.0015). The levels of G-CSF were significantly correlated with CRP, ESR, ferritin and systemic score in AoSD (p<0.0001). Significant correlations between the levels of G-CSF and circulating neutrophils (p<0.0001), neutrophil-to-lymphocyte ratio (p<0.0001), percentages of LDGs in the PBMCs (p=0.0042) as well as absolute numbers of circulating LDGs (p=0.0180) were identified. Patients with fever, evanescent rash, sore throat, arthralgia, myalgia, lymphadenopathy or hepatomegaly/elevated liver enzymes displayed significantly higher levels of G-CSF compared to patients without these manifestations (p<0.05). Conclusion Our findings indicate that G-CSF is implicated in the pathogenesis of AoSD, and targeting G-CSF may have therapeutic potential for AoSD. In addition, introducing circulating G-CSF levels into the clinical assessment system may help to monitor disease activity.

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.

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