Listeriosis presenting with fever, arthralgia, elevated liver enzymes, and hyperferritinaemia in pregnancy: a critical mimicker of adult-onset Still’s disease

2021 ◽  
pp. 1-3
Author(s):  
T Kitada ◽  
K Kadoba ◽  
R Watanabe ◽  
T Koyama ◽  
Y Nakayama ◽  
...  
Keyword(s):  
Liver Enzymes ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Elevated Liver Enzymes ◽  
Adult Onset Still's Disease ◽  
In Pregnancy

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.

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 Adult Onset Still’s Disease Associated withMycoplasma pneumoniaeInfection and Hemophagocytic Lymphohistiocytosis

2016 ◽  
Vol 2016 ◽  
pp. 1-4 ◽  
Author(s):  
Abhishek Agnihotri ◽  
Allison Ruff ◽  
Lauren Gotterer ◽  
Addie Walker ◽  
Amy H. McKenney ◽  
...  
Keyword(s):  
Hemophagocytic Lymphohistiocytosis ◽  
The Body ◽  
Adult Onset Still’S Disease ◽  
Inflammatory Disorder ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Elevated Liver Enzymes ◽  
Mycoplasma Pneumonia ◽  
Adult Onset Still's Disease

Adult Onset Still’s Disease (AOSD) is a systemic inflammatory disorder that can be associated with hemophagocytic lymphohistiocytosis (HLH), a rare but potentially fatal disease of overactive histiocytes and lymphocytes. We present a unique case of AOSD complicated byMycoplasma pneumoniainfection and HLH. A 28-year-old female developed joint pains followed by a diffuse, erythematous, pruritic skin rash that quickly spread throughout the body. The patient deteriorated and developed fever, chills, cough, and dyspnea and had to be intubated. She had hypoalbuminemia, elevated liver enzymes, a very high serum ferritin level, positive anti-Mycoplasma pneumoniaIgG and IgM antibodies, and normal rheumatoid factor and anti-nuclear antibodies. The chest X-ray showed diffuse bilateral infiltrates. Bone marrow biopsy revealed hemophagocytosis. The patient was treated with azithromycin, methylprednisolone, and anakinra and was discharged home on cyclosporine and prednisone. This case highlights that patients can develop features of both AOSD and HLH at the beginning of the disease and early diagnosis and treatment increase the likelihood of recovery.

scholarly journals Adult Onset Still’s Disease: A Diagnostic Dilemma

2021 ◽  
Vol 23 (1) ◽  
pp. 83-86
Author(s):  
Dinesh Koirala ◽  
UN Pathak ◽  
P Adhikari ◽  
R Shrestha ◽  
C Neupane
Keyword(s):  
Case Report ◽  
Liver Enzymes ◽  
Adult Onset Still’S Disease ◽  
Unknown Etiology ◽  
Rare Clinical Entity ◽  
Adult Onset ◽  
Still’S Disease ◽  
Diagnostic Dilemma ◽  
Still's Disease ◽  
Adult Onset Still's Disease

Adult-onset Still’s disease (ASD) is a rare clinical entity with unknown etiology, characterized by arthritis, fever, evanescent rash and other systemic presentations. This case report describes a 19-year-old male who presented with sore throat, fever, arthritis, evanescent rash, raised liver enzymes and hyperferritinemia. It also reveals the diagnostic dilemma faced during the diagnosis of the disease. He was diagnosed to have ASD based on Yamuguchi criteria after the exclusion of other potential diagnoses. Patient was treated with prednisolone and there was a good response with improvement in symptoms and laboratory indices.

Adult-onset Still’s disease in pregnancy

2012 ◽  
Vol 22 (1) ◽  
pp. 163-165 ◽  
Author(s):  
Motohisa Yamamoto ◽  
Tetsuya Tabeya ◽  
Chisako Suzuki ◽  
Yasuyoshi Naishiro ◽  
Hidetaka Yajima ◽  
...  
Keyword(s):  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Adult Onset Still's Disease ◽  
In Pregnancy
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