scholarly journals Pure Red Cell Aplasia with Adult Onset Still’s Disease

2013 ◽  
Vol 2013 ◽  
pp. 1-3 ◽  
Author(s):  
Nicholas Robillard ◽  
Paul Van Nguyen ◽  
Robert Wistaff ◽  
Mikhael Laskine
Keyword(s):  
Clinical Manifestations ◽  
Pure Red Cell Aplasia ◽  
Adult Onset Still’S Disease ◽  
Red Cell ◽  
Adult Onset ◽  
Still’S Disease ◽  
Red Cell Aplasia ◽  
Still's Disease ◽  
Wide Range ◽  
Adult Onset Still's Disease

Adult Onset Still’s Disease (AOSD) is a rare inflammatory syndrome mostly seen in young adults. Known for its wide range of clinical manifestations, AOSD often presents with nonremitting systemic signs and symptoms. Many rare case associations have been described with AOSD, but only few with pure red cell aplasia (PRCA). We are presenting a fourth known case of a young female adult with AOSD and PRCA in the literature.

scholarly journals Pure red cell aplasia in a patient with adult-onset Still's disease

2008 ◽  
Vol 83 (8) ◽  
pp. 687-688 ◽  
Author(s):  
Vikram Comondore ◽  
A. Caroline Patterson ◽  
Leslie Zypchen ◽  
Bakul Dalal
Keyword(s):  
Pure Red Cell Aplasia ◽  
Adult Onset Still’S Disease ◽  
Red Cell ◽  
Adult Onset ◽  
Still’S Disease ◽  
Red Cell Aplasia ◽  
Still's Disease ◽  
Adult Onset Still's Disease

scholarly journals An Update on the Pathogenic Role of Neutrophils in Systemic Juvenile Idiopathic Arthritis and Adult-Onset Still’s Disease

2021 ◽  
Vol 22 (23) ◽  
pp. 13038
Author(s):  
Ji-Won Kim ◽  
Mi-Hyun Ahn ◽  
Ju-Yang Jung ◽  
Chang-Hee Suh ◽  
Hyoun-Ah Kim
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Immune Defense ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Wide Range ◽  
Adult Onset Still's Disease

Neutrophils are innate immune phagocytes that play a key role in immune defense against invading pathogens. The main offensive mechanisms of neutrophils are the phagocytosis of pathogens, release of granules, and production of cytokines. The formation of neutrophil extracellular traps (NETs) has been described as a novel defense mechanism in the literature. NETs are a network of fibers assembled from chromatin deoxyribonucleic acid, histones, and neutrophil granule proteins that have the ability to kill pathogens, while they can also cause toxic effects in hosts. Activated neutrophils with NET formation stimulate autoimmune responses related to a wide range of inflammatory autoimmune diseases by exposing autoantigens in susceptible individuals. The association between increased NET formation and autoimmunity was first reported in antineutrophil cytoplasmic antibody-related vasculitis, and the role of NETs in various diseases, including systemic lupus erythematosus, rheumatoid arthritis, and psoriasis, has since been elucidated in research. Herein, we discuss the mechanistic role of neutrophils, including NETs, in the pathogenesis of systemic juvenile idiopathic arthritis (SJIA) and adult-onset Still’s disease (AOSD), and provide their clinical values as biomarkers for monitoring and prognosis.

scholarly journals Pneumonitis in Adult Onset Still’s Disease: Uncommon or Under Diagnosed?

2017 ◽  
Vol 30 (7-8) ◽  
pp. 578 ◽  
Author(s):  
Silvia Fernandes ◽  
Margarida Almeida ◽  
José Alberto Pereira da Silva ◽  
José Carlos Romeu
Keyword(s):  
Differential Diagnosis ◽  
Case Report ◽  
Clinical Manifestations ◽  
Adult Onset Still’S Disease ◽  
Severe Respiratory Failure ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Pulmonary Parenchyma ◽  
Adult Onset Still's Disease

The adult onset Still’s Disease is an uncommon entity characterized by multiple clinical manifestations. Pneumonitis, less often considered, deserves particular emphasis given the need for differential diagnosis and because it can progress to severe respiratory failure. With the aim to highlight the pulmonary parenchyma involvement in patients with adult onset Still’s Disease, we present a case report which progresses with pneumonitis.

scholarly journals The Association of ATG16L1 Variations with Clinical Phenotypes of Adult-Onset Still’s Disease

Genes ◽  
2021 ◽  
Vol 12 (6) ◽  
pp. 904
Author(s):  
Wei-Ting Hung ◽  
Shuen-Iu Hung ◽  
Yi-Ming Chen ◽  
Chia-Wei Hsieh ◽  
Hsin-Hua Chen ◽  
...  
Keyword(s):  
Skin Rash ◽  
Direct Sequencing ◽  
Differentially Expressed Gene ◽  
Clinical Manifestations ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Autophagosome Formation ◽  
Still's Disease ◽  
Adult Onset Still's Disease

Adult-onset Still’s disease (AOSD) is a rare autoinflammatory disease, which has elevated autophagosome levels regulated by autophagy-related gene (ATG) expression. We investigated the associations of ATG polymorphisms with AOSD susceptibility, clinical manifestations, and disease course. The six-candidate single-nucleotide polymorphisms (SNPs) involved in autophagy were genotyped using direct sequencing on samples from 129 AOSD patients and 129 healthy participants. The differentially expressed gene products were quantified using PCR and ELISA. Significant linkage disequilibrium was noted in three SNPs of autophagy-related 16-like 1 (ATG16L1) gene (rs10210302, rs2241880, and rs1045100). Although the AA/CC/TT haplotype of ATG16L1 was not associated with the susceptibility of our AOSD patients compared with other haplotypes, those carrying this haplotype had lower mRNA expression levels of LC3-II, reflecting by autophagosome formation (p = 0.026). Patients carrying AA/CC/TT haplotype also have a significantly higher proportion of skin rash and a lower proportion of arthritis compared with other haplotypes. The AA/CC/TT haplotype was significantly associated with systemic pattern (odds ratio, 3.25; 95% confidence interval, 1.15–9.14; p = 0.026). In summary, the AA/CC/TT haplotype encoded lower levels of autophagosome formation and was associated with a higher proportion of skin rash and systemic pattern of AOSD compared with other haplotypes.

scholarly journals Diagnostic Challenge: A Report of Two Adult-Onset Still’s Disease Cases

2017 ◽  
Vol 2017 ◽  
pp. 1-4 ◽  
Author(s):  
Sakunee Niranvichaiya ◽  
Daranporn Triwongwaranat
Keyword(s):  
Rheumatoid Factor ◽  
Clinical Manifestations ◽  
Maculopapular Rash ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Positive Rheumatoid Factor ◽  
Salmon Pink ◽  
Still's Disease ◽  
Adult Onset Still's Disease

This study reports two adult-onset Still’s disease (AOSD) cases that met both Yamaguchi’s and Fautrel’s criteria and that presented with notable clinical manifestations. One case presented with atypical dermographism-like rash with an extremely high ferritin level. The other case presented with typical salmon-pink maculopapular rash but had atypical positive rheumatoid factor. This suggests that although negative rheumatoid factor is one of the criteria used for the diagnosis of AOSD, a positive rheumatoid factor result does not exclude AOSD. Beside a classic rash, characterized by transient salmon-pink maculopapular rash, we also find atypical dermographism-like rash. These findings remind us that there exist various types of rash from AOSD.

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