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.

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 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 Adult-onset Still’s disease in the clinical practice of infectious disease physician

2020 ◽  
Vol 4 (11) ◽  
pp. 705-709
Author(s):  
T.V. Kharlamova ◽  
◽  
T.Yu. Smirnova ◽  
V.P. Golub ◽  
I.V. Barysheva ◽  
...  
Keyword(s):  
Infectious Disease ◽  
Clinical Practice ◽  
Maculopapular Rash ◽  
Young Age ◽  
Adult Onset Still’S Disease ◽  
Adult Onset ◽  
Still’S Disease ◽  
Infectious Disease Physician ◽  
Still's Disease ◽  
Adult Onset Still's Disease

Adult-onset Still’s disease (AOSD) is a rare multisystem disease of unknown etiology. According to the literature, AOSD incidence in the world is 0.1–0.4 per 100 thousand population and it has two age-related peaks: the first peak — young age (15–25 years), the second — in persons aged 35–46 years. The causes and pathogenetic mechanism of AOSD are not established. The infectious pathology nature has not received proper scientific confirmation. According to modern concepts, this disease belongs to the group of auto-inflammatory, occurring without autoimmune disorders. It was found that patients with AOSD have a T-cell regulation disorder, increased production of the proinflammatory cytokine, the long-term persistence of which leads to the occurrence of fever and aseptic inflammation in various organs and tissues. The main criteria for this nosology are young age, persistent fever, arthritis, maculopapular rash, elevated ferritin level, neutrophilic leukocytosis, seronegative rheumatoid arthritis. Concomitant diseases include polyserositis, lymphadenopathy, hepatosplenomegaly, and nasopharyngeal infection. The article presents a clinical case of AOSD verified in a young woman at the Infectious Diseases Clinical Hospital. The difficulties of diagnostics are described, taking into account the variety of symptoms and the absence of pathognomonic disease signs. KEYWORDS: Adult-onset Still’s disease, fever, auto-inflammatory disease, arthritis, maculopapular rash, ferritin. FOR CITATION: Kharlamova T.V., Smirnova T.Yu., Golub V.P. et al. Adult-onset Still’s disease in the clinical practice of infectious disease physician. Russian Medical Inquiry. 2020;4(11):705–709. DOI: 10.32364/2587-6821-2020-4-11-705-709.

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