- [Systemic juvenile onset idiopathic arthritis and adult onset still disease]. [Journal Article]
- RMRev Med Suisse 2018 Feb 14; 14(594):372-377
- Still's disease is a rare multifactorial disease associated with systemic inflammation. Systemic-onset juvenile idiopathic arthritis and adult-onset Still's disease are both pediatric and respectivel...
Still's disease is a rare multifactorial disease associated with systemic inflammation. Systemic-onset juvenile idiopathic arthritis and adult-onset Still's disease are both pediatric and respectively adult forms of the disease with a cut-off age of 16 years. The disease is characterized by the following features : hectic fever > 39° C, arthralgia or arthritis, rash, neutrophilia and systemic inflammation. The prognosis of the disease is functional and vital. The evolution over time is variable : regression, evolution by relapses with regression at term and chronic joint evolution. This focus describes the two forms of the disease, their complications and the therapeutic options.
- Convergent pathways of the hyperferritinemic syndromes. [Journal Article]
- IIInt Immunol 2018 Feb 06
- Hyperferritinemia and pronounced hemophagocytosis help distinguish a subset of patients with a particularly inflammatory and deadly systemic inflammatory response syndrome. Two clinically similar dis...
Hyperferritinemia and pronounced hemophagocytosis help distinguish a subset of patients with a particularly inflammatory and deadly systemic inflammatory response syndrome. Two clinically similar disorders typify these hyperferritinemic syndromes: hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS). HLH is canonically associated with a complete disturbance of perforin/granzyme-mediated cytotoxicity, whereas MAS occurs in the context of the related rheumatic diseases systemic juvenile idiopathic arthritis (SJIA) and adult-onset Still's disease (AOSD), with associated IL-1 family cytokine activation. In practice, however, there are accumulating lines of evidence for innate immune dysregulation in HLH as well as partial impairments of cytotoxicity in MAS, and these mechanisms likely represent only a fraction of the host and environmental factors driving hyperferritinemic inflammation. Herein, we present new findings that highlight the pathogenic differences between HLH and MAS, two conditions that present with life-threatening hyperinflammation, hyperferritinemia and hemophagocytosis.
- Response to: 'Is the PET/CT first choice for differential diagnosis of FUO/IUO?' by Armaganet al. [Journal Article]
- ARAnn Rheum Dis 2018 Feb 02
- Impaired autophagic flux and its related inflammation in patients with adult-onset Still's disease. [Journal Article]
- OOncotarget 2018 Jan 02; 9(1):110-121
- The pathogenic role of autophagic immune regulation in adult-onset Still's disease (AOSD) is unclear. We investigated the relative levels of autophagy in AOSD patients and healthy controls, its assoc...
The pathogenic role of autophagic immune regulation in adult-onset Still's disease (AOSD) is unclear. We investigated the relative levels of autophagy in AOSD patients and healthy controls, its association with disease activity or course, and the change in autophagy after 6 months of therapy. Autophagosome levels were determined from the mean fluorescence intensity of autophagosomotropic dye incorporated into circulating immune cells. The fluorescent signal from lymphocytes, monocytes, and granulocytes from AOSD patients was greater than from controls. Levels of p62 fluorescence measured using flow cytometry in lymphocytes and granulocytes from AOSD patients was greater than in the corresponding cells from healthy controls. Expression of Atg5 and LC3-II mRNA and protein levels of p62 and LC3-II were elevated in AOSD patients. Moreover, AOSD activity scores correlated positively with autophagosome levels in monocytes and granulocytes, p62 levels in circulating immune cells, and levels of Beclin-1, Atg5, and LC3-II mRNA. Autophagosome levels and Atg mRNA expression decreased with disease remission in AOSD patients. Elevated autophagosome formation and p62 levels suggest impaired autophagic flux in AOSD.
- Biological therapy of traditional therapy-resistant adult-onset Still's disease: an evidence-based review. [Review]
- TCTher Clin Risk Manag 2018; 14:167-171
- CONCLUSIONS: Our findings suggest that anakinra and tocilizumab may be good choices for the treatment of refractory AOSD considering the effectiveness and safety.
- Purulent constrictive pericarditis caused by Salmonella enteritidis in a patient with adult-onset Still's disease: A case report. [Case Reports]
- MMedicine (Baltimore) 2017; 96(50):e8949
- CONCLUSIONS: This case presents an opportunity to highlight the importance of considering purulent pericarditis in patients previously diagnosed with AOSD. High clinical suspicion, early diagnosis, and prompt management can result in a better outcome in purulent pericarditis.
- Acute appendicitis complicated with necrotizing fasciitis in a patient with adult-onset Still's disease: A case report. [Case Reports]
- MMedicine (Baltimore) 2018; 97(5):e9794
- CONCLUSIONS: (1) The steroid dose was difficult to titrate when AOSD complicated by sepsis. The differential diagnosis from MAS/HLH with bacterial/viral infection related severe sepsis was difficult but critical for decision making from clinicians and rheumatologists. (2) The conservative treatment with antibiotics for perforated appendix is safe but has a higher failure rate in immunocomprised patients such as systemic lupus erythematosus and AOSD. Early surgical intervention might contribute to better outcome. (3) The abdominal wall abscess can be spread from intra-abdominal lesion through the inferior epigastric vessels which were as weak points of abdominal wall. Imaging examinations contribute to acute diagnosis and help surgeons perform surgical interventions to prevent morbidity and mortality.
- Application of the international league against rheumatism classification criteria for systemic juvenile idiopathic arthritis as a prognostic factor in patients with adults-onset Still's disease. [Journal Article]
- PRPediatr Rheumatol Online J 2018 Jan 25; 16(1):9
- CONCLUSIONS: In AOSD patients, there is fair concordance between the Yamaguchi and ILAR criteria for systemic JIA. Positive ILAR criteria may be useful for identifying AOSD patients at high risk for relapse, MAS and the need for ICU care. Further studies including larger populations from several centers are needed to confirm our results regarding the utility of the ILAR criteria in AOSD patients.
- Two Severe Cases of Adult-onset Still's Disease with Persistent Pruritic Eruptions. [Journal Article]
- ADActa Derm Venereol 2018 Jan 24
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- Generalized Purpura as an Atypical Skin Manifestation of Adult-onset Still's Disease in a Patient with Behçet's Disease. [Journal Article]
- ADActa Derm Venereol 2018 Jan 09