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Objective: Adult‐onset Still's disease (AOSD) and secondary hemophagocytic lymphohistiocytosis (sHLH) are both hyperferritinemic cytokine storm syndromes that can be difficult to distinguish from each other in hospitalized patients. The objective of this study was to compare the inflammatory markers ferritin, D‐dimer, C‐reactive protein (CRP), and soluble CD25 (sCD25) in patients with AOSD and sHLH. These four markers were chosen as they are widely available and represent different aspects of inflammatory diseases: macrophage activation (ferritin); endothelialopathy (D‐dimer); interleukin‐1/interleukin‐6/tumour necrosis factor elevation (CRP) and T cell activation (sCD25). Methods: This was a single‐center retrospective study. Patients diagnosed by the Hematology service at Vancouver General Hospital for AOSD or sHLH from 2009 to 2023 were included. Results: There were 16 AOSD and 44 sHLH patients identified. Ferritin was lower in AOSD than HLH (median 11 360 μg/L vs. 29 020 μg/L, p =.01) while D‐dimer was not significantly different (median 5310 mg/L FEU vs. 7000 mg/L FEU, p =.3). CRP was higher (median 168 mg/L vs. 71 mg/L, p <.01) and sCD25 was lower (median 2220 vs. 7280 U/mL, p =.004) in AOSD compared to HLH. The combined ROC curve using CRP >130 mg/L and sCD25< 3900 U/mL to distinguish AOSD from HLH had an area under the curve (AUC) of 0.94 (95% confidence interval 0.93–0.97) with sensitivity 91% and specificity 93%. Conclusions: These findings suggest that simple, widely available laboratory tests such as CRP and sCD25 can help clinicians distinguish AOSD from HLH in acutely ill adults with extreme hyperferritinemia. Larger studies examining a wider range of clinically available inflammatory biomarkers in a more diverse set of cytokine storm syndromes are warranted. [ABSTRACT FROM AUTHOR]

A recent study published in the Journal of Internal Medicine explores the use of IL-1β/DNA complex levels as a diagnostic tool for distinguishing autoinflammatory disorders from autoimmune and infectious diseases. The researchers developed a novel ELISA assay to measure the amount of IL-1β bound to extracellular DNA in plasma samples. They found that circulating IL-1β/DNA complex levels were significantly higher in patients with autoinflammatory disorders compared to those with autoimmune rheumatic diseases, acute infections, and healthy individuals. The assay also showed promise in evaluating treatment response in patients receiving IL-1 inhibitors. Further studies are needed to validate the diagnostic and prognostic utility of this assay in diverse inflammatory disorders. [Extracted from the article]

This document is a case report discussing a patient who presented with symptoms of Multisystem Inflammatory Syndrome in Children (MIS-C), a condition associated with COVID-19. The patient experienced persistent fever, rash, gastrointestinal symptoms, and neurocognitive dysfunction. They were initially diagnosed with Still's Disease but did not respond to treatment. The patient was eventually diagnosed with MIS-C based on the CDC criteria and treated with intravenous immunoglobulin (IVIG) and methylprednisolone. The symptoms improved with treatment. The authors acknowledge the patient and her parents for giving permission to publish the details of the case. [Extracted from the article]

Despite decades of research, Parkinson's disease is still an idiopathic pathology for which no cure has yet been found. This is partly explained by the multifactorial character of most neurodegenerative syndromes, whose generation involves multiple pathogenic factors. In Parkinson's disease, two of the most important ones are the aggregation of α‐synuclein and oxidative stress. In this work, we address both issues by synthesizing a multifunctional nanozyme based on grafting a pyridinophane ligand that can strongly coordinate CuII, onto biodegradable PEGylated polyester nanoparticles. The resulting nanozyme exhibits remarkable superoxide dismutase activity together with the ability to inhibit the self‐induced aggregation of α‐synuclein into amyloid‐type fibrils. Furthermore, the combination of the chelator and the polymer produces a cooperative effect whereby the resulting nanozyme can also halve CuII‐induced α‐synuclein aggregation. [ABSTRACT FROM AUTHOR]

Key Clinical Message: This case details adult‐onset Still's disease (AOSD) onset post‐human papillomavirus (HPV) vaccination and acute gastroenteritis. The timing of HPV vaccine and vaccine‐autoimmune disease literature may potentially confound the well‐established link between infections and AOSD onset. [ABSTRACT FROM AUTHOR]

This article, "Parkinson's disease: Still waiting for a cure," offers a unique perspective on living with Parkinson's disease. The author, who has Young Onset Parkinson's Disease, reflects on their experiences in regulatory and scientific meetings, the impact of the patient voice in drug development, and the challenges of living with a progressive disease. They emphasize the importance of a patient-centric approach in research and drug development, highlighting the need for disease-modifying interventions and the use of biomarkers in clinical trials. The author calls for urgency in the research process and emphasizes the importance of collaboration and data sharing. Overall, the article provides hope for the future of Parkinson's disease research and drug development. [Extracted from the article]

This article discusses a study that examines the potential use of soluble programmed death 1 (sPD-1) as a biomarker for predicting mortality in patients with adult-onset Still's disease (AOSD). AOSD is a systemic inflammatory disease with a varied clinical course, and currently, there are no established biomarkers for predicting prognosis. The study included 69 patients with AOSD and found that higher levels of sPD-1 were associated with worse survival outcomes. The authors suggest that sPD-1 could be a valuable indicator for mortality in patients with AOSD. However, further research is needed to confirm the usefulness of sPD-1 levels as an indicator of diagnosis and disease activity in AOSD patients. [Extracted from the article]

The article discusses Still's disease, also known as systemic juvenile idiopathic arthritis (SJIA), which is a complex autoimmune disorder affecting individuals of all ages. Recent advancements in research and treatment have led to more precise diagnostic tools and personalized treatment strategies, improving outcomes for patients. The use of anti-inflammatory medications and biologic therapies has reshaped the treatment landscape, offering hope for those with SJIA and adult-onset Still's disease (AOSD). Collaboration between pediatric and adult rheumatologists is crucial in providing comprehensive care and developing innovative treatment approaches. The principles of precision medicine, personalized care, and collaboration guide efforts to enhance the management of Still's disease and improve the lives of affected patients. [Extracted from the article]

This document is a collection of abstracts from the Australasian Journal of Dermatology. The articles cover a range of topics, including the diagnosis of bacterial skin infections, the economic viability of genotyping in epilepsy patients, and the diagnosis of fungal infections. Other articles discuss the complications of certain skin conditions, such as Stevens-Johnson Syndrome and toxic epidermal necrolysis, and the potential association between a COVID-19 vaccine and toxic epidermal necrolysis. The document also includes case studies and discussions on the treatment of different dermatological conditions, such as morphoea and lymphomatoid papulosis. Additionally, there are articles on the impact of bariatric surgery on dermatology drugs, the treatment of livedoid vasculopathy, and the role of interleukin-17 in pyoderma gangrenosum. [Extracted from the article]

Background: Adult‐onset Still's disease (AOSD) is a rare systemic inflammatory disorder that is characterized by quotidian fevers, arthritis, and an evanescent rash. Occurrence of concurrent thrombotic microangiopathy (TMA) in AOSD is rare. The treatment aspects of TMA in AOSD are actively being debated. Methods: Medline search using MeSH terms and snowballing yielded a total of 29 articles with co‐occurrence of AOSD and thrombotic thrombocytopenic purpura (TTP) including our own. Pooled data were synthesized for descriptive analysis. Results: Median age was 35 years with a majority of females (68.96%). A majority of these studies/patients were either Asian (34.48%) or Caucasian (31.03%). Concurrent TMA at the time of AOSD diagnosis was seen in 65.51% patients. Only 3/29 patients had ADAMTS13 level less than 10%, consistent with TTP and 3/29 were diagnosed with hemolytic uremic syndrome (HUS). The remainder were diagnosed clinically. Complication rate was high, and 15/29 (51.72%) patients died or had permanent neurological/renal/vision/gangrenous complications. Median and mean ferritin peak was observed to be higher (7458 and 12 349, respectively) in patients who either died/had partial remission, compared to those who had complete response (3257 and 10 899, respectively), p =.829. Conclusions: A majority of patients with AOSD‐associated TMA either died or had permanent complications. TMA was diagnosed alongside AOSD in 65% patients, while the rest developed TMA during the course of their disease. Blurred vision may precede TMA and could help risk‐stratify high‐risk AOSD patients clinically. Glycosylated ferritin remains low several weeks to months after disease remission and may be used to monitor severity of disease process. Further studies are necessary to confirm the existing vascular endothelial growth factor hypothesis in AOSD‐associated TMA. [ABSTRACT FROM AUTHOR]

Aim: This study addresses the challenge of predicting the course of Adult‐onset Still's disease (AoSD), a rare systemic autoinflammatory disorder of unknown origin. Precise prediction is crucial for effective clinical management, especially in the absence of specific laboratory indicators. Methods: We assessed the effectiveness of combining traditional biomarkers with the k‐medoids unsupervised clustering algorithm in forecasting the various clinical courses of AoSD—monocyclic, polycyclic, or chronic articular. This approach represents an innovative strategy in predicting the disease's course. Results: The analysis led to the identification of distinct patient profiles based on accessible biomarkers. Specifically, patients with elevated ferritin levels at diagnosis were more likely to experience a monocyclic disease course, while those with lower erythrocyte sedimentation rate could present with any of the clinical courses, monocyclic, polycyclic, or chronic articular, during follow‐up. Conclusion: The study demonstrates the potential of integrating traditional biomarkers with unsupervised clustering algorithms in understanding the heterogeneity of AoSD. These findings suggest new avenues for developing personalized treatment strategies, though further validation in larger, prospective studies is necessary. [ABSTRACT FROM AUTHOR]

This article in Experimental Dermatology discusses a study that investigated the potential use of serum leucine-rich α2-glycoprotein (LRG) as a biomarker for generalized pustular psoriasis (GPP). The study compared the levels of LRG in patients with GPP to those with other skin conditions and healthy individuals. The results indicated that serum LRG levels may be a useful biomarker for GPP, but further research is needed. The article provides detailed information about the study's methodology and the characteristics of the participants. However, it is important to note that the study has limitations due to its small sample size and retrospective design. [Extracted from the article]

Key Clinical Message: We report an extremely rare occurrence of adult‐onset Still's disease (AOSD) in a patient with ulcerative colitis. The possibility of autoinflammatory conditions such as AOSD should be considered when evaluating or treating symptoms suspected as side effects of the severe acute respiratory syndrome coronavirus 2 vaccination, regardless of the associated comorbidities. Summary: A woman in her 50s with a history of stable ulcerative colitis (UC) for 20 years, managed using salazosulfapyridine, presented with migratory rashes, spiking fever, edema, and joint pain that started 1 week after receiving the BNT162B2 mRNA vaccine against severe acute respiratory syndrome coronavirus 2 (SARS‐CoV‐2). Laboratory tests revealed extremely high serum ferritin levels. The patient was diagnosed with adult‐onset Still's disease (AOSD) based on the relevant classification criteria after ruling out other diseases. Detection of high levels of interleukin‐18, an inflammatory cytokine related to AOSD, supported the diagnosis. Nonsteroidal anti‐inflammatory drug monotherapy alone resulted in significant improvements in both the abovementioned symptoms and the elevated inflammatory marker levels. AOSD in a patient with UC is extremely rare. Only one case of AOSD with UC was reported before the coronavirus disease 2019 era. This case indicates that SARS‐CoV‐2 vaccination can trigger a hyperinflammatory response, classified as AOSD, in a patient with UC, which is extremely rare. [ABSTRACT FROM AUTHOR]

This article discusses a case of Methotrexate (MTX)-induced Macrophage Activation Syndrome (MAS) in a patient with Still's disease. MAS is a severe complication characterized by uncontrolled activation and proliferation of macrophages, leading to multi-organ dysfunction. The patient presented with symptoms such as mucosal bleeding, cracked lips, and a high fever. Laboratory tests revealed pancytopenia and increased levels of inflammatory markers. The patient was treated with methylprednisolone and subsequently with prednisolone and cyclosporine. The article emphasizes the importance of early recognition and treatment of MAS and highlights the potential association between MTX and MAS. Further research and reporting of similar cases are needed to improve patient care and outcomes. [Extracted from the article]

This article presents a case study of a man in his 30s who presented with a 6-day history of fevers, rash, pharyngitis, and joint pain. The patient was diagnosed with adult-onset Still disease (AOSD), a rare systemic idiopathic autoinflammatory disorder characterized by high spikes of daily fever, a salmon-colored rash, arthralgia, and neutrophilic leukocytosis. The article discusses the diagnostic criteria for AOSD and highlights the importance of recognizing flagellate erythema, a poor prognostic sign in AOSD. The patient was treated with colchicine, ibuprofen, and corticosteroids, and showed significant improvement at follow-up. [Extracted from the article]

Key Clinical Message: The present case indicates that cryoglobulinemia vasculitis should be considered in the differential diagnosis of purpura in patients with adult‐onset Still's disease (AOSD). The presence of purpura is suggested in adult‐onset Still's disease (AOSD) hematological complications of hemophagocytic syndrome, disseminated intravascular coagulation, or thrombotic microangiopathy. We herein report a case of AOSD complicated by cryoglobulinemia vasculitis presenting with purpura. [ABSTRACT FROM AUTHOR]

This case indicates that nocardiosis can aggravate the gravity of rheumatoid arthritis, so potential infection such as nocardiosis may need to be considered when treating difficult-to-treat arthritis. In addition to these, due to the high infectious tendency to CNS of nocardiosis, we suggest that patients with pulmonary nocardiosis or who are immunocompromised may need brain MRI examination even when typical CNS symptoms were not shown. As for treatment, SMZ-TMP is the optimal medication against nocardiosis, and a combination of drugs is required for patients with SMZ-TMP resistance or severe infections. The use of corticosteroids, immunosuppressants, and/or biologics in patients with rheumatic disease may suppress their immune system, and therefore induce opportunistic infections including nocardiosis. [Extracted from the article]

Aim: Intravenous tocilizumab (TCZ‐IV) was approved for the treatment of adult Still's disease (ASD) in Japan in May 2019 based on its efficacy and safety in a phase III randomized controlled trial. This study determined treatment patterns in patients with ASD and assessed oral glucocorticoid (GC) dose changes after TCZ‐IV administration in Japanese clinical practice. Methods: Patients in the Medical Data Vision database aged 16 years or older with one or more of International Classification of Diseases, 10th revision codes M061 (ASD) or M082 (systemic juvenile idiopathic arthritis) during January 2017–March 2021 (cohort 1) and those initiating TCZ‐IV during May 2019–March 2021 (cohort 2) were included. Results: In cohort 1, the proportion of patients who were prescribed interleukin‐6 inhibitors (mainly TCZ‐IV) increased from 10.8% (January–April 2019 [before TCZ‐IV approval]; n = 2002) to 18.3% (January–March 2021 [after TCZ‐IV approval]; n = 2008). In cohort 2 (n = 193), 84.5% of patients were on oral GCs (≤5 mg/day: 23.8%) at index date (initial TCZ‐IV prescription date); 46/70 (65.7%) were on oral GC at 5 mg/day or higher 12 months after TCZ‐IV treatment (primary outcome). After 12 months of treatment, the TCZ‐IV retention rate was 73.6% and the TCZ‐IV administration interval was every 4 weeks and every 2 weeks in 31.9% and 27.7% of patients, respectively. Conclusion: The use of interleukin‐6 inhibitors increased by 7.5% points in Japanese patients with ASD ~2 years after TCZ‐IV approval, suggesting that an unmet medical need existed. This study suggests the potential GC‐sparing effect of TCZ‐IV in patients with ASD in clinical practice. [ABSTRACT FROM AUTHOR]

Genetic architecture distinguishes systemic juvenile idiopathic arthritis from other forms of juvenile idiopathic arthritis: clinical and therapeutic implications. ANA-positive patients with persistent oligoarticular, extended oligoarticular, or polyarticular JIA have the same disease entity and differ only in disease severity.[4] Corroborating studies suggest that HLA associations and peripheral blood transcriptomic signatures differ at the boundary of 6 years old. Familial aggregation of juvenile idiopathic arthritis with other autoimmune diseases: impact on clinical characteristics, disease activity status and disease damage. Juvenile idiopathic arthritis (JIA) refers to a group of heterogeneous diseases sharing unknown original chronic inflammatory arthritis over 6 weeks. [Extracted from the article]

Key Clinical Message: Hemophagocytic lymphohistiocytosis (HLH) is a rare hematologic disease that occurs due to immune system dysfunction. Clinical manifestations of this disease are fever, increased ferritin level, cytopenia, and hemophagocytosis in the biopsy report of the bone marrow. We report a 36‐year‐old woman referred to our hospital with persistent fever, arthralgia in interphalangeal joints, and cutaneous rash on the trunk, was subsequently diagnosed as an adult‐onset Still's disease (AOSD), and after bone marrow aspiration, HLH was diagnosed with her. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Idiopathic multicentric Castleman disease (iMCD) is challenging to diagnose due to clinical similarities with other conditions, such as Still's disease. Once diagnosed, iMCD may be effectively managed with the anti‐interleukin‐6 antibody siltuximab. Here, we present the case of a 19‐year‐old Polish woman with persistent fever and enlarged lymph nodes and whose diagnosis remained inconclusive following initial clinical assessments and extensive laboratory analyses. The patient had subsequent complaints of joint pain and erythema which were suspicious of Still's disease and resolved with treatment with tocilizumab. Later, the progression of symptoms, such as lymphadenopathy, and elevated interleukin‐6 levels were consistent with Castleman disease, leading to the diagnosis of idiopathic multicentric Castleman disease seven years after the patient first reported symptoms. Treatment with the anti‐interleukin‐6 antibody siltuximab resulted in complete symptom resolution and normalization of inflammatory parameters. No adverse events were reported due to treatment with siltuximab. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Young patients with persistent rash and fevers despite antibiotic treatment should be evaluated for non‐infectious etiologies. In our patient's case, these findings led to a diagnosis of MAS, which ultimately affected how she was managed. Adult‐onset Still's disease (AOSD) is a rare, often difficult to diagnose autoimmune disease that typically presents as a rash, unresolving fevers and joint pains capable of mimicking a number of autoimmune diseases. Here, we present the case of a young postpartum woman whose clinical presentation, which included a pruritic maculopapular rash that evolved to include a flagellate component, and serological studies, chief among them cytopenias and a Ferritin >15,000 nm/mL) allowed us to make an early diagnosis of AOSD complicated by macrophage activation syndrome. We discuss the treatment for AOSD complicated by MAS with Hydrocortisone and Anakinra, the final discharge regimen prescribed for our patient, and report on her state 3 months post‐hospitalization, which was favorable. Our case is unique because we ultimately believe that pregnancy itself triggered her ASOD, because of how the quality of the flagellate component of her rash allowed us to narrow the differential diagnosis, and because of how the significant cytopenias and significant liver dysfunction alerted us to the possibility of MAS. [ABSTRACT FROM AUTHOR]

This article discusses a case report of a patient with Sjögren's disease (SjD) who also exhibited autoinflammatory features. SjD is an autoimmune disease characterized by the presence of autoantibodies and lymphocytic infiltration into affected glands. Autoinflammatory diseases, on the other hand, involve problems with innate immunity. The patient in this case had symptoms resembling adult Still's disease and Behçet's disease, as well as large-vessel involvement, which are uncommon in SjD. The presence of certain HLA class I alleles may have contributed to the development of these unique manifestations. Treatment with prednisolone and colchicine improved the symptoms. [Extracted from the article]

Key Clinical Message: Adult‐onset Still's disease is a rare inflammatory condition with diverse clinical features. Yamaguchi criteria aid diagnosis, and pleural effusion and elevated ferritin levels are important markers. Steroids are the first‐line treatment. Adult‐onset Still's disease (AOSD) is a rare systemic inflammatory condition with an unknown etiology. It is characterized by, spiking fever, arthritis, evanescent rash, sore throat, serositis, hepatomegaly, splenomegaly, and lymphadenopathy. It is a diagnosis of exclusion and has infections, systemic autoimmune and inflammatory rheumatic diseases, malignancy, and adverse drug reactions as its differential diagnosis. Because of these characteristics, diagnosis is frequently delayed, posing a significant challenge for physicians. While several classification criteria can be used to diagnose Still's disease, they have limitations in terms of sensitivity and specificity. The Yamaguchi criteria are considered the most sensitive and commonly used, requiring the presence of at least five characteristics, with at least two being major diagnostic criteria. Steroid therapy is the first‐line treatment for AOSD patients. In this case report, we present a 56‐year‐old female patient who developed pleurisy a few months after a car accident, subsequently diagnosed with adult‐onset Still's disease. [ABSTRACT FROM AUTHOR]

Finally, we diagnosed a VEXAS syndrome following COVID-19 vaccination. VEXAS syndrome following COVID-19 mRNA vaccination Dear Editor, VEXAS syndrome (vacuoles in myeloid progenitors, E1 ubiquitin-activating enzyme, X-linked, autoinflammatory manifestations and somatic) is a recently described adult-onset autoinflammatory disease.[1] A 76-year-old man was vaccinated against SARS-CoV-2 with the Tozinameran/BNT162b2 (Pfizer/BioNTech) on 1 April 2021. [Extracted from the article]

Key Clinical Message: The characteristics of liver dysfunction due to adult‐onset Still's disease are not specific. Differentiating from autoimmune hepatitis is important in deciding whether to continue corticosteroid therapy, and also in terms of management of cirrhosis and surveillance of hepatocellular carcinoma. Liver biopsy is thought to be the most important determinant for differential diagnosis. [ABSTRACT FROM AUTHOR]

What the genes tell us in the VEXAS syndrome? Tocilizumab for treatment of cutaneous and systemic manifestations of vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome without myelodysplastic syndrome. 7 Huang H, Zhang W, Cai W. VEXAS syndrome in myelodysplastic syndrome with autoimmune disorder. VEXAS syndrome in a female patient with constitutional 45,X (turner syndrome). [Extracted from the article]

Adult-onset Still's disease presenting with "pencil sketch-like" arrangement of papules and geographic pigmentation Adult-onset Still's disease is a rare entity with varied clinical manifestations. We encountered two cases of adult-onset Still's disease (AOSD) with bizarre pigmentation and grouped papules. [Extracted from the article]

Acquired hemophilia A (AHA) is a potentially life‐threatening hemorrhagic disorder with many etiologies. We report the first case in the literature describing the association of AHA with adult‐onset Still's disease (AOSD). [ABSTRACT FROM AUTHOR]

Adult‐onset Still's disease (AOSD) is an uncommon inflammatory disorder. AOSD and SARS‐Cov‐2 infection share clinical and laboratory features, including systemic inflammation. A 19‐year‐old woman had prolonged fever for 3 weeks, joint pain, and biological inflammatory syndrome. Post COVID‐19 AOSD was diagnosed. SARS‐Cov‐2 infection induces many inflammatory diseases including AOSD. COVID‐19 infection is a systemic disease mimicking auto‐inflammatory diseases and a differential diagnosis. SARS‐Cov‐2 infection induces many inflammatory diseases including adult‐onset Still's disease. [ABSTRACT FROM AUTHOR]

This clinical correspondence discusses a case of amyopathic dermatomyositis (ADM) with flagellate dermatitis in a 23-year-old male patient. The patient presented with erythematous papules on his face, limbs, and trunk, as well as a long flagellate streak on the left wrist and large erythematous lesions on the lower back. The patient had typical skin manifestations of dermatomyositis, and a diagnosis of ADM was established. Treatment with hydroxychloroquine sulfate tablets significantly improved the symptoms. Flagellate dermatitis is a rare rash characterized by intense pruritus and whiplash-like streaks, and its pathogenesis in DM is not well understood. This case highlights the diversity of skin presentations in patients with ADM and the importance of considering ADM in cases with negative myositis-specific antibodies. [Extracted from the article]

Aim: To analyze the clinical patterns of new‐onset inflammatory arthritis after COVID‐19 vaccination among patients without pre‐existing rheumatic or autoimmune diseases. Method: Case reports and series of new‐onset inflammatory arthritis after COVID‐19 vaccination were collected before April 2022. Clinical characteristics including diagnosis, age, gender, vaccine types, time interval between events, joint involvement (poly‐ or oligo−/monoarthritis), and laboratory data reflecting inflammatory status were sorted and P values between these parameters are calculated with independent sample Student's t test or 2 × 2 Fisher's exact test. Results: Among 39 cases with new‐onset post‐vaccination arthritis including 25 females and 13 males (1 unknown), the most common diagnosis is adult‐onset Still's disease (AoSD, 10 cases), and the most common vaccine types are BNT162b2 (16 cases) and AZD‐1222 (or ChAdOx1‐nCoV19, 15 cases). Sub‐analysis reveals that post‐vaccination polyarthritis is more common among females (P =.016, by 2 × 2 Fisher's exact test, compared with male patients) and older patients (P =.006, by Student's t test). The C‐reactive protein level is significantly higher in cases with post‐vaccination inflammatory polyarthritis than oligoarthritis (P =.029), as well as in cases with AoSD than other causes of post‐vaccination arthritis (P =.004). However, serum level of erythrocyte sedimentation rate in patients with post‐vaccination AoSD are independent of other clinical variables in the analysis. Conclusion: New‐onset post‐vaccination polyarthritis are more common in females and older patients. Although COVID‐19 vaccines may lead to inflammatory arthritis, the benefits of vaccination substantially outweigh the potential risks of such serious adverse effects due to their rarity. [ABSTRACT FROM AUTHOR]

Adult onset still's disease (AOSD) is a rare autoinflammatory disease displaying with a wide range of non‐specific symptoms and budd‐chiari syndrome (BCS) is an uncommon disorder characterized by obstruction of hepatic venous outflow. We present the case of a young patient who presented with persistent fever, sore throat, elbow, hand fingers and knees arthralgia with abdominal pain. The patient's symptoms had started 7 days before the referral. Imaging and laboratory data led to the diagnosis of BCS in the context of AOSD. The patient treated with corticosteroid in combination of warfarin with favorable outcome and complete improvement of signs and symptoms. We came to this conclusion AOSD complicated with BCS is a rare but potentially life‐threatening entity. Clinicians should be aware of this complication. [ABSTRACT FROM AUTHOR]

The phenomenon is known as Still's murmur.[2] Still published more than 100 scientific papers on diverse topics, such as rickets, tuberculosis and speech disorders. This is a rare multifactorial systemic autoinflammatory disease, characterised by high spiking fevers, an intermitted salmon-coloured I rash called i Still's rash and elevated ferritin levels. He was also chairman of the National Society for the Prevention of Infant Mortality from 1917 to 1937.[4] Still also worked in private practice in London and often waived his fee for patients if their families could not afford to pay. [Extracted from the article]

As illustrated in this case, a biopsy is essential to diagnose NUD and HUVS, but vasculitis may not be captured on biopsy which can hinder diagnostic certainty. Regarding her rash, our differential diagnoses were neutrophilic urticarial dermatosis (NUD) or hypocomplementemic urticarial vasculitis syndrome (HUVS), with vasculitis not captured on our biopsy secondary to sampling error. (b) Neutrophilic infiltrate of the dermis with no associated vasculitis (hematoxylin and eosin stain, ×100) gl At initial presentation, the patient had stage 2 hypertension (151/100 mmHg). [Extracted from the article]

Future studies and experiences are needed in order to determine the best strategy for allo-HSCT in patients with the VEXAS syndrome. Recently, a cohort of six patients who were retrospectively diagnosed with the VEXAS syndrome was reported and received allo-HSCT for severe inflammatory syndromes ( I n i = 4) or MDS ( I n i = 2).4 Of these patients, three showed long-term complete remission. Recently, Beck et al. reported a novel monogenic auto-inflammatory syndrome called the VEXAS syndrome (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic syndrome).1 A cohort of 25 men was found to have a myeloid lineage-restricted somatic mutation in I UBA1 i , a gene located on the X chromosome encoding the ubiquitin-activating enzyme 1. Azacitidine for patients with vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic syndrome (VEXAS) and myelodysplastic syndrome: data from the French VEXAS registry. [Extracted from the article]

Aim: Adult‐onset Still's disease (ASD) is a rare, potentially life‐threatening autoinflammatory condition. As reported prevalence shows regional variation and long‐term outcome data are scarce, we investigated epidemiology and long‐term health outcomes of ASD in Western Australia (WA). Methods: Population‐based cohort study using longitudinally linked administrative health data from all WA hospitals between 1999 and 2013 for ASD patients (ICD‐10‐AM M06.1) and controls matched for age, gender, and index year. Rate ratios and odds ratios (RR/OR) with 95% confidence intervals (CI) compared ASD patients with controls. Results: The average ASD incidence (n = 52) was 0.22/100 000 with 2.4/100 000 point‐prevalence as of December 31, 2013. ASD patients (median age 41.5 years, 59.6% female) had higher odds of previous liver disease (OR 2.67, 95% CI 1.31‐5.45), fever (OR 54.10, 95% CI 6.60‐433.0), rash (OR 15.70, 95% CI 4.08‐60.80), and serious infections (OR 4.36, 95% CI 2.11‐22.80) than controls. Despite biological disease‐modifying antirheumatic drugs in 27% of patients, ASD patients had higher odds for joint replacement (n = 7, 13.5%) (OR 45.5, 95% CI 4.57‐93.70), osteoporosis (OR 31.3, 95% CI 3.43‐97), and serious infections (RR 5.68; 95% CI 6.61‐8.74) during follow up. However, crude mortality (11.5% vs 7.5%; P = 0.34), survival at 1 and 5 years (P= 0.78), and last modified Charlson Comorbidity score (median 2 vs 2) were similar between groups. Conclusion: The epidemiology and demographics of ASD in Western Australia fall within the internationally reported range. ASD patients present increased rates of liver disease, rash, and serious infections before disease onset. Mortality following ASD was not increased for 5 years despite high rates of chronic arthritis requiring joint replacement, serious infections, and osteoporosis. [ABSTRACT FROM AUTHOR]

Aim: Macrophage activation syndrome (MAS), a severe complication of systemic adult‐onset Still's disease (AOSD), has been reported to occur during interleukin‐6 (IL‐6) inhibitor treatment. However, predictors for MAS development are unknown. Therefore, this study investigated predictive features for MAS development after starting IL‐6 inhibitor treatment in systemic AOSD patients. Method: In a single‐center retrospective study involving systemic AOSD patients who were refractory to high‐dose glucocorticoids with immunosuppressants and started IL‐6 inhibitor treatment between April 2008 and March 2020, we compared the baseline clinical features between patients who developed AOSD flare with MAS features (MAS group) and those who did not (non‐MAS group) during IL‐6 inhibitor treatment. Results: Only tocilizumab was used as an IL‐6 inhibitor. Six of 14 refractory systemic AOSD patients developed AOSD flares with MAS features during tocilizumab treatment, including 4 who developed them shortly after initiation. The MAS group had significantly lower neutrophil counts, fibrinogen, and higher IL‐18/C‐reactive protein (CRP) ratio at starting tocilizumab (baseline) than the non‐MAS group. Before starting tocilizumab, neutrophil counts were trending downward and upward in the MAS and non‐MAS groups, respectively, with significant differences in changes. Receiver operating characteristic analysis showed that baseline neutrophil counts and fibrinogen and their changes before tocilizumab treatment and baseline IL‐18/CRP ratio had significant discriminatory abilities for subsequent MAS development. Conclusion: We identified baseline laboratory features associated with MAS development after initiating an IL‐6 inhibitor in refractory systemic AOSD patients. These features may reflect the suppression of IL‐6 signaling, and further suppression of IL‐6 signaling might trigger early‐onset MAS. [ABSTRACT FROM AUTHOR]

The article discusses a case of a 39-year-old Mexican woman who presented with symptoms of fever, arthritis, and a rash. After evaluation, she was diagnosed with anti-Ku syndrome, a condition characterized by the presence of anti-Ku antibodies. This syndrome is challenging to categorize due to the lack of classification criteria and limited published information. The patient's symptoms resolved after treatment with prednisone and methotrexate. The article highlights the need to describe the clinical phenotype of patients with anti-Ku syndrome to establish accurate disease phenotypes, prognosis, and treatment. [Extracted from the article]

Systemic juvenile idiopathic arthritis (sJIA, also called Still's disease) is a rare childhood auto‐inflammatory disease with significant morbidity. This case report illustrates the clinical course and highlights diagnostic challenges. FDG‐PET/CT imaging may be beneficial in the diagnostic process for some cases, in order to achieve rapid diagnosis and early treatment. [ABSTRACT FROM AUTHOR]

Background: Rice body synovitis (RBS) is a rare disease. It is prone to be developed due to rheumatoid disorder or tuberculosis infection. Additional infectious arthritis (non‐tuberculous mycobacterial infection and fungal infection), juvenile arthritis, the onset of adult Still's disease, systemic lupus erythematosus (SLE), seronegative arthritis, and non‐specific arthritis. The clinical imaging, histopathological features, and surgical treatment process of a patient were documented combined with literature. Furthermore, differentiation was performed with additional synovitis diseases so that the cognition of synovitis could be enhanced for clinical reference. Case Presentation: The present study reported a 50‐year‐old female patient who suffered from intermittent left knee pain with limited movement for 9 years. The conditions were aggravated after long‐term standing or walking and remitted after taking a rest, accompanied by noose and jamming. The specialist range of motion (ROM) examinations of the left knee revealed: 30° ‐ 0° ‐ 110° and left McMurray sign (+). Plain MRI scanning revealed that in the left knee cavity and the popliteal fossa area, a large number of low signals on free rice‐like bodies were visible inside and the lower femur and the upper tibia exhibited abnormally high signals of patchy lipography. Surgical exploration revealed numerous rice‐like free bodies in the suprapatellar bursa, the intercondylar fossa, and the posterior articular capsule. The patient presently has resolution of symptoms after surgical treatment. Conclusions: The RBS of the knee joint is very rare in the clinic. As MRI examination can provide valuable information, clinicians should actively perform MRI examination. Once the disease is diagnosed by examination, surgery is the optimal treatment. [ABSTRACT FROM AUTHOR]

The association of Thrombotic thrombocytopenic purpura (TTP) and adult‐onset Still's disease (AOSD) is very uncommon. Hereby, we present a case of TTP occurring in patient with a known AOSD and the successful outcome after plasma exchanges. [ABSTRACT FROM AUTHOR]

Ménière's disease is an inner ear disorder, associated with episodes of vertigo, fluctuant hearing loss, tinnitus, and aural fullness. Ménière's disease is associated with endolymphatic hydrops. Clinical evidences show that this disease is often incapacitating, negatively affecting the patients' everyday life. The pathogenesis of Ménière's disease is still not fully understood and remains unclear. Previous numerical studies available in the literature related with endolymphatic hydrops, are very scarce. The present work applies the finite element method to investigate the consequences of endolymphatic hydrops in the normal hearing, associated with the Ménière's disease. The obtained results for the steady state dynamics analysis are in accordance with clinical evidences. The results show that the basilar membrane is not affected in the same intensity along its length and that the lower frequencies are more affected by the endolymphatic hydrops. From a clinical point of view, this work shows the relationship between the increasing of the endolymphatic pressure and the development of hearing loss. [ABSTRACT FROM AUTHOR]

A systematic review of autoimmune arthritis occurring after COVID-19 vaccination was conducted, identifying 39 cases in the literature, with 10 diagnosed as adult-onset Still's disease. The small number of cases compared to the global scale of vaccination raises ethical concerns about the balance between preventing deaths and potential adverse effects. The scientific community must investigate any possible adverse effects of vaccines thoroughly, including collecting samples and using a systems biology approach. The rates of disease flare-ups post-vaccination in patients with autoimmune rheumatic diseases are not different from unvaccinated individuals, suggesting that the cases of autoimmune arthritides may be occurring in addition to the background incidence. The vaccines may have led to the precipitation or initiation of autoimmunity, and the exact timeline for the development of arthritis after vaccination has not been standardized. It is unclear if the manifestations relapsed after resolution or became chronic. The safety of COVID vaccination in individuals with autoimmunity has been proven, suggesting that the vaccines are less likely to precipitate autoimmunity. However, further research and international collaboration are needed to document and follow up on autoimmune phenomena reported after COVID-19 vaccination. [Extracted from the article]

A 23‐year‐old woman followed for adult‐onset Still's disease (AOSD) presented fever and chest pain. Clinical examination showed erythematous papules suggestive of flagellate dermatitis. Laboratory findings showed increased Cardiac troponin. Myocarditis due to AOSD was therefore suspected. The patient was treated with prednisone and methotrexate with significant clinical improvement. [ABSTRACT FROM AUTHOR]

Objective: To investigate the relationship between interferon‐γ (IFN‐γ), IFN‐γ‐producing immunocompetent cells, their related cytokines, and the clinical features in adult‐onset Still's disease (AOSD). Methods: Twenty‐five patients with AOSD before initiating treatment (acute AOSD), 9 patients after remission (remission AOSD), and 12 healthy controls (HC) were included. Circulating IFN‐γ‐producing CD4+ and CD8+ cells, natural killer (NK) cells, and IFN‐γ production in NK cells were evaluated by flow cytometry. Serum levels of IFN‐γ, interleukin (IL)‐6, IL‐12, IL‐15, and IL‐18 were also measured. The obtained results were statistically analyzed with clinical findings. Results: Serum levels of IFN‐γ, IL‐6, IL‐12, IL‐18, intracellular expression of IFN‐γ in CD4+, CD8+, and NK cells were significantly higher in acute AOSD than in HC. The proportion of NK cells was significantly lower in acute AOSD than in HC. Serum levels of IFN‐γ and IFN‐γ expression in CD4+ cells were significantly correlated with serum ferritin levels. The proportion of NK cells had a significant inverse correlation with serum IFN‐γ levels. A lower proportion of NK cells was significantly noted in patients refractory to initial immunosuppressive treatment. In remission AOSD, serum levels of IL‐6, IL‐12, and IL‐18 were significantly higher than in HC. Conclusion: Increased serum levels of IFN‐γ, increased expression of IFN‐γ in CD4+ cells, and decreased NK cell proportion correlate with disease activity in AOSD. Moreover, a lower proportion of NK cells may be useful for predicting a refractory clinical course. Meanwhile, increased serum levels of IL‐6, IL‐12, and IL‐18 may persist after clinical remission. [ABSTRACT FROM AUTHOR]

A 38-year-old gentleman with a history of Adult-Onset Still's Disease (AOSD) presented with a 4-month history of hypopigmented patches on his dorsum hands before spreading to his face, trunk, and lower extremities. His medications included Tocilizumab, Cyclosporin, Prednisone for his AOSD as well as Span-K and Vitamin D. His AOSD had been in remission with no fevers. Thus we present a visually striking case of vitiligo developing in the same distribution as a previous AOSD eruption in a patient without a previous history of vitiligo. [Extracted from the article]

Adult‐onset Still's disease may cause intestinal pseudo‐obstruction via a cytokine storm. Early diagnosis and treatment are the key for patient survival before the development of serious complications such as macrophage activation syndrome. [ABSTRACT FROM AUTHOR]

LINKED CONTENT: This article is linked to Atia et al paper. To view this article, visit https://doi.org/10.1111/apt.17419 [ABSTRACT FROM AUTHOR]