Adult Onset Still Disease

Introduction. Adult Onset Still Disease (AOSD) is a systemic inflammatory disease, of unknown etiology, affecting young adults. It is, at least in part, a diagnosis of exclusion. Characterized by high fever with spikes, with or without complete defervescence outside of said spikes, arthritis, and evanescent rash. Also, sore throat, hepatomegaly, splenomegaly, serositis, lymphadenopathy might be present. Fatal risk is mainly attributed to Macrophage Activation Syndrome. Biologically its main trait is a marked inflammatory syndrome with unusually elevated ferritin levels. Case presentation. 31 year old male, known with a history of alopecia areata, treated with Diprophos, last dose one month prior to admission, with fever, odynophagia, sore throat, diffuse myalgia, debuted twelve days before. On blood samples, at admission and during his hospital stay, marked inflammatory signs are present (elevated ESR up to 100mm/h, C-reactive protein up to 37.4mg/dl and marked ferritin of 6240 ng/ml) accompanied by leukocytosis with neutrophilia, lymphopenia, low grade normochromic, normocytic anemia, thrombocytosis, slightly elevated liver function tests, cholestasis (GGT 502 U/L, ALKP 255 U/L) with normal bilirubin, D-dimers over 3000 with lowering values to 1344, spontaneous INR at 1.57. Normal values for rheumatoid factor, no antinuclear antibodies present, negative serology for hepatitis B, C, HIV, atypical bacterial infections (Chlamydia, Coxiella, Mycoplasma) Epstein-Barr Virus, syphilis, vasculitis markers (pANCA, cANCA) and anti-double stranded DNA, all coupled with 5 different hemocultures and 3 different urocultures all negative. Repeated ENT evaluation was within normal, chest X-Ray, echocardiography and CT scans of neck, thorax and abdomen reported back no significant abnormalities. Thus, by applying Yamaguchi criteria, after a complex evaluation of the case, the diagnosis is AOSD, and during his stay at the Internal Medicine department of Col ea Clinical Hospital, despite antiinflammatory, antithermic, antibiotic and corticosteroid treatment, the patient remains symptomatic, with high fever (up to 38.8 degrees Celsius) with chills and diffuse myalgias. At the indication of a rheumatologist, inside a specialty clinic, pulse-therapy with Methylprednisolone is initiated (500mgs a day for 5 days) with fever remission for more than 72 hours. The corticosteroid treatment is continued at home, with the patient self-monitoring for symptoms. Two months after corticoid therapy was initiated, the patient is almost without any symptoms (alopecia areata still present partially) and the inflammatory syndrome is greatly diminished. Conclusion. We present the case of a 31 year old male, with AOSD, defined by Yamaguchi criteria, with persistent symptomatology, mainly as high fever, without response to usual treatment, with the exception of pulse-therapy with Methylprednisolone. The diagnosis proved to be difficult, in part being one of exclusion, but also the clinical presentation, so unspecific, might easily lead to a different diagnosis.

ADULT ONSET STILL’S DISEASE PRESENTING AS PYREXIA OF UNKNOWN ORIGIN

Adult onset Still’s disease (AOSD) is a rare inammatory disease characterized by fever, arthralgia, rash, sore throat, lymphadenopathy, hepatomegaly and/or splenomegaly. It is a rare cause of pyrexia of unknown origin (PUO) and should be considered once the more common infectious, rheumatological and malignant causes have been excluded. This is usually a diagnosis of exclusion. Yamaguchi criteria can be used for diagnosis. Patients of AOSD show an excellent response to Non-steroidal anti-inammatory drugs (NSAIDs), however some may not have a sustained effect, in which case glucocorticoids have to be considered. Here’s a case of a young male patient who was evaluated for the cause of pyrexia of unknown origin.

Adult-onset Still’s disease in a 73-year-old Maltese man

Adult-onset Still’s disease (AOSD) is a rare autoinflammatory condition diagnosed by Yamaguchi criteria. We report an atypical presentation of a 73-year-old man, who was admitted with fever, sore throat and pleurisy that were unresponsive to three courses of antibiotics. Fever persisted in a quotidian pattern and the typical salmon-coloured rash consistent with AOSD appeared at 4 weeks from symptoms onset. These features in addition to neutrophilia and hyperferritinaemia in the absence of concurrent infectious and neoplastic causes satisfied Yamaguchi criteria for a diagnosis of AOSD. Epstein-Barr antigen was initially detected at moderate titre levels, but was undetectable after 1 week. Complete resolution of symptoms was reported on initiation of steroid treatment. A relationship between disease onset and viral syndromes has been documented to occur and few similar cases preceded by Epstein-Barr virus detection have been reported.

Adult Onset Still´s Disease (AOSD): A rare condition with a classic presentation. Case Report

Introduction: Adult Onset Still´s Disease (AOSD) is a rare systemic inflammatory disease of unclear etiology, with low incidence and prevalence among the general population. AOSD is a common cause of fever of unknown origin (FUO) in up to 20% of cases. Due to the scarce knowledge about this disease and its diagnosis, it is usually unrecognized in the differential diagnoses, worsening the prognosis and increasing complications in some patients.Case presentation: This is the case of a 32-year-old female patient with prolonged febrile illness, who did not respond to the antimicrobial treatments previously established. She was diagnosed with AOSD according to the Yamaguchi criteria after an extensive exclusion process. She was treated with first-line treatment with corticosteroids, achieving satisfactory results.Conclusions: The diagnosis of AOSD is an exhaustive process. Regardless of the availability of cutting-edge diagnostic tools, the medical history of the patient and an adequate physical examination are the most important aspects to consider.

AB1016 RATE OF PREDEFINING DISEASE ACTIVITY, REMISSION AND RELAPSE OF ADULT-ONSET STILL’S DISEASE: SYSTEMATIC LITERATURE REVIEW

Background:Adult-onset Still’s disease (AOSD) is a rare, multisystem and auto-inflammatory disorder. Although several scoring systems are proposed to describe AOSD disease activity, there is no agreement on the definition of disease activity, remission or relapse of AOSD.Objectives:Aim of this literature review was to determine the rates of defining ‘’disease activity’’, ‘’remission’’ and ‘’relapse’’ of AOSD.Methods:We performed a systematic review (May 2019) on Pubmed by using the MeSh word ‘’ Adult-onset Still’s disease’’; results were restricted to human studies and English language. We excluded case reports, reviews and studies including less than 10 patients; 215 articles were recruited for final assessment. Of this 215 article, we reached the full-texts of the 181 articles. Final assessment was done with 181 articles. In these articles, we searched whether the definitions of disease activity, remission or relaps were made or not.Results:Total of 181 articles were included to analysis. Mean age was 37.2±6.6 and 73.6% of patients was female. Articles were devided to 4 main groups according to main focus related to AOSD: Observational studies, laboratory studies, genetic studies and clinical trials. Diagnosis of AOSD was according to Yamaguchi criteria in 159 (87.8%) of articles and Yamaguchi criteria was the most commonly used criteria set. Disease activity was defined in 96 (54.1%) of articles. Remission and relapse were defined in 52 (28.7%) and 18 (10%) of articles, respectively. Most commonly used definition of disease activity was the ‘’systemic score’’ which was defined by Pouchet et al. Rates of defining ‘’disease activity’’, ‘’remission’’ and ‘’relapse’’ according to 4 main groups were given in Table 1.Table 1.Rates of disease activity, remission and relapse reporting based on 4 main article groupsArticle groupn (%)Disease activity (n,%)pRemission (n,%)pRelapse (n,%)pObservational Studies70 (38.7)18 (25.7)<0.00117 (24.3)<0.0018 (11.4)<0.001Laboratory Studies72 (39.8)46 (63.9)15 (20.8)2 (2.8)Genetic Studies17 (9.4)5 (29.4)2 (11.8)0 (0)ClinicalTrial22 (12.2)14 (63.6)18 (81.8)8 (36.4)Conclusion:In recent years, new treatment options become available for AOSD. However, data about how to measure the AOSD activity in largely missing. Definitions of disease activity, remission or relapse are missing in the most of the studies, including clinical trials, in current literature. Future studies are needed for this issue.References:NoneDisclosure of Interests:Emre Bilgin: None declared, Umut Kalyoncu Consultant of: Abbvie, Amgen, Janssen, Lilly, Novartis, UCB

A case report of Adult-onset Still’s disease presenting with acute fibrinous and organising pneumonia

Adult-onset Still’s disease is a rare inflammatory disorder characterised by fever, arthritis and rash. It can present in a number of ways and is associated in 5% of cases with parenchymal lung involvement. We present the case of a 37-year-old man who initially presented with fever, weight loss and pancytopaenia. He gradually deteriorated requiring non-invasive ventilation with a Computerised tomography of his chest showing bilateral nodular infiltrates. An open lung biopsy showed acute fibrinous organising pneumonia, which responded well to corticosteroid treatment. He then re-presented over three years later with a similar systemic illness although with less severe lung involvement. Following extensive further investigations, he was diagnosed with Adult-onset Still’s disease fulfilling the Yamaguchi criteria. We feel this case is important due to the rare association of Adult-onset Still’s disease and interstitial lung disease. More specifically, we are not aware of any published cases of Adult-onset Still’s disease with acute fibrinous organising pneumonia.

Clinical characteristics and comorbidities in adult-onset Still’s disease using a large US administrative claims database

Objectives We aimed to describe clinical characteristics, treatment patterns and major comorbidities of a US-based adult-onset Still’s disease (AOSD) cohort. Methods Administrative claims data from Truven MarketScan were collected from 2009 to 2015. An AOSD case was defined as ≥1 M06.1 International Classification of Diseases 10th revision (ICD-10) medical claim code. We extracted data for the AOSD cohort (n = 106) and 1:5 matched controls (n = 530) without AOSD. Outcomes of interest and a novel claims-based set of Yamaguchi criteria were identified by relevant ICD 9th revision (ICD-9) and ICD-10 codes. Bivariate descriptive analyses were conducted on all variables. Comorbidity rates and rate ratios were calculated in AOSD cases and matched controls. Statistical significance of cohort differences was determined to compare AOSD cases and matched controls. Results The AOSD cohort, with a mean age of 43.08 (standard deviation, s.d. 13.9) years and with female predominance (68.9%) was observed over a mean of 750.12 (637.6) days. A total of 35.9% of AOSD patients fulfilled claims-based Yamaguchi criteria compared with 0.4% matched controls (P&lt; 0.05). We identified severe AOSD-related complications, including macrophage activation syndrome (4.7%) and acute respiratory distress syndrome (12.3%). Treatment commonly involved systemic glucocorticoids (62.2%), MTX (51%) and anakinra (24.5%). Compared with matched controls, serious infections were significantly increased (rate ratio 2.58, 95% CI: 1.53, 4.37, P = 0.0004), while hyperlipidaemia (0.54, 95% CI: 0.35, 0.85; P = 0.008) and obesity (0.30, 95% CI: 0.15, 0.62; P = 0.001) were significantly decreased in AOSD patients. Conclusion We characterized a first US-based AOSD cohort using a large national administrative claims database, and identified key complications, treatments and comorbidities.

Adult-onset Still’s disease with macrophage activation syndrome diagnosed and treated based on cytokine profiling: a case-based review

Adult-onset Still’s disease (AOSD) is a relatively rare systemic inflammatory disorder and is diagnosed using various sets of classification criteria, with the Yamaguchi criteria as the most widely used criteria. Herein, we present the case of a 21-year-old woman admitted with a high fever, lasting for over 1 month, who did not fulfill the Yamaguchi criteria. However, by analyzing the inflammatory cytokine profile, we defined this case as AOSD based on a greatly elevated serum interleukin-18 level. In addition, we predicted the occurrence of macrophage activation syndrome by a characteristic increase in the soluble tumor necrosis factor receptor II level, which allowed a timely intervention for this malicious complication. Therefore, we suggest that cytokine profiling will be useful for the diagnosis and management of AOSD.

Expand the differential…think beyond rheumatoid arthritis

A 31-year-old male patient with severe, migratory arthralgias presented to our academic medical centre after being erroneously diagnosed and treated for rheumatoid arthritis for over 1 year. Multiple immunomodulatory therapies for rheumatoid arthritis were attempted with no relief of symptoms. Eventually, the pain was so bothersome that the patient became bedridden for 1 month prior to presenting to our facility. Our assessment revealed that the patient met the diagnostic criteria, known as the Yamaguchi criteria, needed to diagnose adult-onset Still’s disease. Yamaguchi criteria include migratory inflammatory arthritis, quotidian fevers, leucocytosis and a salmon-coloured maculopapular rash. These signs and symptoms may go unnoticed or overlooked if adult-onset Still’s disease is not considered. The patient was treated with anakinra (a recombinant human IL-1 receptor antagonist) and had rapid improvement in his symptoms, with the restoration of mobility.