scholarly journals Adult onset still’s disease: a diagnostic dilemma

2019 ◽  
Vol 6 (6) ◽  
pp. 1950
Author(s):  
Dugganapalli Dinesh Kumar Reddy ◽  
Veeraballi Sindhusha ◽  
Metta Jagadeesh
Keyword(s):  
Adult Onset Still’S Disease ◽  
Inflammatory Disorder ◽  
Adult Onset ◽  
Unknown Aetiology ◽  
Still’S Disease ◽  
High Grade Fever ◽  
Diagnostic Dilemma ◽  
Still's Disease ◽  
Clinical Triad ◽  
Adult Onset Still's Disease

Adult onset still’s disease is a rare systemic inflammatory disorder of unknown aetiology, characterized by clinical triad (high spiking fever, evanescent rash and arthritis) and biological triad with lack of serological markers as a true gold standard makes diagnosis difficulty. Here is a case of 32year old male presented with high grade fever for 2 months, joint pains and swelling for 1month rash for 3 days. O/E: pallor and B/L tender, swollen ankle and knee joints, and P/A: splenomegaly. Investigations showed-Hb%. 8 gms, neutrophilic leucocytosis with thrombocytosis, ESR:72 mm/1h, CRP elevated. ASO-titre, RA factor and Anti CCP antibodies are negative. Adult onset still’s disease is a heterogenous and rare systemic inflammatory disorder of unknown aetiology with lack of serological diagnostic modalities.

scholarly journals Adult onset Still’s disease in the elderly: a case-based literature review

2021 ◽  
Vol 5 (1) ◽  
Author(s):  
Arash Mollaeian ◽  
Jingjing Chen ◽  
Nina N. Chan ◽  
Gregory A. Nizialek ◽  
Christopher J. Haas
Keyword(s):  
Delayed Diagnosis ◽  
The Elderly ◽  
Adult Onset Still’S Disease ◽  
Inflammatory Disorder ◽  
Adult Onset ◽  
Still’S Disease ◽  
Diagnosis And Management ◽  
Still's Disease ◽  
Area Of Interest ◽  
Adult Onset Still's Disease

Abstract Background Adult onset Still’s disease (AOSD) is a rare inflammatory disorder that classically presents with high spiking fevers, evanescent rash, and arthritis. The diagnosis is one of exclusion and can be further complicated by atypical presentations, particularly in elderly patients in whom AOSD is very rare. Case presentation A case of AOSD in a 73-year-old woman with a non-classic presentation, leading to delayed diagnosis and management, is presented along with a review of the English literature for AOSD cases in elderly people over 70 years of age. Thirty nine case reports and series were identified and the current case was added, totaling 42 individual cases. Significant findings included a four-times higher prevalence in females, a higher prevalence of macrophage activation syndrome despite lower mortality, the presence of pruritic rash in almost one fifth of the cases, and high prevalence of delayed diagnosis. Conclusions AOSD in the elderly may vary from the classic criteria described in the medical literature and may lead to delayed diagnosis and management. Further evaluation and better characterization of AOSD in the elderly remains an area of interest.

scholarly journals Adult-onset Still’s disease, the most internistic of the rheumatic diseases: current concepts and a report of six cases

2013 ◽  
pp. 259-264
Author(s):  
Roberto Boni ◽  
Pier G. Rabitti
Keyword(s):  
Serum Ferritin ◽  
Hemophagocytic Syndrome ◽  
Unknown Origin ◽  
Essential Elements ◽  
Adult Onset Still’S Disease ◽  
Inflammatory Disorder ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Adult Onset Still's Disease

Adult-onset Still’s disease (AOSD) is a rare systemic inflammatory disorder of unknown origin. It is characterized by spiking fever, evanescent rash, arthralgia/arthritis, and leukocytosis. The differential diagnosis includes a number of other conditions, and management is complicated by the lack of course predictors and the risks associated with complications and treatments. This report examines recent advances in our understanding of adult-onset Still’s disease (pathogenesis, diagnosis, complications, treatment). Current research in this field is focused on the significance of serum ferritin in AOSD, mechanisms underlying the hemophagocytic syndrome, and use of biologic therapies in patients who are refractory to conventional treatment. Six cases of AOSD diagnosed by our staff between 2002 and 2009 are also analyzed and compared with other cases reported in the literature. This analysis showed that Still’s rash and serum ferritin levels were not essential elements for diagnosis. In addition, the course of the disease showed little relation to the severity / characteristics of the presenting picture, but the evolution worsened with the age of the patient at diagnosis.

scholarly journals Pulmonary Onset of Adult Onset Still’s Disease Complicated with Kikuchi Lymphadenitis

2020 ◽  
Vol 2020 ◽  
pp. 1-6
Author(s):  
G. D. E. Kodithuwakku ◽  
C. L. Fonseka ◽  
S. Nanayakkara ◽  
H. M. M. Herath
Keyword(s):  
Ferritin Level ◽  
Severe Pneumonia ◽  
Poor Response ◽  
Complete Recovery ◽  
Adult Onset Still’S Disease ◽  
Inflammatory Disorder ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Adult Onset Still's Disease

Background. Adult onset Still’s disease (AOSD) is a rare inflammatory disorder with a variety of clinical presentations. Even though pneumonitis and pleurisy are known to occur in AOSD, pulmonary onset presentations are exceedingly rare. Case Presentation. We present a 40-year-old male, presenting with fever and bilateral alveolar shadows with pleural effusions mimicking community-acquired severe pneumonia. He was initially treated as severe pneumonia with poor response to broad spectrum antibiotics. Subsequently, he was managed as AOSD-induced pneumonitis, as he fulfilled Yamaguchi criteria. Few weeks later, he developed macular rash and arthralgia with generalized lymphadenopathy with lymph node histology, showing Kikuchi lymphadenitis. He responded well to steroids and had a complete recovery. Conclusion. Non-infective causes of pneumonitis should be suspected in the setting of poorly resolving pneumonias, especially when microbiological and serological investigations does not support an infective etiology. Presence of systemic symptoms with arthralgia, rash, and disproportionately elevated ferritin level supports the diagnosis of AOSD. Kikuchi lymphadenitis is a reported association with AOSD, and there could be a causal link between the two disorders.

AB1031 DYSKERATOTIC CELLS IN PERSISTENT PRURITIC SKIN LESIONS AS A PROGNOSTIC FACTOR IN ADULT-ONSET STILL’S DISEASE.

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1808.1-1808
Author(s):  
S. Inotani ◽  
Y. Taniguchi ◽  
H. Nishikawa ◽  
N. Maeda-Aoyama ◽  
K. Nakajima ◽  
...  
Keyword(s):  
Inflammatory Infiltrate ◽  
Skin Lesions ◽  
Terminal Deoxynucleotidyl Transferase ◽  
Adult Onset Still’S Disease ◽  
Inflammatory Disorder ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Pruritic Skin ◽  
Adult Onset Still's Disease

Background:Adult-onset Still’s disease (AOSD), a systemic inflammatory disorder, is characterized by high fever, evanescent rash, arthritis, and hyperferritinaemia. AOSD is also reported to be associated with other skin lesions, including persistent pruritic papules and plaques.Objectives:This study aimed to assess the significance of dyskeratotic skin lesions in Japanese AOSD patients.Methods:We retrospectively assessed the histology of persistent pruritic skin lesions and evanescent rashes and the relationship between dyskeratotic cells, serum markers, and outcomes in 20 Japanese AOSD patients, comparing AOSD histology with that of dermatomyositis (DM), drug eruptions, and graft-versus-host disease (GVHD).Results:Persistent pruritic lesions were characterized by scattered single keratinocytes with an apoptotic appearance confined to the upper layer of the epidermis and horny layer without inflammatory infiltrate. In contrast to AOSD, the histology of DM, drug eruption, and GVHD demonstrated dyskeratotic cells in all layers of the epidermis with inflammatory infiltrate. AOSD with evanescent rash showed no dyskeratotic cells. The dyskeratotic cells in pruritic AOSD lesions stained positive for ssDNA and terminal deoxynucleotidyl transferase-mediated dUTP nick end-labeling, indicating apoptosis. Serum IL-18 was significantly higher in AOSD patients with dyskeratotic cells than those without, and generally required higher doses of glucocorticoids, immunosuppressants, and biologic agents. Two of ten AOSD patients with dyskeratotic cells died from haemophagocytic lymphohistiocytosis.Conclusion:Persistent pruritic AOSD skin lesions are characterized by dyskeratotic cells with apoptotic features, involving the upper layers of the epidermis. There may be a link to elevated IL-18. This dyskeratosis may be a negative prognostic indicator.Disclosure of Interests:None declared

scholarly journals SAT0544 USE OF BIOLOGICAL DMARDS IN PATIENTS WITH ADULT-ONSET STILL’S DISEASE: RESULTS FROM TURKBIO REGISTRY

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1229.1-1229
Author(s):  
A. Yazici ◽  
E. Dalkiliç ◽  
M. Birlik ◽  
M. A. Öztürk ◽  
S. Akar ◽  
...  
Keyword(s):  
Disease Duration ◽  
Biological Agents ◽  
Adult Onset Still’S Disease ◽  
Inflammatory Disorder ◽  
Adult Onset ◽  
Still’S Disease ◽  
Switching Rate ◽  
Drug Survival ◽  
Still's Disease ◽  
Adult Onset Still's Disease

Background:Adult-onset Still’s disease (AOSD) is a rare multisystemic inflammatory disorder, and is diagnosed by exclusion. AOSD is generally treated with nonsteroidal antiinflammatory drugs, corticosteroids, and conventional disease modifiying antirheumatic drugs (cDMARDs). Biological disease modifiying antirheumatic drug (bDMARD) therapy are recommended in AOSD patients who are refractory to tradional therapy, and bDMARDs is becoming increasingly important in AOSD treatment.Objectives:To evaluate the use of bDMARDs and drug survival in AOSD patients.Methods:TURKBIO registry is the Turkish version of Danish DANBIO rheumatological database which has been established in 2011. All patients with AOSD who received biological agents registered in TURKBIO registry between dates of October 2011 and October 2019 were included in this study. The demographic data, response of therapy, frequency of using and switching biological agents were collected.Results:As of October, 21 AOSD patients were recruited. Mean age of patients was 34.6±7.3 (min-max: 24-49) years, mean disease duration was 9.3±7.4 (min-max: 1-22) years, and 57.1% of patients was female. Mean duration from onset to start of bDMARDs was 7±6.1 (min-max: 0.5-21) years. It was observed that 13 patients (61.9%) received tocilizumab (TCZ), 6 patients (28.6%) received IL-1 inhibitors (5 anakinra and one canakinumab), 2 patients (9.5%) received certolizumab and one patient (4.8%) received etanercept as a first-line bDMARDs. The most frequently used biological agents in current treatment were as follows: 52.4% of patients received TCZ and 33.3% received IL-1 inhibitors (4 anakinra, 3 canakinumab), and the most frequently used concomitant drugs were methotrexate (47.6%) and hydroxychloroquine (14.3%). The switching rate was 33.3%, and in half of them the reason of switching was adverse events. The median drug survival for bDMARDs was 28.6 months (Table).Table.Demographic, laboratory features and management of AOSD(median;25-75)n=21Age (year)34.7 (28.3-40.6)Gender (Female) n(%)12 (57.1)Disease duration (year)8 (2-17)Duration from onset to start of bDMARs (year)6 (1.5-10)ESR (on onset)44 (21-66)CRP (on onset)65 (3.1-108)Current bDMARDs n(%) Tocilizumab11(52.4) IL-1 inhibitors7 (33.3) Etanercept1 (4.8) Certolizumab2 (9.5)Concomitant cDMARD n(%) Methotrexate10 (47.6) Leflunomide4 (19) Sulfasalazine1 (4.8) Hydroxychloroquine3 (14.3)bDMARDs Survival (months)28.6 (5.5-75)Switching Rate n(%)7 (33.3)Adverse Event n(%)3 (14.3)Conclusion:This is the first evaluation of AOSD patients who used biological agents from TURKBIO registry. According our data, TCZ and anti-IL1 agents were the most frequent biological choices. The limitation of this study was the low number of the patients with AOSD who used biological agents.References:[1].Zhou S, Qiao J, Bai J, Wu Y, Fang H. Biological therapy of traditional therapy-resistant adult-onset Still’s disease: an evidence-based review. Ther Clin Risk Manag 2018;14:167-71.Acknowledgments:NoneDisclosure of Interests:None declared

scholarly journals Adult Onset Still’s Disease Associated withMycoplasma pneumoniaeInfection and Hemophagocytic Lymphohistiocytosis

2016 ◽  
Vol 2016 ◽  
pp. 1-4 ◽  
Author(s):  
Abhishek Agnihotri ◽  
Allison Ruff ◽  
Lauren Gotterer ◽  
Addie Walker ◽  
Amy H. McKenney ◽  
...  
Keyword(s):  
Hemophagocytic Lymphohistiocytosis ◽  
The Body ◽  
Adult Onset Still’S Disease ◽  
Inflammatory Disorder ◽  
Adult Onset ◽  
Still’S Disease ◽  
Still's Disease ◽  
Elevated Liver Enzymes ◽  
Mycoplasma Pneumonia ◽  
Adult Onset Still's Disease

Adult Onset Still’s Disease (AOSD) is a systemic inflammatory disorder that can be associated with hemophagocytic lymphohistiocytosis (HLH), a rare but potentially fatal disease of overactive histiocytes and lymphocytes. We present a unique case of AOSD complicated byMycoplasma pneumoniainfection and HLH. A 28-year-old female developed joint pains followed by a diffuse, erythematous, pruritic skin rash that quickly spread throughout the body. The patient deteriorated and developed fever, chills, cough, and dyspnea and had to be intubated. She had hypoalbuminemia, elevated liver enzymes, a very high serum ferritin level, positive anti-Mycoplasma pneumoniaIgG and IgM antibodies, and normal rheumatoid factor and anti-nuclear antibodies. The chest X-ray showed diffuse bilateral infiltrates. Bone marrow biopsy revealed hemophagocytosis. The patient was treated with azithromycin, methylprednisolone, and anakinra and was discharged home on cyclosporine and prednisone. This case highlights that patients can develop features of both AOSD and HLH at the beginning of the disease and early diagnosis and treatment increase the likelihood of recovery.

scholarly journals Hemophagocytic syndrome, a rare variant of Still’s disease

2017 ◽  
Vol 5 (10) ◽  
pp. 4649
Author(s):  
Neha Bhave ◽  
Dinesh Gupta ◽  
Prashant Kashyap
Keyword(s):  
Musculoskeletal Diseases ◽  
Adult Onset Still’S Disease ◽  
Unknown Etiology ◽  
Inflammatory Disorder ◽  
Adult Onset ◽  
Still’S Disease ◽  
Diagnostic Challenge ◽  
Serological Tests ◽  
Still's Disease ◽  
Adult Onset Still's Disease

Adult onset still’s disease is a rare systemic inflammatory disorder of unknown etiology that is responsible for a significant number of cases of fever of unknown origin (FUO) and musculoskeletal diseases. The diagnosis in adult onset still’s disease is mainly clinical and requires exclusion of other infections. Laboratory tests are nonspecific and treatment mainly comprises of corticosteroids, NSAIDS, immunosuppressive drugs, iv gamma globulin, anti-tumour necrosis factor, anti-interleukin. AOSD (adult onset stills disease) is a diagnostic challenge. Discovery of new serological tests and a specific diagnostic criterion may help the clinician in faster diagnosis and better management of the disease. 

scholarly journals Case Report: Adult Onset Still’s Disease after vaccination against Covid-19

2021 ◽  
Vol 6 ◽  
pp. 333
Author(s):  
Ujjwol Risal ◽  
Anup Subedee ◽  
Raju Pangeni ◽  
Rakshya Pandey ◽  
Suravi Pandey ◽  
...  
Keyword(s):  
Adult Onset Still’S Disease ◽  
Temporal Association ◽  
Adult Onset ◽  
Still’S Disease ◽  
High Grade Fever ◽  
Serious Adverse Events ◽  
Mortality And Morbidity ◽  
Still's Disease ◽  
Adult Onset Still's Disease ◽  
Multiple Antibiotics

Vaccination against the virus responsible for COVID-19 has become a key in preventing mortality and morbidity related to the infection. Studies have shown that the benefits of vaccination outweigh the risks. However, there are concerns regarding serious adverse events of some vaccines,      although they are fortunately      rare. Here, we report a case of a 47-year-old female from Kathmandu who presented with high grade fever, dry cough and erythematous rash a week after exposure to the Oxford-AstraZeneca vaccine. She had hepatosplenomegaly, persistent leucocytosis, anaemia and thrombocytosis along with markedly raised inflammatory markers. Her tests for infectious causes and haematological malignancies was negative and she showed no response to multiple antibiotics. Finally, she had a dramatic response to steroids with disappearance of fever and normalization of other laboratory parameters. Hence, she was diagnosed with       Adult-onset Still’s      Disease (AOSD). She was under methotrexate and prednisolone tapering dose and doing well as at time of writing. The trigger for the disease was hypothesized to be the vaccine because of the strong temporal association.

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