Adult-onset Still's Disease: A Case Report

Adult-onset Still’s Disease is a rare auto inflammatory disorder of unknown etiology characterized mainly by high spiking fever, arthritis, evanescent rash and lymphadenopathy. It is a form of systemic onset juvenile rheumatoid arthritis that is encountered in adults, typically between 15-25 years and 36-45years. We here describe a 28 years lady with fever, arthritis of multiple large joints, lymphadenopathy and rash, with negative Rheumatoid factor and evidence of past infection with Ebstein-Barr virus and Parvovirus B19. History, examination findings and investigations showed several features consistent with adult-onset Still’s disease along with high ferritin level. After exclusion of probable other diagnosis and use of Yamaguchi criteria, she was diagnosed with adult-onset Still’s disease. All the major and minor criteria of Yamaguchi for diagnosis were met. Her disease responded well with steroid, she achieved remission and is currently under maintenance therapy.

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

Case Reports in Rheumatology ◽

10.1155/2020/8327068 ◽

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.

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Hemophagocytic syndrome, a rare variant of Still’s disease

International Journal of Research in Medical Sciences ◽

10.18203/2320-6012.ijrms20174615 ◽

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.

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A rare clinical presentation: Adult-onset Still’s disease

Journal of Kathmandu Medical College ◽

10.3126/jkmc.v9i1.33546 ◽

2020 ◽

Vol 9 (1) ◽

pp. 56-59

Author(s):

Prakash Banjade ◽

Prakash Poudel Jaishi ◽

Jonas Malla

Keyword(s):

Clinical Manifestations ◽

Differential Diagnoses ◽

Treatment Modalities ◽

Adult Onset Still’S Disease ◽

Unknown Etiology ◽

Inflammatory Disorder ◽

Adult Onset ◽

Still’S Disease ◽

Still's Disease ◽

Adult Onset Still's Disease

Adult -onset Still’s disease (AOSD) is a rare, idiopathic, inflammatory disorder of unknown etiology and pathogenesis that presents in 5 to 10% of patients as fever of unknown origin (FUO )exclusion characterized by generalized migratory joint ache, blanchable rash, fever and other systemic manifestations. We report an interesting case of a 23 year old Nepalese lady from Okhaldunga who presented with one-month duration of FUO along with sore throat, fever, vomiting, generalized joint ache, erythematous blanchable rash, headache and visual impairment. On examination there was hepatomegaly and investigations showed raised liver enzymes, serum ferritin and fibrinogen.After extensive workup, potential differential diagnoses were ruled out She was diagnosed to have Adult -onset Still’s disease based on Yamuguchi criteria after exclusion of other potential differentials.The patient partially responded to prednisolone and later methotrexate was prescribed which improved her symptoms. The case history, incidence, pathogenesis, clinical manifestations, differential diagnoses, diagnostic workup, treatment modalities, and prognosis of AOSD are discussed in this case report.

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Adult onset Still’s disease: a diagnostic challenge

International Journal of Research in Dermatology ◽

10.18203/issn.2455-4529.intjresdermatol20180119 ◽

2018 ◽

Vol 4 (1) ◽

pp. 87

Author(s):

Rahul Jain ◽

V. K. Joglekar ◽

Kriti Jain

Keyword(s):

Disease Diagnosis ◽

Adult Onset Still’S Disease ◽

Unknown Etiology ◽

Inflammatory Disorder ◽

Adult Onset ◽

Still’S Disease ◽

Diagnostic Challenge ◽

Still's Disease ◽

Adult Still's Disease ◽

Adult Onset Still's Disease

<p class="abstract"><span lang="EN-IN">Adult Still’s disease (ASD) is a systemic inflammatory disorder of unknown etiology, typically characterized by a clinical triad of daily spiking high fevers, evanescent rash, and arthritis. This report described a 26-year-old male who presented with these symptoms along with raised liver enzymes and hyperferritinemia. After ruling out systemic infections, localized infections, malignancies and other rheumatological diseases, Adult onset Still’s disease diagnosis was made according to Yamaguchi criteria (having 4 major features and 3 minor features). AOSD is a heterogeneous and rare disease and the lack of serologic markers as a true gold standard makes diagnosis difficult.</span></p>

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Adult Onset Still's Disease: A Case Report

Anwer Khan Modern Medical College Journal ◽

10.3329/akmmcj.v4i2.16944 ◽

2013 ◽

Vol 4 (2) ◽

pp. 52-54

Author(s):

Mahmudur Rahman Siddiqui ◽

Golam Kibria Khan ◽

Md. Safiqul Islam ◽

KM Ahasan Ahmed

Keyword(s):

Skin Rash ◽

Ferritin Level ◽

Adult Onset Still’S Disease ◽

Inflammatory Disorder ◽

Adult Onset ◽

Still’S Disease ◽

Laboratory Findings ◽

Medical College ◽

Still's Disease ◽

Adult Onset Still's Disease

Adult onset Still's disease (AOSD) is a chronic multi-system inflammatory disorder characterized by high spiking fever, polyarthralgia and skin rash. Lymphadenopathy is another prominent feature of adult onset Still's disease. We describe a 22 years old lady presented with fever, skin rashes and polyarthritis for 3 months. Examination revealed fever, typical skin rash, generalized lymphadenopathy and polyarthritis. On investigation there were neutrophilic leukocytosis, high ESR, high ferritin level, but RA test and ANA test were negative. All of her history, clinical examinations and laboratory findings fulfill the diagnostic Yamaguchi criteria for AOSD. With proper treatment, now she is completely symptoms free and leaving a healthy life. Anwer Khan Modern Medical College Journal Vol. 4, No. 2: July 2013, Pages 52-54 DOI: http://dx.doi.org/10.3329/akmmcj.v4i2.16944

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

BMC Rheumatology ◽

10.1186/s41927-021-00183-6 ◽

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.

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

Italian Journal of Medicine ◽

10.4081/itjm.2010.259 ◽

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.

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Significant association of past parvovirus B19 infection with cytopenia in both adult-onset Still's disease and systemic lupus erythematosus patients

Clinica Chimica Acta ◽

10.1016/j.cca.2012.01.027 ◽

2012 ◽

Vol 413 (9-10) ◽

pp. 855-860 ◽

Cited By ~ 12

Author(s):

Der-Yuan Chen ◽

Yi-Ming Chen ◽

Joung-Liang Lan ◽

Bor-Show Tzang ◽

Chi-Chen Lin ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Lupus Erythematosus ◽

Parvovirus B19 ◽

Adult Onset Still’S Disease ◽

Adult Onset ◽

Still’S Disease ◽

Systemic Lupus ◽

Still's Disease ◽

Parvovirus B19 Infection ◽

Adult Onset Still's Disease

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Adult Onset Still’s Disease: A Review on Diagnostic Workup and Treatment Options

Case Reports in Rheumatology ◽

10.1155/2016/6502373 ◽

2016 ◽

Vol 2016 ◽

pp. 1-6 ◽

Cited By ~ 5

Author(s):

Rajesh Gopalarathinam ◽

Eric Orlowsky ◽

Ramesh Kesavalu ◽

Sreeteja Yelaminchili

Keyword(s):

Diagnostic Workup ◽

Differential Diagnoses ◽

Treatment Modalities ◽

Adult Onset Still’S Disease ◽

Unknown Etiology ◽

Adult Onset ◽

Still’S Disease ◽

Still's Disease ◽

Systemic Inflammatory Disease ◽

Adult Onset Still's Disease

Adult onset Still’s disease (AOSD) is a rare systemic inflammatory disease of unknown etiology and pathogenesis that presents in 5 to 10% of patients as fever of unknown origin (FUO) accompanied by systemic manifestations. We report an interesting case of a 33-year-old African-American male who presented with one-month duration of FUO along with skin rash, sore throat, and arthralgia. After extensive workup, potential differential diagnoses were ruled out and the patient was diagnosed with AOSD based on the Yamaguchi criteria. The case history, incidence, pathogenesis, clinical manifestations, differential diagnoses, diagnostic workup, treatment modalities, and prognosis of AOSD are discussed in this case report.

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