Beneficial Effect of Interleukin 1 Inhibition with Anakinra in Adult-onset Still’s Disease. An Open, Randomized, Multicenter Study

Nordström, Dan; Knight, Ann; Luukkainen, R; Vollenhoven, Ronald van; Rantalaiho, Vappu; Kajalainen, Anna; Brun, Johan G.; Prøven, Anne; Ljung, Lotta; Kautiainen, Hannu; Pettersson, Tom

doi:10.3899/jrheum.111549

Cited by 141 publications

(141 citation statements)

References 9 publications

(10 reference statements)

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“…Anti-IL-1 therapy is useful in CAPS but also in AOSD 7 , although patients with AOSD usually do not present mutations in the NLRP3 gene 8,9 . The symptoms of AOSD and CAPS partly overlap (Table 1), so different sets of diagnostic criteria may be challenging to use.…”

Section: Rheumatologymentioning

confidence: 99%

A Patient with a Phenotype of Adult-onset Still Disease, But a Genotype Typical of Cryopyrin-associated Periodic Fever Syndrome

Eriksson

Jacobs²,

Söderkvist³

2013

J Rheumatol

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Section: Rheumatologymentioning

confidence: 99%

A Patient with a Phenotype of Adult-onset Still Disease, But a Genotype Typical of Cryopyrin-associated Periodic Fever Syndrome

Eriksson

Jacobs²,

Söderkvist³

2013

J Rheumatol

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“…There is a constant balance between over suppressing the immune system and under suppressing the immune system. Over suppressing the immune system subsequently better controls the AOSD, but predisposes the patient to systemic and local infection [15,16]. Under suppressing the immune system prevents many infectious from occurring by humoral and innate immunity, however allows for the patients autoimmune process to flare, and thus leads to more symptoms associated with the disease.…”

Section: Discussionmentioning

confidence: 99%

Non Infectious Cavitary Lung Lesions in a Patient with Adult Onset Still’s Disease: A Case Report

Mitchell¹,

Helen²

2017

J Infect Dis Preve

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Acute Onset Still's Disease (AOSD) is a disease of immunologic and hematologic dysfunction, whereby a percentage of cases develop further into Macrophage Activating Syndrome (MAS). This patient is a 35 year old Caucasian male with Acute Onset Still's Disease without MAS was hospitalized for multiple cavitary lung lesions. Lesions included a larger estimated 2.5 cm x 2.5 cm cavitary lesion in the Right Middle Lobe and smaller estimated 1.3 x 1.4 cm and 1.8 x 1.2 cm lesions in the Left Lower Lobe of the lungs. The larger lesion was seen on chest X-ray a further detailed on a chest CT scan, which revealed the remaining two smaller pulmonary lesions. The patient experienced undulating fevers as high as 101°F. The patient was put on isolation and received serial blood cultures, sputum cultures, acid fast stains of sputum, urine cultures, viral film array serology all resulting in negative results. Over seven acid fast bacilli stains of sputum were negative in the setting of multiple cavitary lung lesions with high suspicion of active Tuberculosis. The patient received immediate broad spectrum antibiotic and antifungal intravenous antibiotics without relief in fever or symptoms. The patient received a full body Indium scan and MRI of the brain looking for a source of possible infection and further classification of his disease; both of which were negative. The blood and sputum fungal and bacterial cultures were negative. Serial chest CT and chest X-ray demonstrated slightly smaller cavitary lung lesions with thinner walls. The suspicion of Tuberculosis remained high and a lung biopsy was obtained of the large cavitary lesion in the Left Middle Lobe, which did not demonstrate acid fast staining on three separate samples. This patient was concluded to have noninfectious cavitary lung lesions and discharged home with close follow up. He is the first patient reported with AOSD Disease without MAS hospitalized for undulating fevers and multiple cavitary lung lesions without evidence of Tuberculosis of active infection.

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“…In particular, IL-1β has been implicated in AOSD etiology [13]. IL-1 antagonists (Anakinra) induced more beneficial responses than disease modifying anti-rheumatic drugs (DMARD) in patients with AOSD [14]. In case series of AOSD complicated by MAS, anakinra leads to complete recovery in most patients [15].…”

Section: Discussionmentioning

confidence: 99%

Electroconvulsive Therapy as a Potential Trigger of Adult Onset Still’s Disease

Rauber¹,

Fm²,

Blank³

et al. 2017

J Clin Case Rep

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Beneficial Effect of Interleukin 1 Inhibition with Anakinra in Adult-onset Still’s Disease. An Open, Randomized, Multicenter Study

Abstract: Anakinra induced more beneficial responses than DMARD in patients with AOSD and was favored in the OLE phase. (ClinicalTrials.gov Protocol Registration NCT01033656).

Cited by 141 publications

References 9 publications

A Patient with a Phenotype of Adult-onset Still Disease, But a Genotype Typical of Cryopyrin-associated Periodic Fever Syndrome

A Patient with a Phenotype of Adult-onset Still Disease, But a Genotype Typical of Cryopyrin-associated Periodic Fever Syndrome

Non Infectious Cavitary Lung Lesions in a Patient with Adult Onset Still’s Disease: A Case Report

Electroconvulsive Therapy as a Potential Trigger of Adult Onset Still’s Disease

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