Adult Still disease (ASD) is a rare illness that causes high fevers, rash, and joint pain. It may lead to long-term (chronic) arthritis.
Adult Still disease is a severe version of juvenile idiopathic arthritis (JIA), which occurs in children. Adults can have the same condition, although it is much less common. It is also called adult-onset Still disease (AOSD).
Still's disease - adult; Adult-onset Still's disease; AOSD; Wissler-Fanconi syndrome
Fewer than 1 out of 100,000 people develop ASD each year. It affects women more often than men.
The cause of adult Still disease is unknown. No risk factors for the disease have been identified.
Almost all people with the disease will have fever, joint pain, sore throat, and a rash.
Additional symptoms include:
The spleen or liver may become swollen. Lung and heart inflammation may also occur.
The goal of treatment for adult Still disease is to control the symptoms of arthritis. Aspirin and other nonsteroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen, are most often used first.
Prednisone may be used for more severe cases.
If the disease is severe or persists for a long time (becomes chronic), medicines that suppress the immune system might be needed. Such medicines include:
Bruno Fautrel practices in Paris, France. Fautrel is rated as an Elite expert by MediFind in the treatment of Adult Still's Disease. He is also highly rated in 13 other conditions, according to our data. His top areas of expertise are Rheumatoid Arthritis (RA), Arthritis, Adult Still's Disease, Axial Spondyloarthritis (AxSpA), and Hip Replacement.
Giulio Cavalli practices in Milan, Italy. Cavalli is rated as an Elite expert by MediFind in the treatment of Adult Still's Disease. He is also highly rated in 18 other conditions, according to our data. His top areas of expertise are Erdheim-Chester Disease, Adult Still's Disease, Non-Langerhans-Cell Histiocytosis, Heart Transplant, and Lung Transplant.
Serena Colafrancesco practices in Rome, Italy. Colafrancesco is rated as an Elite expert by MediFind in the treatment of Adult Still's Disease. She is also highly rated in 8 other conditions, according to our data. Her top areas of expertise are Adult Still's Disease, Sjogren Syndrome, Dry Mouth, Dry Eye Syndrome, and Hormone Replacement Therapy (HRT).
In many people, symptoms may come back several times over the next few years.
Symptoms continue for a long time (chronic) in about one third of people with adult Still disease.
A rare form of the disease, called macrophage activation syndrome, can be very severe with high fevers, severe illness and low blood cell counts. The bone marrow is involved and biopsy is needed to make the diagnosis.
Other complications may include:
Call your provider if you have symptoms of adult Still disease.
If you have already been diagnosed with the condition, you should call your provider if you have a cough or difficulty breathing.
There is no known prevention.
Summary: This will be a single centre, Phase 1, First-In-Human, Randomized, Double-Blind, Placebo-Controlled Study to Evaluate the Safety, Tolerability, Pharmacokinetics, and Pharmacodynamics of Single Ascending Dose of APB-R3 in Healthy Participants.
Summary: The main purpose of the study is to evaluate the safety and tolerability of AEVI-007 in participants with Adult Onset Still's Disease (AOSD).
Published Date: January 31, 2021
Published By: Diane M. Horowitz, MD, Rheumatology and Internal Medicine, Northwell Health, Great Neck, NY. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
Alonso ER, Marques AO. Adult-onset still disease. In: Hochberg MC, Gravallese EM, Silman AJ, Smolen JS, Weinblatt ME, Weisman MH, eds. Rheumatology. 7th ed. Philadelphia, PA: Elsevier; 2019:chap 173.
Gerfaud-Valentin M, Maucort-Boulch D, Hot A, et al. Adult-onset still disease: manifestations, treatment, outcome, and prognostic factors in 57 patients. Medicine (Baltimore). 2014;93(2):91-99. PMID: 24646465 pubmed.ncbi.nlm.nih.gov/24646465/.
Kaneko Y, Kameda H, Ikeda K, et al. Tocilizumab in patients with adult-onset still's disease refractory to glucocorticoid treatment: a randomised, double-blind, placebo-controlled phase III trial. Ann Rheum Dis. 2018;77(12):1720-1729. PMID: 30279267 pubmed.ncbi.nlm.nih.gov/30279267/.
National Organization for Rare Disorders website. Rare diseases.org. Adult onset Still's disease. rarediseases.org/rare-diseases/adult-onset-stills-disease/. Updated 2021. Accessed May 10, 2021.
Ortiz-Sanjuán F, Blanco R, Riancho-Zarrabeitia L, et al. Efficacy of anakinra in refractory adult-onset Still's disease: multicenter study of 41 patients and literature review. Medicine (Baltimore). 2015;94(39):e1554. PMID: 26426623 pubmed.ncbi.nlm.nih.gov/26426623/.