What is macrophage activation syndrome?
Macrophage activation syndrome (MAS) is a severe complication of rheumatic disease in childhood, particularly in systemic Juvenile Idiopathic Arthritis (sJIA). It is characterize by an uncontrolled activation and proliferation of T lymphocytes and macrophages.
What is SoJIA?
Systemic-onset juvenile idiopathic arthritis (SoJIA) is characterized by spiking fever, evanescent rash, hepatosplenomegaly, serositis, lymphadenopathy, and arthritis. The International League Against Rheumatism criteria has classified SoJIA as a subtype of JIA [1].
Is systemic juvenile idiopathic arthritis life threatening?
Rarely, kids with JIA can get a life-threatening problem called macrophage activation syndrome. It happens when the immune system becomes too active and damages some of the body’s organs. It can lead to severe diarrhea, bleeding, hearing loss, confusion, and seizures.
What is systemic JIA?
Systemic juvenile idiopathic arthritis (SJIA) is a rare autoinflammatory disease, affecting only 10% to 15% of children with juvenile idiopathic arthritis (JIA). Formerly called “Still’s disease,” SJIA is considered to be a type of JIA, but the symptoms, underlying cause and treatments for it are all different.
Can adults get macrophage activation syndrome?
Abstract. Macrophage activation syndrome (MAS) is a potentially fatal complication of Adult-Onset Still’s disease (Still’s disease).
Is macrophage activation syndrome life threatening?
Macrophage activation syndrome (MAS) is a life-threatening complication of rheumatic diseases, requiring immediate and appropriate treatment. MAS is a disorder related to hemophagocytic lymphohistiocytosis (HLH), which is divided into primary and secondary HLH.
Can SoJIA be cured?
SoJIA patients are classically treated first with non-steroidal anti-inflammatory drugs, and if the inflammation is not controlled, high dose corticosteroids are used.
Is SoJIA curable?
Treatment. There is no cure for SJIA but remission (little or no disease activity or symptoms) is possible. Early aggressive treatment is key to getting the disease under control as quickly as possible. The past few years have seen a revolution in treatments for SJIA.
What does juvenile arthritis rash look like?
Juvenile PsA rash typically presents as a flushed patch of skin that may appear silvery-white in areas due to an accumulation of dead skin cells. The rash is usually itchy or painful. Although it may develop anywhere on the body, it most commonly affects the following areas: the scalp.
Is JIA rash itchy?
In systemic JIA, the rash is typically flat, pale, and non-itchy, and it tends to disappear within minutes or hours. Systemic JIA rashes also tend to be accompanied by a high fever. The treatment for JIA rash involves targeting the underlying inflammation and using topical medications to ease skin symptoms.
What are the three types of JIA?
The three major types include: Oligoarticular JIA (arthritis in four joints or less) Polyarticular JIA (arthritis in five or more joints) Systemic JIA (arthritis plus fever, rash and large lymph nodes)
How common is macrophage activation syndrome?
The estimated prevalence of MAS in SJIA was ~10%, and it increased up to 40% in subclinical MAS, which showed the evidence of MAS occurrence only in the bone marrow. The prevalence of MAS in SLE varied from 0.9% to 4.6% and it increased up to 9.4% in those patients with a hepatic dysfunction.
What happens if juvenile idiopathic arthritis goes untreated?
If it is not treated, JIA can lead to: Permanent damage to joints. Interference with a child’s bones and growth. Chronic (long-term) arthritis and disability (loss of function)
How many people have AOSD?
Slightly more than 1 person per 100,000 people are affected each year. Males and females 16- to 35-years old are mostly likely to contract AOSD. The condition seems to affect men and women equally, although some studies suggest women are affected slightly more than men.
What is the difference between Still’s disease and rheumatoid arthritis?
While it is unusual for RA to present with skin problems, cutaneous features are not uncommon. Still’s disease, also known as juvenile rheumatoid arthritis, is characterised by intermittent high fevers associated with a salmon-coloured rash, and arthritis.
What is evanescent rash?
Evanescent skin lesions, like wheals, are those that last for less than 24 hours before resolving.
What is Still’s disease?
Adult Still’s disease is a rare type of inflammatory arthritis that features fevers, rash and joint pain. Some people have just one episode of adult Still’s disease. In other people, the condition persists or recurs. This inflammation can destroy affected joints, particularly the wrists.
What does rash with juvenile arthritis look like?
What does arthritis rash look like?
Patches can appear red or burgundy and have a red-violet hue on darker skin tones. They may appear as multiple pinpoint dots. RA-related vasculitis of the skin commonly appears on the fingers and toes. Sometimes, rheumatoid vasculitis rashes develop into painful ulcers.