AIIMS May 2011 – MCQ 104
Which of the following is not true about JRA?
A. Fever
B. Uveitis
C. Rheumatoid nodules
D. Raynaud’s phenomenon
Please contribute to the discussion by posting the answer with references and pointing out any errors in the question!
5 Comments
Its D
rheumatoid nodules and rf is usually absent [robins 7th edition p1309]
raynaud’s phenomenon
Types of JRA
The three major types of JRA are:
Oligoarticular (pronounced: awl-li-go-ar-tik-yoo-lur) JRA. The prefix oligo means “few” and articular means “associated with a joint or joints.” This kind of JRA involves four or fewer joints and usually affects larger joints such as the knee. About half the kids and teens with JRA have the oligoarticular type. It generally begins in young children between infancy and age 5. Sometimes iridocyclitis (pronounced: ire-ih-doe-si-kleye-tis), an inflammation in front of the eye near the iris (the colored part of the eye) also occurs. For this reason, eye exams are important, even when the arthritis is not active.
Polyarticular (pronounced: pa-lee-are-tick-yoo-lur) JRA. The prefix poly means “many.” Polyarticular JRA affects five or more joints, usually the small ones in the hand and fingers. It also can affect the knees, hips, ankles, feet, and neck. Polyarticular JRA may have symmetric involvement of joints, meaning it affects the same joint on each side of the body. Involved joints are usually swollen. Symptoms may include a low-grade fever, tiredness, poor appetite, and rheumatoid nodules (bumps) on the affected joints. This form of JRA can lead to long-term joint problems and frequently requires treatment with strong medications.
Systemic (pronounced: sis-teh-mick) JRA. Systemic JRA can affect several parts of the body, including internal organs and joints. This is the least common form of JRA. The first signs of systemic JRA are usually high fevers, chills, and a rash. Arthritis may begin with the fevers or not until weeks or months later. Fever usually occurs late in the afternoon or evening and can go up to 103° Fahrenheit (39.4° Celsius) or higher and return to normal within a few hours. The person may feel very sick when his or her temperature is high but fine during the rest of the day. About half of the kids and teens with systemic JRA recover completely, whereas the other half experience joint symptoms, such as pain and stiffness, which may persist for many years. In some cases the effects of the disease, such as decreased movement, stiffness, and pain in the joints, may last into adulthood
Methotrexate-associated appearance and rapid progression of rheumatoid nodules in systemic-onset juvenile rheumatoid arthritis.
Falcini F, Taccetti G, Ermini M, Trapani S, Calzolari A, Franchi A, Cerinic MM.
Source
University of Florence, Italy.
Abstract
Rheumatoid nodules are a rare extraarticular manifestation of juvenile rheumatoid arthritis (JRA), usually detected in patients with polyarticular-onset disease and positive rheumatoid factor (RF). To date, there has not been a published report of rheumatoid nodules in systemic-onset JRA. Low-dose methotrexate (MTX) is generally considered to be the most useful second-line drug in the treatment of polyarticular JRA. In adult RA, MTX has been shown to be associated with appearance and progression of rheumatoid nodules. This report describes a 3-year-old girl with RF-negative, antinuclear antibody-negative systemic JRA who developed multiple rheumatoid nodules on the scalp and trunk during MTX therapy. The first nodule developed on the scalp 6 months after MTX treatment was initiated. Previous treatment with azathioprine was not associated with nodulosis. This represents an atypical case of MTX-associated accelerated nodulosis in systemic JRA, and raises the problem of treatment plan modification in the presence of this side effect. SO ANS IS D
Research Notes on Juvenile Rheumatoid Arthritis and Raynaud’s Disease
Written by the OrganizedWisdom Team with Nicole Kinsey as the reviewer. All original content is supervised by the The OrganizedWisdom Medical Review Team
For individuals with juvenile rheumatoid arthritis (JRA), Raynaud’s disease occurs when the blood vessels that feed the fingers and toes constrict in reaction to cold or emotional stress.1 Raynaud’s disease can occur on its own or it can occur in the presence of another underlying disorder.1 JRA, also called juvenile idiopathic arthritis (JIA) and juvenile chronic arthritis (JCA), is the most common form of arthritis in children.2
Fast Facts:
In Raynaud’s disease, the body reduces blood flow to the fingers and toes under relatively minor cold stress, such as holding a glass of ice water or being in an air-conditioned room.1
Raynaud’s disease can occur in the presence of another underlying disorder such as a connective tissue disease like lupus or scleroderma.1
Almost any rheumatic disease can be associated with Raynaud’s including rheumatoid arthritis, erythematosus, and dermatomyositiS
There are two different types of pauciarticular JRA: one type usually affects little girls under age 7 and is associated with the development of eye inflammation (chronic iridocyclitis or uveitis) in about 1/3 of these children. These children should be tested for antinuclear antibodies (ANA). This tells the rheumatologist and the ophthalmologist (eye doctor) whether your child has a higher risk of developing uveitis (when the ANA is positive the risk is higher). Unfortunately, this eye condition is silent so only proper monitoring by an eye doctor who is familiar with this complication of JA may find the changes in the eyes.
The second type of pauciarticular JRA usually affects boys who are somewhat older, typically after age 8 and tends to involve the lower spine (sacroiliac joints), hips, knees, ankles and tendons. Sometimes, the spots at which tendons and ligaments attach to bones are also inflamed (enthesitis). These children may also develop eye problems, but this is usually acute uveitis (rather than chronic uveitis) and often causes redness and pain in the eyes. This type of JRA may be the first sign of another arthritis-related condition, such as one of the spondyloarthropathies. This group of diseases usually affects the spine, tendons, and eyes, and is associated with a genetic factor, HLA-B27 in some people.
Symptoms of systemic onset JRA usually include: daily high spiking fevers (103 degrees or higher) that may last for weeks or even months; a rash of pale red or pink spots that appear on the child’s chest, thighs and sometimes other parts of the body (the rash may accompany the fever and may come and go for many days in a row); joint pain and inflammation that may accompany the fever or begin weeks or months later. Joint problems can become a major long-term symptom. Other possible features of this form of arthritis include inflammation of the outer lining of the heart (pericarditis) or the lungs (pleuritis); anemia and high white blood cells and platelets (cells that help with clotting); and enlarged lymph nodes, liver or spleen. Blood work is usually checked frequently in the first few weeks and months of this illness to watch your child closely.
In about half the children with systemic JRA, the illness seems to disappear within 1 year of onset. Flare-ups, or a return of the illness, can happen without warning or after some viral infections (mononucleosis and chicken pox, for example). In most children with systemic JA, medications must be used for months to years to control both parts of this illness – the systemic part (fever, rash, anemia, etc.) as well as the arthritis
SO FEVER UVEITES ARE COMMON WHILE OTHER TWO ARE UNCOMMON