Paediatric Rheumatology Flashcards
what is the most common chronic rheumatologic disease in children?
JIA
what is JIA?
Juvenile idiopathic arthritis (JIA) is the most common type of arthritis in kids and teens. It typically causes joint pain and inflammation in the hands, knees, ankles, elbows and/or wrists. But, it may affect other body parts too
what features and effects are seen in JIA?
Arthritis for at least 6 weeks
Morning stiffness or gelling
irritability or refusal to walk in toddlers
School absence or limited ability to participate in physical activity
Rash /fever
Fatigue
Poor appetite/wt loss
Delayed puberty
what are the differential diagnosis of JIA?
Septic arthritis
Osteomyelitis
Transient synovitis
Malignancies i.e lymphoma, neuroblastoma, bone tumours
Recurrent haemarthrosis
Vascular abnormalities
Trauma
others
what are the signs seen in JIA?
Swelling: periarticular soft tissue edema/intraarticular effusion/hypertrophy of synovial membrane
Tenosynovitis (swollen tendons)
pain
Joint held in position of maximum comfort
range of motion limited at extremes
what is Oligoarticular Juvenile Arthritis? and who does it occur in?
Affects four or fewer joints, typically the large ones (knees, ankles, elbows). Most common subtype of JIA.
what is the pattern of onset in children with oligoarticular juvenile arthritis?
The antibodies that target “normal” proteins within the nucleus of a cell are called antinuclear antibodies (ANA). Most of us have autoantibodies, but typically in small amounts. The presence of large amount of autoantibodies or ANAs can indicate an autoimmune disease. ANAs could signal the body to begin attacking itself which can lead to autoimmune diseases. A positive ANA can also be seen in juvenile arthritis.
what is polyarticular juvenile arthritis?
Affects five or more joints, often on both sides of the body (both knees, both wrists, etc.). May affect large and small joints. Affects about 25% of children with JIA.
what are the features and symptoms of polyarticular JA
enthesitis related JIA should have at least 2/6 signs which are what?
Enthesitis is inflammation of the entheses, the sites where tendons or ligaments insert into the bone
Also known as spondyloarthritis. Affects where the muscles, ligaments or tendons attach to the bone (entheses). Commonly affects the hips, knees and feet, but may also affect the fingers, elbows, pelvis, chest, digestive tract (Crohn’s disease or ulcerative colitis) and lower back (ankylosing spondylitis). More common in boys; typically appears in children between the ages of eight and 15.
what features are seen in psoriatic JIA?
Joint symptoms and a scaly rash behind the ears and/or on the eyelids, elbows, knees, belly button and scalp. Skin symptoms may occur before or after joint symptoms appear. May affect one or more joints, often the wrists, knees, ankles, fingers or toes
what is Systemic JIA?
Affects the entire body (joints, skin and internal organs). Symptoms may include a high spiking fever (103°F or higher) that lasts at least two weeks and rash. Affects about 10% of children with JIA
Accounts for 5-15% of JIA
what symptoms and signs are seen in systemic JIA?
Unwell
Arthritis
Intermittent fever>2 weeks
Salmon pink erythematous rash
Generalized lymphadenopathy
Serositis
Hepatomegaly/splenomegaly
High inflammatory markers
how do you diagnose JIA?
History
Physical examination findings
Investigation
no specific test for JIA, but your doctor will take blood tests and x-rays. They may also do other tests, including:
ultrasound or MRI scans to try to see if there’s arthritis and to rule out other conditions
removing fluid from a joint (aspiration) to rule out joint infection
bone marrow examination to rule out some rare conditions, especially if they think you have systemic-onset JIA
to be diagnosed with JIA, what is someone required to have?
you’ve had arthritis for six weeks or more
your symptoms started before your 16th birthday
your doctor has ruled out other conditions that can cause arthritis
what investigations can be done for JIA?
Labs
Plain x ray
US
MRI with contrast
whats the differentce between osteoarthritis and rheumatoid arthritis?
Osteoarthritis occurs when the smooth cartilage joint surface wears out. Osteoarthritis usually begins in an isolated joint. Rheumatoid arthritis is an autoimmune disease, which means that the immune system malfunctions and attacks the body instead of intruders.
The aim of treatment for juvenile idiopathic arthritis (JIA) is to what?
control the symptoms of arthritis
enable you to lead an active life at school or college
enable you to enjoy an active family and social life
help you become an independent adult.
what is the pharmacological manegement of JIA?
Pharmacologic management consisting of nonsteroidal anti-inflammatory drugs (NSAIDs), disease-modifying antirheumatic drugs (DMARDs), biologic agents, and intra-articular and oral steroids
Drugs can’t cure arthritis, but they can control the symptoms and help to reduce the possibility of joint damage. An increasing number of medicines are now available
Disease-modifying anti-rheumatic drugs (DMARDs) dampen down inflammation and can reduce the joint damage caused by arthritis.Methotrexate is the most commonly used DMARD in JIA
steroids control inflammation, pain and stiffness
what is non-pharmacological management of JIA?
Psychosocial factors, including counselling for patients and parents
School performance, such as school-life adjustments, and physical education adjustments
Nutrition, particularly to address anemia and generalized osteoporosis
Physical therapy to relieve pain and to address range of motion, muscle strengthening, activities of daily living, and conditioning exercises
Occupational therapy, including joint protection, a program to relieve pain, range of motion, and attention to activities of daily living
are Intraarticular steroids(IAS) useful?
IAS highly efficacious
Remission>6 months 84%
Greater success in oligoarticular JIA
Safe and effective
No long term side effects
Disease modifying anti inflammatory drugs(DMARD) - which one is commonly used and when?
Methotrexate is DMARD of choice
Poor response to IAS in oligo JIA
Should be used early for good outcome.
Most given injectable(subcutaneous) form
Not many side effects
Blood monitoring
when are Biological agents used?
Failure to respond to DMARD
Anti TNF agents commonly used
Good safety profile
Newer biologics
Uveitis associated with JIA
what is uveitis?
Uveitis is a form of eye inflammation. It affects the middle layer of tissue in the eye wall (uvea)
If untreated can progress to chronic uveitis
All children diagnosed with JIA undergo screening.
Early detection prevents complications
who get uveitis and what symptoms does it cause?
Uveitis more common in ANA positive oligo JIA
< 5 years
Rarely symptomatic
Red eyes, headache, reduced vision.
Cataracts, glaucoma and blindness
how do you treat uveitis?
Needs slit lamp examination
All JIA pts to be seen within 6 weeks of diagnosis
High risk children
Initially topical steroids to reduce inflammation. More severe need systemic steroids
Poor response to steroids
DMARD and biologics
Early detection and treatment prognosis good
what are the complications of JIA?
Poor growth
Localised growth disturbances
Micrognathia (very small lower jaw)
contractures
Ocular complications
