Paediatric Rheumatology

Question 1

what is the most common chronic rheumatologic disease in children?

Answer 1

JIA

Question 2

what is JIA?

Answer 2

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

Question 3

what features and effects are seen in JIA?

Answer 3

— 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

Question 4

what are the differential diagnosis of JIA?

Answer 4

—Septic arthritis

—Osteomyelitis

—Transient synovitis

—Malignancies i.e lymphoma, neuroblastoma, bone tumours

—Recurrent haemarthrosis

—Vascular abnormalities

—Trauma

—others

Question 5

what are the signs seen in JIA?

Answer 5

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

Question 6

what is Oligoarticular Juvenile Arthritis? and who does it occur in?

Answer 6

Affects four or fewer joints, typically the large ones (knees, ankles, elbows). Most common subtype of JIA.

Question 7

what is the pattern of onset in children with oligoarticular juvenile arthritis?

Answer 7

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.

Question 8

what is polyarticular juvenile arthritis?

Answer 8

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.

Question 9

what are the features and symptoms of polyarticular JA

Answer 9

Question 10

enthesitis related JIA should have at least 2/6 signs which are what?

Answer 10

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.

Question 11

what features are seen in psoriatic JIA?

Answer 11

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

Question 12

what is Systemic JIA?

Answer 12

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

Question 13

what symptoms and signs are seen in systemic JIA?

Answer 13

—Unwell

—Arthritis

—Intermittent fever>2 weeks

—Salmon pink erythematous rash

—Generalized lymphadenopathy

—Serositis

—Hepatomegaly/splenomegaly

High inflammatory markers

Question 14

how do you diagnose JIA?

Answer 14

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

Question 15

to be diagnosed with JIA, what is someone required to have?

Answer 15

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

Question 16

what investigations can be done for JIA?

Answer 16

Labs

Plain x ray

US

MRI with contrast

Question 17

whats the differentce between osteoarthritis and rheumatoid arthritis?

Answer 17

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.

Question 18

The aim of treatment for juvenile idiopathic arthritis (JIA) is to what?

Answer 18

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.

Question 19

what is the pharmacological manegement of JIA?

Answer 19

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

Question 20

what is non-pharmacological management of JIA?

Answer 20

—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

Question 21

are Intraarticular steroids(IAS) useful?

Answer 21

IAS highly efficacious

Remission>6 months 84%

Greater success in oligoarticular JIA

Safe and effective

No long term side effects

Question 22

Disease modifying anti inflammatory drugs(DMARD) - which one is commonly used and when?

Answer 22

—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

Question 23

when are Biological agents used?

Answer 23

—Failure to respond to DMARD

—Anti TNF agents commonly used

—Good safety profile

—Newer biologics

Question 24

Uveitis associated with JIA

what is uveitis?

Answer 24

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

Question 25

who get uveitis and what symptoms does it cause?

Answer 25

—Uveitis more common in ANA positive oligo JIA

—< 5 years

—Rarely symptomatic

—Red eyes, headache, reduced vision.

—Cataracts, glaucoma and blindness

Question 26

how do you treat uveitis?

Answer 26

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

Question 27

what are the complications of JIA?

Answer 27

Poor growth

Localised growth disturbances

Micrognathia (very small lower jaw)

contractures

Ocular complications