FAQs - Information on Specific Diseases

• Juvenile Idiopathic Arthritis (JIA)
• Systemic Lupus Erythematosus (SLE)
• Dermatomyositis
• Scleroderma

Juvenile Idiopathic Arthritis (JIA)
JIA is the childhood chronic arthritis that is days in and days out. Like adult onset arthritis, arthritis in children is caused by inflammation of the lining of the joint capsule, called synovium.

Synovium is supposed to be with a thickness of 2-3 cell depth covering over a loose connective tissue matrix. In joints with arthritis, synovium is quite expanded and thickened due to influx of thousands of blood borne cells like neutrophils and lymphocytes for reasons poorly understood. These influx cells remain in the synovium, release inflammatory mediators like IL-1 (interleukin-1) and TNF (tumor necrosis factor), which results with swelling of the joint.

Clinically, the child may feel discomfort and stiffness of the affected joints. The child may also experience morning stiffness. Please keep an eye on the duration of morning stiffness as it may correlate with disease activity.

There are different subtypes of JIA differing in pattern of age onset, joints involved:

1. Pauciarticular JIA
   Usually affects toddlers and school age children
   Girls more than boys
   Involves up to 4 joints (usually large joints – knees and ankles)
   Can cause inflammation of eyes as well (uveitis)
   If child is ANA positive, it may mean there is increased risk for developing uveitis
2. Polyarticular JIA
   Can happen in young children as well as teens
   More than 4 joints are affected that can be small and large joints of hands and feet as well as knees, ankles, wrists, elbows
   Usually affects joints in symmetric fashion (i.e. both hands may have arthritis)
   Rheumatoid factor can be positive among the teens with arthritis, which can indicate child is likely to carry arthritis into the adult life
   These children can also develop uveitis and also need regular follow up by eye doctor (ophthalmologist)
3. Systemic onset JIA
   Can cause fever, rash, and arthritis
   Children can be very ill with systemic JIA
   Please keep a fever diary including duration and time of the day
   It would be very helpful to document rashes with a digital camera
4. JIA with axial pattern i.e. spondyloarthopathy pattern
   Usually effects the spine and the joints around the spine like hips, shoulder, and knees
   Can also lead to inflammation of tendons at the site of attachment to the bone (enthesitis)
   This type of arthritis can run in families and can be associated with HLA B27 (Human leukocyte antigen type B, subtype 27)
5. JIA with bowel disease
   Individuals with inflammatory bowel disease can develop arthritis
   These children need to be followed closely by the GI service
   Please let your physician know if you have any stomach concerns, weight loss, or changes in bowel habits
6. JIA with psoriatic skin disease
   Psoriasis can lead to arthritis which may be before, after or along with development of skin rash
   There is usually family history of psoriasis
   Patients will be followed closely by dermatologist as well (skin doctors)

Systemic Lupus Erythematosus (SLE)

• Lupus affects 1 out of 100,000 children at ages less than 16 years of age
• Females are affected almost 10 times more commonly than males in adults
• The diagnosis of lupus is based on history, physical exam, and laboratory values
• The main course of treatment is steroids that are usually combined with DMARDs.
• There are a couple of things I like to emphasize: Lupus is a serious disease and may involve risk for long-term organ damage and disability. Few suggestions to patients and families:

• Establish close follow ups with your rheumatologist
• Please have good compliance to medicationsKeep a diary of concerns
• It is so important to have positive attitude, and allow your child to keep up with peers at school and home.
• Use sunscreen at all times!! Avoid excessive sun exposure.
• It always helps to develop healthy eating habits and regular exercise routine.
• Most importantly, please see a doctor right away or Emergency roomThe hospital department that provides emergency services to patients who need immediate medical attention. visit if there is any fever!!

Dermatomyositis

• This is a rare disease that affects about 1 out of 200,000/300,000 children a year
• Causes inflammation in the skin and muscles manifesting with rash and weakness
• It can be dangerous if it causes trouble swallowing and breathing or there are severe abdominal concerns.
• The skin rash is very sensitive to sun exposure. Use sunscreen at all times!
• These children are treated with steroids and DMARDs for a minimum of 12-24 months.
• Your rheumatologist may work closely with Dermatology (skin doctors) and physical therapist.

Scleroderma

• This is a disease that can be found in different forms among children
• Also called systemic scleroderma if it affects most of the skin as well as lungs, GI tract and kidneys
• Systemic scleroderma usually causes Raynaud’s of hands, the skin over the hands can be puffy and shinny, the patient may have fatigue, joint pain, shortness of breath, difficulty swallowing, diarrhea or constipation, and weight loss.
• Patients with systemic scleroderma are followed by rheumatology, pulmonary (lungs), cardiology (heart), GI, and nephrology (kidney) doctors.
• It can be form of more limited disease affecting only small patches of skin; if the skin patch is round it is called “Morphea” and if the skin patch is linear then it is called “linear scleroderma.” Morphea or linear scleroderma usually, but not always, is not associated with any organ involvement.