July brings awareness to juvenile arthritis: Learn about it to ensure proper diagnosis coding.
When people hear that someone has arthritis, they generally assume that person is older. The truth is, this disease also affects children and teens, and sometimes goes undetected. Children may express discomfort or pain in their joints or muscles, but their complaints are easily dismissed or overlooked as “growing pains.” Unfortunately, they could be living with one of the diseases that fall under the umbrella term juvenile arthritis.
This month, we’ll bring to light juvenile arthritis. You’ll learn about the types of arthritis young people may develop and the signs and symptoms with which they may present. This awareness will enable you to code a juvenile arthritis diagnosis with certain specificity.
Juvenile Arthritis Has Many Forms
Juvenile arthritis, also referred to as pediatric rheumatic disease, encompasses inflammatory and rheumatic diseases in children under 16 years of age. It affects nearly 300,000 children in the United States. Most forms of juvenile arthritis are autoimmune or autoinflammatory diseases, which means the immune system — whose job is to fight against viruses and germs — gets confused and releases inflammatory chemicals that attack healthy cells and tissue. According to the Arthritis Foundation, the types of juvenile arthritis include:
Juvenile idiopathic arthritis – This is the most common type of arthritis in children. Idiopathic means “from unknown causes.” Juvenile idiopathic arthritis includes six subtypes: oligoarthritis, polyarthritis, systemic, enthesitis-related, juvenile psoriatic arthritis, and undifferentiated.
Juvenile myositis – This inflammatory disease causes muscle weakness. The two types are juvenile polymyositis and juvenile dermatomyositis. Juvenile dermatomyositis causes a skin rash on the eyelids and knuckles.
Juvenile lupus – This autoimmune disease may affect the joints, skin, heart, kidneys, blood, and other parts of the body. The most common form is systemic lupus erythematosus.
Juvenile scleroderma – This condition causes the skin to tighten and harden. The word scleroderma means “hard skin.”
Vasculitis – This disease causes blood vessel inflammation that can lead to heart complications. The most common kinds in children and teens are Kawasaki disease and Henoch-Schonlein purpura.
Fibromyalgia – This arthritis-related condition is a chronic pain syndrome that can cause widespread muscle pain and stiffness, fatigue, disrupted sleep, and other symptoms. It is more prevalent in girls after the onset of puberty.
Spot Genetic Causes and Symptoms
Although the precise cause of juvenile arthritis is hard to pinpoint, researchers have found certain genes may be activated by bacteria, viruses, or environmental factors, causing it to manifest. There is no hard evidence that certain foods, allergies, toxins, poor nutrition, or lack of vitamins cause the disease.
The symptoms of juvenile idiopathic arthritis will differ depending on which of the six subtypes the patient is experiencing. Like other forms of arthritis, juvenile idiopathic arthritis can affect one or more joints and is characterized by when symptoms flare up and when symptoms subside. Signs and symptoms include:
- Joint inflammation and swelling: Joint inflammation is common, and is most notable in large joints (i.e., knees, elbows, ankles).
- Pain and tenderness: Although the patient may not mention joint pain, the parent may notice limping, especially when the patient wakes up in the morning or after a nap.
- Stiffness or clumsiness: The parent may notice that their child appears clumsier than usual, particularly in the morning or after naps.
- Fever, swollen lymph nodes, and rash: Sometimes a high fever, swollen lymph nodes, or a rash on the trunk may occur and get worse in the evenings.
Some forms of juvenile arthritis have minimal or no joint symptoms and, instead, affect internal and external organs. Symptoms include:
- Scaly, red rash (psoriatic arthritis)
- Light spotted pink rash (systemic arthritis)
- Butterfly-shaped rash across the cheeks and bridge of the nose (juvenile lupus)
- Thick, hard patches of skin (scleroderma)
- Eye dryness, redness, pain, sensitivity to light, and vision impairment may be symptoms of uveitis (chronic eye inflammation)
- Diarrhea and bloating may be symptoms when internal organs, such as the digestive tract, are affected
- Shortness of breath when the lungs and heart are affected
- Fatigue; feeling very tired or rundown
- Appetite loss
- High fever, which may get worse in the evening
Early Diagnosis and Treatment Are Key
Pediatricians who suspect a patient’s symptoms are related to juvenile arthritis may refer the child to a pediatric rheumatologist. This specialist will ask about the child’s onset of symptoms and how long they last, and their medical history, as well as family history. They may perform a range of motion exam to look for signs of juvenile arthritis. They may check the patient for rash, eye abnormalities, and joint swelling, tenderness, and pain. The specialist may order laboratory tests to look for inflammatory markers. They also may order X-rays, computed tomography scans, or magnetic resonance imaging to detect signs of joint damage that is not caused by trauma or infection.
Although there is no cure for juvenile arthritis, early diagnosis and aggressive treatment can send it into remission, with little to no disease activity or symptoms. According to the Arthritis Foundation, a successful treatment plan for juvenile idiopathic arthritis includes medication, physical activity, therapies, and healthy eating. These measures are said to:
- Slow or stop inflammation and prevent disease progression;
- Relieve symptoms, control pain, and improve quality of life;
- Prevent joint and organ damage;
- Preserve joint function and mobility for adulthood; and
- Reduce long-term health effects.
Medications and Pain Management
Medications used to treat juvenile arthritis focus on either controlling disease activity or relieving symptoms. Drugs used for controlling disease activity, such as flare-ups, are corticosteroids and disease-modifying antirheumatic drugs (DMARDs).
Corticosteroids (for example, prednisone) are quick-acting, anti-inflammatory injections given in a physician’s office. They manage the disease until other medications or treatments start working. DMARDs (for example, methotrexate, sulfasalazine) and biologics relieve symptoms by suppressing the immune system to stop it from attacking the joints. Biologics may be available in pill form, but usually are injected or infused in a physician’s office.
An aggressive approach to treatment involves starting right away with a biologic or DMARD/biologic combo to combat inflammation and disease progression quickly. Drugs may be added or removed, depending on the results.
Drugs that help to relieve arthritis pain include analgesics and nonsteroidal anti-inflammatories (NSAIDs). These drugs do not reduce joint damage or change the progression or course of the disease.
Exercise, Therapy, and Other Treatments
Low-impact exercise can help to keep joint pain and stiffness at bay. Swimming, yoga, and walking are joint-friendly and recommended by most physicians. Other more intense physical activities can be done in moderation if a physical therapist says symptoms are controlled enough to participate.
Physical and occupational therapists teach patients with juvenile arthritis how to be active and coordinated, how to use assistive devices, and how to perform daily tasks easily. They show patients strengthening and flexibility exercises to improve the child’s quality of life.
If there are pain flare-ups, heat pads and warm baths can soothe stiff joints and tired muscles. For acute pain, cold treatment is best to reduce inflammation and numb painful areas. Creams, patches, and gels may also be used to help joint or muscle pain. Sometimes massage or acupuncture is used to help reduce pain, stress, or anxiety that juvenile arthritis brings.
Patients with juvenile arthritis should understand that healthy food choices — such as fruits, vegetables, whole grains, fatty fish, and cooking with extra virgin olive oil — can help decrease inflammation. Foods high in fat and sugar and processed foods may have the opposite effect and should be avoided. Doctors may suggest supplements and vitamins.
Surgery is usually not performed on juvenile arthritis patients unless a joint replacement is necessary due to a damaged joint or the child is in severe pain. These surgeries may be performed as outpatient procedures.
Kids and teens with this chronic disease often need a strong support system to help them cope and navigate through the difficulties that juvenile arthritis brings. They need to know they are not alone and learn strategies to remain positive.
Look to M08 for Juvenile Arthritis Diagnosis Codes
ICD-10-CM classifies M08.- Juvenile arthritis into three major subtypes defined by symptoms present during the first six months following onset: systemic onset (Still’s disease, juvenile-onset), polyarticular onset, and pauciarticular onset.
M08.- Juvenile arthritis
M08.0- Unspecified juvenile rheumatoid arthritis
M08.1- Juvenile ankylosing spondylitis
M08.2- Juvenile rheumatoid arthritis with systemic onset
M08.3- Juvenile rheumatoid polyarthritis (seronegative)
M08.4- Pauciarticular juvenile rheumatoid arthritis
M08.8- Other juvenile arthritis
M08.9- Juvenile arthritis, unspecified
Be sure to also code any associated underlying conditions, such as K50.- Crohn’s disease [regional enteritis] or K51.- Ulcerative colitis.
Arthritis Foundation: www.arthritis.org/diseases/juvenile-arthritis
Arthritis Foundation, News Blog, “July Is National Juvenile Arthritis Awareness Month:”
AAPC Knowledge Center, “A&P Tip: Juvenile Arthritis,” July 1, 2015:
The Mayo Clinic, Juvenile Idiopathic Arthritis: https://www.mayoclinic.org/diseases-conditions/juvenile-idiopathic-arthritis/symptoms-causes/syc-20374082
WebMD, Treating Rheumatoid Arthritis with Disease-Modifying Drugs (DMARDs):