Department of Pediatric Rheumatology
Disease-specific information
The following list contains the more common diseases that Pediatric Rheumatologists treat at UCLA. It does not, however, represent all of the conditions that are cared for by our team. The information provided is aimed to increase awareness of the diagnosis, causes and treatments for rheumatic diseases. It should not take the place of consultation with a Pediatric Rheumatologist.
Juvenile rheumatoid arthritis (JRA)
Systemic lupus erythematosus (SLE)
Juvenile dermatomyositis (JDM)
Juvenile rheumatoid arthritis (JRA)
What is JRA?
Juvenile rheumatoid arthritis (JRA) is a chronic condtion that begins in children age less than age 16 years and causes inflammation (swelling, warmth, and pain with movement) and stiffness of joints for more than six weeks. It is likely that the name will soon be changed to juvenile idiopathic arthritis (JIA) since we do not know the cause of the arthritis (idiopathic means of unknown cause). It is one of the most common chronic childhood diseases- about as common as type 1 diabetes mellitus (juvenile diabetes). The length of the disease and the severity of illness varies depending on many factors, including the type of JRA (there are three different types), the age at which the disease starts, and other factors. Treatment is essential since the inflammation can affect joint, muscle and bone development in the growing child.
What are the three types of JRA?
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pauciarticular
This is the most common form of JRA, involving four or fewer joints. Large joints, such as the knees, ankles, or elbows are typically affected and the arthritis is usually asymmetrical. This type of disease most often affects girls and often presents as early as 1 year of age (mean 2-4 years of age). The eye is most frequently involved in this type of JRA with inflammation that if untreated may lead to blindness. It is essential that children with this type of JRA see an ophthomologist every three months. -
polyarticular
This is the next most common type of JRA and this type is the most like adult rheumatoid arthritis (RA) since it affects 5 or more joints in a symmetrical pattern. Polyarticular disease is more serious and tends to affect the small joints, such as the hands and feet, as well as, the large joints such as shoulders, elbows, wrists, hips, knees, and ankles. This type of JRA affects girls more often than boys. -
systemic
Systemic onset JRA (also called Still's disease) is the most serious, but least common form of the disease. It affects one or more joints and causes inflammation of internal organs, including the heart, liver, spleen, and lymph nodes. Boys and girls are equally likely to have systemic JRA.
What causes JRA?
Like adult rheumatoid arthritis, JRA is an autoimmune disease, which means the body's immune system attacks its own healthy cells and tissues.
JRA is considered to be a multifactorial condition. Multifactorial means that "many factors" are involved in causing a health problem. The factors are usually both genetic and environmental, where a combination of genes from both parents, in addition to unknown environmental factors, produce the trait or condition. Often one gender (either males or females) is affected more frequently than the other in multifactorial traits. Multifactorial traits do occur in families because they are partly caused by genes.
What are the symptoms of JRA, and how is it diagnosed?
Symptoms of juvenile rheumatoid arthritis may appear during episodes (flare-ups) or may be chronic and continuous. The following are the most common symptoms of juvenile rheumatoid arthritis. However, each child may experience symptoms differently. Symptoms may include:
- swollen, stiff, and painful joints, especially in the morning or after a nap (joints in the knees, hands, and feet are most commonly affected); as well as warmth and redness in a joint
- high fever and rash, if systemic juvenile rheumatoid arthritis
- swollen lymph nodes, if systemic juvenile rheumatoid arthritis
- eye inflammation
- fatigue
- decreased appetite, poor weight gain, and slow growth
Symptoms of juvenile rheumatoid arthritis may resemble other medical conditions or problems. Always consult your child's physician for a diagnosis.
Currently, there is no diagnostic test that definitively tells that a child has JRA. Diagnosis of juvenile rheumatoid arthritis is usually confirmed based on the presence of the following:
-
reported
symptoms
Joint inflammation usually must be present for at least six weeks, continuously. -
laboratory
tests
Laboratory tests, such as certain blood tests, can help rule out other conditions and help determine the type of juvenile rheumatoid arthritis present. Frequent tests that are checked when suspecting JRA include the ANA (antinuclear antibody), RF (rheumatoid factor), CBC (complete blood count), blood chemistries (including liver and kidney function tests), urine analysis, ESR (erythrocyte sedmentation rate), and CRP (c-reactive protein). -
arthrocentesis (joint aspiration)
It is sometimes necessary to confirm the diagnosis of JRA and rule out infection of the joint by removing fluid from the joint space (synovial fluid) and examining it under the microscope for signs of inflammation. -
radiology
studies
Different radiology studies are often used to aide in the diagnosis of JRA and to monitor the progression of the disease. X-rays (plain films), CT scans and MRI scans are used in different situations to closely examine the bones and joints.
How is JRA treated?
The goal of treatment for juvenile rheumatoid arthritis is to maintain a normal activity level for your child. Specific treatment for juvenile rheumatoid arthritis depends on many factors, including the type of JRA, the severity of the involvement, whether or not eye inflammation exists, and how your child responds to or tolerates different therapies. At UCLA, we take a team approach to the care of children with JRA and we feel it is very important to involve both the parents and the child with every decision concerning the treatment plan.
Treatment of JRA begins with an understanding of the disease and the different options available for children. Counseling is an important part of each visit at UCLA. Often, we look to other modes of therapy outside of medications to aide in the healing process. This may include physical therapy (which focuses on the lower body and on improving and maintaining muscle and joint function) and occuptaional therapy (which focuses on the upper body and on improving the ability to perform activities of daily living). In addition, all patients with JRA should be examined by an ophthomologist (eye specialist) to check for inflammation in the eyes. Pediatric rheumatologists often work very closely with ophthomologist to coordinate care for both the child’s eyes and joints.
Although there is no cure for JRA, there are many different medications available to treat it. The UCLA pediatric rheumatologists choose medications based on the most effective therapy with the fewest side effects. Common medications used for JRA include non-steroidal anti-inflammatory drugs or NSAIDs (such as naproxen or ibuprofen), disease-modifying anti-rheumatic medications (such as methotrexate), corticosteroids (such as prednisone), intra-articular corticosteroids or steroid joint injections, and biologic agents (such as Enbrel or Remicaide). For more information on these medicines and there potential side effects, please ask your pediatric rheumatologist.
Systemic Lupus Erythematosus (SLE, or Lupus)
What is lupus?
Systemic lupus erythematosus, also known as SLE, or simply lupus, is a disease that is characterized by periodic episodes of inflammation of and damage to the connective tissues and organs, including the heart, lungs, blood vessels, brain, kidneys, and skin. The kidneys, heart, lungs, and brain are the organs most affected. Lupus affects each individual differently and the effects of the illness range from mild to severe. Lupus potentially can be fatal.
Lupus is more common in young women (late teens to 30s). This may be due to the fact that estrogen (a female hormone) seems to be associated with SLE in some cases. Men can also be affected and have severe disease. Lupus affects more African Americans, Asian Americans, Latinos, and Native Americans than Caucasian Americans. Lupus in children occurs most often at the age of ten and older; lupus is rare in children younger than 5 years of age.
The disease is known to have periods of flare-ups and periods of remission (partial or complete lack of symptoms). Children with lupus frequently have kidney involvement. The severity of the kidney involvement can alter the survival rate of patients with lupus. In some cases, kidney damage is so severe it leads to kidney failure.
What causes lupus?
Lupus is an autoimmune disorder, which means the body's immune system attacks its own healthy cells and tissues.
Lupus is considered to be a multifactorial condition. Multifactorial means that "many factors" are involved in causing a health problem. The factors are usually both genetic and environmental, where a combination of genes from both parents, in addition to unknown environmental factors, produce the trait or condition. Often one gender (either males or females) is affected more frequently than the other in multifactorial traits. Multifactorial traits do occur in families because they are partly caused by genes. Females are affected with lupus three to 10 times more often than males.
What are the symptoms of lupus and how is it diagnosed?
Lupus symptoms are usually chronic and recurring. The following are the most common symptoms of lupus, however, each child may experience symptoms differently. Symptoms may include:
- malar rash - a rash shaped like a butterfly that is usually found over the bridge of the nose and on the cheeks.
- discoid rash - a raised rash found on the head, arms, chest, or back.
- fever
- inflammation of the joints
- sunlight sensitivity
- hair loss
- mouth ulcers
- fluid around the lungs, heart, or other organs
- kidney problems
- anemia, low white blood cell, or low platelet count
- Raynaud's phenomenon - a condition in which the blood vessels of the fingers and toes go into spasm and cause them to change color to purple or white when triggered by factors such as cold, stress, or illness.
- weight loss
- nerve or brain dysfunction
Symptoms of lupus may resemble other medical conditions or problems. Always consult your child's physician for a diagnosis.
Lupus is difficult to diagnose because the symptoms may be vague. There is no single test that can diagnose lupus. To fullfil criteria for a diagnosis of lupus, a child must have a specific combination of certain symptoms, physical exam findings, and laboratory tests. Some of these tests include: a CBC (complete blood count), blood chemistries (including liver and kidney function), inflammatory blood tests (ESR or erythrocyte sedementation rate and CRP or c-reactive protein), urine analysis (to check for blood or protein in the urine), complement levels (blood proteins that help to destroy foreign substances and are often low in active lupus). Specific tests for lupus include autoantibodies such as ANA (antinuclear antibody test), double-stranded DNA, SSA/SSB, RNP, sm, and SCL-70. Further tests including x-rays, ultrasound of the heart, lung function tests, skin biopsy or kidney biopsy may be needed depending on the presentation of the disease.
How is lupus treated?
There is no cure for lupus. The goal of treatment in lupus is to control inflammation to prevent long-term organ damage and scarring. UCLA Pediatric Rheumatologists use a team approach to caring for patients with lupus. This involves both active participation on the part of the child and the parents in the decision-making process of treatment. The medications used in treatment will vary depending on the amount and severity of organ involvement.
Treatment of lupus begins with an understanding of the disease and the different options available for children. Counseling is an important part of each visit at UCLA. Often, we look to other modes of therapy outside of medications to aide in the healing process. This may include physical therapy (which focuses on the lower body and on improving and maintaining muscle and joint function) and occuptaional therapy (which focuses on the upper body and on improving the ability to perform activities of daily living). All patients with lupus should avoid sun exposure during the peak sunlight hours of the day (from 10 AM to 3 pm) and wear sunscreen with SPF rating of at least 30. If the kidneys are affected by lupus, pediatric nephrologists (kidney doctors) will often be involved as well. At UCLA, we have a monthly Lupus clinic where lupus patients can be seen simultaneously by both the rheumatologist and the nephrologist to coordinate care.
There are many different medications used in treating lupus. UCLA pediatric rheumatologists will choose medicines based on the most effective therapy with the fewest side effects. Common medicines used in treating lupus include nonsteroidal anti-inflammatory drugs or NSAIDs (such as naproxen or ibuprofen), anti-malarial medicines (such as hydroxychlorquine or plaquenil), blood pressure medications (such as ACE inhibitors like lisinopril), corticosteroids (such as prednisone), and immunosuppressive agents (such as cyclophosphamide or cytoxan, and mycophenolate mofetil or cellcept). To find out more information about these medications and their potential side effects, please ask you pediatric rheumatologist.
Juvenile Dermatomyositis (JDM)
What is juvenile dermatomyositis?
Juvenile dermatomyositis is one of the conditions in a group of conditions called the dermatomyositis/polymyositis complex. The conditions in this complex are characterized by muscle damage due to inflammation process of the blood vessels that lie under the skin and in the muscles. Skin changes around the eyelids (heliotrope rash) and over the knuckles and finger joints (Gottron's papules) are also seen. Juvenile dermatomyositis is the condition most often seen in children.
Juvenile dermatomyositis is rare, and it affects girls more often than boys.
What causes juvenile dermatomyositis?
The cause of juvenile dermatomyositis has not yet been determined. However, factors which are thought to be associated with dermatomyositis include dysfunction of the immune system, resulting in inflammation of the blood vessels and infections.
What are the symptoms of juvenile dermatomyositis and how is it diagnosed?
The symptoms of juvenile dermatomyositis often appear gradually. At times, there may be a more acute, or intense, onset of symptoms. Most patients with JDM have muscle pain or tenderness, muscle weakness, and skin rash usually around the eyelids, knuckles, elbows or knees. In addition, children can also have fevers, fatigue, weight loss, joint pain and inflammation, and calcium depositis under the skin. Often, patients will have different phases of illness, including: a prodromal period (nonspecific symtpoms like fever and fatigue that can last from weeks to months), progressive muscle weakness and rash (which can last up to two years), persistent muscle weakness, inflammation and rash ( which can last up to another two years), and recovery (which can occur without consequence or with chronic changes and calcium deposition due to the disease).
There is no single test that can diagnose JDM. The diagnosis is based on patient symptoms, physical exam findings of rash, specific muscle weakness, and laboratory tests. Lab tests used to help diagnose JDM include a CBC (complete blood count), blood chemistries (including liver and kidney tests), inflammatory markers (ESR or erythrocyte sedmentation rate and CRP or c-reactive protein), muscle enzyme tests (including total CK and aldolase levels), a urine analysis, and antibody testing. Often, further tests are used to confirm the diagnosis such as EMG (electromyography), MRI scans, and muscle or skin biopsies. It is important to establish a clear diagnosis of JDM so that the treatment plan will be effective.
How is juvenile dermatomyositis treated?
Juvenile dermatomyositis cannot be cured. However, with supportive therapy and a multidisciplinary team approach to treatment, remission of the disease may be achievable in time. UCLA pediatric rheumatologists use a team approach to caring for patients with JDM. This involves active participation of both the parents and the child in the decision-making process of treating JDM. The type of treatment will vary depending on the severity of disease involvement.
Treatment of juvenile dermatomyositis begins with an understanding of the disease and the different options available for children. Counseling is an important part of each visit at UCLA. Often, we look to other modes of therapy outside of medications to aide in the healing process. This may include physical therapy (which focuses on the lower body and on improving and maintaining muscle and joint function) and occuptaional therapy (which focuses on the upper body and on improving the ability to perform activities of daily living). In addition, patients with skin rashes are encouraged to avoid sun exposure during peak sunlight hours (10 am to 3 pm) and wear sunscreen with an SPF rating of at least 30.
There are many different medications used in treating JDM. UCLA pediatric rheumatologists will choose medicines based on the most effective therapy with the fewest side effects. Common medicines used in treating JDM include nonsteroidal anti-inflammatory drugs or NSAIDs (such as naproxen or ibuprofen), anti-malarial medicines (such as hydroxychlorquine or plaquenil), disease modifying anti-rheumatic drugs (such as methotrexate), corticosteroids (such as prednisone), and immunomodulating agents (such as intravenous immunoglobulin). To find out more information about these medications and their potential side effects, please ask you pediatric rheumatologist.
What is vasculitis?
Vasculitis is a term that refers to several different conditions, all of which involve inflammation and/or damage to the blood vessels, ranging from moderate to severe. Some conditions, such as Kawasaki disease, involve inflammation and death of tissue in arteries. Other conditions, such as Henoch-Schönlein purpura (HSP), may affect both arteries and veins. Other conditions, such as Wegener’s granulomatosis or microscopic polyangiitis, are associated with certain antibodies (P or C ANCA antibodies). HSP is the most common form of vasculitis seen in children, and occurs most often in children between ages 3 and 15 years.
What causes vasculitis?
The exact cause of vasculitis is not known. However, research indicates that causes may include infectious agents and dysfunction of the immune system.
What are the symptoms of vasculitis, and how is it diagnosed?
Symptoms related to vasculitis may vary, depending on the specific type of vasculitis. Often times, children with vasculitis will have nonspecific symptoms early in the disease such as fevers, fatigue, and weight loss. Many types of vasculitis have skin rashes associated, including Kawasaki’s disease, Henoch-Schönlein purpura, and others. Vasculitis can affect many different organs, including the brain, the lungs and respiratory system, the heart, the liver, the kidneys and the joints. Because of this fact, symptoms will vary depending on the organs involved.
There is no single test that can diagnose vasculitis. Vasculitis often mimics other diseases such as infections and cancer, and these diseases should be looked for in patients who have similar symptoms to those described above. Laboratory tests used to aide in the diagnosis often include: a CBC (complete blood count), blood chemistries (including liver and kidney tests), inflammatory markers (ESR or erythrocyte sedmentation rate and CRP or c-reactive protein), a urine analysis, and antibody testing. In addition, x-rays, ultrasound of the heart, CT scan (cat scan) and MRI are often used to look closer at specific areas that may have inflammation of the blood vessels. Biopsy of the skin or other involved organs is often necessary to help confirm the diagnosis of vasculitis.
How is vasculitis treated?
The course of the disease varies greatly with the type of vasculitis a patient has. Some types (such as Henoch-Schönlein purpura) will have relatively self-limited courses and not require any specific treatment. Others may be life-threatening if they are untreated. UCLA pediatric rheumatologists use a team approach to caring for patients with vasculitis. This involves active particpation of both the parents and the child in the decision-making process of treating vasculitis.
Treatment of vasculitis begins with an understanding of the specific disease and the different options available for children. Counseling is an important part of each visit at UCLA. Often, we look to other modes of therapy outside of medications to aide in the healing process. This may include physical therapy (which focuses on the lower body and on improving and maintaining muscle and joint function) and occuptaional therapy (which focuses on the upper body and on improving the ability to perform activities of daily living). In addition, patients with skin rashes are encouraged to avoid sun exposure during peak sunlight hours (10 am to 3 pm) and wear sunscreen with an SPF rating of at least 30.
There are many different medications used in treating vasculitis. UCLA pediatric rheumatologists will choose medicines based on the most effective therapy with the fewest side effects. Common medicines used in treating vasculitis include nonsteroidal anti-inflammatory drugs or NSAIDs (such as naproxen or ibuprofen), aspirin, disease modifying anti-rheumatic drugs (such as methotrexate), corticosteroids (such as prednisone), and immunomodulating agents (such as intravenous immunoglobulin), and immunosuppresive medications (such as cyclophosphamide or cytoxan). To find out more information about these medications and their potential side effects, please ask you pediatric rheumatologist.
What is scleroderma?
Scleroderma means “hardening of the skin.” This is a relatively rare condition in children that often presents first with characteristic skin changes. There are two main types of scleroderma- “systemic” scleorsis, meaning widespread with multiple organ involvement, and “localized” disease, mainly involving the skin. Systemic sclerosis can be a life-threatening disorder, depending on the amount and severity of organ involvement. Localized scleroderma, which can include “morphea” and “linear scleroderma,” often is restricted to the skin but can cause problems with joints and muscles.
What causes scleroderma?
The cause of all types of scleroderma is not known. Research indicates that cells in our bodies called fibroblasts are inappropriately activated to produce too much collagen, a substance that normally is distributed in skin. Consequently, scarring forms in the skin, lungs and other organs. It is thought that this may be caused by an unknown infection or immune system dysregulation.
What are the symptoms or scleroderma and how is it diagnosed?
Symptoms of scleroderma vary with the different types. Localized scleroderma patients usually have distinct areas of hard skin that may appear shiny and are sometimes discolored. If the skin affected crosses a joint line, there can be difficulty in moving that joint. There can be some pain or tenderness associated as well. Systemic scleroderma patients tend to have more skin involvement in addition to other organs involved. Patients often will have Raynaud’s phenomenon, which is a condition where the fingers and toes will become discolored (white or purple) and painful when exposed to cold weather. Systemic sclerosis can also affect the lungs, the kidneys, the heart, and the digestive tract.
There is no blood test that can diagnose scleroderma. The diagnosis is usually made based on patient history, findings on physical exam, and laboratory tests. Common lab tests used include: CBC (complete blood count), blood chemistries (including liver and kidney tests), inflammatory markers (ESR or erythrocyte sedmentation rate and CRP or c-reactive protein), a urine analysis, ANA (antinuclear antibody test) and other antibody testing (SCL-70 and centromere antibodies). Often a skin biopsy is used to confirm the diagnosis of scleroderma. Other tests such as x-rays, lung function tests, ultrasound, CT scan, and MRI scans are used in certain situations to further define the disease.
How is scleroderma treated?
There is no cure for scleroderma. The goal of treatment is to reduce the amount and severity of involvement and to prevent further damage to the skin and other organs. UCLA pediatric rheumatologists use a team approach to caring for patients with scleroderma. This involves active participation by both the parents and the child in the decision-making process of treating scleroderma.
Treatment of scleroderma begins with an understanding of the disease and the different options available for children. Counseling is an important part of each visit at UCLA. Often, we look to other modes of therapy outside of medications to aide in the healing process. This may include physical therapy (which focuses on the lower body and on improving and maintaining muscle and joint function) and occuptaional therapy (which focuses on the upper body and on improving the ability to perform activities of daily living). Patients with scleroderma are often also cared for by dermatologists, who the pediatric rheumatologist work closely with at UCLA. In addition, patients are encouraged to avoid sun exposure during peak sunlight hours (10 am to 3 pm) and wear sunscreen with an SPF rating of at least 30.
There are many different medications used in treating scleroderma. UCLA pediatric rheumatologists will choose medicines based on the most effective therapy with the fewest side effects. Common medicines used in treating scleroderma include skin creams (such as Dovenex), disease modifying anti-rheumatic drugs (such as methotrexate), corticosteroids (such as prednisone), and immunosuppresive medications (such as cyclophosphamide or cytoxan). To find out more information about these medications and their potential side effects, please ask you pediatric rheumatologist.
What is fibromyalgia?
Fibromyalgia, is a chronic, widespread pain in muscles and soft tissues surrounding the joints throughout the body, accompanied by fatigue. The disease is fairly common, affecting approximately 2 percent of the US population.
Although its symptoms are similar to other joint diseases, such as arthritis, fibromyalgia is actually a condition of the soft tissue or muscular system that causes pain in the muscles and soft tissues.
Fibromyalgia is one of several pain syndromes included in the classification of musculoskeletal pain syndrome (MSPS), or pain amplification syndrome.
What causes or triggers fibromyalgia?
Although the cause of fibromyalgia is unknown, researchers believe there may be a link with sleep disturbance, psychological stress, or immune, endocrine, or biochemical abnormalities. Fibromyalgia mainly affects the muscles and the points at which the muscles attach to the bone (at the ligaments and tendons).
What are the symptoms of fibromyalgia and how is it diagnosed?
Pain is the most common and chronic symptom of fibromyalgia. Pain may begin in one area of the body, such as the neck and shoulders, but eventually the entire body may become affected. The pain ranges from mild to severe and may be described as burning, soreness, stiffness, aching, or gnawing pain. Fibromyalgia is usually associated with characteristic tender spots of pain (or trigger points) in the muscles. The following are other common symptoms of fibromyalgia. However, each child may experience symptoms differently. Symptoms may include:
- moderate to severe fatigue
- decreased exercise endurance
- sleep problems at night
- depressed mood
- anxiety
- poor school attendance
- headaches
There are no laboratory tests that can confirm a diagnosis of fibromyalgia. Instead, diagnosis is usually based on reported symptoms. Laboratory tests and other tests, such as x-rays, may be performed in order to rule out other causes of the symptoms shown by your child.
How is fibromyalgia treated?
There is no cure for fibromyalgia. The goal of treatment is to control the pain associated with fibromyalgia, improve sleep patterns, and maintain a healthy and active lifestyle for the child. UCLA pediatric rheumatologists use a team approach to caring for patients with fibromyalgia. This involves active participation by both the parents and the child in the decision-making process of treating fibromyalgia.
Treatment of fibromyalgia begins with an understanding of the disease and the different options available for children. Counseling is an important part of each visit at UCLA. Often, we look to other modes of therapy outside of medications to aide in the healing process. This may include physical therapy (which focuses on the lower body and on improving and maintaining muscle and joint function) and occuptaional therapy (which focuses on the upper body and on improving the ability to perform activities of daily living). Sleep hygiene and relaxation techniques are often taught during clinic visits. Patients with fibromyalgia are often also cared for by pain management physicians, with whom the Pediatric Rheumatologist work closely at UCLA.
There are many different medications used in treating fibromyalgia. UCLA pediatric rheumatologists will choose medicines based on the most effective therapy with the fewest side effects. Common medicines used in treating fibromyalgia include nonsteroidal anti-inflammatory drugs or NSAIDs (such as naproxen or ibuprofen) and restorative sleep medications (such as amitryptiline or Elavil). To find out more information about these medications and their potential side effects, please ask you pediatric rheumatologist.