Childhood rheumatic diseases and musculoskeletal examination Flashcards
What is the most common type of vasculitis in childhood? What causes it?
Henoch-Schoenlein purpura.
It is caused by IgA immune complex deposits in small vessels in the skin, kidney, joints and gut.
How is Henoch-Schoenlein purpura diagnosed?
It is primarily a clinical diagnosis of a child presenting with purpura + 1 of the following 4:
- arthralgia/arthritis.
- abdominal pain.
- kidney symptoms: proteinuria and/or hematuria.
- skin biopsy showing leucocytoclastic vasculitis with IgA deposition.
What are differential diagnosis of Henoch-Schoenlein purpura?
Meningococcal disease / meningitis.
Thrombocytopenia.
True or false: Most patients with Henoch-Schoenlein purpura will need hospital admittance.
False. The good prognosis is good in spite of dramatic skin manifestations. The patient can in most cases be receive supportive treatment (NSAIDs, paracetamol, prednisolon) at home.
What is the role of urine dipstick tests evaluating Henoch-Shoenlein purpura?
Urine stix for should be performed in the acute phase of the disease and every 2 weeks until no more clinical
signs of purpura or abdominal pain attacks. (There is usually a complete recovery.)
What is Kawasaki disease?
A febrile immunemediated multisystem vasculitis. Vasculitis in small and medium size arteries, the coronary arteries are especially vulnerable.
What are typical findings/symptoms of Kawasaki disease?
High fever.
Bilateral non-purulent conjunctivitis.
Polymorphous exanthema without vesicles or crusts.
Mucosal changes in lips and mouth: Red cracked lips, «strawberrytongue», rubor in mouth- and pharyngeal mucosa.
Rubor and edema in palms of hands and feet, followed by skin peeling in fingers and toes.
Cervical lymphadenopathy (at least one gland > 1,5 cm).
What is the definition of complete and incomplete Kawasaki disease?
Complete: High fever > 5 days + at least 4/5 classical symptoms.
Incomplete: High fever and mucosal findings, but otherwise not fulfilling criteria for the diagnosis.
What is the treatment for Kawasaki disease?
Intravenous immunoglobulins, followed by acetylsalicylic acid orally (first in an anti-inflammatory dose and then in an anti-thrombotic dose).
Both Henoch-Schoenlein purpura and Kawasaki disease have complete recovery. There are however some diseases that increase the risk of complications. What are these?
Henoch Schoenlein purpura: Kidney disease.
Kawasaki disease: Heart disease.
What is the role of echocardiography in the follow up of Kawasaki disease?
Echocardiography should be performed in the acute phase if the disease, and after 2 and 6 weeks, and then after 1 year. (Use of echocardiography should be individualized if coronary artery aneurisms develops.)
What is PFAPA?
PFAPA (Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis) is a childhood syndrome that affects both boys and girls. It causes repeated episodes of fever, mouth sores, sore throat, and swollen lymph nodes. PFAPA usually starts in early childhood between ages 2 and 5.
What is the definition of arthritis?
Hydrops (fluid in the joint) or limitation of movement:
In addition to pain during movement and/or tenderness over the joint.
What are differential diagnostics associated to arthritis in children?
Septic arthritis
Transient coxitis, CLP (Perthe), Epiphysiolysis
Juvenile idiopathic arthritis
Reactiv arthritis (streptococci, gut bacteria, chlamydia, etc.)
Chronic pain conditions
Cancer (Leukemia, sarcoma)
Chronic inflammatory connective tissue diseases
What is juvenile idiopathic arthritis defined as?
Arthritis with unknown cause, duration longer than 6 weeks, and onset before 16 years of age. (It is an umbrella term including many types of arthritis.)
