Part XV. Rheumatologic Disorders Flashcards
What is the most common rheumatic disease in children and one of the more common chronic illnesses of childhood?
Juvenile Idiopathic arthritis
What is a cornerstone of therapy in pediatric rheumatology because of its sustained effectiveness and relative low toxicity over prolonged periods of treatment?
Methotrexate (MTX)
Naproxen, in its use for treatment in rheumatologic disorders in childhood, is more likely than other NSAIDs to cause a unique skin reaction called?
Pseudoporphyria, characterized by small, hypopigmented depressed scars occurring in areas of minor skin trauma, such as fingernail scratches. Pseudoporphyria is more likely to occur in fair-skinned individuals and on sun-exposed areas. If pseudoporphyria develops, the inciting NSAID should be discontinued because scars can persist for years or may be permanent.
A rare but fatal side effect of Rituximab?
Progressive multifocal leukoencephalopathy
What is the most common subtype of JIA?
Oligoarthritis is the most common subtype (40–50%), followed by polyarthritis (25–30%) and systemic JIA (5–15%). There is no sex predominance in systemic JIA (sJIA), but more girls than boys are affected in both oligoarticular (3 : 1) and polyarticular (5 : 1) JIA. The peak age at onset is 2-4 yr for oligoarticular disease.
The more predominantly affected joints in oligoarthritic JIA?
It predominantly affects the large joints of the lower extremities, such as the knees and ankles. Isolated involvement of upper-extremity large joints is less common.
In JIA, the presence of a positive ____ test confers increased risk for asymptomatic anterior uveitis, requiring periodic slit-lamp examination
antinuclear antibody (ANA)
This is characterized by arthritis, fever, rash, and prominent visceral involvement, including hepatosplenomegaly, lymphadenopathy, and serositis (pericarditis).
Systemic JIA (sJIA)
Describe the characteristic fever of sJIA.
The characteristic fever, defined as spiking temperatures to ≥39°C (102.2°F), occurs on a daily or twice-daily basis for at least 2 wk, with a rapid return to normal or subnormal temperatures. The fever is often present in the evening and is frequently accompanied by a characteristic faint, erythematous, macular rash. The evanescent salmon-colored lesions, classic for sJIA, are linear or circular and are usually distributed over the trunk and proximal extremities. The classic rash is nonpruritic and migratory with lesions lasting <1 hr.
This a cutaneous hypersensitivity in pts with sJIA in which classic lesions are brought on by superficial trauma.
Koebner phenomenon
This is a rare but potentially fatal complication of sJIA that can occur at any time (onset, medication change, active or remission) during the disease course.
Macrophage activation syndrome (MAS), also referred to as secondary hemophagocytic syndrome or hemophagocytic lympho- histiocytosis (HLH).
It classically manifests as acute onset of high-spiking fevers, lymphadenopathy, hepatosplenomegaly, and encephalopathy. Laboratory evaluation shows thrombocytopenia and leukopenia with elevated liver enzymes, lactate dehydrogenase, ferritin, and triglycerides.
sJIA
What is a feature useful in distinguishing MAS from a flare of systemic disease?
erythrocyte sedimentation rate (ESR) falls because of hypofibrinogenemia and hepatic dysfunction
These are used to treat children with sJIA with an inadequate response to methotrexate, with poor prognostic factors, or with severe disease onset.
TNF-α antagonists (e.g., etanercept, adalimumab)
Common clinical features of patients with psoriatic arthritis are?
3, besides arthritis
- nail pitting,
- onycholysis, and
- dactylitis (sausage-like swelling of fingers or toes)
The gold standard for early visualization of sacroiliitis is ?
evidence of bone marrow edema adjacent to the joint on MRI with fluid-sensitive sequences such as short-T1 inversion recovery (STIR)
Factors associated with disease progression in Juvenile ankylosing spondylitis (JAS) include?
4
- tarsitis,
- HLA-B27 positivity,
- hip arthritis within the 1st 6 mo, and
- disease onset after age 8.
How does Poststreptococcal arthritis differ from rheumatic fever?
Poststreptococcal arthritis may follow infection with either group A or group G streptococcus. It is typically oligoarticular, affecting lower-extremity joints, and mild symptoms can persist for months. Poststreptococcal arthritis differs from rheumatic fever, which typically manifests with painful migratory polyarthritis of brief duration.
