ABP Pediatric Rheumatology flashcards

32 cards. Read the term and the definition here. The deck at the top of this page is optional.

HLA class I function

HLA-A, -B, and -C molecules present endogenous peptides to CD8+ cytotoxic T cells; HLA-B27 is strongly associated with enthesitis-related arthritis and reactive arthritis.

Inflammasome activation

Cytosolic NOD-like receptors assemble inflammasomes that activate caspase-1, which cleaves pro-IL-1 beta into active IL-1 beta; gain-of-function NLRP3 mutations cause cryopyrin-associated periodic syndromes.

Complement deficiency and lupus

Hereditary deficiency of early classical complement components such as C1q, C2, or C4 strongly predisposes to SLE, whereas deficiency of terminal components C5 through C9 predisposes to recurrent Neisseria infection.

Oligoarticular JIA definition

Arthritis in four or fewer joints during the first six months of disease; persistent oligoarticular disease never exceeds four joints, while extended disease involves more than four after six months.

Systemic JIA features

Daily spiking fever for at least two weeks, evanescent salmon-colored rash, arthritis, lymphadenopathy, and serositis; ferritin, CRP, and ESR are elevated and macrophage activation syndrome is a major complication.

Uveitis screening in JIA

Chronic anterior uveitis is usually asymptomatic; slit-lamp screening frequency depends on JIA category, ANA status, and age at onset, ranging from every three to every twelve months.

Anti-CCP in JIA

Anti-cyclic citrullinated peptide antibody is less sensitive than rheumatoid factor but more specific for rheumatoid factor-positive polyarticular JIA and predicts erosive disease.

EULAR/ACR lupus criteria

The entry criterion is an ANA of at least 1:80; weighted clinical and immunologic domains are scored, and a total of at least 10 points with one clinical domain classifies SLE.

Neonatal lupus risk

Maternal anti-Ro/SSA or anti-La/SSB antibodies cross the placenta and can cause congenital complete heart block, transient rash, cytopenias, and hepatitis in the newborn.

Juvenile dermatomyositis features

Proximal muscle weakness with Gottron papules, heliotrope rash, and elevated creatine kinase, aldolase, and lactate dehydrogenase; myositis-specific antibodies help define subtypes and prognosis.

MCTD hallmark antibody

Mixed connective tissue disease is defined by high-titer anti-U1-RNP antibodies plus overlapping features of lupus, scleroderma, and polymyositis, commonly Raynaud phenomenon and puffy hands.

Kawasaki disease treatment

Intravenous immunoglobulin 2 g/kg as a single infusion plus aspirin is given within ten days of fever onset; corticosteroids are added for high-risk patients to reduce coronary artery aneurysm risk.

IgA vasculitis tetrad

Palpable purpura on dependent skin, arthritis or arthralgia, abdominal pain, and renal involvement; IgA deposition on biopsy confirms the diagnosis and urinalysis is monitored for months.

ANCA staining patterns

Proteinase-3 ANCA produces a cytoplasmic pattern and is linked to granulomatosis with polyangiitis; myeloperoxidase ANCA produces a perinuclear pattern and is linked to microscopic polyangiitis.

Takayasu arteritis clues

Adolescent girls with hypertension, unequal arm blood pressures, diminished pulses, or claudication; MR or CT angiography shows stenosis or wall thickening of the aorta and its branches.

Familial Mediterranean fever

MEFV variants cause one- to three-day episodes of fever, peritonitis, pleuritis, and erysipelas-like rash; daily colchicine prevents attacks and prevents AA amyloidosis.

PFAPA syndrome

Periodic fever with aphthous stomatitis, pharyngitis, and cervical adenitis in children aged two to five years; attacks last three to six days and a single corticosteroid dose aborts an episode.

CAPS and IL-1 blockade

NLRP3 gain-of-function mutations cause cold-induced urticaria, fever, and arthralgia; anakinra or canakinumab block IL-1 and control the inflammation.

Growing pains

Benign bilateral evening leg pain in children aged three to twelve years that responds to massage; examination, growth, and function are normal and management is reassurance alone.

Complex regional pain syndrome

Disproportionate regional pain with allodynia, temperature and color changes, and functional loss after minor injury; treatment is intensive physical and occupational therapy with psychological support.

Hypermobility assessment

The Beighton score quantifies joint hypermobility; management of symptomatic hypermobility focuses on muscle strengthening, proprioceptive training, and activity modification rather than restriction.

Septic arthritis urgency

Acute monoarthritis with fever requires urgent joint aspiration for cell count, Gram stain, and culture; bacterial infection needs drainage and intravenous antibiotics to prevent joint destruction.

Lyme arthritis

Monoarthritis, usually of the knee, in a patient from an endemic area with positive Borrelia burgdorferi serology; it responds to oral antibiotics and usually resolves without chronic damage.

Acute rheumatic fever

Follows group A streptococcal pharyngitis; Jones criteria require evidence of preceding infection plus migratory polyarthritis, carditis, chorea, erythema marginatum, or subcutaneous nodules.

ANA testing caveats

Indirect immunofluorescence on HEp-2 cells reports titer and pattern; low titers occur in 5 to 15 percent of healthy children, so ANA must be interpreted with clinical features and never alone.

MRI in musculoskeletal disease

MRI detects early synovitis, bone marrow edema, and osteomyelitis before radiographs change; it is the most sensitive imaging test for sacroiliitis and occult inflammatory joint disease.

Synovial fluid analysis

Cell count and differential, Gram stain, culture, and crystal examination distinguish septic, inflammatory, and mechanical effusions; a white cell count above 50,000 per microliter suggests bacterial infection.

Methotrexate monitoring

Given weekly with folic acid; check CBC, liver enzymes, and creatinine at baseline and periodically; toxicity includes hepatotoxicity, stomatitis, and pneumonitis, and the drug is teratogenic.

TNF inhibitor pretreatment

Before starting a TNF inhibitor, screen for latent tuberculosis and hepatitis B; live vaccines are contraindicated during therapy and the risk of serious infection is increased.

Hydroxychloroquine safety

Generally safe in pregnancy and used for cutaneous and systemic lupus; annual ophthalmologic examination is recommended after five years because of rare retinal toxicity.

Transition readiness

Structured transition programs begin in early adolescence, assess self-management skills, and transfer care to adult rheumatology by age eighteen to twenty-one to prevent loss of follow-up.

CHAQ purpose

The Childhood Health Assessment Questionnaire measures physical function and disability across eight domains in children with rheumatic disease and tracks response to treatment over time.