BONE AND CONNECTIVE TISSUE DISORDERS 1.3 (AB) Flashcards
What is the most common rheumatic disease in children?
Juvenile idiopathic arthritis (JIA)
What clinical manifestation is shared by all JIA subtypes?
Arthritis
What term did the former classification scheme of the American College of Rheumatology use for JIA?
Juvenile rheumatoid arthritis
What organization proposed a standardized classification using the term JIA?
The International League of Associations for Rheumatology (ILAR)
What are the age criteria for JIA diagnosis?
Onset before age 16
What are the joint criteria for JIA diagnosis?
Arthritis in ≥ 1 joint with swelling or effusion, or ≥2 of limited ROM, tenderness/pain on motion, increased heat
What is the required duration of arthritis for JIA diagnosis?
At least 6 weeks
How is onset type of JIA defined?
By type of articular involvement in the first 6 months
What are the three onset types of JIA?
Polyarthritis, Oligoarthritis, Systemic-onset disease
Which JIA subtype is most common?
Oligoarthritis (40-50%)
What is the incidence range of JIA worldwide?
0.8–22.6 per 100,000 children per year
What is the prevalence range of JIA worldwide?
7–401 per 100,000
Is there sex predominance in systemic JIA?
No
What is the sex ratio in oligoarticular JIA?
3:1 (girls to boys)
What is the sex ratio in polyarticular JIA?
5:1 (girls to boys)
What is the peak age of onset for oligoarticular JIA?
2–4 years
What is the bimodal age distribution for polyarticular JIA?
2–4 years and 10–14 years
What immune systems are altered in JIA?
Both humoral and cell-mediated immunity
How is systemic JIA different from other subtypes in terms of immunity?
sJIA involves dysregulation of the innate immune system
What is a characteristic pathology of inflammatory synovitis in JIA?
Villous hypertrophy and hyperplasia with hyperemia and edema
What cells are predominantly infiltrated in JIA synovium?
T lymphocytes and plasma cells
What is pannus formation in JIA?
Inflamed tissue that erodes cartilage and bone
What causes joint pain and stiffness in elderly with arthritis?
Depletion of synovial fluid leading to friction
What is the definition of arthritis in JIA?
Swelling or ≥2 of: limited ROM, tenderness/pain on motion, warmth
What are common initial symptoms of JIA?
Morning stiffness, limp, gelling after inactivity
How does arthritis in large joints affect limb growth?
Causes lengthening initially, then premature growth plate closure
What causes limb length discrepancy in JIA?
Initial accelerated growth followed by premature closure of growth plates
What type of diagnosis is JIA?
Clinical diagnosis
Are there diagnostic lab tests for JIA?
No, only supportive or prognostic tests like ANA and RF
What multisystem rheumatic diseases can mimic JIA?
SLE, juvenile dermatomyositis, sarcoidosis, vasculitic syndromes
What condition’s joint limitation may mimic chronic arthritis?
Scleroderma
What are features of acute rheumatic fever that help differentiate it from JIA?
Exquisite joint pain, remittent fever, migratory polyarthritis
What liver condition can present with acute arthritis?
Autoimmune hepatitis
What are the goals of JIA treatment?
Disease remission, prevent joint damage, support normal growth and development
How long is NSAID trial before escalating treatment in oligoarthritis?
4–6 weeks
What is the role of intraarticular corticosteroids in JIA?
Used for functional limitations or poor NSAID response
What long-acting corticosteroid is used in JIA?
Triamcinolone hexacetonide
What are second-line treatments for JIA if NSAIDs and steroids fail?
DMARDs (e.g., methotrexate), TNF inhibitors
What is systemic lupus erythematosus (SLE)?
A chronic autoimmune disease characterized by multisystem inflammation and autoantibodies against self-antigens.
Which population is most affected by SLE?
Females of reproductive age.
Which organs are most commonly involved in SLE?
Skin, joints, kidneys, blood-forming cells, blood vessels, and CNS.
How does pediatric SLE differ from adult SLE?
Pediatric SLE is more severe and involves more organs.
What genetic factors are associated with SLE?
Congenital deficiencies of C1q, C2, and C4; polymorphisms in IRF5 and PTPN22; familial clustering.
What are constitutional symptoms of SLE?
Fatigue, anorexia, weight loss, fever, lymphadenopathy.
What are musculoskeletal symptoms of SLE?
Arthritis, myositis, tendonitis, arthralgias, myalgias, avascular necrosis, osteoporosis.
What are common skin manifestations in SLE?
Malar rash, discoid rash, photosensitive rash, cutaneous vasculitis, livedo reticularis, Raynaud phenomenon, alopecia, oral and nasal ulcers.
What renal findings are seen in SLE?
Hypertension, proteinuria, hematuria, edema, nephrotic syndrome, renal failure.
What cardiovascular manifestations can SLE cause?
Pericarditis, myocarditis, conduction abnormalities, Libman-Sacks endocarditis.
List some neuropsychiatric symptoms of SLE.
Seizures, psychosis, cerebritis, stroke, myelitis, depression, cognitive impairment, migraines, neuropathies.
What pulmonary symptoms can occur in SLE?
Pleuritis, interstitial lung disease, hemorrhage, hypertension, embolism.
What hematologic abnormalities are seen in SLE?
Hemolytic anemia, thrombocytopenia, leukopenia, anemia of chronic disease, hypercoagulability.
What GI symptoms may be associated with SLE?
Hepatosplenomegaly, pancreatitis, bowel vasculitis, peritonitis.
What ocular findings may occur in SLE?
Retinal vasculitis, scleritis, episcleritis, papilledema, dry eyes, optic neuritis.
What is required for diagnosis of SLE?
Presence of 4 of 11 ACR 1997 criteria over time.
Is ANA required to diagnose SLE?
No, but ANA-negative lupus is rare.
What is the sensitivity and specificity of ANA for SLE?
Sensitivity: 95–99%, Specificity: ~50%.
Which antibodies are specific for SLE?
Anti-dsDNA and anti-Smith.
What are the 11 ACR criteria for SLE diagnosis?
Malar rash, discoid rash, photosensitivity, oral/nasal ulcers, arthritis, serositis, renal disorder, neurologic disorder, hematologic disorder, immunologic disorder, positive ANA.
What is a notable feature of SLICC criteria compared to ACR?
Higher sensitivity (93% vs 77%), lower specificity (85% vs 99%).
Which antibody correlates with lupus nephritis?
Anti–double-stranded DNA.
Which antibody is linked to Raynaud phenomenon and pulmonary disease?
Anti-RNP.
What antibodies are associated with neonatal lupus and congenital heart block?
Anti-Ro (SSA) and Anti-La (SSB).
What antibody is present in most drug-induced lupus cases?
Antihistone antibody.
What antibodies increase risk of thrombosis in SLE?
Antiphospholipid antibodies (lupus anticoagulant, anticardiolipin, anti-beta-2 glycoprotein).
What lifestyle advice should all SLE patients follow?
Use sunscreen and avoid prolonged sun/UV exposure.
What drug is recommended for all SLE patients?
Hydroxychloroquine.
What are toxicities of hydroxychloroquine?
Retinal toxicity, vision loss.
What is the max recommended dose of hydroxychloroquine?
400 mg/day or ≤6.5 mg/kg.
Why are corticosteroids used in SLE?
To rapidly control significant manifestations and flares.
Name some corticosteroid side effects in adolescents.
Growth disturbance, weight gain, acne, striae, osteoporosis.
What immunosuppressive drugs are used to spare steroids?
Methotrexate, leflunomide, azathioprine, MMF, tacrolimus, cyclophosphamide, rituximab, belimumab.
Which drugs are used for lupus nephritis?
Cyclophosphamide, MMF, azathioprine.
Which drug is reserved for severe, life-threatening SLE?
Cyclophosphamide.
What are common early causes of death in pediatric SLE?
Infection, glomerulonephritis, neuropsychiatric complications.
What renal morbidities are associated with childhood lupus?
Hypertension, dialysis, transplantation.
What cardiovascular issues can occur in SLE?
Atherosclerosis, MI, cardiomyopathy, valvular disease.
Name some musculoskeletal complications of SLE.
Osteopenia, compression fractures, avascular necrosis.
What are potential endocrine complications in SLE?
Diabetes, obesity, growth failure, infertility, fetal wastage.
What is the 5-year survival rate in pediatric SLE?
Approximately 95%.
Why is long-term monitoring important in pediatric SLE?
Due to high risk of future morbidity, complications, and medication side effects.
Who should manage pediatric SLE patients ideally?
Pediatric rheumatologists in a multidisciplinary clinic.
