BONE AND CONNECTIVE TISSUE DISORDERS 1.3 (AB)

Question 1

What is the most common rheumatic disease in children?

Answer 1

Juvenile idiopathic arthritis (JIA)

Question 2

What clinical manifestation is shared by all JIA subtypes?

Answer 2

Arthritis

Question 3

What term did the former classification scheme of the American College of Rheumatology use for JIA?

Answer 3

Juvenile rheumatoid arthritis

Question 4

What organization proposed a standardized classification using the term JIA?

Answer 4

The International League of Associations for Rheumatology (ILAR)

Question 5

What are the age criteria for JIA diagnosis?

Answer 5

Onset before age 16

Question 6

What are the joint criteria for JIA diagnosis?

Answer 6

Arthritis in ≥ 1 joint with swelling or effusion, or ≥2 of limited ROM, tenderness/pain on motion, increased heat

Question 7

What is the required duration of arthritis for JIA diagnosis?

Answer 7

At least 6 weeks

Question 8

How is onset type of JIA defined?

Answer 8

By type of articular involvement in the first 6 months

Question 9

What are the three onset types of JIA?

Answer 9

Polyarthritis, Oligoarthritis, Systemic-onset disease

Question 10

Which JIA subtype is most common?

Answer 10

Oligoarthritis (40-50%)

Question 11

What is the incidence range of JIA worldwide?

Answer 11

0.8–22.6 per 100,000 children per year

Question 12

What is the prevalence range of JIA worldwide?

Answer 12

7–401 per 100,000

Question 13

Is there sex predominance in systemic JIA?

Answer 13

No

Question 14

What is the sex ratio in oligoarticular JIA?

Answer 14

3:1 (girls to boys)

Question 15

What is the sex ratio in polyarticular JIA?

Answer 15

5:1 (girls to boys)

Question 16

What is the peak age of onset for oligoarticular JIA?

Answer 16

2–4 years

Question 17

What is the bimodal age distribution for polyarticular JIA?

Answer 17

2–4 years and 10–14 years

Question 18

What immune systems are altered in JIA?

Answer 18

Both humoral and cell-mediated immunity

Question 19

How is systemic JIA different from other subtypes in terms of immunity?

Answer 19

sJIA involves dysregulation of the innate immune system

Question 20

What is a characteristic pathology of inflammatory synovitis in JIA?

Answer 20

Villous hypertrophy and hyperplasia with hyperemia and edema

Question 21

What cells are predominantly infiltrated in JIA synovium?

Answer 21

T lymphocytes and plasma cells

Question 22

What is pannus formation in JIA?

Answer 22

Inflamed tissue that erodes cartilage and bone

Question 23

What causes joint pain and stiffness in elderly with arthritis?

Answer 23

Depletion of synovial fluid leading to friction

Question 24

What is the definition of arthritis in JIA?

Answer 24

Swelling or ≥2 of: limited ROM, tenderness/pain on motion, warmth

Question 25

What are common initial symptoms of JIA?

Answer 25

Morning stiffness, limp, gelling after inactivity

Question 26

How does arthritis in large joints affect limb growth?

Answer 26

Causes lengthening initially, then premature growth plate closure

Question 27

What causes limb length discrepancy in JIA?

Answer 27

Initial accelerated growth followed by premature closure of growth plates

Question 28

What type of diagnosis is JIA?

Answer 28

Clinical diagnosis

Question 29

Are there diagnostic lab tests for JIA?

Answer 29

No, only supportive or prognostic tests like ANA and RF

Question 30

What multisystem rheumatic diseases can mimic JIA?

Answer 30

SLE, juvenile dermatomyositis, sarcoidosis, vasculitic syndromes

Question 31

What condition's joint limitation may mimic chronic arthritis?

Answer 31

Scleroderma

Question 32

What are features of acute rheumatic fever that help differentiate it from JIA?

Answer 32

Exquisite joint pain, remittent fever, migratory polyarthritis

Question 33

What liver condition can present with acute arthritis?

Answer 33

Autoimmune hepatitis

Question 34

What are the goals of JIA treatment?

Answer 34

Disease remission, prevent joint damage, support normal growth and development

Question 35

How long is NSAID trial before escalating treatment in oligoarthritis?

Answer 35

4–6 weeks

Question 36

What is the role of intraarticular corticosteroids in JIA?

Answer 36

Used for functional limitations or poor NSAID response

Question 37

What long-acting corticosteroid is used in JIA?

Answer 37

Triamcinolone hexacetonide

Question 38

What are second-line treatments for JIA if NSAIDs and steroids fail?

Answer 38

DMARDs (e.g., methotrexate), TNF inhibitors

Question 39

What is systemic lupus erythematosus (SLE)?

Answer 39

A chronic autoimmune disease characterized by multisystem inflammation and autoantibodies against self-antigens.

Question 40

Which population is most affected by SLE?

Answer 40

Females of reproductive age.

Question 41

Which organs are most commonly involved in SLE?

Answer 41

Skin, joints, kidneys, blood-forming cells, blood vessels, and CNS.

Question 42

How does pediatric SLE differ from adult SLE?

Answer 42

Pediatric SLE is more severe and involves more organs.

Question 43

What genetic factors are associated with SLE?

Answer 43

Congenital deficiencies of C1q, C2, and C4; polymorphisms in IRF5 and PTPN22; familial clustering.

Question 44

What are constitutional symptoms of SLE?

Answer 44

Fatigue, anorexia, weight loss, fever, lymphadenopathy.

Question 45

What are musculoskeletal symptoms of SLE?

Answer 45

Arthritis, myositis, tendonitis, arthralgias, myalgias, avascular necrosis, osteoporosis.

Question 46

What are common skin manifestations in SLE?

Answer 46

Malar rash, discoid rash, photosensitive rash, cutaneous vasculitis, livedo reticularis, Raynaud phenomenon, alopecia, oral and nasal ulcers.

Question 47

What renal findings are seen in SLE?

Answer 47

Hypertension, proteinuria, hematuria, edema, nephrotic syndrome, renal failure.

Question 48

What cardiovascular manifestations can SLE cause?

Answer 48

Pericarditis, myocarditis, conduction abnormalities, Libman-Sacks endocarditis.

Question 49

List some neuropsychiatric symptoms of SLE.

Answer 49

Seizures, psychosis, cerebritis, stroke, myelitis, depression, cognitive impairment, migraines, neuropathies.

Question 50

What pulmonary symptoms can occur in SLE?

Answer 50

Pleuritis, interstitial lung disease, hemorrhage, hypertension, embolism.

Question 51

What hematologic abnormalities are seen in SLE?

Answer 51

Hemolytic anemia, thrombocytopenia, leukopenia, anemia of chronic disease, hypercoagulability.

Question 52

What GI symptoms may be associated with SLE?

Answer 52

Hepatosplenomegaly, pancreatitis, bowel vasculitis, peritonitis.

Question 53

What ocular findings may occur in SLE?

Answer 53

Retinal vasculitis, scleritis, episcleritis, papilledema, dry eyes, optic neuritis.

Question 54

What is required for diagnosis of SLE?

Answer 54

Presence of 4 of 11 ACR 1997 criteria over time.

Question 55

Is ANA required to diagnose SLE?

Answer 55

No, but ANA-negative lupus is rare.

Question 56

What is the sensitivity and specificity of ANA for SLE?

Answer 56

Sensitivity: 95–99%, Specificity: ~50%.

Question 57

Which antibodies are specific for SLE?

Answer 57

Anti-dsDNA and anti-Smith.

Question 58

What are the 11 ACR criteria for SLE diagnosis?

Answer 58

Malar rash, discoid rash, photosensitivity, oral/nasal ulcers, arthritis, serositis, renal disorder, neurologic disorder, hematologic disorder, immunologic disorder, positive ANA.

Question 59

What is a notable feature of SLICC criteria compared to ACR?

Answer 59

Higher sensitivity (93% vs 77%), lower specificity (85% vs 99%).

Question 60

Which antibody correlates with lupus nephritis?

Answer 60

Anti–double-stranded DNA.

Question 61

Which antibody is linked to Raynaud phenomenon and pulmonary disease?

Answer 61

Anti-RNP.

Question 62

What antibodies are associated with neonatal lupus and congenital heart block?

Answer 62

Anti-Ro (SSA) and Anti-La (SSB).

Question 63

What antibody is present in most drug-induced lupus cases?

Answer 63

Antihistone antibody.

Question 64

What antibodies increase risk of thrombosis in SLE?

Answer 64

Antiphospholipid antibodies (lupus anticoagulant, anticardiolipin, anti-beta-2 glycoprotein).

Question 65

What lifestyle advice should all SLE patients follow?

Answer 65

Use sunscreen and avoid prolonged sun/UV exposure.

Question 66

What drug is recommended for all SLE patients?

Answer 66

Hydroxychloroquine.

Question 67

What are toxicities of hydroxychloroquine?

Answer 67

Retinal toxicity, vision loss.

Question 68

What is the max recommended dose of hydroxychloroquine?

Answer 68

400 mg/day or ≤6.5 mg/kg.

Question 69

Why are corticosteroids used in SLE?

Answer 69

To rapidly control significant manifestations and flares.

Question 70

Name some corticosteroid side effects in adolescents.

Answer 70

Growth disturbance, weight gain, acne, striae, osteoporosis.

Question 71

What immunosuppressive drugs are used to spare steroids?

Answer 71

Methotrexate, leflunomide, azathioprine, MMF, tacrolimus, cyclophosphamide, rituximab, belimumab.

Question 72

Which drugs are used for lupus nephritis?

Answer 72

Cyclophosphamide, MMF, azathioprine.

Question 73

Which drug is reserved for severe, life-threatening SLE?

Answer 73

Cyclophosphamide.

Question 74

What are common early causes of death in pediatric SLE?

Answer 74

Infection, glomerulonephritis, neuropsychiatric complications.

Question 75

What renal morbidities are associated with childhood lupus?

Answer 75

Hypertension, dialysis, transplantation.

Question 76

What cardiovascular issues can occur in SLE?

Answer 76

Atherosclerosis, MI, cardiomyopathy, valvular disease.

Question 77

Name some musculoskeletal complications of SLE.

Answer 77

Osteopenia, compression fractures, avascular necrosis.

Question 78

What are potential endocrine complications in SLE?

Answer 78

Diabetes, obesity, growth failure, infertility, fetal wastage.

Question 79

What is the 5-year survival rate in pediatric SLE?

Answer 79

Approximately 95%.

Question 80

Why is long-term monitoring important in pediatric SLE?

Answer 80

Due to high risk of future morbidity, complications, and medication side effects.

Question 81

Who should manage pediatric SLE patients ideally?

Answer 81

Pediatric rheumatologists in a multidisciplinary clinic.