Pediatric Rheumatology

Juvenile idiopathic arthritis (JIA)

What is the most common rheumatological disease of childhood?

synovitis, lymphocytes, thickened, proteases, destruction, >, 1, 8

Juvenile Idiopathic Arthritis: Background

-Most common rheumatological disease of childhood

-Etiology → autoimmune disease

-Pathophysiology → Chronic __________

• Synovium infiltrated by ____________ and inflammatory cytokines → becomes ___________ and hypervascular → production/release of tissue __________/collagenases → tissue/bone ____________

-Epidemiology

• Females _ males

• Peak at _-3 and _-12 years old (but can occur at any age)

swelling, edema, ROM, uveitis

Juvenile Idiopathic Arthritis: General Symptoms

-Joint _________, pain, stiffness

• The joint swelling is often more acute and triggered by some sort of event

-Physical exam may reveal joint ______/effusion, erythema, limited ___, tenderness to palpation

-__________ is a potential ocular manifestation, as it is associated with other rheumatologic disorders

<, medium, knee, ankle, not

Oligoarticular Juvenile Idiopathic Arthritis

-Most common form of JIA

-Defined as arthritis in _5 joints within 6 months of diagnosis

-________-large joints are affected → _______ is most common, followed by ______ and wrist

-Systemic symptoms are ___ common

>, symmetric, small, feet, systemic

Polyarticular Juvenile Idiopathic Arthritis

-Arthritis in _ 5 joints

-__________ arthritis is common

-Can affect any sized joint, but _______ joints are the most common

• Classically seen in the hands, _______, and c-spine

-________ symptoms are more common, such as fever and malaise

systemic, fever, pericarditis, 6, sacroiliac, ankylosing, psoriatic, IBD

Systemic JIA and Spondyloarthropathies

-Systemic JIA (uncommon)

• _________ symptoms first → ______ sometimes with rash, failure to thrive, organ inflammation (pleuritic, ____________, hepatosplenomegaly)

• Arthritis starts later (_ weeks to 6 months after systemic symptoms)

-Spondyloarthropathies

• Axial skeleton, __________ joints, tendinous insertions

• Subgroups → _________ spondylitis, _________ arthritis, arthritis of ___

normal, leukemia, elevated, CRP, oligoarticular, uveitis, erosions, septic arthritis, negative

Juvenile Idiopathic Arthritis: Diagnosis

-Patients with oligoarticular JIA often have _______ labs

-CBC → exclude ____________

• WBCs more likely to be _________ in polyarticular and systemic disease

-ESR and ___ → inflammatory markers

• Elevated in polyarticular and systemic disease

-ANA

• More likely to be positive in ____________ or polyarticular

• If positive, more likely to develop _______

-Rheumatoid factor and anti-CCP

• Can be positive in polyarticular

-Xrays → usually normal early on

• May show periarticular osteopenia or _________ of the articular surface if disease progresses

-Arthrocentesis → rule out _______ __________

• JIA has lower WBCs with predominance in lymphocytes and ________ culture

NSAIDs, organ, arthritis, Methotrexate, polyarticular, unresponsive, PT

Juvenile Idiopathic Arthritis: Treatment

-______ (1st line) → Naproxen, ibuprofen, indomethacin

-Systemic corticosteroids → reserved for extreme cases

• Severe systemic JIA with ______ involvement or severe _________

-___________, Hydroxychloroquine, Sulfasalazine (2nd line)

• Methotrexate is drug of choice for ___________ and systemic JIA ____________ to NSAIDs/corticosteroids

-TNF Inhibitors → Etanercept, infliximab, adalimumab

-__ and OT → improve joint function/mobility

destruction, systemic, remission

Juvenile Idiopathic Arthritis: Complications and Prognosis

-Complications → ____________ of joints, blindness (uveitis), polyarticular and _______ JIA more likely to have complications

-Prognosis → 85% complete __________ rate

Henoch-Schonlein Purpura (IgA Vasculitis)

What is the most common systemic vasculitis in childhood?

inflammation, hemorrhage, hypersensitivity, IgA, >, winter

Henoch-Schonlein Purpura: Background

-Most common systemic vasculitis in childhood

-Characterized by ____________ of small blood vessels with leukocytic infiltration of tissue, ___________, and ischemia

-Etiology → unknown, possible ____________ process that involves immune complexes (mostly ___)

-Epidemiology → 3-15 y/o, males _ females, more common in ________

purpura, waist, macules, calves, lower, knee, painful, pain, diarrhea

Henoch-Schonlein Purpura: Symptoms

-Palpable ________ (hallmark)

• Most often below the ______, found on the buttocks and lower extremities

• May start as _________ or urticarial lesions then progress to purpura

• Non-blanching, non-thrombocytopenic

-Edema

• Mostly of _______, dorsum of feet, scalp, scrotum, and/or labia

-Arthritis

• Mostly in ______ extremity, with the ankle and ______ being the most common sites

• Acute and very _________ (often won’t bear weight)

-GI Symptoms

• Mild-moderate crampy abdominal _____

• Bloody _______, distention, and perforation are possible but rare

-Renal disease

• Glomerulonephritis → hematuria, HTN, acute renal failure

-Rarely associated with encephalopathy, pancreatitis, and orchitis

elevated, normal, creatinine, 3-4, NSAIDs, corticosteroids, ischemia

Henoch-Schonlein Purpura: Diagnosis, Treatment, Complications

-Diagnosis

• ESR and CRP are _______

• CBC shows an elevated white count, but platelets are _________

• Renal function (BUN/___________)

• UA (look for hematuria)

-Treatment

• Most cases resolve within __-__ weeks without complications

• Supportive therapy → ________ for pain, systemic ____________ (GI or renal issues)

-Complications

• Rare, but bowel ________, perforation, intussusception, HTN, and renal disease are possible

inflammation, arteries, aneurysm, acquired, Asian, 5

Kawasaki Disease: Background

-Second most common vasculitis in children

-Multisystem involvement and ____________ of small to medium sized _______ with resulting _________ formation, particularly of coronary arteries

-Leading cause of _________ heart disease in US children

-Etiology → unknown

-Epidemiology → more common in individuals of _______ descent, and those younger than _ years old (peak 2-3 y/o)

fever, conjunctivitis, dry, strawberry, swelling, rash, desquamation, coronary, Beau

Kawasaki Disease: Symptoms

-Acute Phase (1-2 weeks)

• High _____, bilateral ____________, mucosal changes with ____ cracked lips and _________ tongue, cervical LAD, __________ of hands and feet, and ____ on the chest and inguinal area

-Subacute Phase (until 4th week)

• Gradual resolution of fever and other symptoms, ___________ of skin (fingers/toes MC), and ________ artery aneurysms

-Convalescent Phase (until 6-8 weeks)

• Disappearance of symptoms, _____ lines of fingernails

elevated, WBC, cultures, ECHO, 2-3, 6-8

Kawasaki Disease: Workup

-CRP and ESR are _________

-CBC → ___ elevated, platelets are normal to low in acute phase and elevated in subacute phase

-CMP → abnormal hepatobiliary functions are common

-Blood/urine _________ → assess for other potential causes

-CXR

-LP → depending on symptoms and to assess for other causes

-Monitor for development of coronary artery aneurysms with _____, which are usually performed at __-__ weeks and __-__ weeks post-diagnosis. This frequency increases if aneurysms are found

5, conjunctivitis, mucus, dry, strawberry, erythema, desquamation, rash

Kawasaki Disease: Diagnostic Criteria

-Fever > _ days duration associated with 4 out of the 5 of the following:

• Bilateral nonsuppurative __________

• One or more changes of the ____ membranes of the upper respiratory tract, including pharyngeal injection, ____ fissured lips, injected lips, and “__________” tongue

• One or more changes of the extremities, including peripheral __________, peripheral edema, periungual ___________, and generalized desquamation

• Polymorphous ____, primarily truncal

• Posterior cervical LAD > 1.5 cm in diameter

• Disease cannot be explained by some other known disease process

IVIG, ASA, acute, subacute, complications

Kawasaki Disease: Treatment

-____ → single dose of 2g/kg over 12 hours

• Helps with symptoms and decreases incidence of aneurysms

-___

• Anti-inflammatory dose: 80-100 mg/kg/day Q6H (_____ phase)

• Anti-thrombotic dose: 3-5 mg/kg/day (________ and convalescent phase)

-Most cases resolve without _____________ but the complications can be serious