BOARD REVIEWBOARD REVIEW

RHEUMATOLOGYRHEUMATOLOGY

Dennis A. PeacockDennis A. Peacock

April 9, 2008April 9, 2008

CASE PRESENTATIONCASE PRESENTATION

8 month old male with swollen left 8 month old male with swollen left ankle for several daysankle for several days

No Fever and no signs of being illNo Fever and no signs of being ill

Normal growth and developmentNormal growth and development

Physical ExamPhysical Exam

Alert, Playful, NADAlert, Playful, NAD

Left ankle and dorsum of the foot is Left ankle and dorsum of the foot is markedly swollenmarkedly swollen

Left ankle is erythematous & warmLeft ankle is erythematous & warm

Tender to palpation and movementTender to palpation and movement

Limited Range of MotionLimited Range of Motion

22ndnd toe with edema and erythema but toe with edema and erythema but non-tender and full ROMnon-tender and full ROM

LABSLABS

WBC – 9.1 WBC – 9.1 52% segs, 40% lymphs, 7% monos, 1% eos52% segs, 40% lymphs, 7% monos, 1% eos

H/H – 12/36, Plts – 454H/H – 12/36, Plts – 454

ESR – 69ESR – 69

U/A – normalU/A – normal

ASO – negativeASO – negative

ParvoB19 – negativeParvoB19 – negative

Lyme titers – negativeLyme titers – negative

Labs cont.Labs cont.

ANA – 1:640 (diffuse pattern)ANA – 1:640 (diffuse pattern)

Left Foot X-ray – widening of the Left Foot X-ray – widening of the tibiofibular with soft-tissue swellingtibiofibular with soft-tissue swelling

Bone Scan – Left ankle inflammationBone Scan – Left ankle inflammation

Case ContinuedCase Continued

Two weeks later signs of arthritis are Two weeks later signs of arthritis are seen in both ankles, both knees, and seen in both ankles, both knees, and both wristsboth wrists

WHAT IS THE DIAGNOSIS?WHAT IS THE DIAGNOSIS?

– JUVENILE RHEUMATOID ARTHRITISJUVENILE RHEUMATOID ARTHRITIS

JRAJRA

BasicsBasics– Most common rheumatic dz in childrenMost common rheumatic dz in children– Incidence ~14 per 100,000Incidence ~14 per 100,000– Prevalence ~115 per 100,000Prevalence ~115 per 100,000– First described by George Still in 1897First described by George Still in 1897

Diagnosis of JRADiagnosis of JRA

CriteriaCriteria– Age at onset <16Age at onset <16– ArthritisArthritis– At least 6 weeks durationAt least 6 weeks duration– Type defined by disease in 1Type defined by disease in 1stst 6 months 6 months

Polyarthritis: 5 or more jointsPolyarthritis: 5 or more joints

Oligoarthritis: <5 inflammed jointsOligoarthritis: <5 inflammed joints

Systemic: characteristic feverSystemic: characteristic fever

– Exclusion of other types of arthritisExclusion of other types of arthritis

Major Presentations of JRAMajor Presentations of JRA

Joint effusions/swelling/painJoint effusions/swelling/pain

Loss of normal anatomic landmarksLoss of normal anatomic landmarks

Gradual onset of symptomsGradual onset of symptoms

Morning StiffnessMorning Stiffness

Gel phenomeonGel phenomeon– Napping and Prolonged sitting after Napping and Prolonged sitting after

inactivity (Stiffness)inactivity (Stiffness)

PICTURESPICTURES

Oligoarthritis (Pauciarticular)Oligoarthritis (Pauciarticular)

Inflammation of 4 or fewer jointsInflammation of 4 or fewer joints

~60% of all cases of JRA~60% of all cases of JRA

Two sub-classesTwo sub-classes– Early-onset Pauciarticular (EOPA)Early-onset Pauciarticular (EOPA)

Female to male predominanceFemale to male predominance

Onset usually before 5 years of ageOnset usually before 5 years of age

Usually ANA+ in young femalesUsually ANA+ in young females

Rheumatoid Factor NegativeRheumatoid Factor Negative

Strong association with Uveitis/IridocyclitisStrong association with Uveitis/Iridocyclitis

UveitisUveitis

50% with EOPA have chronic 50% with EOPA have chronic asymptomatic iridocyclitisasymptomatic iridocyclitis– Inflammation of Iris and ciliary bodyInflammation of Iris and ciliary body

Untreated uveitis may develop Untreated uveitis may develop synechiae (adhesions) between iris synechiae (adhesions) between iris and lensand lens

May progress to band keratopathy May progress to band keratopathy (calcium deposits in cornea)(calcium deposits in cornea)

UveitisUveitisMust adhere to guidelines for examsMust adhere to guidelines for exams– Oligo and Polyarticular disease Oligo and Polyarticular disease ANA+ ANA+

and onset < 7y are at greatest riskand onset < 7y are at greatest riskMust examine eyes every 3-4 monthsMust examine eyes every 3-4 months

– Oligo and Polyarticular disease that are Oligo and Polyarticular disease that are ANA- or onset > 7y are at medium RiskANA- or onset > 7y are at medium Risk

Examine eyes every 6 monthsExamine eyes every 6 months

– Systemic are at least risk (q 12 months)Systemic are at least risk (q 12 months)

Uveitis PicturesUveitis Pictures

OligoarthritisOligoarthritis

Late Onset Pauciarticular (LOPA)Late Onset Pauciarticular (LOPA)– Generally affects boys > 8 years oldGenerally affects boys > 8 years old– Involves Hips/SI joints, Knees, Ankles, Involves Hips/SI joints, Knees, Ankles,

Feet (especially Achilles Tendinitis)Feet (especially Achilles Tendinitis)– Associated with family history of Associated with family history of

spondyloarthropathies or psoriasisspondyloarthropathies or psoriasis– May be associated with HLA-B27 +May be associated with HLA-B27 +– May progress to be categorized as other May progress to be categorized as other

spondyloarthropathiesspondyloarthropathies

PolyarthritisPolyarthritis

5 or more joints5 or more joints

30% of all cases of JRA30% of all cases of JRA

Females > MalesFemales > Males

Less associated with extraarticular Less associated with extraarticular involvementinvolvement

Usually RF –Usually RF –

May be ANA+ (especially if RF+) May be ANA+ (especially if RF+) associated with worse disease and joint associated with worse disease and joint destructiondestruction

Systemic Onset JRASystemic Onset JRA

Still’s Disease (~15% cases JRA)Still’s Disease (~15% cases JRA)

Males > Females (?)Males > Females (?)

Characterized by fever, rash, Characterized by fever, rash, irritability, arthritis, and visceral irritability, arthritis, and visceral involvementinvolvement

Fever (>39) that occurs twice dailyFever (>39) that occurs twice daily

Rash: 2-6mm, evanescent, salmon Rash: 2-6mm, evanescent, salmon colored (trunk/proximal extremeties)colored (trunk/proximal extremeties)

Systemic JRASystemic JRA

Systemic JRASystemic JRA

Associated with Serositis, Pleuritis, Associated with Serositis, Pleuritis, Pericarditis, Hyperbilirubinemia, Pericarditis, Hyperbilirubinemia, elevated transaminases, anemia, elevated transaminases, anemia, leukocytosis, hepatosplenomegalyleukocytosis, hepatosplenomegaly

25% progress to chronic 25% progress to chronic inflammatory arthritisinflammatory arthritis

Usually ANA and RF negativeUsually ANA and RF negative

Differential DiagnosisDifferential Diagnosis

Often Seronegative Often Seronegative so often a so often a disease of exclusiondisease of exclusion

Must differentiate between Septic Must differentiate between Septic arthritisarthritis

Must exclude lyme disease (may Must exclude lyme disease (may mimic oligoarthritismimic oligoarthritis

Distinguish between Leukemia and Distinguish between Leukemia and JRA (bone pain v. joint pain)JRA (bone pain v. joint pain)

Differential DiagnosisDifferential Diagnosis

SLESLE

LUPUSLUPUS

Rheumatic FeverRheumatic Fever

HSPHSP

PANPAN

IBDIBD

VirusesViruses

Joint Joint hypermobilityhypermobility

Reiter syndromeReiter syndrome

Reactive arthritisReactive arthritis

Psoriatic arthritisPsoriatic arthritis

Enthesitis Enthesitis syndromesyndrome

Treatment of JRATreatment of JRA

Anti-inflammatory Anti-inflammatory – NSAIDSNSAIDS

Obviously risk of GI bleed, increased LFT’s, Obviously risk of GI bleed, increased LFT’s, Reye-like syndrome or encephalopathyReye-like syndrome or encephalopathy

– Immunosuppressives (Steroids)Immunosuppressives (Steroids)Used if severe diseaseUsed if severe disease

Cardiac InvolvementCardiac Involvement

– New immunomodulatorsNew immunomodulatorsEnbrelEnbrel

SpondyloarthropathiesSpondyloarthropathies

Juvenille Ankylosing SpondylitisJuvenille Ankylosing Spondylitis– Male:female ratio of 3:1Male:female ratio of 3:1– Affects Axial Joints (SI JOINT!!)Affects Axial Joints (SI JOINT!!)

Ossification of anterior spinal ligament and Ossification of anterior spinal ligament and fusion of the facets “bamboo spine”fusion of the facets “bamboo spine”

Night pain/morning stiffness/pain with restNight pain/morning stiffness/pain with rest

– ANA/RF negativeANA/RF negative– HLA-B27 + in 90%HLA-B27 + in 90%– Can be associated with uveitis/iritisCan be associated with uveitis/iritis

Ankylosing SpondylitisAnkylosing Spondylitis

SpondyloarthropathiesSpondyloarthropathies

Reiter’s SyndromeReiter’s Syndrome– ““Can’t see, can’t pee, can’t climb a tree”Can’t see, can’t pee, can’t climb a tree”– Urethritis, Iritis, ArthritisUrethritis, Iritis, Arthritis– Post-infectious (1-3 weeks after)Post-infectious (1-3 weeks after)

Enteric pathogens (yersinia, shigella, Enteric pathogens (yersinia, shigella, salmonella)salmonella)

Non-gonococcal urethritis (Chlamydia)Non-gonococcal urethritis (Chlamydia)

– NSAIDS and antibiotics to treat underlying NSAIDS and antibiotics to treat underlying illnessillness

– May be associated with HLA-B27May be associated with HLA-B27

SpondyloarthropathiesSpondyloarthropathies

IBDIBD– Chron’s and UC may be associated with Chron’s and UC may be associated with

arthritisarthritis– Tends to affect limb jointsTends to affect limb joints– May be associated with HLA-B27 and May be associated with HLA-B27 and

spine involvementspine involvement– Affects about 1 in 5 with IBDAffects about 1 in 5 with IBD– Treat underlying diseaseTreat underlying disease

Joint HypermobilityJoint Hypermobility

Increased mobility of jointsIncreased mobility of joints– >10° hyperextension elbows/knees>10° hyperextension elbows/knees– Thumb to forearm… etc.Thumb to forearm… etc.

May be associated with Ehlers-danlosMay be associated with Ehlers-danlos

Increased risk of dislocations and Increased risk of dislocations and joint painsjoint pains

Treat with NSAIDSTreat with NSAIDS

Reassure ParentsReassure Parents

Functional Joint ComplaintsFunctional Joint Complaints

Growing PainsGrowing Pains– Likely due to overuse of muscles/jointsLikely due to overuse of muscles/joints– Occur later in the dayOccur later in the day– Feels better with touch/massageFeels better with touch/massage– Girls more likely to complain of painsGirls more likely to complain of pains– Normal growth/developmentNormal growth/development– Pain does not awaken from sleepPain does not awaken from sleep– Treat with supportive care Treat with supportive care massage, massage,

heating pad, stretching, NSAIDSheating pad, stretching, NSAIDS