BOARD REVIEW RHEUMATOLOGY Dennis A. Peacock April 9, 2008

CASE PRESENTATION 8 month old male with swollen left ankle for several days No Fever and no signs of being ill Normal growth and development

Physical Exam Alert, Playful, NAD Left ankle and dorsum of the foot is markedly swollen Left ankle is erythematous & warm Tender to palpation and movement Limited Range of Motion 2 nd toe with edema and erythema but non-tender and full ROM

LABS WBC – % segs, 40% lymphs, 7% monos, 1% eos H/H – 12/36, Plts – 454 ESR – 69 U/A – normal ASO – negative ParvoB19 – negative Lyme titers – negative

Labs cont. ANA – 1:640 (diffuse pattern) Left Foot X-ray – widening of the tibiofibular with soft-tissue swelling Bone Scan – Left ankle inflammation

Case Continued Two weeks later signs of arthritis are seen in both ankles, both knees, and both wrists WHAT IS THE DIAGNOSIS? –JUVENILE RHEUMATOID ARTHRITIS

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

Diagnosis of JRA Criteria –Age at onset <16 –Arthritis –At least 6 weeks duration –Type defined by disease in 1 st 6 months Polyarthritis: 5 or more joints Oligoarthritis: <5 inflammed joints Systemic: characteristic fever –Exclusion of other types of arthritis

Major Presentations of JRA Joint effusions/swelling/pain Loss of normal anatomic landmarks Gradual onset of symptoms Morning Stiffness Gel phenomeon –Napping and Prolonged sitting after inactivity (Stiffness)

PICTURES PICTURES

Oligoarthritis (Pauciarticular) Inflammation of 4 or fewer joints ~60% of all cases of JRA Two sub-classes –Early-onset Pauciarticular (EOPA) Female to male predominance Onset usually before 5 years of age Usually ANA+ in young females Rheumatoid Factor Negative Strong association with Uveitis/Iridocyclitis

Uveitis 50% with EOPA have chronic asymptomatic iridocyclitis –Inflammation of Iris and ciliary body Untreated uveitis may develop synechiae (adhesions) between iris and lens May progress to band keratopathy (calcium deposits in cornea)

Uveitis Must adhere to guidelines for exams –Oligo and Polyarticular disease  ANA+ and onset < 7y are at greatest risk Must examine eyes every 3-4 months –Oligo and Polyarticular disease that are ANA- or onset > 7y are at medium Risk Examine eyes every 6 months –Systemic are at least risk (q 12 months)

Uveitis Pictures

Oligoarthritis Late Onset Pauciarticular (LOPA) –Generally affects boys > 8 years old –Involves Hips/SI joints, Knees, Ankles, Feet (especially Achilles Tendinitis) –Associated with family history of spondyloarthropathies or psoriasis –May be associated with HLA-B27 + –May progress to be categorized as other spondyloarthropathies

Polyarthritis 5 or more joints 30% of all cases of JRA Females > Males Less associated with extraarticular involvement Usually RF – May be ANA+ (especially if RF+)  associated with worse disease and joint destruction

Systemic Onset JRA Still’s Disease (~15% cases JRA) Males > Females (?) Characterized by fever, rash, irritability, arthritis, and visceral involvement Fever (>39) that occurs twice daily Rash: 2-6mm, evanescent, salmon colored (trunk/proximal extremeties)

Systemic JRA

Associated with Serositis, Pleuritis, Pericarditis, Hyperbilirubinemia, elevated transaminases, anemia, leukocytosis, hepatosplenomegaly 25% progress to chronic inflammatory arthritis Usually ANA and RF negative

Differential Diagnosis Often Seronegative  so often a disease of exclusion Must differentiate between Septic arthritis Must exclude lyme disease (may mimic oligoarthritis Distinguish between Leukemia and JRA (bone pain v. joint pain)

Differential Diagnosis SLELUPUS Rheumatic Fever HSPPANIBDViruses Joint hypermobility Reiter syndrome Reactive arthritis Psoriatic arthritis Enthesitis syndrome

Treatment of JRA Anti-inflammatory –NSAIDS Obviously risk of GI bleed, increased LFT’s, Reye-like syndrome or encephalopathy –Immunosuppressives (Steroids) Used if severe disease Cardiac Involvement –New immunomodulators Enbrel

Spondyloarthropathies Juvenille Ankylosing Spondylitis –Male:female ratio of 3:1 –Affects Axial Joints (SI JOINT!!) Ossification of anterior spinal ligament and fusion of the facets “bamboo spine” Night pain/morning stiffness/pain with rest –ANA/RF negative –HLA-B27 + in 90% –Can be associated with uveitis/iritis

Ankylosing Spondylitis

Spondyloarthropathies Reiter’s Syndrome –“Can’t see, can’t pee, can’t climb a tree” –Urethritis, Iritis, Arthritis –Post-infectious (1-3 weeks after) Enteric pathogens (yersinia, shigella, salmonella) Non-gonococcal urethritis (Chlamydia) –NSAIDS and antibiotics to treat underlying illness –May be associated with HLA-B27

Spondyloarthropathies IBD –Chron’s and UC may be associated with arthritis –Tends to affect limb joints –May be associated with HLA-B27 and spine involvement –Affects about 1 in 5 with IBD –Treat underlying disease

Joint Hypermobility Increased mobility of joints –>10° hyperextension elbows/knees –Thumb to forearm… etc. May be associated with Ehlers-danlos Increased risk of dislocations and joint pains Treat with NSAIDS Reassure Parents

Functional Joint Complaints Growing Pains –Likely due to overuse of muscles/joints –Occur later in the day –Feels better with touch/massage –Girls more likely to complain of pains –Normal growth/development –Pain does not awaken from sleep –Treat with supportive care  massage, heating pad, stretching, NSAIDS