juvenile idiopathic arthritis & rheumatic fever

8/3/2019 Juvenile Idiopathic Arthritis & Rheumatic Fever

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Rachel State

PGY3, Triple Board


2/26

13 year old male with fever, rash, jointpain x 6 months who presented withworsening symptoms over the last 3

weeks.


3/26

Fever:

Initially intermittent tactile temperatures.

Progressed to high fevers in the evening, improved

by morning. Associated with diaphoresis, shakingchills.

Rash:

Initially diffuse, puritic, and erythematous

Rash would pop up randomly, family noted acorrelation to onset of fever

Returned on arms and spread to chest, back, andface. Faded, but did not completely resolve during 3

weeks prior to presentation.


4/26

Pain (generally worse in the morning, better inthe afternoon):

Started as generalized malaise with headaches and

mild stiffness in arms and legs 3 weeks ago developed pain in left wrist.

The following day he had difficulty walking due tobilateral ankle pain.

Pain progressively worsened to include bilateralwrists, ankles, arms, shoulders, back, and neck.

Escalated the day prior to admission and patient wasunable to ambulate due to severe pain in the left

knee


5/26

Nonproductive cough, sore throat, andcongestion that started the day prior toadmission

Mild diarrhea alternating with constipation.Over last week with diffuse abdominal painthat comes and goes.

5 pound weight loss over last 3 weeks

No nausea, vomiting, localized weakness,dysphagia, conjunctivitis, or lymphadenopathy


6/26

Positive rapid strep in January, treated withAzithromycin

Bilateral Genu Valgum s/p surgical repair in

August, no complications Amblyopia with left eye visual impairment,

corrected with glasses

Imms: UTD


7/26

FMHx

Paternal grandfather with rheumatoid arthritis andSLE

Paternal uncle with rheumatoid arthritis Brother with Type I Diabetes

Mother with hypothyroidism

Maternal grandmother with multiple myeloma

SHx Lives with 4 brothers.

Went camping no tick bites or unusual exposures


8/26

Vitals: Temp 39.8 C, HR 125, RR 30, BP 104/42, on Room airsaturating 97%

General: Weight 79kg (98%tile) ; Height 64.57 in (48 %tile); BMI: 30

(>97%) Communicative and polite in no acute distress, lying in bed

covered with blanket, febrile, with shaking chills.

HEENT: Erythematous posterior oropharynx, no lesions, tonsils

1+ with no exudate. Erythematous tongue. Flushed cheeks. Necksupple, no LAD.

Cardio: Systolic murmur 2/6 heard best left upper sternalborder, tachycardic with regular rhythm. Normal S1 and S2. Distalpulses 2+ bilaterally


9/26

EXTREMITIES/MS: WWP. Swelling with mild tenderness to palpation in bilateral

knees L>R, well healing surgical scars bilaterally. No erythemaor warmth. No swelling or erythema of other joints. Range of

motion in bilateral wrists and dorsal flexion of bilateral feetlimited by pain. Minimal rotation of hips but no pain on exam.

Skin:

Blanching maculopapular rash on upper arms, chest,

back; less prominent on lower extremities andforearms and abdomen; spares palms and soles. Nonscaly, no open lesions/wounds, no discharge, nofocal areas of erythema.


10/26

Seen by PCP at onset of fevers:

Leukocytosis, elevated ESR and CRP, normal BMP,negative rapid strep.

Referred to cardiology for new murmur on PE EKG - slightly prolonged PR interval

TTE - possible vegetation on aortic valve vs. partialleaflet fusion with trace aortic insufficiency, trivialmitral insufficiency with no evidence of carditis

Followed regularly by PCP

Blood cultures negative x 3


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12/26

Rheumatic Fever

Malaria

JIA

Periodic Fever Syndrome

Osteomyelitis Mixed Connective Tissue

Disease

Septic Arthritis

Vasculitis

Kawasaki

SLE

Osetosarcoma

Bacterial Endocarditis

Reactive Arthritis

CMV/EBV/Adeno

Polyarteritis Nodosa

Adeno

Post Infectious Arthritis

(Parvo B19) Enterovirus

TB

Leukemia

Sarcoidosis

Lymphoma

Dermatomyositis

Lyme Disease

Complex Regional Pain

Syndrome


13/26

CBC: WBC 14.9 (14%B, 71%N, 9%L, 1%M,1%E), Plts 549, Hgb/Hct wnl

CMP: elevated alk phos, otherwise wnl

CRP 22.4, ESR 96 ASO titer elevated at 452

DNAse Ab negative at 130

ANA negative Ddimer 3939, LDH 1000

CMV negative, EBV negative


14/26

MRI w/o contrast of left knee: No evidence of osteomyelitis.Moderate joint effusion with moderate synovitis, a finding ofuncertain significance given relatively recent epiphysiodesis.

CT abdomen and pelvis w/ contrast: Minimal free fluid in the rightparacolic gutter and pelvis. No abscess. No HSM. Enlarged bilateralpelvic lymph nodes and prominent inguinal and mesenteric lymphnodes.

Chest X ray: No abnormalities noted

TTE: No vegetations seen but depending on index of suspicion, onecould consider a TEE for more complete evaluation. Trivial aorticvalve regurgitation. Normal right and left ventricular function.


15/26

Dx with Stills Disease after markedreduction in symptoms with high dose

NSAIDs


16/26


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Three Major Subtypes

Systemic onset

Pauciarticular onset

Polyarticular onset


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10-15 % of JIA patients

Females = Males

Occurs in children less than 16

No specific joint involvement Fever/Rash/Heptosplenomegaly/LAD/Pericardial

Effusion/Pleural Effusion

Destructive Arthritis > 50% percent

Lab Abnormalities Marked leukocytosis (elevated or normal platelet count)

Severe Anemia

Elevated ESR/CRP

+ Rheumatoid Factor, rare


19/26


20/26

50% of JIA patients

Female > Male

Peak age 2-3 years, rare > 10

Typically large joint involvement, rarely hips Less than 5 joints in the first 6 months

20% with Uveitis

Labs Mildly elevated ESR

ANA +

Negative RF


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30 40% of JIA patients Females > Males Bimodal peaks: 2 5 years, 10 14 years Any joint, usually small joints, rarely starts in hip

More than 5 joints in the first 6 months Uveitis is rare Destructive Arthritis > 50% of patients Labs

Mild anemia Mildly elevated ESR Low ANA titer in younger patients RF positive in 10-20% of those > 10 yo


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2 4 weeks after Strep pharyngitis

5 15 year olds

Developing Countries

470,000 new cases of yearly 233,000 deaths attributable to rheumatic fever or

rheumatic heart disease each year

Incidence: 19 in 100,000

United States

2 10 in 100,000

Localized outbreaks


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Major Criteria Migratory Arthritis (predominately large joints)

Carditis/Valvulitis

Sydenham Chorea

Erythema Marginatum Subcutaneous Nodules (small, painless, over bony

surface)

Minor Criteria

Arthralgia Fever

Elevated inflammatory markers

Prolonged PR interval


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JIA NSIADS Corticosteroids in acute phase (less than 6 months) Methotrexate IL-I or IL-6 inhibitors refractory systemic symptoms

Anakinra Retuximab

TNA alpha inhibitors adjunct for arthritis in refractory disease Etanercept Infliximab

Hematopoietic cell transplantation relentless disease

ARF Treat Strep infections! Penicillin G monthly for 10 years after initial attack or until age 18


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Carapetis, JR, Steer, AC, Mulholland, EK, Weber, M. The global burden of group A streptococcaldiseases. Lancet Infect Dis 2005; 5:685.

Carapetis, JR. Rheumatic heart disease in developing countries. N Engl J Med 2007; 357:439. Tibazarwa, KB, Volmink, JA, Mayosi, BM. Incidence of acute rheumatic fever in the world: a

systematic review of population-based studies. Heart 2008; 94:1534. Miyake, CY, Gauvreau, K, Tani, LY, et al. Characteristics of children discharged from hospitals in the

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Criteria for the classification of juvenile rheumatoid arthritis. Bull Rheum Dis 1972; 23:712. Cassidy, JT, Petty, RE. Juvenile rheumatoid arthritis. In: Textbook of Pediatric Rheumatology, 4th ed,

Cassidy, JT, Petty, RE (Eds) W.B. Saunders Company, Philadelphia, 2001. p. 218. Cush, JJ, Medsger, TA, Christy, WC, et al. Adult-onset Still's disease: Clinical course and outcome.

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rheumatoid arthritis: data from the Pennsylvania Systemic Onset Juvenile Arthritis Registry(PASOJAR). J Rheumatol 2008; 35:343.

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