Juvenile Idiopathic Arthritis & Rheumatic Fever
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Transcript of Juvenile Idiopathic Arthritis & Rheumatic Fever
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Rachel State
PGY3, Triple Board
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13 year old male with fever, rash, jointpain x 6 months who presented withworsening symptoms over the last 3
weeks.
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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.
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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
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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
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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
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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
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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
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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.
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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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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
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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
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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.
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Dx with Stills Disease after markedreduction in symptoms with high dose
NSAIDs
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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
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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
United States in 2000 with the diagnosis of acute rheumatic fever. Pediatrics 2007; 120:503. Gordis, L. The virtual disappearance of rheumatic fever in the United States: Lessons in the rise and
fall of disease. Circulation 1985; 72:1155. Guidelines for the diagnosis of rheumatic fever. Jones Criteria, 1992 update. Special Writing Group of
the Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease of the Council onCardiovascular Disease in the Young of the American Heart Association. JAMA 1992; 268:2069.
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.
Arthritis Rheum 1987; 30:186 Behrens, EM, Beukelman, T, Gallo, L, et al. Evaluation of the presentation of systemic onset juvenile
rheumatoid arthritis: data from the Pennsylvania Systemic Onset Juvenile Arthritis Registry(PASOJAR). J Rheumatol 2008; 35:343.
Still, GF. On a form of chronic joint disease in children. Med Chir Trans 1897; 80:47. (Reprinted in:Arch Dis Child 1941;16:156).
Bywaters, EG. Still's disease in the adult. Ann Rheum Dis 1971; 30:121.