Rachel State PGY3, Triple Board

13 year old male with fever, rash, joint pain x 6 months who presented with worsening symptoms over the last 3 weeks.

Fever:

Initially intermittent tactile temperatures. Progressed to high fevers in the evening, improved by morning. Associated with diaphoresis, shaking chills.
Initially diffuse, puritic, and erythematous Rash would pop up randomly, family noted a correlation to onset of fever Returned on arms and spread to chest, back, and face. Faded, but did not completely resolve during 3 weeks prior to presentation.

Rash:

Pain (generally worse in the morning, better in the 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 to bilateral ankle pain. Pain progressively worsened to include bilateral wrists, ankles, arms, shoulders, back, and neck. Escalated the day prior to admission and patient was unable to ambulate due to severe pain in the left knee

Nonproductive cough, sore throat, and congestion that started the day prior to admission Mild diarrhea alternating with constipation. Over last week with diffuse abdominal pain that comes and goes. 5 pound weight loss over last 3 weeks No nausea, vomiting, localized weakness, dysphagia, conjunctivitis, or lymphadenopathy

Positive rapid strep in January, treated with Azithromycin Bilateral Genu Valgum s/p surgical repair in August, no complications Amblyopia with left eye visual impairment, corrected with glasses

Imms: UTD

FMHx

Paternal grandfather with rheumatoid arthritis and SLE Paternal uncle with rheumatoid arthritis Brother with Type I Diabetes Mother with hypothyroidism Maternal grandmother with multiple myeloma
Lives with 4 brothers. Went camping no tick bites or unusual exposures

SHx

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

saturating 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. Neck supple, no LAD.

Cardio: Systolic murmur 2/6 heard best left upper sternal

border, tachycardic with regular rhythm. Normal S1 and S2. Distal pulses 2+ bilaterally

EXTREMITIES/MS:

WWP. Swelling with mild tenderness to palpation in bilateral knees L>R, well healing surgical scars bilaterally. No erythema or warmth. No swelling or erythema of other joints. Range of motion in bilateral wrists and dorsal flexion of bilateral feet limited 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 and forearms and abdomen; spares palms and soles. Non scaly, no open lesions/wounds, no discharge, no focal areas of erythema.

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. partial

leaflet fusion with trace aortic insufficiency, trivial mitral insufficiency with no evidence of carditis

Followed regularly by PCP

Blood cultures negative x 3

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

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

MRI w/o contrast of left knee: No evidence of osteomyelitis. Moderate joint effusion with moderate synovitis, a finding of uncertain significance given relatively recent epiphysiodesis. CT abdomen and pelvis w/ contrast: Minimal free fluid in the right paracolic gutter and pelvis. No abscess. No HSM. Enlarged bilateral pelvic lymph nodes and prominent inguinal and mesenteric lymph nodes. Chest X ray: No abnormalities noted

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

Dx with Stills Disease after marked reduction in symptoms with high dose NSAIDs

Three Major Subtypes

Systemic onset

Pauciarticular onset
Polyarticular onset

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

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

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

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

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

JIA

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

TNA alpha inhibitors adjunct for arthritis in refractory disease Hematopoietic cell transplantation relentless disease Treat Strep infections! Penicillin G monthly for 10 years after initial attack or until age 18

ARF

Carapetis, JR, Steer, AC, Mulholland, EK, Weber, M. The global burden of group A streptococcal diseases. 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 on Cardiovascular 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.