Transcript Staff Conference

One disease entity which
explains all the signs and
symptoms
Staff Conference
R.C.
Cherie Marie A. Tecson, M.D.
18 July 2008
General Data
9 year old male
 Filipino
 Roman Catholic
 Chief complaint: Joint pain

History of Present Illness
2 years PTC
(+) joint pains, swelling and
warmth, migratory, for 2-3 days
(+) fever
7 months PTC
(+) chest pain
(+) easy fatigability
(+) persistent joint pains, swelling
and warmth
3 months PTC
(+) easy fatigability
(+) 2-pillow orthopnea
(+) joint pains, swelling and warmth
1 month PTC
Persistence of joint pains,
swelling and warmth
Review of Systems
(+) weight loss, ~10%
 (-) oral ulcers
 (-) skin lesions
 (-) blurring of vision

Past Medical History

(+) Measles at 1 yo
Family Medical History

(-) similar illness
Physical Examination
Physical Examination
Physical Examination
Dynamic precordium
 Apex beat at the 5th left intercostal
space, midclavicular line
 (+) grade 3/6 holosystolic murmur
at the apex
 (+) grade 2/6 systolic murmur over
the left parasternal border

Physical Examination
Initial Impression

Congestive heart failure
functional class I secondary to
Acute Rheumatic Fever with
Severe Carditis
Course in the OPD
Problem 1: Acute Rheumatic
Fever with Severe Carditis
 Aspirin, 300mg/tab, 2-2-2-1
 Captopril (1.3) 25mg/tab, ½ tab
BID
2d Echo
Course at the OPD
Problem 1: Acute Rheumatic
Fever with Severe Carditis
Rheumatic Heart Disease
 Two weeks after initial consult,
(+) persistent joint pains
 Ibuprofen 500mg/cap, 1 cap
TID
 Nonreactive RA factor
 Negative ANA
Course at the OPD
2) Chronic Arthritis
 (+) migratory, polyarticular
 Not relieved by Aspirin and
Ibuprofen
 (+) intermittent fever during
attacks
 RA factor: nonreactive
 ANA factor: negative
Salient Features
9 year old male
 Chronic arthritis
 Treated as a case of RHD
 No resolution of joint pains

Objectives
1) To formulate an approach to the
diagnosis of a patient with joint
pains and cardiac disease
2) To differentiate the clinical and
laboratory findings of JIA and RF
3) To discuss the management plan
of a patient with JIA and RF
Approach to Diagnosis
Systemic Lupus Erythematosus
Episodic, mutisystem,
autoimmune disease
 Inflammation of blood
vessels and connective
tissue
 (+) ANA, antibodies to
dsDNA
 Diagnosis is clinical and
supported by laboratory
abnormalities

Cassidy, J., Petty, R., Laxer, R., Lindsley, C. Textbook of Pediatric Rheumatology. Elsevier
Saunders. Philadelphia, 2005.
Disease
Entity
SLE
Characteristic
Arthritis
•Small joints of the
hands, wrists,
elbows, shoulders,
knees, ankles
•Short in duration
•Myalgia or muscle
weakness
characteristic
Cardiac
Involvement
•Pericarditis (80%)
•Rarely accompanied
by cardiomegaly
•Classic lesion:
Libman-Sacks
endocarditis
Other
Features
Malar rash,
Discoid rash,
Photosensitivity,
Oral Ulcers,
Serositis,
Renal, Neurologic,
Hematologic
Immunologic
disorder, ANA
Cassidy, J., Petty, R., Laxer, R., Lindsley, C. Textbook of Pediatric Rheumatology. Elsevier
Saunders. Philadelphia, 2005.
Crozier IG, Li E, Milne MJ and Nichols G : Cardiac involvement in systemic lupus
erythematosus detected by echocardiography. Am J Cardiol, 65:1145, 1990.
Ankylosing Spondylitis
Chronic
inflammatory
disease of the axial
and peripheral
skeletons
 Radiologic evidence
of bilateral
inflammation of
sacroiliac joints

Cassidy, J., Petty, R., Laxer, R., Lindsley, C. Textbook of Pediatric Rheumatology. Elsevier
Saunders. Philadelphia, 2005.
Disease
Entity
AS
Characteristic
Arthritis
Cardiac
Involvement
Other
Features
•Buttocks, thighs,
groin, heels or around
shoulders
•Spontaneous
resolution
•Joint involvement <4
•Seronegativity to
rheumatoid factor
•Uncommon
•Aortic insufficiency
•Aortitis
•Conduction
disturbance
•Cardiomyopathy
•Altered diastolic
function
Family history,
enthesitis, radiologic
evidence of
sacroiliac joints
Cassidy, J., Petty, R., Laxer, R., Lindsley, C. Textbook of Pediatric Rheumatology. Elsevier
Saunders. Philadelphia, 2005.
Gragham DC and Smythe HA : The carditis and aortitis of ankylosing spondiylitis. Bull
Rheum Dis, 9:171- 1958.
Behçet’s Disease
Relapsing systemic vasculitis
that is rare in children
 Unknown cause
 Usual course is of exacerbations
and remissions

Cassidy, J., Petty, R., Laxer, R., Lindsley, C. Textbook of Pediatric Rheumatology. Elsevier
Saunders. Philadelphia, 2005.
Kliegman, R., Behrman, R., Jenson, H., Stanton, B. Nelson’s Textbook of Pediatrics, 18th
Edition. Saunders, 2007.
Disease
Entity
Behçet’s
disease
Characteristic
Arthritis
•Acute onset
•Recurrent
•Asymmetric
•Polyarticular
•Large joints
Cardiac
Involvement
•Uncommon
•Pericarditis
•Myocarditis
•Arrhytmias
Other
Features
Triad of aphthous
stomatitis, genital
ulceration, uveitis,
superficial
thrombophlebitis
Higashihara M, Mori M, Takeuchi A, et. al. : Myocarditis in Behçet's disease a case report
and review of the literature. J Rheumatol, 9:630, 1982
James DG and Thomson A : Recognition of the diverse cardiovascular manifestations in
Behçet's disease. Am Heart J, 13:45, 1982.
Cassidy, J., Petty, R., Laxer, R., Lindsley, C. Textbook of Pediatric Rheumatology. Elsevier
Saunders. Philadelphia, 2005.
Acute Rheumatic Fever
Most common form
of acquired heart
disease
 Accounts for ~50%
of all cardiovascular
disease, ~50% of all
cardiac admissions
in many developing
countries

Kliegman, R., Behrman, R., Jenson, H., Stanton, B. Nelson’s Textbook of Pediatrics, 18th
Edition. Saunders, 2007.
Acute Rheumatic Fever
Acute Rheumatic Fever
Jaccoud’s Arthritis
 Rare form of nonerosive, deforming
arthropathy ascribed to Rheumatic
Fever
 Subluxation and ulnar deviation at
the metacarpophalangeal joints
 Pathognomonic "hook" lesion on the
radial side of the affected metacarpal
heads
Zvaifer NJ: Chronic post-rheumatic fever (Jaccoud’s) arthritis. N. Engl J Med 267: 10-14,
1962
Cosh, J: The heart and the rheumatic diseases. Rheum. Phys. Med., 1972, 11, 267.
Disease
Entity
Characteristic
Arthritis
Acute
Rheumatic
Fever
•Large joints
•Migratory, additive
•Self-limiting course
•Swelling, warmth,
erythema and pain
•Rarely lasts more
than 1 week in any
one joint
•Hallmark:
sensitivity to
salicylates
Cardiac
Involvement
•Usually occurs
within one week
of arthritis
•Involves all
layers of the
heart
•Hallmark lesion:
rheumatic
carditis, valvular
insufficiency
Other
Features
•Syndenham’s Chorea
•Erythema
marginatum
•Subcutaneous
nodules
•Fever
•Elevated ESR and
CRP
•Prolonged PR interval
Cassidy, J., Petty, R., Laxer, R., Lindsley, C. Textbook of Pediatric Rheumatology. Elsevier
Saunders. Philadelphia, 2005.
Kliegman, R., Behrman, R., Jenson, H., Stanton, B. Nelson’s Textbook of Pediatrics, 18th
Edition. Saunders, 2007.
Review of Signs and Symptoms








Carditis
Arthritis
Nonreactive RA factor
Negative ANA
Persistently elevated ASO titers
Elevated CRP
2D echo: LAE, LVE, thickened noncoaptating mitral valve with severe
MR, mild AR
Failure to respond to salicylates
Juvenile Idiopathic Arthritis
Arthritis persistent for at least 6
weeks in a child <16 years of
age
 Classification is made at 6
months after diagnosis into one
of seven disease categories
 Cardiac involvement is found in
4.5% of cases

Petty RE, Southwood TR, Manners P, Braum J, Glass DN, Goldenberg J, et al, and the International League
of Associations for Rheumatology. International League of Associations for Rheumatology classification of
juvenile idiopathic arthritis: second revision, Edmonton, 2001. J Rheumatol 2004; 31:390–2.
Choy EH, Panayi GS. Cytokine pathways and joint inflammation in
rheumatoid arthritis. N Engl J Med. 2001;344:907-916.
Does our patient have
JIA or RHD
JIA or RHD?
Cardiac involvement
JIA
RHD
Most common lesion
Pericarditis (7-10%)
Valvular endocarditis
2D Echo
Pericardial effusion (50%)
Hallmark: mitral
insufficiency (76%)
Epidemiology
Common in seronegative
patients with systemic form of
disease or seropositive patients
with severe destructive articular
disease
Cardiac involvement is
essential in diagnosis
Symptomatology
Usually asymptomatic
Symptomatic
Valve involvement
Involvement of mitral valve in
seronegative, polyarticular
disease is rare, multivalvular
involvement rarely seen
Multivalvular involvement,
usually mitral and aortic
disease (97%)
Svetsson, H., Bjorkhem, G., Elborgy, R. Cardiac involvement in juvenile rheumatoid
arthritis. Acta Pediatr Scand. 1983, 72, 345-350.
Bernstein, B., Takahashi, M., Hanson, V. Cardiac involvement in juvenile rheumatoid
arthritis. J Pediatr 1974; 85 313-317.
JIA or RHD?
Arthritis
JIA
RHD
Joint involvement
Can involve small joints
Rarely involves small
joints
Laboratory
Absence of
antistreptococcal
antibodies
High or rising titer of
streptococcal antibodies
Duration
Long duration
Resolves spontaneously
Response to Salicylates
Absent or less dramatic
Usually occurs in 48 hrs
Consequence
Osteoporosis, contracture, No residual damage
wasting, neck stiffness,
involvement of small joints
Pain
Mild to moderate
Usually severe
Effusion
May be marked
Usually mild
Erythema
Usually absent
Common
Course
Protracted, recurrent
Resolves spontaneously
JIA or RHD?
“It is possible for a patient with
chronic rheumatic heart disease
to develop rheumatoid arthritis
by coincidence at a later date,
but there is no evidence for an
association between the two
disorders.”
Zvaifer NJ: Chronic post-rheumatic fever (Jaccoud’s) arthritis. N. Engl J Med 267: 10-14,
1962
Cosh, J: The heart and the rheumatic diseases. Rheum. Phys. Med., 1972, 11, 267.
Grahme, R., Mitchell, A. B. S., and Scott, J. T. (1970). Chronic Post-rheumatic Fever
(Jaccoud's) Arthropathy. Ann. rheum. Dis., 29, 622.
Treatment of RHD
Benzathine Penicillin every 21
days to control activity
 Anticongestive measures
 Balloon valvuloplasty
 Surgical replacement or repair
of affected valves

Cassidy, J., Petty, R., Laxer, R., Lindsley, C. Textbook of Pediatric Rheumatology. Elsevier
Saunders. Philadelphia, 2005.
Kliegman, R., Behrman, R., Jenson, H., Stanton, B. Nelson’s Textbook of Pediatrics, 18th
Edition. Saunders, 2007.
Treatment of JIA
Anti-inflammatory medications
 Immunomodulatory medications
 Physical, occupational therapy
 Occasional need for surgery
 Nutritional support
 Psychosocial and educational
partnership

Hashkes, P., Laxer, R. Medical Treatment of Juvenile Idiopathic Arthritis. JAMA, 2000, 23,
13.
Kliegman, R., Behrman, R., Jenson, H., Stanton, B. Nelson’s Textbook of Pediatrics, 18th
Edition. Saunders, 2007.
Treatment of JIA
Objectives of Treatment of Chronic
Arthritis in Children
Immediate
Relieve discomfort
Preserve function
Prevent deformities
Control inflammation
Long-term
Minimize side effects of disease and
treatment
Promote normal growth and development
Rehabilitate
Educate
Kliegman, R., Behrman, R., Jenson, H., Stanton, B. Nelson’s Textbook of Pediatrics, 18th
Edition. Saunders, 2007.
Course at the OPD
Juvenile Idiopathic Arthritis
 Started on the following meds:
1) Naproxen 250mg/tab, 1 tab
BID
2) Methotrexate 7.5mg/wk, Folic
Acid
 Noted relief of joint pains
Additional Plans
Periodic slit-lamp ophthalmologic
examinations
 Dietary evaluation and counselling
 Physical and occupational therapy
 Intensive monitoring of cardiac
function
 Address hematologic
consequences

Prognosis
Juvenile Idiopathic Arthritis
 Outcome is generally good
 70-90% will have a satisfactory
outcome without serious disability
 25 to 50% will need surgery
 Delay in referral associated with a
poorer functional outcome
 Overall death rate is 0.4 to 2%
Fukinawa, S., Okuni, M. A nationwide surveillance study of rheumatic diseases among
Japanese children. Acta Paedriatica Japan 39: 242-244, 1997
Hashkes, P., Laxer, R. Medical Treatment of Juvenile Idiopathic Arthritis. JAMA, 2000, 23, 13.
Prognosis
Rheumatic Heart Disease
 Major morbidity associated
exclusively with degree of cardiac
damage
 Mortality occurs predominantly in
patients with pancarditis
 Cardiac surgery has alleviated
crippling effect of this complication
*Tompkins DG, Boxerbaum B, Liebman J: Long-term prognosis of rheumatic fever patients
receiving regular intramuscular benzathine penicillin. Circulation 45: 543-551, 1972
In Conclusion
Close monitoring is essential
 Cardiac manifestations of JIA are
rare, asymptomatic, and involves
single valves, while its arthritis does
not respond to salicylates
 Two disease entities can exist in one
patient, and both should be
addressed in terms of management

Thank you!