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One disease entity which explains all the signs and
symptoms
DIAGNOSIS
Staff Conference
Cherie Marie A. Tecson, M.D.18 July 2008
R.C.
General Data
9 year old maleFilipinoRoman CatholicChief complaint: Joint pain
History of Present Illness2 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
Consult
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, 2-2-2-1Captopril (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 factorNegative ANA
Course at the OPD
2) Chronic Arthritis(+) migratory, polyarticularNot relieved by Aspirin and
Ibuprofen(+) intermittent fever during
attacksRA factor: nonreactiveANA factor: negative
Salient Features
9 year old maleChronic arthritisTreated as a case of RHDNo 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
Arthralgia
Articular
Arthritis
Cardiac Involvement
Systemic Lupus Erythematosus
Ankylosing Spondylitis
Behcet’s Disease
Acute Rheumatic Fever
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
Characteristic Arthritis
Cardiac Involvement
Other Features
SLE •Small joints of the hands, wrists, elbows, shoulders, knees, ankles•Short in duration•Myalgia or muscle weakness characteristic
•Pericarditis (80%)•Rarely accompanied by cardiomegaly•Classic lesion: Libman-Sacks endocarditis
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
Characteristic Arthritis
Cardiac Involvement
Other Features
AS •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 causeUsual 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
Characteristic Arthritis
Cardiac Involvement
Other Features
Behçet’s disease
•Acute onset•Recurrent•Asymmetric•Polyarticular•Large joints
•Uncommon•Pericarditis•Myocarditis•Arrhytmias
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, 1982James 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 FeverMost 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, 1962Cosh, J: The heart and the rheumatic diseases. Rheum. Phys. Med., 1972, 11, 267.
Disease Entity
Characteristic Arthritis
Cardiac Involvement
Other Features
Acute RheumaticFever
•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
•Usually occurs within one week of arthritis•Involves all layers of the heart•Hallmark lesion: rheumatic carditis, valvular insufficiency
•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 non-
coaptating 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?
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%)
JIA or RHD?
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, wasting, neck stiffness, involvement of small joints
No residual damage
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, 1962Cosh, 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 measuresBalloon valvuloplastySurgical 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 medicationsImmunomodulatory medications Physical, occupational therapyOccasional need for surgeryNutritional supportPsychosocial 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 discomfortPreserve functionPrevent deformitiesControl inflammation
Long-term
Minimize side effects of disease and treatmentPromote normal growth and developmentRehabilitateEducate
Kliegman, R., Behrman, R., Jenson, H., Stanton, B. Nelson’s Textbook of Pediatrics, 18th Edition. Saunders, 2007.
Course at the OPD
Juvenile Idiopathic ArthritisStarted on the following meds:1) Naproxen 250mg/tab, 1 tab BID2) Methotrexate 7.5mg/wk, Folic
AcidNoted relief of joint pains
Additional Plans
Periodic slit-lamp ophthalmologic examinations
Dietary evaluation and counsellingPhysical and occupational therapy Intensive monitoring of cardiac
functionAddress 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, 1997Hashkes, P., Laxer, R. Medical Treatment of Juvenile Idiopathic Arthritis. JAMA, 2000, 23, 13.
Prognosis
Rheumatic Heart DiseaseMajor 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!