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!