Download Rheumatic fever: a potentially fatal strep infection com

Submission template for Capstone Project Case Report
TITLE OF CASE
”Rheumatic fever: a potentially fatal strep infection complication”
AUTHORS OF CASE Please indicate corresponding author by *
Maya Harp
SUMMARY Up to 150 words summarising the case presentation and outcome
Patient was a 9 year old girl who initially presented in June of 2006 for c/o “Bleeding”.
Patient was a transfer new to the office; the office had no previous history on patient or her past
conditions.
Patient presented with a fever of 101, abdominal pain, blood in urine, and headache. On PE,
patient’s u/a revealed 3+ bloodDiagnosis of Acute glomerulonephritis was made.
2 months later, August 2006, patient comes in for a c/o of “swelling-bee sting, slurring of
speech, and weird wavering swing movement of hand”. On physical exam, there was a mild
mitral regurgitation noted. Immediate investigation was done and diagnosis of Rheumatic fever
with possible rheumatic heart disease was made. Diagnosis was made under the criteria of
JONES with 3 major manifestations. The major manifestations were not apparent until the
previous diagnoses were re evaluated and ruled out to consider them really a manifestation fo RF
ie. JRA was re evaluated to be reactive arthritis, hx of abnormal wavering movements-chorea,
and anxiety along with murmur was carditis plus recurrent labs showing elevated ASO titres, and
acute GN.
Patient is now 11 y/o and doing well. She is currently on erythromycin prophylaxis for the heart
condition, continues to see a cardiologist yearly, she left her previous PCP whom failed to catch
her early symptoms and is now under the care of her PCP whom diagnosed her.
BACKGROUND Why you think this case is important – why you decided to write it up
Rheumatic fever is a potentially fatal but preventable condition that has lost much of its
recognition in clinical practice. It is something that can easily be prevented if we continue to
maintain its high suspicion and appropriately investigate its sequela as presented.
This case reminds us to not overlook one presenting symptom, especially if it is one that can be
associated with a greater aetiology. This case also illustrates a true complication of the most
common diagnosed outpatient conditions in the ped’s clinic today, Pharyngitis.
This case study will educate us on how to recognize the variability in symptoms of this disease,
understand its progression, diagnosis, treatment, and complications.
CASE PRESENTATION Presenting features, medical/social/family history
Patient History Prior to presentation with the new physician:
Labs :
-April 28:
WBC-13.9 H(4-10 )
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RDW-14.4 H(10-14)
Granulocytes -9.91 H(1.2-6.8 )
Basophils
Sed Rate-42 H(0-20) with mod blood,
RBCs 10-20 H
elevated ASO titre-401-800(<200)
IgG -1713 (690-1400)
p-anca/c-anca 6.7, 1.7 H(<.9)
US of the kidneys no renal abnormality was shown,
-June 6, 2006:
Blood : DNASE-B ab titre was high 480 (<170 nl)
Sed Rate : 48 (less than 20 mm /hr)
U/A : cloudy urine
Blood
Leukocyte Esterase : 2+
Wbc of 6-10 (<5/hpf )
Rbc-10-20 (<3/hpf)
ASO titre-520
CRP 3.55
Hyaline Cast-0-5
Dx:
PSGN and Hx of recurrent infections.
Initial presentation to new Physician
HPI:
• June 23,2006
Patient is a 9 year old female who presented on June 23, 2006 after switching from her original
physician.
Patient had c/o “Bleeding”. On PMI, patient had a fever of 101, abdominal pain, blood in urine,
and headache. On exam, U/A revealed 3+ blood. Diagnosis of Acute glomerulonephritis was
made; patient was treated with abx, and referred to a nephrologist.
• July7-2006
Follow up from last visit, patient is still complaining of blood in urine
UA : 2+ blood ; hazy amber color.
Dx : acute pyelonephritis
•
August 14, 2006
Patient comes in for a bee sting on left hand with localized swelling. In addition, mom mentioned
she noticed a slurring in the childs speech and abnormal “waving swing” movement of the hand.
:: Immediate attention was then brought to the patient, and warrant for the diagnosis of Rheumatic
fever was made.
On physical exam a mild mitral regurge murmur was auscultated.
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Past medical history:
Chronic hypertension, JRA, anxiety disorder, glomerulonephritis, gastritis, temporal lobe
epilepsy.
Current Meds:
enalapril 2.5 mg, zantac, vitamin D
Allergies:
Penicillin, shellfish
Past social history:
Patient lives in Grand Blanc with both parents, one sibling and 2 dogs
Past family history:
Father: mitral valve prolapse, resolved PDA, Mother: mild mitral regurge
Notes:
With review of the patients past history and current complications the diagnosis of rheumatic
fever and possible rheumatic heart disease. It was apparent that with the Jones criteria, patient
manifested the joints (arthritis) componnet, Syendhams Chorea component, as well as serial
elevated ASO Titres and antiDNase Ab titres. Past Glomerulonephritis also proved to show that
the patient was suffering from the sequela of previous streptococcal infections.
Plan:
Patient was put on orapred 8ml po bid 5 days for her current PSGN diagnosis
And referred to a rheumatologist and cardiologist at the children heart center for an echo with the
diagnosis of Rheumatic fever with possible rheumatic heart disease.
Outcome:
Echo results showed a diagnosis with mild mitral valve regurge more than physiologic
Rheumatologist ruled out juvenile rheumatoid arthritis, thus the joint problems were a type of
reactive arthritis due to the fever.
Follow up health maintenance exam:
• September 8-2006
Patient now carried the diagnoses of rheumatic fever with rheumatic heart disease
under the criteria of arthritis, chorea, carditis-mitral regurge, and minor
manifestations such as, PSGN, anxiety, and current elevated ASO titre, and fever.
•
Oct 2,06,
Patient had fever of unknown origin and rash over upper chest-dx-scarlet fever.
Patient was sent to ER for culture and IV abx to rule out infective endocarditis
Progress notes:
Patient currently continues to see a cardiologist and is placed on erythromycin 250mg
pO bid for at least 5 years (ptnt has a pcn allergy)
Patient now has to watch for any bouts of FUO to avoid infective endocarditis due to
being a high risk patient from the rheumatic heart disease.
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INVESTIGATIONS If relevant
1. PMHx
2. Comprehensive Blood panel
3. Serial ASO titre; possible anti DNase B titre
4. U/A and culture
5. JRA W/U for full blood count and film, ESR, CRP, ANA, plain XRay to rule out trauma,
tumor, infection, arthrocentesis is septic arthritis suspected
6. Involvement of nephrologist for acute glomerulonephritis,
7. Involvement of rheumatologist for arthritic like pains in joints,
8. Involvement of cardiologist for murmur; and heart management
DIFFERENTIAL DIAGNOSIS If relevant
1. JRA
2. Glomerulonephritis,
3. Mitral valve prolapse, mitral regurge
4. UTI
5. Chorea
6. Infective endocarditis
7. Scarlitenna
8. Vitamin D deficiency
TREATMENT If relevant
1. Prophylaxis for rheumatic heart disease with erythromycin 250 mg bid qd for 5 years.
2. Vitamin D 2000 iU, qD
3. Blood pressure and U/A checked yearly
4. Cardiologist evaluation yearly
OUTCOME AND FOLLOW-UP
Patient is 11 years old now, and is currently in the 6th grade. Patient is doing well and has not had
complaints of any joint disturbances or hematuria. Patient was last followed up by Motts
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Children’s hospital on march 24, 2008, in the pediatric cardiologist clinic. She has stopped taking
Norvasc for the high blood pressure. Patient still lives at home with both parents and a 9y/o
sister. She has 2 dogs.
She will continue to follow her cardiologist as well as a repeat echo in 2 years.
Vitals:
WT: 41 kg, HT: 154 cm, HR: 68 bpm, and regular, RR: 16 breaths per min, Bp: 106/70.
PE:
General- WDWN, NAD, AAO x
Respiratory-Lungs were CTA bilaterally
Cardiovascular- Exam revealed a quiet precordium. S1 was normal and s2 was physiologically
split. No s3/s4 or click heard. A 1/6 soft systolic ejection murmur was auscultated at the left
sternal border. No murmurs were auscultated on diastole. Pulses were 2/4 without delay.
Abdomen- Abdomen was soft and nondistended with normo-active bowel sounds. No
hepatosplenomegaly was noted.
Extremitites-There was no edema, cyanosis, or clubbing of the extremities.
DISCUSSION including very brief review of similar published cases (how many similar cases
have been published?)
Very minimal recent cases have been published on rheumatic fever. One was found which had a
case study within its article.
1.
2.
3.
4.
5.
What is the diagnostic criteria for RF?
What is the appropriate work up for suspicious RF via arthritis presentation?
What is the treatment of RF?
Why is RF a concern?
Incidence of RF
1. Clinical, via-JONES Criteria1
Major manifestations
Carditis
Minor manifestations
Fever
Polyarthritis
Arthralgia
Sydenham’s chorea
Elevated acute phase reactants
Erythema marginatum
Prolonged PR interval
Subcutaneous nodules
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2. Labs2
Acute phase reactants
• Help recognize ARF
• CRP and ESR for monitor of inflammatory activity
Laboratory evidence of a preceding GAS infection via:
• GAS in the throat by culture or rapid streptoccocal antigen test
• Elevated or rising titers of antistreptolysin O (ASO)
• occur in more than 80% of patients with acute GAS pharyngitis
ECG
•
•
Prolonged P-R interval relative to heart rate is a nonspecific finding, present in
more than one third of the patients.
Low-voltage QRS complexes and ST segment changes may be found in the
presence of pericarditis and pericardial effusion.
Cardiac scanning scintigraphy
• Reliable to determine acute from chronic vs. inactive RHD and also in the
follow-up of active carditis
Case 1 with presenting symptoms of arthritis include:
“A 3 year old girl was taken to an accident and emergency department with a painful swelling of her right knee that
came on quickly. She had had a mild cough, sore throat, and low grade temperature for 10 days. There was no
history of trauma. No fracture was seen on a radiograph, and the girl was sent home. Two days later the girl's right
wrist swelled. She was admitted to the Nuffield Orthopaedic Centre for assessment. When she was examined her
temperature was 37.4°C and her pulse was regular (80 beats per minute). She had large infected tonsils, posterior
cervical lymphadenopathy, and a grade 2/6 basal ejection systolic murmur. Her wrist and knee were warm, red,
swollen, and tender, but she had no rashes or subcutaneous nodules. By the following morning she was afebrile and
her joint swelling had resolved spontaneously. A provisional diagnosis of viral arthritis was made and she was
discharged home. Her general practitioner prescribed a course of amoxicillin for her cough and at follow up two
weeks later she was completely well.
The heart murmur persisted, however, and as it had not been noted previously she was referred for paediatric
cardiological assessment. Her electrocardiogram was normal. An echocardiogram showed a small posterior
pericardial effusion with thickening of the mitral valve leaflets and shortening of the chordae tendinae. Blood
samples taken two weeks after the initial presentation were strongly positive for the antistreptolysin O test (800 Todd
units) but were negative for antinuclear antibodies and viral serology. A family history of rheumatic fever in both
maternal grandparents was elicited. A diagnosis of acute rheumatic fever was made, based on the presence of
migratory polyarthritis, carditis, and serological evidence of streptococcal infection. She was treated with antiinflammatory doses of aspirin for six weeks, until her inflammatory markers and antistreptolysin O test results
returned to normal. Repeat echocardiography showed resolution of the pericardial effusion but residual thickening of
the mitral valve and slightly shortened chordae tendinae. The murmur at presentation was a pulmonary flow murmur,
not related to her carditis. We plan for her to continue prophylactic treatment with penicillin at least until she leaves
school.
penicillin.”2
3. Treatment2
•
ANTIBIOTICS: Children who have previously contracted rheumatic fever are often
given continuous (daily or monthly) antibiotic treatments to prevent future attacks of
rheumatic fever and lower the risk of heart damage.
PCN V- 250 mg, bid-tic po (500 mg if adolescent)
If allergic: Erythromycin 20-40mg/kg/d 2-4 times daily, PO
•
ANTI INFLAMMATORIES: If inflammation of the heart has developed, children may
be placed on bed rest. Medications are given to reduce the inflammation, as well as
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antibiotics to treat the Streptococcus infection. Other medications may be necessary to
handle congestive heart failure.
•
SURGERY: Valve replacement (if severe-complicated): If heart valve damage
occurs, surgical repair or replacement of the valve may be considered.
4. Complications
Rheumatic heart disease is a condition in which permanent damage to heart valves is
caused by rheumatic fever. The heart valve is damaged by a disease process that generally
begins with a strep throat caused by bacteria called Streptococcus, and may eventually
cause rheumatic fever. Scarring of the heart valves, forcing the heart to work harder to
pump blood.3
The damage may resolve on its own, or it may be permanent, eventually causing
congestive heart failure 3
•
•
•
Symptoms vary greatly. Often the damage to heart valves isn't immediately noticeable.
A damaged heart valve either doesn't fully close or doesn't fully open.
Eventually, damaged heart valves can cause serious, even disabling, problems. These
problems depend on how bad the damage is and which heart valve is affected. The
most advanced condition is congestive heart failure. This is a heart disease in which
the heart enlarges and can't pump out all its blood.3
This is the complication we want to prevent from occurring, which is why catching
rheumatic fever early is so important
5. Incidence
12 per 1000 (NHIS95)4
approx 1 in 83 or 1.20% or 3.3 million people in USA4
LEARNING POINTS/TAKE HOME MESSAGES 3 to 5 bullet points
•
Mnemonics do help :: remember as much of them as possible
•
Symptoms may not arise concurrently :: it is important to continue follow up and always
try to make connections of new complaints with that of previous ones
•
Never underestimate the power of the patient :: they will tell you exactly what is wrong
with them, just listen!
•
Just because many disease states have been put under the bridge does not mean they do
not still exist
•
Know the most common complaints as well as ruling out their serious complications.
REFERENCES
1. Dajanii AS, Ayoub E, Bierman FZ, Bisno AL, Denny FW, Durack DT, et al. Guidelines for the
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diagnosis of rheumatic fever: Jones criteria, updated 1992. JAMA 1992; 268: 2069-2073
2. http://www.aafp.org/afp/20010415/1557.html
3. Da Silva NA, Pereira BA. Acute rheumatic fever: still a challenge. Rheum Dis Clin North Am
1997
4. http://www.wrongdiagnosis.com/r/rheumatic_fever/prevalence.htm
5. http://www.americanheart.org/presenter.jhtml?identifier=4712
6. Hafner JW - Ann Evidence-based emergency medicine/rational clinical examination abstract.
The clinical diagnosis of streptococcal pharyngitis. Emerg Med - 01-JUL-2005
7. Stead, Latha. First Aid for the Pediatric Clerkship: A Student to Student Guide. McGraw
Hill, 2003.
8. Veasy LG, Tani L, Hill H. Persistence of acute rheumatic fever in the intermountain area of
the United States. J Pediatrics 1994
9. Vincent MT, Pharyngitis. - Am Fam Physician - 15-MAR-2004
10. Binotto MA, Guilherme L, Tanaka AC. Rheumatic Fever. Images Paediatr Cardiol 2002
Date:
October 31, 2008
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