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PROF DR: FAWZY MEGAHED
ASST LEC: RAAFAT SAEID
A 20-Year-Old Man with Sore
Throat, Fever, Myalgias, and a Pericardial
Effusion
A 20-year-old man was admitted to this hospital because
of fever and a pericardial effusion.
The patient had been well until 5 weeks before this
admission, when sore throat,
subjective fever, malaise, and diffuse myalgias
developed.
a clinical diagnosis of streptococcal
pharyngitis was made, and a 7-day course of oral
penicillin was prescribed.
Three days later, the patient was seen in an urgent care
clinic because of fatigue
and worsening myalgias. He reported that the sore
throat and fever had improved.
A rapid antigen test for group A streptococcus was
negative, and the patient returned home.
Fifteen days later, the patient returned to the urgent care
clinic with persistent myalgias and malaise and a 5-day
history of increased sore throat and fever. A throat
culture for group A beta-hemolytic streptococcus was
positive, and a blood culture was negative.
Oral levofloxacin was prescribed, and the patient returned
home.
Four days later, shortness of breath and pain on the left side of the
chest developed.
The patient returned to his primary care physician. The chest pain,
which he rated at 6 on a scale of 0 to 10 (with 10 indicating the
most severe pain), increased with deep inspiration and radiated
to the left shoulder and back. The sore throat had improved, but
fever, myalgias, and malaise persisted. The patient appeared ill.
The white-cell count was 23,800 per cubic millimeter
(reference range, 4700 to 10,800), the C-reactive
protein level was 29.5 mg per deciliter (reference value,
<0.8), the erythrocyte sedimentation rate was 96 mm
per hour (reference value, <15), and a blood culture
was obtained.
Tests for influenza virus and
respiratory syncytial virus were negative. A computed
tomographic (CT) scan of the neck and a transthoracic
echocardiogram were reportedly normal.
Oral azithromycin and intravenous vancomycin,
ceftriaxone, and hydromorphone hydrochloride
were administered.
The patient’s pain improved somewhat, but
fever and shortness of breath persisted. On the
third hospital day, CT of the chest reportedly
revealed right hilar and mediastinal adenopathy,
small bilateral pleural effusions, bibasilar atelectasis,
and no evidence of acute pulmonary embolism.
A test for human immunodeficiency virus
was negative. Antibodies indicative of past
infection with Epstein–Barr virus were detected.
Over the next 2 days, abdominal pain developed.
On the fifth hospital day, CT of the abdomen
and pelvis was performed; no abnormalities
were noted in the abdomen or pelvis, but moderate
bilateral pleural effusions, dependent bibasilar
opacities, and a moderate pericardial effusion
.
A transesophageal echocardiogram
that was obtained the next day showed a
small circumferential pericardial effusion with
some fibrinous strands; no vegetations were seen,
and the estimated ejection fraction was 65%. A
tuberculin skin test was negative. Indomethacin
and colchicine were administered orally.
Swelling of the shoulders and elbows, stiffness
of the shoulders, and a faint erythematous
rash on the face, neck, and arms developed. Fever
(with temperatures between 38.9°C and 39.4°C)
and myalgias persisted, and dyspnea progressively
increased. The administration of intravenous
fluids and supplemental oxygen .
An antistreptolysin O titer was 289 IU
per milliliter (reference value, <530). On the 11th
hospital day, a repeat transthoracic echocardiogram
showed a large anterior pericardial effusion
and a moderate posterior pericardial effusion,
as well as marked respiratory variation of tricuspid
inflow. The patient was transferred to the cardiac
intensive care unit at this hospital.
On admission to this hospital, the patient
reported pain on the left side of the chest and in
the left shoulder, dyspnea, diffuse myalgias, anorexia,
and loose stools. His parents also reported
that he had had jerking movements during sleep
over the past 2 days.
He was sexually active and consistently used condoms.
He had no exposure to animals and had not traveled
outside during the previous 4 years.
He did not use tobacco, but he smoked marijuana
approximately two times per month and
drank five or six beers once per week.
He had no known allergies. His brother had recently
died at 32 years of age from intravenous-drug use that
was complicated by fungal endocarditis with an
abscess of the mitral-valve ring.
On examination, the patient appeared pale
and fatigued. The temperature was 37.7°C, the
pulse 102 beats per minute, the blood pressure
162/83 mm Hg, the respiratory rate 30 breaths
per minute, and the oxygen saturation 94% while
he was receiving supplemental oxygen through a
nasal cannula at a rate of 2 liters per minute.
Pulsus paradoxus measured 14 mm Hg. The heart
sounds were distant. The jugular venous pressure
was greater than 19 cm of water, and Kussmaul’s
sign (distention of the jugular veins during
inspiration) was present. There was dullness
on percussion at both lung bases, and Ewart’s sign
There was pitting edema of the legs below
the knees and mild swelling around the shoulders
and elbows, without effusions. There were
faint pink macules on the forearms and the right
antecubital fossa (Fig. 1) that were no longer visible
after 1 hour.
The remainder of the examination
was normal. Results of renal-function tests
were normal, as were blood levels of magnesium,
phosphorus, glucose, globulin, alanine
aminotransferase,aspartate aminotransferase, total
bilirubin, direct bilirubin, amylase, and lipase. Other
laboratory test results are shown in Table 1.
The results of urinalysis were normal. A blood
culture,chest radiograph, electrocardiogram, and
echocardiogram were obtained.
Differential Diagnosis
A previously healthy young man with a subacute
accelerating illness characterized by serositis,
polyarthralgia, fever, marked leukocytosis, and
elevated inflammatory markers
the pericardial process is the most life threatening and
should be at the top of our list of symptoms, signs, and
findings
.
Infection
RHUMATIC DISEASE (SLE-R.A)
Rheumatic fever
Adult-Onset Still’s Disease
Cancer
Hereditary periodic fever syndromes.
Infection
The protracted (5-week) course of the patient’s illness
argues against a viral cause, as does the markedly
elevated white-cell count with neutrophil
predominance. The presumably negative bacterial
cultures and the disease progression, despite
the administration of broad-spectrum antibiotics,
argue against a bacterial infection.
RHUMATIC DISEASE
The findings are not typical of rheumatoid arthritis,
and the rheumatoid factor was negative.
Although the patient had serositis and joint
involvement,
he did not have other features to suggest
systemic lupus erythematosus, and a test for
antinuclear antibody was negative.
Cancer
Cancer could be considered,
but he had no dominant lymphadenopathy to suggest
lymphoma, no laboratory findings to suggest a
myeloproliferative disorder, and no clinical or
radiographic evidence of a solid tumor.
Rheumatic fever
Rheumatic fever should also be considered given the
recent pharyngitis, the positive throat culture for
group A streptococcus, and the reported jerking
movements during sleep..
The positive throat culture for group A
beta-hemolytic streptococcus most likely reflected
a carrier state; tests for anti–deoxyribonuclease
B (anti–DNase B) and antistreptolysin O antibodies,
which are highly sensitive markers for
acute rheumatic fever, were negative.
Furthermore, the presence of pericarditis without
concomitant myocarditis or valvulitis would be highly
atypical in a patient with acute rheumatic fever.
Adult-Onset Still’s Disease
We should try to explain the rash, which
disappeared 1 hour after it was initially described,
Adult-onset Still’s disease is classically characterized
by four cardinal symptoms: spiking fever, evanescent
salmon-pink maculopapular rash, arthritis, and a
white-cell count greater than 10,000 per cubic
millimeter, with predominance
of neutrophilic polymorphonuclear cells.
In addition, many affected patients
present with sore throat, lymphadenopathy, anemia,
and abnormal results of liver-function tests,
and approximately one quarter of patients have
pleuritis or pericarditis. Most patients have
markedly elevated ferritin level
this diagnosis explains all
the major findings, with the exception of the
jerking movements, which were most likely myoclonic
jerks that were accentuated by his illness.
Hereditary periodic fever syndromes
The patient’s presentation of fevers, arthritis,
serositis, without a history of recurrent inconsistent with
hereditary periodic fever syndromes
.
This patient met at least seven of the Yamaguchi criteria
but met only three of the minor Jones criteria, all of
which overlap with Yamaguchi criteria.
Adult-onset Still’s disease has two clinical
phenotypes — a systemic form, which is predominantly
characterized by systemic symptom
(e.g., high fever and rash), and a chronic form,
which is predominantly characterized by arthritis
that may become deforming. This patient had
the systemic form, which is associated with a
better prognosis and may be monophasic
Adult-onset Still’s disease is on the same
spectrum as systemic juvenile idiopathic arthritis,
although their association is not completely
understood.
Both diseases are characterized by inflammation,
and interleukin-1 appears to be the dominant mediator.
Biologic agents that block
interleukin-1 activity are highly effective in the
treatment of persons with adult-onset Still’s disease.
MANAGEMENT
For mild disease, glucocorticoids and
nonsteroidal antiinflammatory drugs may be effective.
For moderate disease, glucocorticoids are
typically combined with methotrexate for chronic
Forms or combined with biologically active
agents .
THANKS