Transcript Title of Study Authors university or hospital affiliation

PERICARIDAL AND PLEURAL EFFUSIONS AS THE INITIAL
PRESENTATION OF SYSTEMIC LUPUS ERYTHEMATOSUS
1
SAMUEL ASH, M.D. AND
2
MARGARET NEFF, M.D.
1DEPARTMENT
OF INTERNAL MEDICINE, UNIVERSITY OF WASHINGTON MEDICAL CENTER
2DIVISION OF PULMONARY AND CRITICAL CARE MEDICINE, HARBORVIEW MEDICAL CENTER
INTRODUCTION
Systemic lupus erythematosus
(SLE) is an inflammatory
autoimmune disease that
affects multiple organ systems.
The clinical course of patients
with SLE is variable and
difficult to predict, and its
protean manifestations and
variable presentation may
make diagnosis difficult.
OBJECTIVES
PLEURAL DISEASE
• Pleural effusion occurs in
approximately 30% of
patients with SLE and may be
present in up to 93% of cases
at autopsy but it is the initial
manifestation in only 1% of
SLE cases
• The pathogenesis of lupus
pleuritis is not well
understood, but pleural
biopsy shows chronic
inflammation with
lymphocyte and plasma cell
infiltration
• The typical clinical
presentation of lupus pleuritis
includes days to weeks of
fever, pleuritic chest pain,
cough and dyspnea
• Other pulmonary
complications of SLE include
infection, reaction to drug
therapy, interstitial lung
disease, diffuse alveolar
hemorrhage, acute lupus
pneumonitis, bronchiectasis,
pulmonary arterial
hypertension, shrinking lung
syndrome and pulmonary
embolus
PERICARDIAL DISEASE
• Pericarditis is the most
common cardiac complication
of SLE
• Pericardial effusion is less
common and occurs in 9 to
54% of SLE cases
• Cardiac tamponade
associated with SLE is rare,
with a reported incidence of 1
to 2.5%
• Cardiac tamponade as an
initial manifestation of SLE is
even more uncommon. A
review of the literature
revealed only 26 other case
reports of cardiac tamponade
as an initial manifestation of
SLE
• Case reports suggest that SLE
cardiac tamponade may be
treated with indomethacin,
but high dose steroids remain
the mainstay of the acute
treatment
CASE REPORT
DISCUSSION
A 19 year old man with history of anorexia nervosa and bulemia presented to an outside
hospital with two weeks of worsening pleuritic chest pain, cough, rhinorrhea and
generalized fatigue. He was diagnosed with community acquired pneumonia complicated
by parapneumonic effusion and sepsis. He was briefly intubated for central venous
catheter placement, but within 48 hours he was extubated and left against medical advice
with oral antibiotics. He presented to our hospital with similar complaints as well as
shortness of breath.
PHYSICAL EXAM
• T 36.6, HR 124, RR 26, BP 92/56
• Gen: thin, NAD
• Cardiac: tachycardic. no murmurs, rubs
or gallops. nl s1/s2. 2+ bilateral
pitting edema
• Resp: diminished breath sounds at the
right base
• Skin: no rash or breakdown
INITIAL LABORATORY STUDIES
• WBC 10120 (nl 4300-10000)
• Hemoglobin 9.6 (nl 13.0-18.0)
• INR 1.5
• BNP 186 (nl less than 100)
Figure 1-2: PA and left lateral decubitus chest
radiographs showing moderate right and small left
pleural effusions
Clinical Course
The patient’s effusions and tamponade
were initially felt to be reactive and
secondary to an underlying infectious
process. A pericardial drain was placed
with an initial output of 480 mL of
serosanguinous fluid, which resulted in
significant improvement in the patient’s
blood pressure and tachycardia. A
pleural drain was also placed. However,
despite adequate antibiosis his
pericardial and pleural drainage
continued and remained significant.
Figure 3: TTE showing large pericardial effusion with
echocardiographic evidence of tamponade.
ADDITIONAL LABORATORY STUDIES
• Blood, urine, sputum, pericardial fluid
and pleural fluid cultures without
growth
• ANA 1:40
• Anti-smith antibody positive
Diagnosis
A diagnosis of systemic lupus
erythematosus was made and the patient
was treated with pulse dose steroids
followed by maintenance steroid dosing.
With steroid therapy, he rapidly
improved and after removal of his drains
he was discharged home with
rheumatology follow-up.
CONCLUSIONS
•The variable presentation of SLE can make
its diagnosis difficult, particularly in
atypical cases
•SLE can have multiple effects on the lungs
including serositis resulting in significant
pleural effusions
•Pericardial tamponade is a rare but life
threatening complication of SLE and is
even more uncommon as an initial
manifestation of the disease
•Atypical presentations of a broad
differential of diseases should be
considered when a patient’s clinical course
does not progress as anticipated
The most common presenting
syndrome of SLE includes a
mixture of constitutional
symptoms, with skin,
musculoskeletal and mild
hematologic involvement.
However, the symptoms that
brought this patient to medical
attention were primarily those
secondary to his pleural and
pericardial effusions, the latter
of which resulted in cardiac
tamponade.
Further discussion with this
patient and detailed history
yielded no other signs or
symptoms concerning for SLE
except for possible monocular
iritis, which was treated
topically 3 years prior to this
presentation. In retrospect,
his hematologic findings were
consistent with SLE as were
his pleuritis and cardiac
tamponade. However it was
the patient’s lack of initial
clinical improvement that led
to further evaluation and
resulted in the appropriate
diagnosis of a rare
presentation of an uncommon
disease.
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Clinical manifestations of SLE.
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• Keane MP, Lynch JP.
Pleuropulmonary
manifestations of SLE. Thorax
2000; 55:159-66.
• Mohseni MM, Rogers ER.
Cardiac tamponade as the
initial manifestation of SLE. J
Em Med 2010. Epub ahead of
print.
• Porcel JM et al. Resolution of
cardiac tamponade in SLE with
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