ACUTE CHEST

SYNDROME

Andre Coleman, (Almost) M.B.B.S.

CASE

31 year old black male with Hb SS

PC:
Pain crisis
HPC:
Pain is identical to typical pain crisis.
Pain started yesterday and has not responded to
analgesics.

CASE
PMH
Avascular necrosis of both hips, priapism, NSTEMI,
cholecystectomy, one prior history of Acute Chest
Syndrome (ACS).
Visits ED once every month
SH
Smokes cigarettes
Drinks beer occasionally

CASE
Observation and Examination
Temp 99.6 C BP 113/65

Pulse 98/min RR 20/min

O2 93% on room air

Patient in respiratory distress
Mild conjunctival icterus
Abdomen soft with diffuse tenderness
Tender over both hips and lateral thighs

CASE
Emergency Department course
O2 fell to 80% after pain medication
O2 responded to oxygen therapy
Leukocytosis WBC = 17,000
Haemoglobin 8.9 (baseline)

CASE
Patient is admitted to ward
The next day he becomes tachypneic and
tachycardic
Oxygen requirements increase
Transferred to ICU
Receives observation and exchange transfusion
Discharged after day 10

Diagnosis???

ACUTE CHEST
SYNDROME
Acute

Acute cardiopulmonary symptom

PLUS
Chest New pulmonary radiographic infiltrate

Syndrome

Complex and multifactorial pathogenesis

SICKLE CELL DISEASE

Hereditary haemoglobinopathy
Results in abnormal red blood cells that sickle
under poor oxygen tension
Sickled red cells can occlude blood vessels and
disrupt blood supply

EPIDEMIOLOGY
Second most common cause of hospitalization
Responsible for 25% of deaths from Sickle Cell Disease
Most common in the 2-4 years age group
Mortality rate of
<1% for children up to 9%
2% for children 10-19 years
9% for adults

EPIDEMIOLOGY
Hb SS = Hb S 0 Thalassemia > Hb SC = Hb S +
Thalassemia
50% will have at least one episode and some will
have many
50% develop the disorder during hospitalization
for another complication of SCD

CASE

31 year old black male with Hb SS

PC:
Pain crisis
HPC:
Pain is identical to typical pain crisis.
Pain started yesterday and has not responded to
analgesics.

INCIDENCE
Inversely proportional to HbF levels
Directly proportional to hematocrit and WBC levels

CASE
Emergency Department course
O2 fell to 80% after pain medication
O2 responded to oxygen therapy
Leukocytosis WBC = 17,000
Haemoglobin 8.9 g/dL (baseline)

RISK FACTORS
History of Acute Chest Syndrome
Winter months
Asthma and airway hyper-responsiveness
Surgery and Anesthesia

Decline in pulmonary function

Smoking
Opioid use

CASE
PMH
Avascular necrosis of both hips
Priapism
NSTEMI
Cholecystectomy
One prior history of Acute Chest Syndrome (ACS).
Visits ED once every month
SH
Smokes cigarettes
Drinks beer occasionally

ETIOLOGY
Causes are many hence the term syndrome
Etiologies can be classified as those related to Hb S
and those unrelated

DIRECT CONSEQUENCE
OF HB S
Pulmonary infarct
Fat embolism syndrome
Pulmonary oedema induced by narcotics and fluid
overload ***
Hypoventilation
Secondary to rib or sternal bone infarct
Secondary to narcotic administration
***

INDIRECT
CONSEQUENCE OF HB S
Infections
Bacterial
Viral
Fungal
Protozoan

UNRELATED TO HB S
Fibrin thromboembolism
Foreign Body or Intrinsic bronchial obstruction
HIV related opportunistic infections
Acute sarcoidosis
Other common pulmonary diseases
Asthma, aspiration, trauma, etc.

PULMONARY VASCULAR
OCCLUSION
May be the primary cause or the final common pathway
Endothelial dysfunction with
Increased expression of vascular adhesion molecules
Increased platelets and plasma coagulation
Disordered nitric oxide metabolism
Leads to thromboembolism and perhaps hemolysis
Increased secretion of inflammatory cytokines due to infection or
other causes may result in ischemia

FAT EMBOLISM
SYNDROME
Associated with severe vaso-occlusive painful crisis
involving multiple bones
Fat, marrow cells and bony spicules are released into
the circulation where they reach to the lung
Results in severe pulmonary inflammation, vasoocclusion and acute pulmonary hypertension
Formation of free fatty acids by the action of secretory
phospholipase A2

SIGNS AND SYMPTOMS

Common symptoms are fever, cough and chest
pain
Other symptoms include wheezing, chills,
shortness of breath and hemoptysis
Symptoms vary with age

SIGNS AND SYMPTOMS

The most common finding on examination is a normal
exam
Rales, dullness to percussion and wheezing are frequent
findings
Neurologic abnormalities may be present and hint to
infectious etiologies such as Mycoplasma or Chlamydia
Physical Examination is an unreliable tool for
detecting ACS

INVESTIGATIONS

RADIOGRAPHS
Prior to radiological diagnosing, 61% of ACS cases were not
clinically suspected
Do a chest radiograph in a SCD patient who has fever, chest pain or
respiratory symptoms though infiltrates may not appear until 48-72
hours
Patients admitted for severe painful crisis with hypoxemia at rest
should have a repeat chest radiograph 24-48 hours after admission
Bilateral infiltrates or multi-lobar involvement may predict poor
prognosis
Pleural effusions are not usually associated with infectious etiology
High resolution CT scan is more specific than plain chest film

BLOOD WORK
Hemoglobin falls on average 0.7 g/dL
WBC increase on average 69%

BLOOD GAS ANALYSIS

70% present with PO2 < 80 mm Hg
25% present with PO2 < 60 mm Hg
Pulse oximetry overestimates oxygen levels because of
methemoglobin and carboxyhemoglobin
Co-oximetry is the most accurate method
PaO2 is useful to determine PaO2/FiO2 ratio to assess for
frank ARDS

BLOOD CULTURE
Bacteremia occurs is about 3.5% of cases
Chalmydia pneumoniae and Mycoplasma pneumoniae
are the most common documented causes
Other causes include
Streptococcus pneumoniae
Staphylococcus aureus
Klebsiella pneumoniae
Adenovirus
Influenza virus

BRONCHOSCOPIC
CULTURE
Bronchoscopy is the method of choice to obtain
high quality material for culture
Bronchoscopy can also be used to detect lipidladen macrophages

COMPLICATIONS OF ACS

Longer hospital stay and frequent ICU admission

Chronic lung disease
Respiratory failure

TREATMENT

TREATMENT
Supportive measures
Antibiotics
Incentive spirometry

SUPPORTIVE THERAPY
Oxygen therapy if hypoxic
Maintain a PaO2 less than or equal to 100 mmHg in intubated patients to avoid
suppression of erythropoiesis
Adequate hydration
Avoid over-hydration
Pain therapy
Avoid NSAIDS
Avoid over-sedation
Intercostal nerve block with local anesthetic
Patient controlled anesthesia

ANTIBIOTICS
Coverage for the likely causative organism
Third generation cephalosporin
S. pneumoniae
H. Influenzae
K. Pneumoniae

Macrolide
M. Pneumoniae
C. Pneumoniae

A full course of antibiotics is recommended regardless of culture

results

INCENTIVE SPIROMETRY
10 puffs every 2 hours while
awake
Improves ventilation
compromised by opioids

TRANSFUSION
THERAPY
Indication determined clinically
Consider for
Hypovolemia
Hypoxemia
Multilobar pneumonia
Do not exceed Hb of 10 g/dL or hematocrit of 35%
Transfusion rate should be slow
Exchange transfusion is indicated in patients with
PaO2 less than 70 mmHg and who do not respond
to supplemental oxygen

EXCHANGE
TRANSFUSION

Haematology consult should be obtained

If unavailable on emergent basis
Phlebotomize 500 ml.
Infuse 300 ml normal saline.
Phlebotomize additional 500 ml.
Infuse 4-5 units of packed red blood cells.

EXPERIMENTAL
THERAPY
Corticosteroids
Reduces the length of hospitalization
Reduces the length of oxygen and opioid therapy
Useful for asthamtics
Increased readmission within 72 hours
Nitrous Oxide
Reduces pulmonary pressures
Improves oxygenation
Decreases expression of adhesion molecules
Improves haemoglobin saturation

CHRONIC BLOOD
TRANSFUSION
Reduces the incidence of ACS events in patients with
a history of recurrent or severe episodes
Aim to keep sickled cells <30% and hematocrit <35%
Increases risk of
Transfusion reactions
Alloimmunization
Infections
Iron overload

HYDROXYUREA
Ribonuclease reductase inhibitor
Increase Hb F production
Reduces
Episodes of Painful crises
Episodes of Acute chest syndrome
Need for transfusion
Mortality

HEMATOPOIETIC STEM CELL

TRANSPLANTATION
Only available curative option
Limited by toxicity and availability

REFERENCES
http://www.turnerwhite.com/memberfile.php?
PubCode=hp_jan07_sickle.pdf
http://bloodjournal.hematologylibrary.org/content/117/20/5297?ssochecked=1
http://www.ed.bmc.org/library/corecurriculum/JHematologyACSreview.pdf
http://www.acep.org/Clinical---Practice-Management/Focus-On--AcuteChest-Syndrome---The-Critical-Cough/
http://sickle.bwh.harvard.edu/acutechest.html