Acute chest syndrome: Difference between revisions

Revision as of 19:19, 22 March 2025

A complication of sickle cell disease

Acute chest syndrome (ACS) is a serious complication of sickle cell disease (SCD) characterized by chest pain, fever, and pulmonary infiltrates on a chest X-ray. It is a leading cause of morbidity and mortality in patients with sickle cell disease.

Pathophysiology

Acute chest syndrome is primarily caused by the sickling of red blood cells in the pulmonary vasculature, leading to vaso-occlusion and subsequent ischemia and infarction of lung tissue. This process can be triggered by various factors, including infection, fat embolism, and pulmonary embolism. The resulting inflammation and hypoxia further exacerbate the sickling process, creating a vicious cycle.

Clinical Presentation

Patients with acute chest syndrome typically present with sudden onset of chest pain, dyspnea, and fever. Other symptoms may include cough, tachypnea, and hypoxemia. The condition can rapidly progress to acute respiratory distress syndrome (ARDS) if not promptly treated.

Diagnosis

The diagnosis of acute chest syndrome is primarily clinical, supported by imaging and laboratory findings. A chest X-ray typically shows new pulmonary infiltrates, which may be bilateral or unilateral. Laboratory tests often reveal leukocytosis, anemia, and elevated inflammatory markers. Pulse oximetry may show decreased oxygen saturation.

Management

The management of acute chest syndrome involves supportive care and specific treatments to address the underlying causes. Key components of management include:

Oxygen therapy to maintain adequate oxygenation.
Intravenous fluids to prevent dehydration and reduce sickling.
Pain management with analgesics to control chest pain.
Antibiotics if an infectious cause is suspected.
Blood transfusion to reduce the proportion of sickled cells and improve oxygen delivery.
Bronchodilators and incentive spirometry to improve lung function.

Prognosis

The prognosis of acute chest syndrome varies depending on the severity of the episode and the timeliness of treatment. Early recognition and aggressive management are crucial to prevent complications such as respiratory failure and multi-organ failure. Recurrent episodes of acute chest syndrome can lead to chronic lung disease and decreased life expectancy in patients with sickle cell disease.

Prevention

Preventive strategies for acute chest syndrome include regular vaccination against pneumococcus and influenza, hydroxyurea therapy to reduce sickling episodes, and chronic transfusion therapy in high-risk patients. Patient education on recognizing early symptoms and seeking prompt medical attention is also essential.

Related pages

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-{{short description|Potentially lethal blockage of lung vasculature in sickle cell anaemia}}
+{{Short description|A complication of sickle cell disease}}
-{{Infobox medical condition (new)
+{{Use dmy dates|date=October 2023}}
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-The '''acute chest syndrome''' is a [[vaso-occlusive crisis]] of the [[pulmonary]] [[vasculature]] commonly seen in people with [[sickle cell anemia]]. This condition commonly manifests with a new [[Infiltration (medical)|opacification]] of the lung(s) on a chest [[x-ray]].<ref name="Pace2007">{{cite book|author=Betty Pace|title=Renaissance of Sickle Cell Disease Research in the Genome Era|url=https://books.google.com/books?id=mhqswaCtcLQC&pg=PA81|accessdate=15 June 2010|year=2007|publisher=Imperial College Press|isbn=978-1-86094-645-5|pages=81–}}</ref>
-==Signs and symptoms==
+'''Acute chest syndrome''' (ACS) is a serious complication of [[sickle cell disease]] (SCD) characterized by [[chest pain]], [[fever]], and [[pulmonary infiltrates]] on a [[chest X-ray]]. It is a leading cause of morbidity and mortality in patients with sickle cell disease.
-The crisis is a common complication in sickle-cell patients and can be associated with one or more symptoms including fever, cough, excruciating pain, [[sputum]] production, [[dyspnea|shortness of breath]], or [[Hypoxia (medical)|low oxygen levels]].<ref>{{cite web|author= Johnson, CS|year=1995|title=Sickle-Cell Disease: The Acute Chest Syndrome|url=http://sickle.bwh.harvard.edu/acutechest.html}}</ref>
-==Cause==
+==Pathophysiology==
-Acute chest syndrome is often precipitated by a lung infection, and the resulting [[inflammation]] and loss of oxygen saturation leads to further sickling of red cells, thus exacerbating pulmonary and systemic hypoxemia, sickling, and vaso-occlusion.{{citation needed|date=July 2020}}
+Acute chest syndrome is primarily caused by the sickling of [[red blood cells]] in the pulmonary vasculature, leading to [[vaso-occlusion]] and subsequent [[ischemia]] and [[infarction]] of lung tissue. This process can be triggered by various factors, including [[infection]], [[fat embolism]], and [[pulmonary embolism]]. The resulting inflammation and hypoxia further exacerbate the sickling process, creating a vicious cycle.
+==Clinical Presentation==
+Patients with acute chest syndrome typically present with sudden onset of [[chest pain]], [[dyspnea]], and [[fever]]. Other symptoms may include [[cough]], [[tachypnea]], and [[hypoxemia]]. The condition can rapidly progress to [[acute respiratory distress syndrome]] (ARDS) if not promptly treated.
 ==Diagnosis==
-The diagnosis of acute chest syndrome is made difficult by its similarity in presentation with pneumonia.  Both may present with a new opacification of the lung on chest x-ray.  The presence of fevers, low oxygen levels in the blood, increased respiratory rate, chest pain, and cough are also common in acute chest syndrome.  Diagnostic workup includes chest x-ray, complete cell count, reticulocyte count, ECG, and blood and sputum cultures.  Patients may also require additional blood tests or imaging (e.g. a CT scan) to exclude a heart attack or other pulmonary pathology.{{citation needed|date=July 2020}}
+The diagnosis of acute chest syndrome is primarily clinical, supported by imaging and laboratory findings. A [[chest X-ray]] typically shows new pulmonary infiltrates, which may be bilateral or unilateral. Laboratory tests often reveal [[leukocytosis]], [[anemia]], and elevated inflammatory markers. [[Pulse oximetry]] may show decreased [[oxygen saturation]].
-==Prevention==
-[[Hydroxycarbamide|Hydroxyurea]] is a medication that can help to prevent acute chest syndrome. It may cause a [[Leukopenia|low white blood cell count]], which can predispose the person to some types of infection.<ref>[https://www.cdc.gov/ncbddd/sicklecell/treatments.html Sickle cell disease (SCD)]. [[Centers for Disease Control and Prevention]]. Retrieved January 7, 2015.</ref>
-==Treatment==
+==Management==
-Broad spectrum antibiotics to cover common infections such as ''[[Streptococcus pneumoniae]]'' and [[mycoplasma]], pain control, and [[blood transfusion]]. Acute chest syndrome is an indication for [[exchange transfusion]].
+The management of acute chest syndrome involves supportive care and specific treatments to address the underlying causes. Key components of management include:
-[[Bronchodilators]] may be useful but have not been well studied.<ref>{{cite journal|last1=Knight-Madden|first1=JM|last2=Hambleton|first2=IR|title=Inhaled bronchodilators for acute chest syndrome in people with sickle cell disease.|journal=The Cochrane Database of Systematic Reviews|date=Aug 2, 2014|volume=8|issue=8|pages=CD003733|pmid=25086371|doi=10.1002/14651858.CD003733.pub3}}</ref>
+* [[Oxygen therapy]] to maintain adequate oxygenation.
+* [[Intravenous fluids]] to prevent dehydration and reduce sickling.
+* [[Pain management]] with [[analgesics]] to control chest pain.
+* [[Antibiotics]] if an infectious cause is suspected.
+* [[Blood transfusion]] to reduce the proportion of sickled cells and improve oxygen delivery.
+* [[Bronchodilators]] and [[incentive spirometry]] to improve lung function.
 ==Prognosis==
-It may result in death,<ref>{{DorlandsDict|nine/20711638|acute chest syndrome}}</ref> and it is one of the most common causes of death for people with sickle cell anemia.<ref>Kumar, Abbas, Fausto. Robbins and Cotran: The Pathologic Basis of Disease, Page 631</ref>
+The prognosis of acute chest syndrome varies depending on the severity of the episode and the timeliness of treatment. Early recognition and aggressive management are crucial to prevent complications such as [[respiratory failure]] and [[multi-organ failure]]. Recurrent episodes of acute chest syndrome can lead to chronic lung disease and decreased life expectancy in patients with sickle cell disease.
+==Prevention==
+Preventive strategies for acute chest syndrome include regular [[vaccination]] against [[pneumococcus]] and [[influenza]], [[hydroxyurea]] therapy to reduce sickling episodes, and [[chronic transfusion therapy]] in high-risk patients. Patient education on recognizing early symptoms and seeking prompt medical attention is also essential.
-==References==
+==Related pages==
-{{reflist}}
+* [[Sickle cell disease]]
-== External links ==
+* [[Vaso-occlusive crisis]]
-{{Medical resources
+* [[Pulmonary embolism]]
-|  DiseasesDB     =
+* [[Acute respiratory distress syndrome]]
-|  ICD10          =
-|  ICD9           = {{ICD9|517.3}}
-|  ICDO           =
-|  OMIM           =
-|  MedlinePlus    =
-|  eMedicineSubj  =
-|  eMedicineTopic =
-|  MeshID         =
-}}
-{{Respiratory pathology}}
-{{DEFAULTSORT:Acute Chest Syndrome}}
+[[Category:Hematology]]
-[[Category:Red blood cell disorders]]
+[[Category:Pulmonology]]
-[[Category:Syndromes]]
+[[Category:Medical emergencies]]
-{{dictionary-stub1}}
-{{No image}}