First AD

Second AD

A 33-year-old woman with sickle cell disease is admitted with a 2-day history of high fevers, chills, and severe right-sided pleuritic chest pain. She states that she was well until last week, when she developed upper respiratory–type symptoms, which progressed to severe right-sided pleuritic pain, a cough productive of green phlegm, a temperature as high as 103°F (39.4°C), and fatigue. 

Her past medical history is significant for a sickle cell crisis 1 month ago, but she states she is now asymptomatic. Laboratory values include white blood cell count of 27.5 x 10

 followed by a CT scan of the chest. A representative axial CT image is shown in 

This patient presents with an acute febrile illness, and an elevated white blood cell count suggestive of infection. Although pulmonary embolism commonly presents with pleuritic chest pain, pleural effusions, if present, are usually small and uncomplicated.

Acute chest syndrome is a syndrome of fever, hypoxemia, and chest pain, seen in patients with sickle cell disease. It is usually associated with a crisis, and therapy is directed toward alleviation of the crisis (fluids, pain medication, supportive care, and if necessary, exchange transfusion). Patients may have concurrent pneumonia and antibiotics are also prescribed. This patient did not present in crisis, and acute chest syndrome is predominantly parenchymal, not pleural disease. Malignancy involving the pleura would not typically present as an acute febrile illness. 

Patients with sickle cell disease have functional asplenia, and thus are predisposed to severe infection, particularly with encapsulated organisms. Thus empyema would be a logical choice for this presentation. This patient eventually required surgical decortication and subsequently recovered and did well.

• Vichinsky EP, Neumayr LD, Earles AN, at al. National Acute Chest Syndrome Study Group. Causes and outcomes of the acute chest syndrome in sickle cell disease. 

 2000;342:1855-1865. Full text available at: 

http://www.nejm.org/doi/pdf/10.1056/NEJM200006223422502

• Colice GL, Curtis A, Deslauriers J, et al. Medical and surgical treatment of parapneumonic effusions: an evidence-based guideline. 

2000;118:1158-1171. Full text available at: 

http://journal.publications.chestnet.org/data/Journals/CHEST/21953/1158.pdf. 

Respiratory Diseases

A young woman experienced acute onset of high fever, productive cough, and right-sided pleuritic pain. She was not in sickle cell crisis. Do the images here shed light on cause of symptoms?  

In Sickle Cell Disease, What Cause of Acute Respiratory Symptoms?