Diagnosis
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If a patient develops symptoms of IPS after a stem-cell transplant, IPS is a de facto diagnosis if there is no identifiable cause of infection.
| Idiopathic pneumonia syndrome | |
|---|---|
| Specialty | Pulmonology |
| Causes | Stem cell transplant |
Idiopathic pneumonia syndrome (IPS) is a term that defines a set of pneumonia-like symptoms that occur with no sign of infection in the lung. Idiopathic pneumonia syndrome is a serious condition that occurs as a complication of a stem cell transplant. [1]
The symptoms are pneumonia-like, and include fever, chills, coughing, and breathing problems. [2]
IPS is a complication of a stem cell transplant. The incubation period ranges between 4 and 106 days, but mostly is about 22 days from transplant. [2]
Additional risk factors for IPS include total body irradiation dose, age, graft vs host disease, and multi organ failure. [2] [1]
While older reports suggest a rate of as high as 15% of stem cell transplants resulting in IPS, recent studies suggest this is lowering. [3]
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If a patient develops symptoms of IPS after a stem-cell transplant, IPS is a de facto diagnosis if there is no identifiable cause of infection.
Treatment is supportive. IPS is associated with morbidity and poor long-term recovery. [1] While steroids are often used and help with short term outcomes, but do not improve 1-year mortality. [2] [1] Etanercept, a TNFa binding protein also improves short term outcomes, but with no effect on 1-year mortality. [1]
This article incorporates public domain material from Dictionary of Cancer Terms. U.S. National Cancer Institute.