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Idiopathic pneumonia syndrome

Field	Value
specialty	pulmonology
causes	Stem cell transplant

Idiopathic pneumonia syndrome (IPS) is 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.

Symptoms

The symptoms are pneumonia-like, and include fever, chills, coughing, and breathing problems.

Risk factors

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.

Additional risk factors for IPS include total body irradiation dose, age, graft vs host disease, and multi organ failure.

While older reports suggest a rate of as high as 15% of stem cell transplants resulting in IPS, recent studies suggest this is lowering.

Diagnosis

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

Treatment is supportive. IPS is associated with morbidity and poor long-term recovery. While steroids are often used and help with short term outcomes, but do not improve 1-year mortality. Etanercept, a TNFa binding protein also improves short term outcomes, but with no effect on 1-year mortality.

References

Idiopathic pneumonia syndrome entry in the public domain NCI Dictionary of Cancer Terms

References

(February 2020). "Incidence, Risk Factors, and Outcomes of Idiopathic Pneumonia Syndrome after Allogeneic Hematopoietic Cell Transplantation". Biology of Blood and Marrow Transplantation: Journal of the American Society for Blood and Marrow Transplantation.
(2015). "Idiopathic pneumonia syndrome following hematopoietic stem cell transplantation". Journal of Pediatric Intensive Care.
(2023-09-06). "It's All in the Timing". New England Journal of Medicine.

Info: Wikipedia Source

This article was imported from Wikipedia and is available under the Creative Commons Attribution-ShareAlike 4.0 License. Content has been adapted to SurfDoc format. Original contributors can be found on the article history page.

pneumonia syndromes-affecting-the-lung

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