Acute interstitial pneumonitis

Symptoms and signs

The most common symptoms of acute interstitial pneumonitis are highly productive cough with expectoration of thick mucus, fever, and difficulties breathing. These often occur over a period of one to two weeks before medical attention is sought. The presence of fluid means the person experiences a feeling similar to 'drowning'. Difficulties breathing can quickly progress to an inability to breathe without support (respiratory failure).

Acute interstitial pneumonitis typically progresses rapidly, with hospitalization and mechanical ventilation often required only days to weeks after initial symptoms of cough, fever, and difficulties breathing develop. Also, tachypnea and crackling noises are commonly heard when listening to the lungs of patients with this disease.

Diagnosis

Rapid progression from initial symptoms to respiratory failure is a key feature. An X-ray that shows ARDS is necessary for diagnosis (fluid in the small air sacs (alveoli) in both lungs). In addition, a biopsy of the lung that shows organizing diffuse alveolar damage is required for diagnosis. This type of alveolar damage can be attributed to nonconcentrated and nonlocalized alveoli damage, marked alveolar septal edema with inflammatory cell infiltration, fibroblast proliferation, occasional hyaline membranes, and thickening of the alveolar walls. The septa are lined with atypical, hyperplastic type II pneumocytes, thus leading to the collapse of airspaces. Other diagnostic tests are useful in excluding other similar conditions, but history, X-ray, and biopsy are essential. These other tests may include basic blood work, blood cultures, and bronchoalveolar lavage. The clinical picture is similar to ARDS, but AIP differs from ARDS in that the cause for AIP is not known. File:CRIM.PULMONOLOGY2012-678249.001.jpg|Acute interstitial pneumonia showing a marked reduction in lung capacity File:Acute interstitial pneumonia (AIP) Idiopathic DAD 3.jpg File:Acute interstitial pneumonia (AIP) Idiopathic DAD 2.jpg File:Acute interstitial pneumonia (AIP) Idiopathic DAD.jpg

Treatment

Treatment is primarily supportive. Management in an intensive care unit is required and the need for mechanical ventilation is common. Therapy with corticosteroids is generally attempted, though their usefulness has not been established. Lung transplantation has been shown to improve survival and quality of life in selected patients with advanced or end-stage interstitial lung disease, including cases of acute interstitial pneumonitis, with median post-transplant survival reported at around five years.

Prognosis

Sixty percent of people with acute interstitial pneumonitis will die in the first six months of illness. The median survival is months. However, many people who recover from the initial course of the disease experience recurrent episodes of the disease or develop chronic progressive interstitial lung disease. Although some studies have observed partial or (very rarely) complete recovery of lung function in some survivors of acute interstitial pneumonitis, the majority of survivors will continue to endure progressive lung complications.

Epidemiology

Acute interstitial pneumonitis occurs most frequently among people older than forty years old. It affects men and women equally. There are no known risk factors; but smoking is implicated in the clinical behaviour of the disease.

History

Acute interstitial pneumonitis was first described in 1935 by Louis Hamman and Arnold Rich, and given the name Hamman–Rich syndrome.

References

References

1. "Robbin's Pathological Basis of Disease".
2. (1944). "Acute diffuse interstitial fibrosis of the lungs". Bull. Johns Hopkins Hosp..
3. Mrad, Ali. (2025). "Acute interstitial pneumonia". Treasure Island (FL): StatPearls Publishing.
4. (2025). "Acute Interstitial Pneumonia (AIP)". Merck Manual Professional Edition.
5. Leong, Swee. (2023). "Lung transplantation for interstitial lung disease: evolution over three decades". BMJ.
6. Bouros, D. (2000). "Acute interstitial pneumonia". Eur. Respir. J..
7. (31 August 2015). "Smoking and interstitial lung diseases". European Respiratory Review.
8. Hamman, L. (1935). "Fulminating diffuse interstitial fibrosis of the lungs". European Respiratory Society.