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Acute interstitial pneumonia
Acute interstitial pneumonia (AIP) is an acute, rapidly progressive idiopathic pulmonary disease that often leads to fulminant respiratory failure and acute respiratory distress syndrome (ARDS)
Prevalence
1-9 / 100 000
Age of Onset
ICD-10
J84.1
Inheritance
This condition does not appear to have a clear pattern of inheritance.
5 Facts you should know
FACT
An acute, rapidly progressive idiopathic pulmonary disease that often leads to respiratory failure and acute respiratory distress syndrome (ARDS)
FACT
Symptoms usually start as viral-like prodrome followed with shortness of breath with cough, fever, and progress rapidly to acute respiratory distress
FACT
The physical exam typically shows findings of hypoxia, tachypnea, and bilateral diffuse crackles
FACT
Chest X-ray usually shows a pattern that is similar to ARDS
FACT
The mortality rate is greater than 50%, either in the initial presentation or within 6 months after onset
Interest over time
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Common signs & symptoms
Bronchiectasis
Dyspnea
Ground-glass opacification on pulmonary HRCT
Hypoxemia
Interlobular septal thickening on pulmonary HRCT
Nodular pattern on pulmonary HRCT
Peribronchovascular interstitial thickening
Pulmonary infiltrates
Current treatments
Management of ADULT syndrome typically involves addressing individual symptoms and complications as they arise.
Oxygen Therapy
Patients with AIP often require supplemental oxygen to maintain adequate oxygen levels in the blood
Mechanical Ventilation
In severe cases where respiratory failure occurs, mechanical ventilation may be necessary to assist with breathing. This can involve invasive methods such as intubation and mechanical ventilation or non-invasive methods such as continuous positive airway pressure (CPAP) or bilevel positive airway pressure (BiPAP)
Corticosteroids
While the evidence supporting the use of corticosteroids in AIP is limited, they are sometimes used early in the disease course to reduce inflammation in the lungs. However, their efficacy in improving outcomes is debated
Empirical Antibiotics
Antibiotics may be prescribed initially to treat or prevent bacterial infections, as respiratory infections can exacerbate AIP
Immunosuppressive Therapy
In some cases, immunosuppressive medications may be prescribed to modulate the immune response and reduce lung inflammation. These may include drugs like cyclophosphamide or azathioprine
Pulmonary Rehabilitation
Pulmonary rehabilitation programs can help improve respiratory function and quality of life through exercises, education, and support
Extracorporeal Membrane Oxygenation (ECMO)
In very severe cases of respiratory failure refractory to conventional ventilation, ECMO may be considered as a temporary life-supporting measure