Rare Pulmonology News

Advertisement

Spotlight On

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

3,310-29,790

US Estimated

5,135-46,215

Europe Estimated

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

1

An acute, rapidly progressive idiopathic pulmonary disease that often leads to respiratory failure and acute respiratory distress syndrome (ARDS)

 

FACT

2

Symptoms usually start as viral-like prodrome followed with shortness of breath with cough, fever, and progress rapidly to acute respiratory distress

 

FACT

3

The physical exam typically shows findings of hypoxia, tachypnea, and bilateral diffuse crackles

 

FACT

4

Chest X-ray usually shows a pattern that is similar to ARDS

 

FACT

5

The mortality rate is greater than 50%, either in the initial presentation or within 6 months after onset

 

Acute interstitial pneumonia is also known as...

Acute interstitial pneumonia is also known as:

 
  • Acute interstitial pneumonitis
  • Hamman-Rich syndrome
 
 

What’s your Rare IQ?

What is the suspected cause of the lung injuries in acute interstitial pneumonia?

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

Top Clinical Trials

Top Treatments in Research