Hamman-Rich syndrome

Acute interstitial pneumonia is a rare but important diagnosis, associated with a high mortality rate and important to identify early.

A 76 year-old individual presented to hospital with a two-week history of shortness of breath, fevers and a non-productive cough. Treatment initially was for lower respiratory tract infection but returned to hospital three days later as her shortness of breath and peripheral oedema was worsening despite diuretic treatment. Arterial blood gas showed Type 1 Respiratory Failure (p02 was only 10 kPa on 4 L per minute of oxygen). A computed tomography pulmonary angiography (CTPA) was performed to rule out a pulmonary embolism (PE), which showed multifocal diffuse areas of consolidations bilaterally involving all lobes. Bronchoalveolar lavage cellular analysis was also done. The patient was treated as nonspecific interstitial pneumonia. This case study highlights this rare condition presenting similarly to common pulmonary conditions.

The disease is often preceded by a flu-like prodromal illness lasting one to two weeks prior to presentation. Acute respiratory failure develops in previously healthy individuals without pre-existing lung disease. Diagnosis is also supported by high-resolution computed tomography (HRCT). The effects of high flow ventilation in patients with idiopathic pulmonary fibrosis are associated with improvement in respiratory parameters, improving the efficiency of breathing.

Acute interstitial pneumonitis can be a difficult diagnosis, associated with a high mortality rate up to 60%. It is also difficult to treat; however supportive treatment with high flow oxygen therapy along with pulsatile high dose Corticosteroids can aid recovery.