Hamman-Rich Syndrome

Definition

Hamman-Rich Syndrome which is also referred to as acute interstitial pneumonia is a rare kind of pneumonia that could possibly affect healthy individuals on both genders. The disease can get worse without any symptoms appearing for a long span of time; but when symptoms do present, they just appear all of a sudden and acutely.

 

Hamman-Rich Syndrome has symptoms that are almost identical to acute respiratory distress (ARDS) that is why the condition is sometimes misdiagnosed. The disease can occur for a long time which impacts the connective tissue of the lungs. Plasma and white blood cells develop in the alveoli which causes an inflammation in the lungs. With Hamman-Rich Syndrome, the inflammation stretches out into the bronchioles.

Symptoms of Hamman-Rich Syndrome

Clinical symptoms of Hamman-Rich Syndrome consist of:

• Sudden onset of fever
• Shortness of breath
• Coughing
• Cyanosis or the bluish discoloration of the skin
• Fast, shallow breathing
• Wheezing sounds in the lungs
• Problem of the brain and heart functions such as forgetfulness, brisk heart rate, and difficulty concentrating which is due to the long-term reduced supply of oxygen

Causes

Hamman-Rich Syndrome is an idiopathic condition since researchers still do not know what the exact cause is. Based on a histological study, Diffuse Alveolar Damage (DAD) is a main diagnostic criterion among patients with AIP.  DAD is a common histological pattern found on surgical lung biopsies and could result from inhalational injuries, infections, drugs, and other forms of insult. If DAD occurs without any underlying condition or known cause, it is called acute interstitial pneumonia or Hamman-Rich Syndrome.

Treatment

Hamman-Rich Syndrome has no proven and effective therapy. The primary goal of the treatment is to fend off a complete respiratory failure and at the same time, keep the patients alive and relaxed until the disease resolves. Treatments for patients are usually supportive care and mechanical ventilation.

In mechanical ventilation, there has been a lung-protective method that is publicly recommended by physicians based on its recognized benefits in ARDS. A lot of individuals are treated with high-dose corticosteroids that are taken intravenously although the efficacy of these medications is not established. A proven-based therapy of Hamman-Rich Syndrome is really difficult due to the rarity of the diagnosis and because the reported cases of the condition have small, detailed case series.

Prognosis

The prognosis of Hamman-Rich Syndrome patients is poor. Mortality is less than 60% and most patients die in no more than 6 months of presentation. Death is typically due to its complications or severe respiratory failure even with the help of high-dose corticosteroid therapy and mechanical ventilation. About half of the patients do not survive within two months in general.

However, there are some survivors that had been reported in most series. A few well-documented cases of patients with the disease survive the initial hospitalization; but afterwards they die of congestive heart failure, recurrence of the condition, or pneumonia within a few months after being discharged. There is still a possibility of a long-term survival from Hamman-Rich Syndrome, with documented survival up to two to four years after its diagnosis.

References:

1. Acute Interstitial Pneumonia (Accelerated Interstitial Pneumonia; Hamman-Rich Syndrome) at http://www.msdmanuals.com/professional/pulmonary-disorders/interstitial-lung-diseases/acute-interstitial-pneumonia
2. http://www.medscape.com/viewarticle/772355_1
3. http://www.empowher.com/acute-respiratory-distress-syndrome/content/hamman-rich-syndrome-facts-symptoms-and-treatment?page=0,0
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5. Borie R, Quesnel C, Phin S, Debray MP, Marchal-Somme J, Tiev K, et al (2013). Detection of alveolar fibrocytes in idiopathic pulmonary fibrosis and systemic sclerosis. PLoS One. 8(1):e53736.
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