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What is Hamman-Rich Syndrome
Hamman-Rich Syndrome is also known as acute interstitial pneumonia. It is an uncommon type of pneumonia for which researchers have not yet found a cause (idiopathic). It affects otherwise healthy individuals. The underlying condition may worsen without symptoms appearing for a long period of time, but when the symptoms do manifest they come on very suddenly and "acutely".
The condition received its name from the doctors who discovered the disease in 1939, Drs. Hamman and Rich.
Acute interstitial pneumonia has similar symptoms to acute respiratory distress syndrome, and may be misdiagnosed as such.
Pneumonia results when tissues of the lungs become inflamed in reaction to infection. Interstitial pneumonia, by comparison, is a long-term condition that affects the connective tissue of the lungs. The inflammation is caused by the build up of white blood cells and plasma in the alveoli (the tiny sac that facilitate the carbon dioxide/oxygen exchange in the bloodstream). With interstitial pneumonia, the inflammation also extends into the bronchioles - small airways that branch off into the lungs.
If the inflammation lasts long enough, the fluid hardens into scar tissue (fibrosis). If there is enough scar tissue, over time alveoli will be destroyed and the space filled with cysts. Over time, the bronchi and the walls of the bronchi widen, or are destroyed resulting in the lungs shrinking.
Sixty percent of those with acute interstitial pneumonia die within six months of the appearance of symptoms.
Symptoms of Hamman-Rich Syndrome
As stated earlier, symptoms of Hamman-Rich Syndrome or acute interstitial pneumonia are similar to those observed in acute respiratory distress syndrome, which are:
- shortness of breath
- rapid, shallow breathing
- crackling or wheezing sounds in the lungs
- cyanosis (blue tinge) to the skin
- heart and brain function issues (rapid heart rate, difficulty concentrating, forgetfulness) because of long-term reduced oxygen supply
Diagnosis and Treatment of Acute Interstitial Pneumonia
Diagnosis of acute interstitial pneumonia is usually confirmed through CT scans, lung biopsy, and tests that measure pulmonary function.
The goal of treatment is to prevent complete respiratory failure and keep the patient alive and comfortable until the condition resolves. Treatment often involves use of a ventilator in the event of respiratory failure, and administration of corticosteroids (although it is not known how effective these medications are).
For those who survive, lung function will improve over time, but the condition may return.
Sources: www.uptodate.com; www.wrongdiagnosis.com; www.medfriendly.com; www.merck.com
Add a Comment72 Comments
My father in law 71 was diagnosed with hammon Rich syndrome Friday April 12th following two weeks of typical treatments for pneumonia. And 5 way heart bypass for blockages that were suspected to be contributing to his lung condition. He under went lung biopsy which showed no infection or fungus. Dr's mentioned a condition call vascularitis which is inflammation of small blood vessels. Now 5 days later following aggressive treatment with high dose steroids they administered a moderately low dose of cyclophosphamide which is a cancer drug that is used to suppress the immune system. This treatment approach is last resort. The Dr stated that if he dosent respond with improved O2 sturation numbers in 48 hours on the first dose dose he likely is terminal and will be treated for comfort. Today is the 24 hour mark and will be heading to hospital soon to spend day with family there boosting his fighting spirit which is critically important to win against this nasty disease.
My father in law does have moderately low diabetes and arthritis. Otherwise relatively health.
Wisconsinite
April 14, 2013 - 5:33amThis Comment
Thank you for sharing your story. Please keep us posted!
April 14, 2013 - 4:21pmThis Comment
Hello All,
April 1, 2013 - 8:51ammy Mom has been diagnostic with AIP this weekend, I'm really scare cause i don't have as much information about the illness; she is 45 years old i think she still strong and can fight against it.
unfortunately in my city (Juarez, México) there is no treatment or specialist on that disease.
next week we need to move to Mexico City to find any doctor who can help us with, but time is running and AIP wont stop.
Hope we find help there!
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It is a scary condition.
Thank you for sharing your story, Dee. I'm constantly amazed how many people have responded to this article. It was a tough article to research because there really isn't that much information out there.
Please keep us posted on your mom's progress.
April 2, 2013 - 1:55pmThis Comment
My father n law was diagnosed with this on Friday night. IT has really been scary, we had to watch him go on a ventilator, be put to sleep, go through a surgical biopsy, which they doctors really didn't want to do because they were not even sure if he would survive the surgery. But he did really well, they were able to get good samples. He is still on the ventilator and he is 67 years old. I'm really scared and not sure what to think. They said we will no soon if the steriods, and other things they are giving him are going to work. This wait and see is driving me crazy, I wish they could just let him be awake, but his oxygen is just to low, on the vent it stays up in the 90's, but when we admitted him into the hospital is oxygen was in the 60's. And he was on home oxygen. It just wasn't working. I will try to remember to post back later to let you all know how he is doing,
January 26, 2013 - 6:36pmThis Comment
He died on February 8 at 2:55am
February 20, 2013 - 2:35pmcause of death on the certificate was Acute Respiratory Failure
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So, sorry for your loss.
Thank you for letting us know.
February 20, 2013 - 2:59pmThis Comment
He is still with us today, his right lung collapsed this morning, and we got that horrible call this morning. He spiked a fever this weekend, and his white blood cell count got up to 31,000
February 5, 2013 - 5:51pmHis BUN is up to 237 today, and Platlet count is 46,00 they believe he is bleeding some where, his stool is black, and his oxygen ventilator PEEP is at 20% which is the reason his lungs collapsed (among many other reasons) His blood oxygen level this morning was at 102, and that is with the ventilator being at 95%, I know all these numbers are crazy, but it seems like that is what are lives have become just asking about the next days numbers.
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as of today they dont expect him to make it, his stats are dropping
January 29, 2013 - 5:14pmThis Comment
Thank you for letting us know. So sorry.
It really is a terrible illness.
January 29, 2013 - 5:16pmThis Comment