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Hamman-Rich Syndrome: Facts, Symptoms, and Treatment

By HERWriter
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Acute Interstitial Pneumonia (Hamman-Rich Syndrome): Facts, Symptoms, and Treatm Olesya Shelomova/fotolia

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

EmpowHER Guest

My mother was diagnosed with this a month ago. She is on an at home oxygen tank, and also as rheumotoid arthritis, and fibromaylgia. She read some articles and it says the death rate is over 60 percent. Does she stand a chance?

November 14, 2012 - 12:09pm
HERWriter (reply to Anonymous)

Unfortunately, that high percentage death rate is what my research turned up as well. If you want to look at it in a positive light, the survival rate is about 40%.

It's impossible to say what the prognosis is for your mother, and it would be irresponsible and unethical for me to offer such an opinion, as I'm not a doctor, but with all the unknowns regarding this disease, she has just as much chance as anyone of beating this.

The only thing you can do is wait and see, and enjoy every day you have with your mother (we should do that no matter what our mothers' health and/or prognosis).

November 15, 2012 - 12:58pm
EmpowHER Guest

My wife who is 52 was diagnosed with AIP . Should I press the Dr. that i want her to have a lung transplant if possible ?

October 24, 2012 - 10:29pm
HERWriter (reply to Anonymous)

I'm not a doctor, but from my research, a lung transplant isn't a treatment option for AIP. There are a whole slew of potential complications associated with waiting for a lung transplant and a transplant itself.

As my article says, the idea behind treatment of this condition is to help the patient breathe while the condition resolves and the lungs eventually (hopefully) clear.

I know this condition is very scary for loved ones and I can sense that you're worried about her and just want her to be well. Now that they've got a diagnosis, hopefully the doctors will get aggressive with treatment and she will respond positively. Unfortunately, this is one of those extremely frustrating and stressful "wait and see" kind of conditions.

Please let us know what happens.

October 25, 2012 - 6:19am

Everything I know about this disease is contained in the article. Ideally, your doctor should tell you what could or should happen next.

Because I have not experienced it myself, nor has a member of my family, I can't really tell you what happens next from that perspective. Those who have commented on the article who have been through it might be able to answer the "what's next" question.

Perhaps reading some of their comments will be helpful to you.

July 11, 2012 - 1:16pm
EmpowHER Guest

I was diagnose with aip last Thursday. I do not have shirtness in breath but did about 4 weeks ago. I'm so scared. I have 2 kids and need to know what to expect.. Please someone tell me what should be happening....

July 7, 2012 - 5:35pm
EmpowHER Guest

A very dear long time friend is fighting for her life with this and we are all very shocked to see her hooked up to all these machines and that we have never heard of this before and how radip this syndrom occurs, it is very scary and we pray for her every day. I agree much more reaserch is needed in this area for sure.

May 11, 2012 - 5:49am
HERWriter (reply to Anonymous)

Sorry to hear about your friend, Anonymous.


Please keep us posted as to her progress.

May 11, 2012 - 10:52am
EmpowHER Guest
Anonymous (reply to Anonymous)

Was your friend taking Bactrim (ANTI-BIOTIC) right before this happened? That was the case for me and a guy that lived near me that had this happen to us. We were both diagnosed with hammen-rich syndrome.

May 11, 2012 - 7:00am
HERWriter (reply to Anonymous)

That's an interesting question, though I must warn against jumping to the conclusion that taking the antibiotic was a cause or contributing factor. Bactrim is often used as a treatment for pneumonia, as well as other infections such as ear infections and urinary tract infections. Here's some more information from WebMD: Bactrim.

As my article states, Hamman-Rich Syndrome is often mistakenly diagnosed as pneumonia and treated as such. It's only when the symptoms becomes more serious (acute) that doctors realize that it is something else and requires different treatment.

So, at the outset, I would venture to say that there is no causal or catalytic association between the use of Bactrim, Septra or other similar antibiotics and the onset of Hamman-Rich Syndrome.

One of the rare side effects associated with this drug, however, is lung injury; whether this is what happened in your case (or your friend's case) is really impossible to say and, as I am not a medical professional, it would be irresponsible and unethical for me to offer anything other than an opinion. Part of the frustration of this disease is that no one knows how or why it starts and in apparently otherwise healthy individuals with no immediately prior history of lung issues. It certainly can't help to discuss this with your family doctor though and the medical staff that treated you, then they can rule out for certain whether or not Bactrim played any role in your particular case.

May 11, 2012 - 11:09am
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We value and respect our HERWriters' experiences, but everyone is different. Many of our writers are speaking from personal experience, and what's worked for them may not work for you. Their articles are not a substitute for medical advice, although we hope you can gain knowledge from their insight.

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