Diagnosing AERD (Samter's Triad)
How is AERD Diagnosed?
The symptoms of Aspirin Exacerbated Respiratory Disease (AERD, Samter's Triad) usually develop over several years. Because of this, many patients are not diagnosed until the disease has become unmanageable. Primary care doctors are likely to treat the symptoms of asthma and nasal congestion separately, without recognizing the underlying cause. It is estimated that up to 20% of patients with AERD go undiagnosed.
A doctor familiar with the disease can often make a diagnosis based on the patient's medical history alone. Sometimes, an aspirin challenge is performed to confirm the diagnosis. If aspirin or NSAID sensitivity is obvious from the patient’s history, performing an aspirin challenge is not necessary. When aspirin sensitivity is suspected but not certain, an aspirin challenge may confirm the diagnosis.
Researchers continue to search for a less invasive means of diagnosing AERD. Research has found that there are differences in the nasal fluids of patients with AERD and those with other types of sinusitis. Another study found that AERD patients may have higher concentrations of urinary leukotriene E4. Further research is needed to determine if AERD can be successfully diagnosed using such methods. In the meantime, an aspirin or NSAID challenge remains the standard means of diagnosis.
If an aspirin challenge is necessary, this must only be done under the care of an allergy or respiratory specialist and in a controlled medical setting with medications, equipment, and support staff available to assist. Reactions can be significant, so don't try this at home! If you need help finding a doctor, check our map!
Aspirin challenge protocols involve cautiously administering gradually increasing doses of a aspirin or another NSAID in an appropriately monitored medical setting. Outside of the United States, this test may be performed using lysine-aspirin, a liquid form of aspirin that can be administered either through inhalation or intranasally. In the United States, where lysine-aspirin is not approved for use, an oral aspirin challege is most often performed. In recent years, challenge with intranasal ketorolac has also become an accepted means of diagnosis.
For patients who have a history of very severe reactions, the aspirin or NSAID challenge may need to be performed in an hospital.
Aspirin challenge is not recommended in patients with any of the following:
Recent respiratory tract infection
Severe underlying cardiovascular, renal, or liver disease
When is an Aspirin Challenge Necessary?
Many patients can be diagnosed by history alone. In patients with asthma and nasal polyps, the following situations may indicate the need for an aspirin challenge:
The patient has not recently taken aspirin or NSAIDs. NSAID reactions develop spontaneously during the progression of the disease. If NSAIDs have not been used recently, the patient may be unaware that a sensitivity has developed.
The patient has taken aspirin or NSAIDs, but they were also using a leukotriene modifying drug such as Singulair or Zyflo. These medications can block reactions.
A patient has such severe sinus and asthma symptoms at baseline that they may be unaware of exacerbations caused by NSAID ingestion.
The patient is taking low dose aspirin. These patients can still have AERD. Patients taking low dose aspirin have effectively desensitized themselves to aspirin, but 81mg per day is not enough to treat the symptoms of the disease.
Research has found that 42% of patients with asthma, nasal polyps, and chronic sinusitis tested positive on an aspirin challenge even though they had no prior history of reactions to NSAID medications.
Diagnosing AERD Can Be Difficult
Even with the availability of these tools, making a diagnosis is not always easy. Many patients are not aware of their sensitivity to aspirin and NSAIDs. 15% of those who tested positive for sensitivity during an aspirin challenge had no prior history of reactions. Doctors may not consider AERD as a potential diagnosis in children, but research has found that the disease does rarely occur in children. This article, published by experts at the Brigham & Women's AERD Center, discusses 3 cases of pediatric patients who developed the disease.
Doctors may rule out AERD because a patient appears to tolerate aspirin or other NSAIDs. Research has shown, however, that many patients are able to tolerate some use of aspirin or NSAIDs. Many patients take a daily low dose aspirin for heart health - these patients may still have AERD. They may have had a minor reaction when beginning aspirin therapy and attributed it to other causes. 81mg of aspirin a day is enough to maintain desensitization, but not enough to alleviate the symptoms of the disease.
To further complicate matters, even those who test negative on an aspirin challenge may have AERD. A minority of patients, even some with a history of reactions to aspirin and NSAIDs, can have a "silent desensitization" or test negative on an aspirin challenge. Research has found that sinus surgery can reduce the severity of NSAID reactions. Having a diagnostic aspirin challenge soon after surgery may increase the chance of having a false negative test.
Doctors may also rule out AERD because a patient does not have asthma. Asthma in AERD can range from nonexistent to very severe and difficult to treat. Research has shown that even patients without asthma may benefit from aspirin desensitization.
I Think I Have AERD, But My Doctor Hasn't Diagnosed Me
Unfortunately, it is very common for patients to live years with this disease before getting diagnosed. As discussed above, the disease can develop insidiously and many doctors do not proactively screen patients who show symptoms. If you have asthma and nasal polyps and respiratory reactions to NSAIDs, you do have this disease. Here are some "diagnosis horror stories" from real patients.
"I have suffered for 18 years and it wasn’t until I researched this myself that I was actually diagnosed properly by my doctor." -Becky M.
"It took my allergist 20 freaking years to diagnose me with Samter's Triad." -Paul C.
"If my daughter, who is an ER doctor, had not diagnosed me last year after 3 years of seeing every kind of doctor, I would still be searching for an answer." –Carol O.
"I was just diagnosed a week ago and I lived for almost 10 years feeling like doctors thought I was a total loon." -Meghan K.
"I can’t even count the number of doctors, ENTs, allergists, or pulmonologists I saw in the nearly 40 years I went undiagnosed." -Jamie P.
"Took four years to diagnose me! It was brutal." - Tara W.
“My doctors only treated my asthma, never any of the other symptoms. It took 10 years and probably at least 9 different doctors until one listened to me for 5 minutes and said, 'It's simple, you have Samter’s Triad'." –Chrisi G.
If these stories sound familiar to you, know that you are not alone! If you believe that you have this disease, the best thing you can do to help yourself is to find a doctor who understands the disease. Getting diagnosed is the first step to feeling better!
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