Desensitization Therapy for Aspirin Exacerbated Respiratory Disease (AERD)

Specialists at the Penn Aspirin Exacerbated Respiratory Disease (AERD) Center are performing aspirin desensitization therapy for patients with aspirin exacerbated respiratory disease.

According to the 2012 Pennsylvania Asthma Burden Report, the estimated annual prevalence of asthma among adults in Pennsylvania increased by approximately 35% between 2001 and 2010. During this time, inpatient hospitalization rates for adults with asthma as the primary discharge diagnosis were more than twice as high in Philadelphia County than for the state as a whole. In addition, chronic rhinosinusitis, a precursor to both AERD and nasal polyposis, affects ~10% of adults 18-44 in the Philadelphia region.

Given the increasing prevalence of asthma and sinusitis in the region, Penn Otorhinolaryngology – Head and Neck Surgery has introduced the first multidisciplinary AERD Center — a program designed to treat aspirin exacerbated respiratory disease, a difficult to treat variant of the disease defined by a complex course and intractable symptoms.

AERD may affect as many as 10% of all asthmatics and up to 40% of asthmatics with nasal polyps. The Penn AERD Center is led by John V. Bosso, MD, a specialist recognized for founding the first Regional AERD program in the New York metropolitan area and one of the first physicians in the country to be trained in the technique which was conceived at Scripps Clinic in the 1980s.

AERD is characterized by a well-established quartet of symptoms consisting of nasal polyps, chronic eosinophilic sinusitis, asthma and a hypersensitivity to aspirin and non steroidal anti-inflammatory drugs (cyclo-oxygenase 1 inhibitors). The disease appears frequently in patients in the third or fourth decade of life, and often in patients without a prior history of asthma or allergy.

The immunologic basis for AERD is not completely understood. Patients with AERD typically display a robust dysfunctional overproduction of inflammatory cysteinyl leukotrienes and tissue eosinophils as well as increased expression of IL4, IL5 and interferon gamma mRNA, all of which may contribute to the inflammatory characteristics of the disease. Diagnosis is based upon the combination of patient history with the distinct symptomatology of the disease and a positive response to aspirin challenge. Medical treatment is greatly enhanced by aspirin desensitization and long-term therapy with aspirin, which has the effect of attenuating the upper and lower respiratory symptoms identified with AERD.

CASE STUDY

At age 35, Mr. K was referred to the Rhinology Division of the Department of Otorhinolaryngology – Head and Neck Surgery at Penn Medicine for a second opinion pending a recommendation for revision sinus surgery from an outside specialist.

In the years prior to his visit, Mr. K experienced an evolving set of symptoms and therapeutic interventions, all initiated in his late 20s by an intractable upper respiratory infection, followed within a year's time by the abrupt onset of asthma. Two years later, Mr. K was found to have diffuse and widespread polyps throughout his sinuses (see Fig. 1), for which he had surgery. His recovery from surgery was complicated by the complete return of the inciting symptoms within six months and an idiopathic and de novo allergy to NSAIDS resulting in a brief hospitalization for acute respiratory distress.

Mr. K's medications at presentation included prednisone 10 mg daily, amoxicillin-clavulanate 875/125 mg twice daily, fluticasone 250/50 (1 puff 2x day), and montelukast 10 mg daily for respiratory symptoms as well as escitalopram 10 mg daily (a selective serotonin reuptake inhibitor) for depression. An endoscopic examination and a series of imaging studies thereafter found diffuse pansinusitis and polyp growth throughout his sinuses with complete opacification. These studies, and a review of his recent history, prompted a referral to the Penn AERD Center, where his diagnosis of aspirin exacerbated respiratory disease was soon confirmed. Mr. K was scheduled for revision sinus surgery to remove his polyp burden, followed a month later by the initiation of aspirin desensitization therapy at the Center.

At the initiation of therapy, Mr. K received a nasal NSAID spray at a low dose with a gradual progression to higher doses, then a switch to oral aspirin at low, then medium, then full doses. During this time, he was closely monitored and his peak expiratory flow/ FEV1 measured to evaluate lung function. At 60 mg of aspirin, Mr. K had a reaction that mimicked a mild asthma attack, and received the appropriate medications to stabilize his condition. Ninety minutes later, he received another.

60 mg dose, but had no reaction. Over the course of the two days of therapy, Mr. K experienced a mild nasal reaction at 150 mg, which was again treated until stabilization. The same procedure, wherein he received the dose to which he reacted, was then repeated until he reached 325 mg, at which point his desensitization was complete.

Six months after desensitization, Mr. K continues to take 325 mg aspirin twice daily. He has not experienced a recurrence of his polyps or respiratory symptoms, and has substantially reduced his dependence on steroids, antibiotics and antidepressant medications.

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Perelman Center for Advanced Medicine
3400 Civic Center Boulevard
South Pavilion, 3rd floor
Philadelphia, PA 19104
215-662-2777

Published on: September 26, 2016

Penn Faculty Team