“Currently, intravenous augmentation therapy is the only specific therapy available to treat AATD,” says Dr.
It’s also important to remember that augmentation therapy is only recommended for individuals with severe deficiency of AAT who have emphysema and fixed airflow obstruction. When there is no fixed airflow obstruction on PFTs or emphysema on imaging, AATD should not cause symptoms and an alternate diagnosis should be considered. However, it is important to look at a patient’s symptoms holistically when considering an AATD diagnosis. But that diagnosis of AATD also blinded clinicians to the underlying pathology of asthma, which was the actual reason for the patient’s symptoms.” TakeawaysĪATD is under-recognized, and patients with AATD commonly experience lengthy times between the initial symptoms and first actual diagnosis. “The diagnosis of AATD helped frame the case and explained the patient’s physiology. “This was a fascinating and instructive case,” says James Stoller, MD, MS, Chair of the Education Institute at Cleveland Clinic and corresponding author of the study. The patient’s post-bronchodilator forced expiratory volume (FEV 1) and forced vital capacity (FVC) values also improved from 65% and 74% of predicted to 77% and 84% of predicted, respectively. He reported resolution of symptoms, and his Asthma Control Test score improved from 19 to 23. The patient was also advised to abstain from drinking alcohol.įollowing a year of therapy, the patient showed marked improvement.
The patient’s family history (allergies, eosinophilia and elevated IgE level) was consistent with moderately persistent asthma, which was the revised diagnosis.įuture plans for augmentation therapy were stopped, and benralizumab was added to his usual regimen for eosinophilic asthma. Since the patient had no indication of emphysema or fixed airway construction, the care team pursued an alternative diagnosis to AATD to account for the patient’s dyspnea and wheezing. Eosinophilia was detected with 630 eosinophils/mL (12.5%), and the patient’s serum IgE level was elevated at 267 kU/L (upper limit of normal = 114 kU/L). However, a chest CT showed mild diffuse peribronchial thickening, scattered tree-in-bud opacities and mucoid impaction with mild air trapping on expiratory images. Pulmonary function testing (PFT) showed some pseudo-restriction, air trapping, a normal diffusion capacity and no obstruction.
VESICULAR BREATH SOUNDS YOUTUBE SKIN
The patient’s skin was also clear of lesions suggestive of panniculitis or vasculitis. Physical examination of the patient showed normal vesicular breath sounds bilaterally. We do not endorse non-Cleveland Clinic products or services Policy Advertising on our site helps support our mission. Cleveland Clinic is a non-profit academic medical center.
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