A 20-year-old man with known cystic fibrosis is evaluated for productive cough, dyspnea, and malaise that have progressively worsened over the last 3 months. He describes daily brownish sputum production and episodes of wheezing/chest tightness partially relieved by albuterol inhaler. One month ago, he was hospitalized and treated with intravenous antibiotics. He received saline nebulizer, azithromycin, inhaled tobramycin, and chest physiotherapy. The patient has no other medical problems. His medications include dornase alfa, pancrelipase, hypertonic saline therapy, and a short-acting albuterol inhaler as needed.
His temperature is 36.6° C (98° F) and BMI is 20 kg/m². Examination shows pale conjunctivae. There are bilateral scattered rhonchi and wheezes with prolonged expiration on auscultation.
Laboratory results are as follows:

Chest x-ray shows new bilateral patchy upper-lobe infiltrates compared to prior x-rays.
Which of the following is the most likely diagnosis?

Question

A 20-year-old man with known cystic fibrosis is evaluated for productive cough, dyspnea, and malaise that have progressively worsened over the last 3 months. He describes daily brownish sputum production and episodes of wheezing/chest tightness partially relieved by albuterol inhaler. One month ago, he was hospitalized and treated with intravenous antibiotics. He received saline nebulizer, azithromycin, inhaled tobramycin, and chest physiotherapy. The patient has no other medical problems. His medications include dornase alfa, pancrelipase, hypertonic saline therapy, and a short-acting albuterol inhaler as needed.
His temperature is 36.6° C (98° F) and BMI is 20 kg/m². Examination shows pale conjunctivae. There are bilateral scattered rhonchi and wheezes with prolonged expiration on auscultation.
Laboratory results are as follows:

Chest x-ray shows new bilateral patchy upper-lobe infiltrates compared to prior x-rays.
Which of the following is the most likely diagnosis?

Quizplus · Accepted Answer

11ec193a_98de_369b_853d_8199812a17f1_MD0002_00 Allergic bronchopulmonary aspergillosis (ABPA) is an immunologic pulmonary disorder caused by hypersensitivity to Aspergillus fumigatus. It often occurs in patients with asthma or cystic fibrosis. Inhalation of fungal spores activates an inflammatory response (secretion of IgE and IgG antibodies), leading to mucoid impaction of bronchi, eosinophilic pneumonia, and bronchocentric granulomatosis. Patients typically present with recurrent episodes of low-grade fever, wheezing, bronchial hyperreactivity, hemoptysis, and productive cough with brownish mucous plugs. Repeated episodes often lead to bronchiectasis and fibrosis. Early diagnosis is important as the onset of bronchiectasis is associated with poor outcomes. A wide spectrum of radiographic changes can be seen in patients with ABPA, including fleeting pneumonias, central bronchiectasis, and mucus-filled bronchi. Once clinical and radiographic findings lead to suspicion of ABPA, the skin prick test is the next step. A negative test with negative intradermal reactivity to Aspergillus helps rule out the diagnosis. Positive results warrant further evaluation via immunochemistry. A diagnosis is made if serum total IgE > 417 IU/mL (> 1000 ng/mL) and serum antibodies against Aspergillus are positive. Once the diagnosis of ABPA is confirmed, corticosteroids and itraconazole are used to control inflammation and prevent irreversible damage. (Choice B) Atypical mycobacterial infection may present with similar symptoms, but these patients tend not to have eosinophilia. (Choice C) Churg-Strauss syndrome is associated with asthma and presents with eosinophilia, mononeuropathy/polyneuropathy, transient pulmonary opacities, paranasal sinus abnormality, and vasculitis. (Choice D) Eosinophilic pneumonia can be caused by medications, including antimicrobials (eg, sulfonamides, ampicillin, azithromycin) and anticonvulsants (eg, phenytoin). Although azithromycin has been associated with drug-induced eosinophilic pneumonia, this patient's symptoms had progressively worsened before he took the drug. (Choice E) Pulmonary findings in sarcoidosis typically include bilateral hilar adenopathy and/or pulmonary reticular opacities with other systemic findings (eg, lesions in the skin/joint/eyes, arthritis). Educational objective: Allergic bronchopulmonary aspergillosis is an immunologic pulmonary disorder caused by hypersensitivity to Aspergillus fumigatus. It often occurs in patients with asthma or cystic fibrosis. Patients present with recurrent episodes of low-grade fever, wheezing, bronchial hyperreactivity, productive cough, and hemoptysis. Diagnosis is made with a positive skin prick test followed by findings of elevated IgE and positive Aspergillus antibodies. __________ References: Allergic bronchopulmonary aspergillosis. (http://www.ncbi.nlm.nih.gov/pubmed/19265090) Allergic bronchopulmonary aspergillosis in cystic fibrosis--state of the art: Cystic Fibrosis Foundation Consensus Conference. (http://www.ncbi.nlm.nih.gov/pubmed/12975753)

A 20-Year-Old Man with Known Cystic Fibrosis Is Evaluated for Productive