RESOLUTION OF ALLERGIC BRONCHOPULMONARY ASPERGILLOSIS AFTER DIAGNOSIS OF COMMON VARIABLE IMMUNODEFICIENCY

Abstract

Introduction Allergic Bronchopulmonary Aspergillosis (ABPA) is characterized by asthma, elevated IgE and a hyperactive immune response to Aspergillus colonization. Low serum IgE is a sensitive and specific marker for Common Variable Immunodeficiency (CVID), but an atopic endotype has been reported. Case Description A 60-year old man had a 30 year history of severe persistent asthma and ABPA-CB, Stage IV with stereotypical annual exacerbations coinciding with high mold counts. He had a positive skin test to Aspergillus fumigatus, elevated serum IgE, precipitating antibodies to A fumigatus, infiltrates on chest radiograph, and bronchiectasis on CT. After 3 decades of stable disease, he began experiencing atypical exacerbations no longer correlating to seasonal mold exposures but instead to severe respiratory infections requiring prolonged courses of antibiotics in addition to prednisone. During these episodes, serum IgE did not rise and he had low levels of IgG and IgA, as well as a poor response to immunizations. The patient was diagnosed with CVID and started on immunoglobulin replacement with resolution of his recurrent respiratory infections. Repeat testing of specific IgE and precipitating antibodies to A fumigatus were negative. Discussion This is the first reported case of a patient developing CVID followed by a loss of clinical and laboratory evidence of ABPA. If exacerbations of asthma in an ABPA patient begin to occur in the absence of elevated mold counts, alternative diagnoses should be considered. Allergic Bronchopulmonary Aspergillosis (ABPA) is characterized by asthma, elevated IgE and a hyperactive immune response to Aspergillus colonization. Low serum IgE is a sensitive and specific marker for Common Variable Immunodeficiency (CVID), but an atopic endotype has been reported. A 60-year old man had a 30 year history of severe persistent asthma and ABPA-CB, Stage IV with stereotypical annual exacerbations coinciding with high mold counts. He had a positive skin test to Aspergillus fumigatus, elevated serum IgE, precipitating antibodies to A fumigatus, infiltrates on chest radiograph, and bronchiectasis on CT. After 3 decades of stable disease, he began experiencing atypical exacerbations no longer correlating to seasonal mold exposures but instead to severe respiratory infections requiring prolonged courses of antibiotics in addition to prednisone. During these episodes, serum IgE did not rise and he had low levels of IgG and IgA, as well as a poor response to immunizations. The patient was diagnosed with CVID and started on immunoglobulin replacement with resolution of his recurrent respiratory infections. Repeat testing of specific IgE and precipitating antibodies to A fumigatus were negative. This is the first reported case of a patient developing CVID followed by a loss of clinical and laboratory evidence of ABPA. If exacerbations of asthma in an ABPA patient begin to occur in the absence of elevated mold counts, alternative diagnoses should be considered.