title: Disseminated bronchiectasis in an adult with common variable immunodeficiency.
abstract: Primary immunodeficiencies (PID) are traditionally considered childhood diseases; however, adults account for 35% of all patients with PID. Antibody deficiencies, especially Common Variable Immunodeficiency (CVID), which have their peak incidence in adulthood, require a high suspicion index. Even though the estimated frequency of CVID is not high (1:25,000), high rates of under diagnosis and under reporting are very likely. The delay in diagnosis increases the morbidity and mortality; therefore, adult physicians should be able to suspect, identify and initiate management of individuals with PID. Here we report the case of a 37 year-old man presenting to the emergency room with dyspnea, fever and cough; he developed respiratory failure requiring mechanical ventilation. He complained of recurring pneumonia associated with widespread bronchiectasis since he was 18 years old. Serum immunoglobulins quantification showed severe hypogammaglobulinemia (total IgG <140 mg/dL; total IgA, 2.9 mg/dL; and total IgM <5 mg/dL). Treatment with Human Intravenous Immunoglobulin (IVIG) 10% was started, and with antibiotic treatment for severe pneumonia (during 14 days) was also prescribed. His clinical evolution has been favorable after one year follow-up. Common Variable Immunodeficiency (CVID) diagnosis was made.
authors.affiliation: Department of Internal Medicine, Faculty of Health, Universidad del Valle. Cali, Colombia ; Research Group VIREM, School of Basic Sciences, Faculty of Health, Universidad del Valle. Cali, Colombia.
authors.forename: Andrés Felipe
authors.initials: AF
authors.lastname: Zea-Vera
authors.affiliation: Research Group VIREM, School of Basic Sciences, Faculty of Health, Universidad del Valle. Cali, Colombia.
authors.forename: Olga Lucia
authors.initials: OL
authors.lastname: Agudelo-Rojas
mesh_terms: D000328:Adult; D000361:Agammaglobulinemia / Q000175:diagnosis / Q000209:etiology*; D001987:Bronchiectasis / Q000175:diagnosis* / Q000188:drug therapy; D017074:Common Variable Immunodeficiency / Q000175:diagnosis* / Q000188:drug therapy; D003371:Cough / Q000209:etiology; D004417:Dyspnea / Q000209:etiology; D005334:Fever / Q000209:etiology; D005500:Follow-Up Studies; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage*; D008297:Male; D011014:Pneumonia / Q000188:drug therapy / Q000209:etiology; D012008:Recurrence
chemical_list: D016756:Immunoglobulins, Intravenous
keywords: Primary immunodeficiency; bronchiectasis; common variable immunodeficiency; hypogammaglobulinemia; recurring pneumonia