Rationale In the natural course of cystic fibrosis (CF), pulmonary exacerbations treated effectively with antibiotics are common. Rapidly progressive bronchiectasis and severe malnutrition despite aggressive antibiotics and nutritional support are atypical manifestations of CF that require investigation of other diseases as comorbid conditions or as more likely etiologies for these symptoms.
Methods A Caucasian female was diagnosed with CF in infancy by mutational analysis and elevated sweat chloride, evaluated due to neonatal meconium ileus. At age 7 years, immunoglobulin levels were obtained because of poor response to aggressive treatment of her CF. Nearly absent levels of IgA (< 10 mg/dL) and IgG (< 50 mg/dL) were found, along with a markedly elevated IgM level of 1,259 mg/dL. T- and B-cell profile was within normal limits.
Results The patient was diagnosed with common variable immunodeficiency (CVID) in addition to CF. Rapid initiation of IVIG therapy showed dramatic improvement in overall health and pulmonary function.
Conclusion Although not frequently associated, CVID and CF, when coexisting in the same patient, may cause rapidly progressive lung disease. Any young CF patient with abnormally widespread bronchiectasis and significant restrictive lung disease or recalcitrant infections should be evaluated for CVID. If these diseases are found to coexist, immediate initiation of IVIG therapy in addition to routine CF care may improve the overall health of the patient.
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