SESSION TYPE: Infectious Disease Cases III
PRESENTED ON: Wednesday, October 24, 2012 at 11:15 AM - 12:30 PM
INTRODUCTION: Non-cystic fibrosis bronchiectasis is uncommon, and the cause of bronchiectasis is rarely found in up to 80% of cases. We describe a case of persistent pulmonary abscess, pneumonia and bronchiectasis in a young man found to have common variable immunodeficiency disease (CVID).
CASE PRESENTATION: The patient is a 36 year old man who nine months prior, began experiencing fevers, malaise, shortness of breath and productive cough. He was treated several times for pneumonia with oral antibiotics without improvement. The patient continued to have fevers, and his sputum became foul smelling with occasional hemoptysis. Right lower lobe pneumonia with findings of a cavitary lesion and bilateral lower lobe bronchiectasis were found on chest computed tomography (CT). Sputum culture grew penicillin resistant streptococcus pneumoniae, and he was initiated on moxifloxacin. He had slow improvement of his chest CT findings on moxifloxacin and continued to experience malaise and productive, purulent sputum. Repeat sputum sample demonstrated a highly resistant streptococcus pneumoniae, including fluoroquinolones. On exam he appeared chronically ill and was afebrile. He had poor dentition. His lungs demonstrated bronchovesicular breath sounds in the right mid-lung. The remainder of the exam was normal. Laboratory evaluation demonstrated very low immunoglobulin levels (IgG <200 mg/dL, IgA<6 mg/dL, IgM 135 mg/dL) with no antibody response to tetanus, diphtheria, or pneumococcus. Alpha one antitrypsin levels, complement activity, rheumatoid factor and anti-neutrophilic antibody tests were all normal. Pulmonary function tests showed a fixed moderate obstructive pattern with a forced expiratory volume (FEV1) of 2.33 liters (61% of predicted), a total lung capacity of 5.96 liters (98% of predicted) and corrected diffusing capacity of 17.4 mL/min/mmHg (54% of predicted). The patient was initiated on intravenous immunoglobulin therapy (IVIG) and been slowly improving.
DISCUSSION: Common variable immunodeficiency disease is a rare, heterogeneous genetic disorder with high morbidity and mortality. Recurrent infections, pulmonary disease, and lymphoma are common complications. Bronchiectasis is highly prevalent in this population. Studies have suggested that a significant delay in diagnosis (four years) from symptom onset to initiation of IVIG. This may contribute to increased mortality. Evidence-based treatments of bronchiectasis are lacking and largely based on expert opinion.
CONCLUSIONS: The evaluation of bronchiectasis presents an opportunity to diagnose the underlying condition with hopes of preserving pulmonary function and decreasing mortality. CVID frequently presents as bronchiectasis and recurrent infections. Although IVIG is the standard treatment, it is unclear whether this affects the outcome underlying lung diseases.
1) Detkova D, et.al. CVID: Association between memory B-cells and lung diseases. Chest 2007; 131:1883-1889
DISCLOSURE: The following authors have nothing to disclose: Patrick Meehan, Jennifer Mccallister, David Hauswirth
No Product/Research Disclosure InformationThe Wexner Medical Center at The Ohio State University, Columbus, OH