Respiratory Research volume 20 , Article number: Cite this article. Metrics details. Bronchiectasis is a heterogeneous disease depending on etiology. It represents the most frequent non-infectious pulmonary complication of primary immunodeficiencies PID. We investigated whether bronchiectasis associated with PID had a distinct course in comparison to bronchiectasis of other causes.
Respiratory functional data and clinical outcomes were compared. Of patients with bronchiectasis diagnosed in Foch Hospital Suresnes, France , 98 patients fulfilled the selected criteria 20 PID-related cases, 39 idiopathic or post-infectious cases, and 39 cases with other causes. Median time of follow-up was 9. Groups were similar concerning initial characteristics female Eighty-five percent of PID patients received immunoglobulin substitution median trough level was measured at Global median FEV1 annual decline was Hemoptysis occurred in Global mortality rate was We did not record any significant difference for all clinical and functional outcomes between patients with PID and other etiologies.
The median decline in FEV1 was similar in the three groups. The course of PID-related bronchiectasis was similar to bronchiectasis of other causes. Provided that patients receive immunoglobulin replacement, the course of PID-related bronchiectasis seems to be independent of the underlying immune disorder. Bronchiectasis is a chronic respiratory disease, defined radiologically by the presence of permanent bronchial dilatation on high resolution chest computerized tomography.
The improvement in their management, mostly immunoglobulin substitution, has allowed us to decrease the prevalence of bacterial pneumoniae and mortality [ 7 , 8 , 9 ]. However, bronchiectasis remains the most frequent non-infectious respiratory complication of PID, making its physiopathology unclear and unrelated to the occurrence of previous bacterial pneumonia [ 10 , 11 , 12 , 13 ].
In bronchiectasis, some factors have been identified as having a poor prognostic value: extension of bronchiectasis [ 14 ], chronic obstructive pulmonary disease COPD [ 14 ] and Pseudomonas aeruginosa colonization [ 15 ]. However, little is known about mean-term outcome and changes in pulmonary function. The aim of our study was to compare initial characteristics, complications and prognosis of PID-related bronchiectasis with a large cohort of patients with bronchiectasis due to other causes.
