Abstract
Interstitial lung disease (ILD) is an important non-infectious complication in several primary immune deficiencies. In common variable immune deficiency (CVID) it is associated with complex clinical phenotypes and adverse outcomes. The histology of ILD in CVID is heterogeneous and mixed patterns are frequently observed within a single biopsy, including non-necrotising granulomatous inflammation, lymphoid interstitial pneumonitis, lymphoid hyperplasia, follicular bronchiolitis, organizing pneumonia, and interstitial fibrosis; ILD has to be differentiated from lymphoma. The term granulomatous-lymphocytic interstitial lung disease (GLILD), coined to describe the histopathological findings within the lungs of patients with CVID with or without multisystem granulomata, is somewhat controversial as pulmonary granulomata are not always present on histology and the nature of infiltrating lymphocytes is variable. In this mini review we summarize the literature on the histology of CVID-related ILD and discuss some of the factors that may contribute to the inter- and intra- patient variability in the histological patterns reported. Finally, we highlight areas for future development. In particular, there is a need for standardization of histological assessments and reporting, together with a better understanding of the immunopathogenesis of CVID-related ILD to resolve the apparent heterogeneity of ILD in this setting and guide the selection of rational targeted therapies in different patients.
Highlights
Common variable immune deficiency (CVID) is the most common of the primary immunodeficiency (PID) syndromes with a prevalence of 1 in 25,000 and 50,000, depending on the population [1, 2]
Various histopathological entities have been reported within lung biopsies of common variable immune deficiency (CVID) Interstitial lung disease (ILD) patients, from those caused by polyclonal lymphocytic inflammation to well-formed granulomata, organizing pneumonia, or pulmonary fibrosis, often with mixed pathology within individual patient biopsies [7, 9, 16, 27, 33, 35, 44]
It is worth reemphasizing that granulomata are not reported in all cases of CVID-related ILD, with frequencies ranging from 0-94% depending on the individual study (Table 1) [7, 33, 44, 47]
Summary
Common variable immune deficiency (CVID) is the most common of the primary immunodeficiency (PID) syndromes with a prevalence of 1 in 25,000 and 50,000, depending on the population [1, 2]. Other related complications, including splenomegaly, autoimmune cytopaenias, persistent lymphadenopathy and lymphoproliferation, but not necessarily granulomata, occur more frequently in patients with CVID-related ILD, supporting at least a role for intrinsic immune dysregulation driving these varied features [6, 9, 16, 21, 27, 32, 33]. Various histopathological entities have been reported within lung biopsies of CVID ILD patients, from those caused by polyclonal lymphocytic inflammation to well-formed granulomata, organizing pneumonia, or pulmonary fibrosis, often with mixed pathology within individual patient biopsies [7, 9, 16, 27, 33, 35, 44].
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