An update on appropriate lung management in patients with primary antibody deficiencies
Helena Buso1, Giulia A M L Costanzo2, Nicola Monaco3
1Rare Diseases Referral Center, Internal Medicine 1, Department of Medicine (DIMED), AULSS2 Marca Trevigiana, Ca' Foncello Hospital, University of Padova, Padova, Italy.
Introduction:
Predominantly antibody deficiencies (PADs) represent the most common inborn errors of immunity (IEIs), constituting over 50% of all IEIs detected globally. Compared to other IEIs, PADs are linked to a more favorable long-term prognosis and are typically diagnosed in adulthood. We analyzed the most recent literature on lung involvement in PAD using PUBMED Central.
Areas Covered:
PADs are characterized by their impact on the respiratory system, which results in significant morbidity and mortality due to the impairment of both humoral and mucosal immune responses. This impact leads to a broad spectrum of complications that could be infection-related, inflammatory, or neoplastic.
Expert Opinion:
Defined guidelines are unavailable, but a strict assessment of pulmonary status should be recommended in patients with characteristic immunologic patterns (low IgA serum level, reduced percentage of switched memory B cells, increased IgM serum levels, increased percentage of CD21 low B cells, autoimmune cytopenia, and splenomegaly).
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