Alpha-1 Antitrypsin Deficiency and Pulmonary Morbidity in Patients with Primary Immunodeficiency Disease: A Single-Center Experience

Table 1

Coexistence of AATD and PID.

Authors	Study patients (n)	Age (years)	AAT phenotype	Immune deficiency	Pulmonary morbidity
Gupta et al. [19]	1	89	PI∗ZZ	IgG/IgM hypogammaglobulinemia	Bronchiectasis, emphysema, obstruction, pulmonary embolus
Elsensohn et al. [20]	1	36	PI∗ZZ	IgG/IgM hypogammaglobulinemia	Pulmonary embolus
Casterline et al. [21]	1	54	PI∗ZZ	Selective IgA deficiency	Bronchiectasis, emphysema
Phung et al. [22]	1	34	PI∗ZZ	CVID	Obstruction, emphysema
Phung et al. [23]	3	NA	PI∗MZ	Hypogammaglobulinemia	NA
	5	NA	PI∗MS	Hypogammaglobulinemia	NA
	1	NA	PI∗SZ	Hypogammaglobulinemia	NA
Sansom et al. [24]	1	71	PI∗SZ	CVID	Bronchiectasis
	1	5	PI∗MZ	CVID	Bronchiectasis
	1	22	PI∗MZ	CVID	Bronchiectasis
	1	32	PI∗MZ	CVID	Bronchiectasis
	1	33	PI∗MS	CVID
Fazlollahi et al. [25]	1	NA	PI∗SS	CVID
	1	NA	PI∗MZ	CVID
	1	NA	PI∗MZ	CVID	Bronchiectasis
Evers et al.	1	25	PI∗ZZ	CVID
	1	45	PI∗ZZ	Unclassified antibody deficiency	Obstruction, emphysema
	1	37	PI∗MZ	CVID	Obstruction, emphysema
	1	47	PI∗MZ	Selective IgM deficiency
	1	54	PI∗MZ	Selective IgM deficiency	Obstruction
	1	18	PI∗MZ	Isolated IgG subclass deficiency	Panbronchiolitis
	1	50	PI∗MZ	Unclassified antibody deficiency