Table 1.

Clinical and laboratory variables

CLINICAL OUTCOME VARIABLE
Infections	Presence of at least one of these: - Recurrent sinusitis, otitis, or high respiratory tract infections - Pneumonia - Recurrent non-life-threatening infections (e.g. Erysipelas, cholangitis) - Gastro-intestinal infection caused by CVID-characteristic pathogen (e.g. Campylobacter, Giardia, Norovirus, etc.) - Severe infection (sepsis, meningoencephalitis, endocarditis etc.) - Opportunistic infection
Autoimmunity	Presence of at least one autoimmune manifestation, including both systemic and organ-specific autoimmune diseases. Dysregulated or inflammatory manifestations such as enteropathies and non-infectious erythema nodosum were also included in this group
Granulomatous disease	Histological evidence, with or without radiological confirmation, of granulomatous lesions with no discernible infectious cause
Lung involvement	Presence of at least one of the following: high-resolution tomography alterations, such as bronchiectasis or interstitial abnormalities, documented reduction in DLCO, or a restrictive pattern observed in pulmonary function testing
Malignancies	The diagnosis should be established within 6 months of the CVID diagnosis. Benign solid tumors are not considered
Atopy	At least one allergic manifestation, encompassing a history of anaphylaxis, allergic rhino-conjunctivitis, asthma, and atopic dermatitis
Splenomegaly	Spleen diameter > 12 cm, evaluated by ultrasound or CT scan
Lymphadenopathy (LA)	Chronic enlargement of at least two different lymphnode anatomical sites demonstrated through ultrasound or CT scan
LABORATORY VARIABLES
Immunoglobulin levels	Total IgG levels before initiation of Ig replacement therapy, as well as total IgA, IgM, and IgE levels at the most recent evaluation, were determined
CMV and EBV blood viral load	CMV viral load was determined by RT-PCR with CMV ELITe MGB Kit EBV viral load was determined by RT-PCR with EBV ELITe MGB Kit
Other infective agents	Each patient underwent screening for HCV, HIV, HBV, HHV6 using RT-PCR tests (in addition, for HIV, a fourth-generation test was also conducted), and anamnestic immune response to MTB

CMV Cytomegalovirus; DLCO Diffusing Capacity of the Lungs for Carbon Monoxide; EBV Epstein-Barr virus; HBV Hepatitis B virus; HCV hepatitis C virus; HHV6 Human herpes virus 6; HIV human immunodeficiency virus; MTB Mycobacterium tuberculosis; RT-PCR Real time polymerase chain reaction