Protease–Antiprotease imbalance in bronchiectasis

M Oriano, F Amati, A Gramegna, A De Soyza… - International journal of …, 2021 - mdpi.com
Airway inflammation plays a central role in bronchiectasis. Protease–antiprotease balance is
crucial in bronchiectasis pathophysiology and increased presence of unopposed proteases …

The pediatric common variable immunodeficiency—from genetics to therapy: a review

A Szczawinska-Poplonyk, E Schwartzmann… - European Journal of …, 2022 - Springer
Common variable immunodeficiency (CVID) is the most prevalent antibody deficiency,
characterized by remarkable genetic, immunological, and clinical heterogeneity. The …

Histology of interstitial lung disease in common variable immune deficiency

F Dhalla, DJM Lochlainn, H Chapel… - Frontiers in …, 2020 - frontiersin.org
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 …

Bronchiectasis is associated with delayed diagnosis and adverse outcomes in the New Zealand Common Variable Immunodeficiency Disorders cohort study

R Ameratunga, A Jordan, A Cavadino… - Clinical & …, 2021 - academic.oup.com
Common variable immunodeficiency disorders (CVID) are multi-system disorders where
target organ damage is mediated by infective, autoimmune and inflammatory processes …

Respiratory Comorbidities Associated with Bronchiectasis in Patients with Common Variable Immunodeficiency in the USIDNET Registry

O Correa-Jimenez, S Restrepo-Gualteros… - Journal of clinical …, 2023 - Springer
Background Bronchiectasis is a major respiratory complication in patients with common
variable immunodeficiency (CVID) and is associated with recurrent pulmonary infections …

Four diseases, PLAID, APLAID, FCAS3 and CVID and one gene (PHOSPHOLIPASE C, GAMMA‐2; PLCG2): Striking clinical phenotypic overlap and difference

N Kutukculer, E Topyildiz, A Berdeli… - Clinical case …, 2021 - Wiley Online Library
We suggest PLAID, APLAID, and FCAS3 have to be considered as different aspects of the
same underlying condition, because of our long‐term clinical and genetical experiences …

Predictive factors for and complications of bronchiectasis in common variable immunodeficiency disorders

JM Sperlich, B Grimbacher, V Soetedjo… - Journal of Clinical …, 2022 - Springer
Bronchiectasis is a frequent complication of common variable immunodeficiency disorders
(CVID). In a cohort of patients with CVID, we sought to identify predictors of bronchiectasis …

Respiratory infections in patients with primary immunodeficiency

HK Lehman, OA Karl, CT Towe, KA Risma - The Journal of Allergy and …, 2022 - Elsevier
Recurrent and life-threatening respiratory infections are nearly universal in patients with
primary immunodeficiency diseases (PIDD). Early recognition, aggressive treatment, and …

Shorter diagnostic delay in Polish adult patients with common variable immunodeficiency and symptom onset after 1999

M Ziętkiewicz, E Więsik-Szewczyk… - Frontiers in …, 2020 - frontiersin.org
Common variable immunodeficiency (CVID) is the most clinically significant primary
antibody immunodeficiency recognized in adulthood. Previously published data have shown …

Variable immunodeficiency score upfront analytical link (VISUAL), a proposal for combined prognostic score at diagnosis of common variable immunodeficiency

K Guevara-Hoyer, A Jiménez-Huete, J Vasconcelos… - Scientific Reports, 2021 - nature.com
The broad and heterogeneous clinical spectrum that characterizes common variable
immunodeficiency (CVID) is associated with quite different disease course and prognosis …