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Appropriate lung management in patients with primary antibody deficiencies. 2019

Francesco Cinetto, and Riccardo Scarpa, and Federica Pulvirenti, and Isabella Quinti, and Carlo Agostini, and Cinzia Milito
Department of Medicine - DIMED, University of Padova , Padova , Italy.

Introduction: Human primary immunodeficiency diseases (PIDs) include a broad spectrum of more than 350 disorders, involving different branches of the immune system and classified as 'rare diseases.' Predominantly antibody deficiencies (PADs) represent more than half of the PIDs diagnosed in Europe and are often diagnosed in the adulthood. Areas covered: Although PAD could first present with autoimmune or neoplastic features, respiratory infections are frequent and respiratory disease represents a relevant cause of morbidity and mortality. Pulmonary complications may be classified as infection-related (acute and chronic), immune-mediated, and neoplastic. Expert opinion: At present, no consensus guidelines are available on how to monitor and manage lung complications in PAD patients. In this review, we will discuss the available diagnostic, prognostic and therapeutic instruments and we will suggest an appropriate and evidence-based approach to lung diseases in primary antibody deficiencies. We will also highlight the possible role of promising new tools and strategies in the management of pulmonary complications. However, future studies are needed to reduce of diagnostic delay of PAD and to better understand lung diseases mechanisms, with the final aim to ameliorate therapeutic options that will have a strong impact on Quality of Life and long-term prognosis of PAD patients.

UI MeSH Term Description Entries
D008168 Lung Either of the pair of organs occupying the cavity of the thorax that effect the aeration of the blood. Lungs
D008171 Lung Diseases Pathological processes involving any part of the LUNG. Pulmonary Diseases,Disease, Pulmonary,Diseases, Pulmonary,Pulmonary Disease,Disease, Lung,Diseases, Lung,Lung Disease
D008279 Magnetic Resonance Imaging Non-invasive method of demonstrating internal anatomy based on the principle that atomic nuclei in a strong magnetic field absorb pulses of radiofrequency energy and emit them as radiowaves which can be reconstructed into computerized images. The concept includes proton spin tomographic techniques. Chemical Shift Imaging,MR Tomography,MRI Scans,MRI, Functional,Magnetic Resonance Image,Magnetic Resonance Imaging, Functional,Magnetization Transfer Contrast Imaging,NMR Imaging,NMR Tomography,Tomography, NMR,Tomography, Proton Spin,fMRI,Functional Magnetic Resonance Imaging,Imaging, Chemical Shift,Proton Spin Tomography,Spin Echo Imaging,Steady-State Free Precession MRI,Tomography, MR,Zeugmatography,Chemical Shift Imagings,Echo Imaging, Spin,Echo Imagings, Spin,Functional MRI,Functional MRIs,Image, Magnetic Resonance,Imaging, Magnetic Resonance,Imaging, NMR,Imaging, Spin Echo,Imagings, Chemical Shift,Imagings, Spin Echo,MRI Scan,MRIs, Functional,Magnetic Resonance Images,Resonance Image, Magnetic,Scan, MRI,Scans, MRI,Shift Imaging, Chemical,Shift Imagings, Chemical,Spin Echo Imagings,Steady State Free Precession MRI
D011379 Prognosis A prediction of the probable outcome of a disease based on a individual's condition and the usual course of the disease as seen in similar situations. Prognostic Factor,Prognostic Factors,Factor, Prognostic,Factors, Prognostic,Prognoses
D011788 Quality of Life A generic concept reflecting concern with the modification and enhancement of life attributes, e.g., physical, political, moral, social environment as well as health and disease. HRQOL,Health-Related Quality Of Life,Life Quality,Health Related Quality Of Life
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000081207 Primary Immunodeficiency Diseases Genetic immunologic deficiency diseases and syndromes due to mutations in genes involved in IMMUNITY generally characterized by an increased susceptibility to infectious diseases. They are often associated with AUTOIMMUNE DISEASE manifestations. Congenital Immunodeficiency Disease,Congenital Immunodeficiency Diseases,Congenital Immunodeficiency Disorder,Congenital Immunodeficiency Disorders,Congenital Immunodeficiency Syndrome,Congenital Immunodeficiency Syndromes,Inherited Immunodeficiency Disease,Inherited Immunodeficiency Diseases,Inherited Immunodeficiency Disorder,Inherited Immunodeficiency Disorders,Inherited Immunodeficiency Syndrome,Inherited Immunodeficiency Syndromes,Primary Antibody Deficiencies,Primary Antibody Deficiency Disorder,Primary Antibody Deficiency Disorders,Primary Antibody Deficiency Syndrome,Primary Antibody Deficiency Syndromes,Primary Immune Deficiency,Primary Immune Deficiency Disease,Primary Immune Deficiency Diseases,Primary Immune Deficiency Disorder,Primary Immune Deficiency Disorders,Primary Immune Deficiency Syndrome,Primary Immune Deficiency Syndromes,Primary Immunodeficiency Disease,Primary Immunodeficiency Disorder,Primary Immunodeficiency Disorders,Primary Immunodeficiency Syndromes,Antibody Deficiency, Primary,Deficiency, Primary Immune,Immune Deficiency, Primary,Immunodeficiency Disease, Congenital,Immunodeficiency Disease, Inherited,Immunodeficiency Disease, Primary,Immunodeficiency Diseases, Congenital,Immunodeficiency Diseases, Inherited,Immunodeficiency Diseases, Primary,Immunodeficiency Disorder, Congenital,Immunodeficiency Disorder, Inherited,Immunodeficiency Disorder, Primary,Immunodeficiency Disorders, Congenital,Immunodeficiency Disorders, Inherited,Immunodeficiency Syndrome, Congenital,Immunodeficiency Syndrome, Inherited,Immunodeficiency Syndrome, Primary,Immunodeficiency Syndromes, Congenital,Immunodeficiency Syndromes, Inherited,Immunodeficiency Syndromes, Primary,Inherited Immunodeficiency Syndrome,Primary Antibody Deficiency,Primary Immune Deficiencies,Primary Immunodeficiency Syndrome
D014943 Global Health A multi- and interdisciplinary field concerned with improving health and achieving equity in health for all people. It transcends national boundaries, promotes cooperation and collaboration within and beyond health science fields, and combines population-based disease prevention with individually-based patient care. International Health Problems,World Health,International Health,Worldwide Health,Health Problem, International,Health Problems, International,Health, Global,Health, International,Health, World,Health, Worldwide,Healths, International,International Health Problem,International Healths,Problem, International Health,Problems, International Health
D015897 Comorbidity The presence of co-existing or additional diseases with reference to an initial diagnosis or with reference to the index condition that is the subject of study. Comorbidity may affect the ability of affected individuals to function and also their survival; it may be used as a prognostic indicator for length of hospital stay, cost factors, and outcome or survival.
D057210 Delayed Diagnosis Non-optimal interval of time between onset of symptoms, identification, and initiation of treatment. Diagnosis Delay,Late Diagnosis,Delayed Diagnoses,Diagnosis Delays,Diagnosis, Delayed,Diagnosis, Late,Late Diagnoses

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