Protocols of Standard of Care for Adult Patients with Primary Antibody Deficiencies Will Improve Timing of Diagnosis, Survival, and Quality of Life

Katarzyna  Napiórkowska-Baran; Ewa  Więsik-Szewczyk; Marcin Ziętkiewicz; Aleksandra  Matyja-Bednarczyk; Sylwia Kołtan; Natalia Bąkowska-Kocik; Tomasz Rosada; Karolina Baranowska; Ewa Alska; Marta Tykwińska; Ewa Szynkiewicz; Robert Zacniewski; Zbigniew Bartuzi

doi:10.18502/ijaai.v21i4.10285

Katarzyna Napiórkowska-Baran Department of Allergology, Clinical Immunology and Internal Diseases, Ludwik Rydygier Collegium Medicum in Bydgoszcz Nicolaus Copernicus University in Toruń, Bydgoszcz, Poland
Ewa Więsik-Szewczyk Department of Internal Medicine, Pulmonology, Allergy and Clinical Immunology, Central Clinical Hospital of the Ministry of National Defense, Military Institute of Medicine, Warsaw, Poland
Marcin Ziętkiewicz Department of Internal Medicine, Connective Tissue Diseases and Geriatrics, Medical University of Gdańsk, Gdańsk, Poland
Aleksandra Matyja-Bednarczyk 2nd Department of Internal Medicine, Jagiellonian University Medical College, Kraków, Poland
Sylwia Kołtan Department of Pediatrics, Hematology and Oncology, Ludwik Rydygier Collegium Medicum in Bydgoszcz Nicolaus Copernicus University in Toruń, Bydgoszcz, Poland
Natalia Bąkowska-Kocik Department of Clinical Oncology, Franciszek Łukaszczyk Oncology Centre, Bydgoszcz, Poland
Tomasz Rosada Department of Allergology, Clinical Immunology and Internal Diseases, Ludwik Rydygier Collegium Medicum in Bydgoszcz Nicolaus Copernicus University in Toruń, Bydgoszcz, Poland
Karolina Baranowska Department of Allergology, Clinical Immunology and Internal Diseases, Ludwik Rydygier Collegium Medicum in Bydgoszcz Nicolaus Copernicus University in Toruń, Bydgoszcz, Poland
Ewa Alska Department of Allergology, Clinical Immunology and Internal Diseases, Ludwik Rydygier Collegium Medicum in Bydgoszcz Nicolaus Copernicus University in Toruń, Bydgoszcz, Poland
Marta Tykwińska Department of Allergology, Clinical Immunology and Internal Diseases, Ludwik Rydygier Collegium Medicum in Bydgoszcz Nicolaus Copernicus University in Toruń, Bydgoszcz, Poland
Ewa Szynkiewicz Department of Allergology, Clinical Immunology and Internal Diseases, Ludwik Rydygier Collegium Medicum in Bydgoszcz Nicolaus Copernicus University in Toruń, Bydgoszcz, Poland
Robert Zacniewski Department of Allergology, Clinical Immunology and Internal Diseases, Ludwik Rydygier Collegium Medicum in Bydgoszcz Nicolaus Copernicus University in Toruń, Bydgoszcz, Poland
Zbigniew Bartuzi Department of Allergology, Clinical Immunology and Internal Diseases, Ludwik Rydygier Collegium Medicum in Bydgoszcz Nicolaus Copernicus University in Toruń, Bydgoszcz, Poland

DOI: https://doi.org/10.18502/ijaai.v21i4.10285

Keywords: Adult; Clinical protocol; Guideline adherence; Primary immunodeficiency diseases; Standard of care

Abstract

The majority of primary immunodeficiencies (PIDs) are antibody deficiencies (PADs), and not all of them are rare diseases; As an example, Caucasian individuals suffer from selective IgA deficiency at a frequency of 1:500.In addition to infections, symptomatic patients with PAD are more likely to develop neoplastic, autoimmune, and allergic diseases. In the event that PAD is neglected or delayed for more than ten years, complications develop, eventually resulting in death. No studies have been conducted to devise and report detailed ready-to-use protocols for managing PAD to date.This study aimed to propose protocols and guidelines for the adult PAD patients’ standard care. Preparing the protocol, we considered the frequency and type of laboratory tests, imaging, endoscopic examinations, specialist consultations, and standardized recommendations for further care in the place of residence. As a result of the proposed monitoring scheme, patients can be provided with complete
care in terms of their underlying conditions and comorbidities, as well as early detection of complications. This protocol will serve as a guide for physicians dealing with these patients and enable comparisons of patient groups across a variety of treatment centers, even far away from each other.
A national consultant in the field of clinical immunology verified the protocol mainly developed by Polish experts from reference immunology centres for adults.