AI Article Synopsis

  • Primary antibody deficiency (PAD) includes several diseases affecting B cell function and leads to various clinical issues, with 461 patients studied to understand these complications.
  • The most common initial symptoms were respiratory infections (63.5%) and chronic diarrhea (17.2%), with common variable immune deficiency (CVID) patients experiencing longer diagnostic delays than others.
  • Non-infectious issues like autoimmunity (26.2%) and splenomegaly (23.4%) were also frequent, highlighting the need for better awareness among practitioners to facilitate earlier diagnosis and intervention.

Article Abstract

Background: Primary antibody deficiency (PAD) comprises a range of diseases from early to late terminal B cells defects and is associated with the various clinical complications.

Methods: A total of 461 patients (311 males and 150 females) with PADs enrolled in the retrospective cohort study and for all patients' demographic information, clinical records and laboratory data were collected to investigate clinical complications.

Results: The most prevalent first presentations of immunodeficiency were respiratory tract infections in 63.5% and chronic diarrhea in 17.2%. Common variable immune deficiency (CVID) patients had a higher diagnostic delay than class switching defect (CSD), and agammaglobulinemia. Among the noninfectious complications, autoimmunity (26.2%), and splenomegaly (23.4%) were the most common. Lymphadenopathy was higher in CSD patients than other PADs, while splenomegaly, hepatomegaly, autoimmunity and bronchiectasis were more common in CVID patients than others. Atopic manifestations were mostly recorded in patients with selective IgA deficiency. Malignancy was only reported in 5.8% of patients with CVID. There was a higher prevalence of autoimmune manifestations in CVID comparing to other PADs.

Conclusion: PADs are relatively rare diseases and these patients have a variety of first clinical manifestations, such as diverse infections, autoimmunity, lymphoproliferation, allergy, enteropathy and malignancy. Practitioner's awareness about the heterogeneous presentations of PAD disorders is poor, therefore patients often are lately diagnosed, and they are complicated with several clinical complications before the certain diagnosis.

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Source
http://dx.doi.org/10.2174/1871530318666180413110216DOI Listing

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