Lung disease in primary antibody deficiency

Area of Science:

• Immunology
• Pulmonology
• Internal Medicine

Background:

• Primary antibody deficiency (PAD) syndromes are a group of disorders characterized by impaired antibody production.
• Pulmonary manifestations are common and a major cause of morbidity and mortality in PAD patients.
• Common variable immunodeficiency (CVID) is the most frequent PAD syndrome and often presents with respiratory symptoms.

Purpose of the Study:

• To summarize current knowledge on the pulmonary manifestations of PAD syndromes in adults.
• To focus on the respiratory complications associated with CVID.
• To review investigation, treatment, and management strategies for lung involvement in PAD.

Main Methods:

• This is a review article, summarizing existing literature and knowledge.
• Focuses on common variable immunodeficiency (CVID) as a primary example of PAD.
• Discusses infectious and non-infectious lung complications.

Main Results:

• Respiratory infections are a frequent presenting feature and complication of PAD syndromes.
• Pulmonary complications include acute infections, bronchiectasis, granulomatous-lymphocytic interstitial lung disease (GLILD), and increased lymphoma risk.
• While CT scans show lung abnormalities in most CVID patients, not all develop severe complications.

Conclusions:

• Pulmonary complications significantly contribute to the morbidity and mortality of PAD syndromes.
• Further research is needed to understand mechanisms of lung health and disease in PAD.
• Effective investigation, treatment, and management strategies are crucial for patients with PAD-related lung disease.