Association Between Common Variable Immunodeficiency and Pulmonary Amyloidosis: Review

Area of Science:

• Immunology
• Pathology

Background:

• Common Variable Immunodeficiency (CVID) is a primary antibody deficiency linked to infections and immune issues.
• Amyloidosis, a severe complication, rarely involves the lungs in CVID patients.

Purpose of the Study:

• To review CVID-associated amyloidosis cases.
• To present a unique case of pulmonary and gastrointestinal amyloidosis in CVID.

Main Methods:

• Literature search for CVID and amyloidosis case reports and observational studies.
• Clinical case presentation of a CVID patient with pulmonary and gastrointestinal amyloidosis.

Main Results:

• Fifteen cases of CVID-associated amyloidosis were identified, predominantly AA type with multi-organ involvement.
• Only one prior case of pulmonary amyloidosis in CVID was reported; no pulmonary AL amyloidosis without plasma cell dyscrasia.
• The presented patient developed systemic AL amyloidosis from an initial AA type, progressing rapidly with a fatal outcome.

Conclusions:

• Consider amyloidosis in CVID patients presenting with unusual symptoms.
• Distinguishing between AA and AL amyloidosis is crucial for treatment selection.
• Early diagnosis and appropriate management of amyloidosis in CVID may improve patient outcomes.