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Hypercalcemia in a patient with common variable immunodeficiency and renal granulomas
Alex Meyer1, Helen J Lachmann, A David Webster
1Department of Nephrology and Immunology, and Histopathology, Royal Free Hospital, London, United Kingdom. ameyer@doctors.net.uk
Insights
Common variable immunodeficiency (CVID), a primary immunodeficiency, can present with rare kidney issues. This study details the first case of CVID with noncaseating renal granulomas and hypercalcemia.
Area of Science:
- Immunology
- Nephrology
- Endocrinology
Background:
- Common variable immunodeficiency (CVID) is the most frequent primary immunodeficiency, characterized by low immunoglobulin levels and impaired B-cell function.
- While granulomatous inflammation is a recognized manifestation of CVID, renal involvement is exceptionally rare.
- Differentiating granulomatous CVID from other granulomatous diseases like sarcoidosis can be clinically challenging.
Observation:
- This report describes the first documented case of CVID presenting with biopsy-proven noncaseating renal granulomas.
- The patient also exhibited hypercalcemia, a metabolic abnormality not typically associated with CVID.
- The renal biopsy confirmed the presence of granulomatous inflammation within the kidney tissue.
Findings:
- The study highlights a rare association between CVID, noncaseating renal granulomas, and hypercalcemia.
- It suggests that granulomatous inflammation in the kidneys can be a manifestation of CVID.
- The findings underscore the complex and varied clinical presentations of CVID.
Implications:
- This case expands the known spectrum of CVID manifestations, particularly renal involvement.
- Understanding the mechanism of hypercalcemia in granulomatous disorders is crucial for patient management.
- The case emphasizes the importance of considering CVID in the differential diagnosis of granulomatous kidney disease, especially when hypercalcemia is present.
Abstract:
Common variable immunodeficiency (CVID) is the most common primary immunodeficiency. A granulomatous form of the condition is recognized. Renal involvement is rare. The authors present the first case of CVID with biopsy-proven noncaseating renal granulomas, in association with hypercalcemia. The mechanism of hypercalcemia in granulomatous disorders, and the difficulty of differentiating granulomatous CVID from sarcoidosis, are discussed.
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