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[Unusual clinical forms of presentation and infrequent pathologic associations of xanthogranulomatous pyelonephritis]
1Servicio de Urología, Hospital Ntra, Sra. de la Esperanza, Barcelona, España.
Objective:
To describe the unusual clinical forms and rare associations of xanthogranulomatous pyelonephritis seen in a variable proportion of cases.
Methods:
Analysis of our series of 83 cases and 2573 cases reported in the literature showed 8 different and less frequent clinical forms and up to 7 rare associations, which are discussed in this study.
Results:
There are different clinical forms under "renal infection", ranging widely from asymptomatic to severe septic forms that affect patients of all ages--from the newborn to the elderly-, preferentially in association with conditions of immunosuppression, and other more uncommon or rare forms in which xanthogranulomatous pyelonephritis is often incidentally discovered.
Conclusions:
These forms are not extremely rare since more than 2500 cases have been reported in the literature. Knowledge about the different forms permits stratification of this condition into different clinical forms of presentation and provides further insight into the associated conditions, some of which are rare. Overall, such forms or associations have been reported to account for 20 to 40% of all cases, according to the studies reported elsewhere. This overall incidence is rather significant and should be taken into account.
Insights
Xanthogranulomatous pyelonephritis presents with diverse clinical forms and rare associations, affecting all age groups. Recognizing these variations is crucial for accurate diagnosis and management of this significant renal condition.
Area of Science:
- Nephrology
- Pathology
- Infectious Diseases
Context:
- Xanthogranulomatous pyelonephritis (XGP) is a rare, chronic destructive inflammation of the kidney.
- It is often associated with long-standing obstruction and infection.
- Unusual clinical presentations and rare associations can complicate diagnosis.
Purpose:
- To delineate the spectrum of unusual clinical forms of xanthogranulomatous pyelonephritis.
- To identify and discuss rare associations linked to XGP.
- To enhance understanding of the varied presentations of this condition.
Summary:
- A comprehensive analysis of 83 cases and 2,573 literature cases identified 8 distinct less frequent clinical forms and 7 rare associations of XGP.
- Presentations range from asymptomatic to severe septic forms, affecting all ages, particularly those with immunosuppression.
- Uncommon or rare forms are often discovered incidentally.
Impact:
- The findings highlight that these unusual forms and associations are not extremely rare, accounting for 20-40% of cases.
- Improved knowledge allows for better stratification of XGP presentations.
- This study provides critical insights for clinicians managing patients with XGP and its associated conditions.