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Calciphylaxis-Associated Cutaneous Vascular Calcification in Noncalciphylaxis Patients
Kristine M Chaudet1, Puja Dutta2, Sagar U Nigwekar3
1Pathology Resident, Pathology Service, Massachusetts General Hospital, Harvard Medical School, Boston, MA.
Insights
Histologic features previously linked to calciphylaxis are not specific and are more common in patients with chronic kidney disease (CKD). These findings suggest these features may be less reliable for diagnosing calciphylaxis in CKD patients.
Area of Science:
- Nephrology
- Dermatopathology
- Vascular Biology
Background:
- Calciphylaxis is a severe condition strongly linked to chronic kidney disease (CKD).
- Established histologic criteria for calciphylaxis diagnosis and their specificity remain unclear.
- Histologic findings associated with calciphylaxis may be present in patients without the condition, particularly those with CKD.
Purpose of the Study:
- To determine the prevalence of histologic features associated with calciphylaxis in patients without the condition.
- To evaluate if these features are more common in patients with chronic kidney disease (CKD).
Main Methods:
- Retrospective analysis of healthy marginal skin tissue from above-the-knee amputation specimens.
- Comparison of tissue samples from patients with CKD (n=23) and without CKD (n=47).
- Von Kossa staining to detect intravascular calcification in capillaries and small-to-medium arterioles.
Main Results:
- Intravascular calcification was detected in 40.0% of all patients.
- Capillary calcification and intravascular thrombosis were more prevalent in patients with CKD.
- Finely stippled capillary calcification was observed in 26.1% of CKD patients vs. 8.5% of non-CKD patients (P=0.0484).
- Intravascular thrombosis occurred in 8.7% of CKD patients vs. 0.0% of non-CKD patients (P=0.0403).
- No patients exhibited clinical calciphylaxis during the study or 1-year follow-up.
Conclusions:
- Histologic features previously attributed to calciphylaxis are nonspecific.
- These nonspecific features are more prevalent in patients with chronic kidney disease (CKD).
- The diagnostic specificity of these histologic findings for calciphylaxis requires further investigation, especially in CKD populations.
Abstract:
Calciphylaxis is a highly morbid disease that is strongly associated with chronic kidney disease (CKD); however, the histologic criteria for diagnosis have not been well established nor have their specificity for calciphylaxis been determined. This retrospective study aimed to examine the prevalence of histologic features commonly associated with calciphylaxis in noncalciphylaxis patients. We also sought to evaluate whether these features may be more prevalent in patients with CKD. To assess this, healthy marginal skin tissue from above-the-knee amputation specimens was compared between patients with CKD (n = 23) and without CKD (n = 47). Intravascular calcification of capillaries or small-to-medium arterioles was detected on von Kossa stain in 40.0% of the entire cohort. Capillary calcification and intravascular thrombosis were more prevalent in patients with CKD. Finely stippled capillary calcification was present in 26.1% of patients with CKD versus 8.5% of patients without CKD (P = 0.0484), and intravascular thrombosis was present in 8.7% of patients with CKD and 0.0% of patients without CKD (P = 0.0403). None of the patients in this study had clinical evidence of calciphylaxis at presentation and in at least a 1-year follow-up period. This study confirms that the histologic features previously associated with calciphylaxis are nonspecific and are more prevalent in patients with CKD.
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