Abstract: PO1937
Dysmorphic Lysosomes, Pathognomonic of Chronic Interstitial Nephritis in Agricultural Communities (CINAC)
Session Information
- Renal Pathology: From Laboratory to Bedside
November 04, 2021 | Location: On-Demand, Virtual Only
Abstract Time: 10:00 AM - 12:00 PM
Category: Pathology and Lab Medicine
- 1600 Pathology and Lab Medicine
Authors
- Olano, Claudia Guadalupe, Harbor-UCLA Medical Center, Torrance, California, United States
- Guevara, Nehemias Antonio, Harbor-UCLA Medical Center, Torrance, California, United States
- Salinas, Carlos Alberto, San Juan de Dios Hospital, nephrology department, Santa Ana, SA, El Salvador
- Orantes, Carlos, Ministry of Health of El Salvador, Santa Ana, SA, El Salvador
- Barba, Lilly M., Harbor-UCLA Medical Center, Torrance, California, United States
- Akram, Sami M., Loma Linda University, Loma Linda, California, United States
Background
The etiology and pathogenesis of CINAC are unknown. Two hypotheses have been put forward. Dysmorphic lysosomes (DL) were described as pathognomonic however some argue that DL are non-specific findings. Therefore, we did an observational study of electron microscopic (EM) findings in CINAC comparing to published cases.
Methods
Sixteen patients from Central America, suspected of CINAC clinically underwent kidney biopsy (KB). Demographic characteristics, blood and urine analysis data were collected. Renal histology was studied using light (LM), immunofluorescence (IF), and electron microscopy (EM). We reviewed the literature in PubMed using the following search terms (a). “Chronic Interstitial Nephritis + Electron Microscopy + Kidney” and identified 8 relevant cases (Group A) (b). “Dysmorphic Lysosomes, Electron Microscopy “ found 14 cases that described the EM findings (Group C).
Results
Of the 8 patients in group A, who had CIN, only 2had DL on EM. One patient had Calcineurin Inhibitors (CNI) and one patient from Sri Lanka had chronic kidney disease of unknown origin, which is also considered as CINAC. In patient who had CNI, the DL had irregular edges unlike the smooth rounded DL of varying sizes noted in all CINAC patients. In the control group, C, the patients who had DL, were 14 but the DLs were present in organs other than kidneys. The two patients had DL in the kidneys, one was Fabry’s disease, and the other was Light chain Proximal tubulopathy (LCPT). In Fabry’s disease, the DL were lamellated. In LCPT the DL were rectangular due to the characteristics of the lambda proteins.
Conclusion
Dysmorphic Lysosomes may occur in multiple disorders, however, in young persons with agricultural exposure, non-nephrotic proteinuria, presence of tubular inflammation, presence of smooth rounded clusters of DL are pathognomonic.
Morphology of the DL is dictated by the contents of the lysosomes as in the case of LCPT; further, evaluation is recommended.
| CIN-GrA | CINAC-GrB | DL-GrC | Total | |
| DL-Kidney Positive | 2 | 16 | 2 | |
| DL-Kidney Negative | 6 | 0 | 12 | |
| 8 | 16 | 14 | 28 |
The Chi-square statistic is 21.3929. The p-value is 000023. DL= Dysmorphic Lymphocytes. GrA: CIN, GrB: CINAC, GrC: Have dysmorphic lymphocytes.