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ABSTRACT: Background
Podocyte infolding glomerulopathy (PIG) is a peculiar and very rare manifestation in renal pathology. Its underlying pathogenesis mechanism and clinical characteristics remain unclear due to sparse reports.Objective
To further elucidate the clinical profile of PIG by carefully reporting our four cases and a comprehensive review of cases in the literature.Methods
This study retrospectively reviewed four cases of PIG from 2010 to 2022 in our centre. Clinical and pathological profiles were reported. PIG cases in the literature were searched in the MEDLINE database and analysed together with our cases.Results
Four cases of PIG identified from our centre and 40 cases from the current literature were reported. The pooled analysis of these 44 cases indicated 79.5% (35/44) were females, 93.2% (41/44) were East Asians, and 63.6% (28/44) were reported in Japan. The average age was 42.0 ± 12.5 years old. The average amount of proteinuria at the time of renal biopsy was 3.06 ± 3.2 g/day. The most reported comorbidities were connective tissue diseases, mainly systemic lupus erythematosus, and 20.5% (9/44) of the cases did not have any contaminant disease. Most of the cases (81.8%, 36/44) had been treated with immunosuppressants, of which a combination of corticosteroids and one other type of immunosuppressant was most commonly reported. In addition, 45.4% (20/44) and 34.1% (15/44) of the cases had achieved complete response and partial response, respectively, after treatment. Whole exosome sequencing indicated mutations in the INF2 gene.Conclusions
PIG is a rare condition and seen in relatively younger populations, often associated with connective tissue diseases clinically and one or two other glomerulopathies histologically. The outcomes following immunosuppressive treatment are relatively good. Mutations in INF2 might be involved in the development of PIG; however, the implications of these results need to be investigated.
SUBMITTER: Feng Y
PROVIDER: S-EPMC9918010 | biostudies-literature | 2023 Jan
REPOSITORIES: biostudies-literature
Feng Yunlin Y Wang Wei W Zou Yurong Y Chen Tingyu T Wang Wei W Li Guisen G Wang Amanda Y AY Zhang Ping P
Journal of clinical medicine 20230130 3
<h4>Background</h4>Podocyte infolding glomerulopathy (PIG) is a peculiar and very rare manifestation in renal pathology. Its underlying pathogenesis mechanism and clinical characteristics remain unclear due to sparse reports.<h4>Objective</h4>To further elucidate the clinical profile of PIG by carefully reporting our four cases and a comprehensive review of cases in the literature.<h4>Methods</h4>This study retrospectively reviewed four cases of PIG from 2010 to 2022 in our centre. Clinical and ...[more]