Pediatric antinuclear antibody negative full-house nephropathy: A clinical conundrum

Abu Bakar, Karmila and Jie, Chuah Ming and Hui, Lim Min and Rao, Prakash Rao Rama and Chin, Yap Yok and Yin, Caroline Eng Siew (2022) Pediatric antinuclear antibody negative full-house nephropathy: A clinical conundrum. Iranian Journal of Pediatrics, 32 (4). ISSN 2008-2142, DOI

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Lupus nephritis is common in pediatric systemic lupus erythematosus (SLE). The presence of full-house im-munoglobulin deposits is a unique feature for histopathological diagnosis in lupus nephritis. Our case report highlights two cases with antinuclear antibody (ANA) negative full-house nephropathy (FHN), their clinical phenotypes and intermediate long-term re-nal outcome. A review of the pediatric literature on this clinical entity ensued.Case Presentation: Two girls, aged three and nine years old presented with acute onset of kidney impairment. One needed tem-porary dialysis support. Both showed hypocomplementemia and biopsies concurred with FHN. Notably, ANA was absent in both patients. They achieved complete remission soon after introducing immunosuppressive therapy and the disease remained quies-cent for many years thereafter. ANA remained negative during surveillance.Conclusions: There have been reports on a possible incomplete form when the characteristic of FHN is seen in isolation. Although natural history of this entity remains uncertain, most literature reports apparent long-term remission following the initial episode. ANA positivity, as an obligatory criterion of diagnosing lupus nephritis (LN), recommended in the new EULAR/ACR 2019 guideline, may raise many uncertainties for this group of patients. There is a need to elucidate whether this should continue to be perceived as part of a lupus continuum or it may be considered a unique clinical entity? Such uncertainties possibly subject the child and his/her caregivers to perpetual mental stress.

Item Type: Article
Uncontrolled Keywords: Negative ANA; Systemic Lupus Erythematosus; full-House Nephropathy; SLE
Subjects: R Medicine
R Medicine > RJ Pediatrics
Divisions: Faculty of Medicine
Depositing User: Ms. Juhaida Abd Rahim
Date Deposited: 20 Sep 2023 07:15
Last Modified: 20 Sep 2023 07:15

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