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We read with interest the Education Review article entitled ‘Kidney manifestations of pediatric Sjögren’s syndrome’ by La Bella et al. published online in the March issue of Pediatric Nephrology [1]. The authors have focused on the kidney manifestations found in Sjögren’s syndrome (SS). Kidney involvement is an uncommon presentation in primary SS (5–20.5%); tubulointerstitial nephritis (TIN) has been the predominant histopathological lesion (37–98%) reported [2]. It can lead to renal tubular acidosis (RTA); distal RTA being more common than proximal. The authors have mentioned that to date, only eight paediatric cases have been reported to have glomerular involvement in primary SS in the form of membranous glomerulopathy, mesangioproliferative glomerulonephritis with IgA deposits, pauci-immune crescentic glomerulonephritis, TIN and focal proliferative glomerulonephritis [1].
We had previously reported a 9-year-old female child who had earlier presented with features of nephrotic syndrome and later had frequent relapsing course being treated with low-dose prednisolone on alternate days. During follow-up, she developed dry skin, recurrent bilateral parotid gland swellings, knee and ankle joint arthralgia and was found to have positive anti‑Sjögren’s syndrome‑related antigen A (anti‑SSA) with normal C3, C4, ANA and anti-dsDNA levels.
Kidney biopsy revealed focal segmental glomerulosclerosis (FSGS) with proliferation of visceral podocytes, pseudo-crescent formation and collapse of glomerular tuft [2]. The child was treated with tacrolimus, prednisolone and hydroxychloroquine and attained remission of proteinuria.
FSGS in primary SS in children has not been reported so far even in the Japanese Renal Biopsy Registry [3], where SS was diagnosed in 35 patients of 110 non-lupus connective tissue disorders, which also included children, and none had FSGS. As such there is no definite protocol of immunosuppression due to rarity of the disease. However, glomerular involvement in SS has been found to have higher mortality as compared to those with TIN [4]. Therefore, early diagnosis and prompt therapy are needed to prevent progression of kidney disease in SS.
References
La Bella S, Vivarelli M, Di Ludovico A, Di Donato G, Chiarelli F, Breda L (2024) Kidney manifestations of pediatric Sjögren’s syndrome. Pediatr Nephrol 39:711–721. https://doi.org/10.1007/s00467-023-06135-1
Mishra OP, Singh A, Gupta P, Prasad R, Batra VV (2023) Focal segmental glomerulosclerosis in primary Sjogren syndrome. Asian J Pediatr Nephrol 6:24–26. https://doi.org/10.4103/ajpn.ajpn_19_22
Ichikawa K, Konta T, Sato H, Ueda Y, Yokoyama H (2017) The clinical and pathological characteristics of nephropathies in connective tissue diseases in the Japan Renal Biopsy Registry (J-RBR). Clin Exp Nephrol 21:1024–1029. https://doi.org/10.1007/s10157-017-1398-5
Kidder D, Rutherford E, Kipgen D, Fleming S, Geddes C, Stewart GA (2015) Kidney biopsy findings in primary Sjögren syndrome. Nephrol Dial Transplant 30:1363–1369. https://doi.org/10.1093/ndt/gfv042
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OPM, AS and RP reviewed the literature and drafted the manuscript.
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Mishra, O.P., Singh, A. & Prasad, R. Focal segmental glomerulosclerosis in primary Sjögren’s syndrome: a rare presentation. Pediatr Nephrol 39, 2271 (2024). https://doi.org/10.1007/s00467-024-06315-7
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DOI: https://doi.org/10.1007/s00467-024-06315-7