Membranous nephropathy with solitary polyclonal IgA deposition: A case report and literature review

Sawamura, Masato; Komatsuda, Atsushi; Kaga, Hajime; Saito, Ayano; Yasuda, Tadashi; Wakui, Hideki; Joh, Kensuke; Takahashi, Naoto

doi:10.5414/cncs109807

Cited by 2 publications

(3 citation statements)

References 10 publications

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“…Although these clinical features are similar to those of the present case, our case is quite different because of the scarce mesangial proliferation and granular IgA deposition along the GBM seen. In addition, our case is also different from two previous ones of MN with solitary polyclonal IgA deposits [14,15]. Although they are different from our case in terms of lacking C3 deposition and subepithelialdominant electron dense deposition, it should be noted that polyclonal IgA can pass through GBM and deposit at subepithelial sites.…”

Section: Discussioncontrasting

confidence: 95%

Glomerulonephritis with severe nephrotic syndrome induced by immune complexes composed of galactose-deficient IgA1 in primary Sjögren’s syndrome: a case report

et al. 2021

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Background Primary Sjögren’s syndrome (pSS) is an auto-immune disease characterized by sialadenitis and dacryoadenitis with lymphoplasmacytic cell infiltration. In pSS, not only sicca symptoms, but also extra-glandular involvement induced by immune abnormalities based on pSS occurs. Renal involvement is one such important life-threatening extra-glandular involvement. Although the aberrant glycosylated IgA in pSS as a product of over-activated B cells is a risk factor of renal involvement, its association has not been clarified. Here we report a case of glomerulonephritis (GN) induced by immune complexes (IC) composed of galactose-deficient IgA1 (Gd-IgA1) in a patient with pSS. Case presentation A 48-year-old Japanese woman with pSS was admitted to our hospital because of a two-month history of nephrotic syndrome. Seven years before she had been diagnosed with pSS from keratoconjunctivitis sicca, elevation of serum anti-Ro/SSA antibody titer and lymphoplasmacytic cell infiltration around salivary ducts of the small salivary glands. Renal biopsy revealed diffuse bubbling appearance in glomerular basement membrane (GBM) with scarce mesangial proliferation. Immunofluorescence showed granular IgA, C3 and Gd-IgA1 staining of GBM. Light chain staining showed no monoclonality. Electron microscopy showed electron dense deposits mainly in the intra-membranous and paramesangial areas and slightly in the subepithelial area. Additional serum analysis confirmed elevation of Gd-IgA1 (13.5 μg/mL), which was comparable with that seen in IgA nephropathy, and qualitative enzyme-linked immunosorbent assay of IgA-containing circulating immune complex (IgA-CIC) was positive. Thus, we diagnosed GN induced by IC composed of Gd-IgA1. Furthermore, retrospectively performed immunofluorescence of the small salivary gland evaluated at the diagnosis of pSS showed positive Gd-IgA1 staining of infiltrating lymphoplasmacytic cells. Therefore, we concluded that Gd-IgA1 produced by over-activated B cells in pSS formed circulating IC and thereby induced GN. After induction therapy with high dose prednisolone and mycophenolate mofetil, the nephrotic syndrome remitted within 3 weeks, the serum Gd-IgA1 level decreased to the normal range (3.8 μg/mL), and serum IgA-CIC disappeared in the 6th month after induction therapy. Conclusions Our findings clearly demonstrate an association between aberrant glycosylated IgA and the renal involvement seen in pSS, thereby helping to clarify the renal significance of aberrant glycosylated IgA in pSS.

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Section: Discussioncontrasting

confidence: 95%

Glomerulonephritis with severe nephrotic syndrome induced by immune complexes composed of galactose-deficient IgA1 in primary Sjögren’s syndrome: a case report

et al. 2021

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“…Of note, only two cases of IMN with solitary polyclonal IgA deposition have been described previously, namely by Kobayashi et al ( 7 ) in 2015 and by Sawamura et al ( 8 ) in 2019. Table summarizes the clinicopathological findings in the previously reported cases and our present case.…”

Section: Discussionmentioning

confidence: 99%

“…However, the involvement of different IG subclasses in the pathogenesis of MN is still not fully elucidated. To our knowledge, only two unusual IMN cases with solitary polyclonal IgA deposition have been reported by Japanese authors (2015 and 2019) ( 7 , 8 ). Findings from these two cases are consistent with regard to the main features of granular deposition of solitary polyclonal IgA on immunofluorescence and quick remission upon the administration of immunosuppressive induction therapy.…”

Section: Introductionmentioning

confidence: 94%

Idiopathic Membranous Nephropathy with Solitary Immunoglobulin A Deposition: A Case Report and a Review of the Literature

Huang

Yang

et al. 2022

Intern. Med.

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A 63-year-old man with an 8-year history of proteinuria was diagnosed with nephrotic syndrome, and a renal biopsy was performed. Light and electron microscopic analyses showed classic features of idiopathic membranous nephropathy (IMN). However, immunofluorescence tests revealed solitary polyclonal granular IgA deposition along the glomerular capillary walls, rather than IgG, which is often dominant in IMN. The combined use of corticosteroids and calcineurin inhibitor was noticeably effective in reducing proteinuria and improving edema in the current case. Two additional rare cases of IMN with solitary IgA deposition were reviewed, and long-term surveillance is still warranted to characterize its clinicopathological features and outcome.

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Membranous nephropathy with solitary polyclonal IgA deposition: A case report and literature review

Cited by 2 publications

References 10 publications

Glomerulonephritis with severe nephrotic syndrome induced by immune complexes composed of galactose-deficient IgA1 in primary Sjögren’s syndrome: a case report

Glomerulonephritis with severe nephrotic syndrome induced by immune complexes composed of galactose-deficient IgA1 in primary Sjögren’s syndrome: a case report

Idiopathic Membranous Nephropathy with Solitary Immunoglobulin A Deposition: A Case Report and a Review of the Literature

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