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


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Article Info

DATE

2021-03-25

AUTHORS

Ryo Nishioka, Satoshi Hara, Hiroyuki Kawahara, Kiyoaki Ito, Ichiro Mizushima, Masayoshi Hirata, Michio Nagata, Mitsuhiro Kawano

ABSTRACT

BackgroundPrimary 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 presentationA 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.ConclusionsOur 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. More... »

PAGES

108

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Identifiers

URI

http://scigraph.springernature.com/pub.10.1186/s12882-021-02306-0

DOI

http://dx.doi.org/10.1186/s12882-021-02306-0

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1136669228

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/33765955


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50 basement membrane
51 biopsy
52 case report
53 cases
54 cases of glomerulonephritis
55 cell infiltration
56 cells
57 complexes
58 dense deposits
59 deposits
60 diagnosis
61 diagnosis of pSS
62 diffuse
63 disease
64 dose prednisolone
65 duct
66 electron microscopy
67 electron-dense deposits
68 elevation
69 enzyme-linked immunosorbent
70 extra-glandular involvement
71 factors
72 findings
73 galactose-deficient IgA1
74 gland
75 glomerular basement membrane
76 glomerulonephritis
77 granular IgA
78 high-dose prednisolone
79 history
80 hospital
81 immune abnormalities
82 immune complexes
83 immunofluorescence
84 immunosorbent
85 induction therapy
86 infiltration
87 involvement
88 keratoconjunctivitis sicca
89 levels
90 light chain staining
91 lymphoplasmacytic cell infiltration
92 lymphoplasmacytic cells
93 membrane
94 mesangial proliferation
95 microscopy
96 mofetil
97 monoclonality
98 months
99 mycophenolate mofetil
100 nephropathy
101 nephrotic syndrome
102 normal range
103 occurs
104 paramesangial area
105 patients
106 prednisolone
107 primary Sjögren's syndrome
108 products
109 proliferation
110 qualitative enzyme-linked immunosorbent
111 range
112 renal biopsy
113 renal involvement
114 renal significance
115 report
116 risk factors
117 salivary ducts
118 salivary glands
119 serum Gd-IgA1 levels
120 serum analysis
121 severe nephrotic syndrome
122 sialadenitis
123 sicca
124 sicca symptoms
125 significance
126 small salivary glands
127 staining
128 subepithelial area
129 symptoms
130 syndrome
131 therapy
132 titers
133 two-month history
134 weeks
135 women
136 years
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