IgA nephropathy in the patient with systemic lupus erythematosus in remission

Fujikura, Emi; Kimura, Tomoyoshi; Otaka, Akihiko; Arima, Shuji; Satoh, Hiroshi; Itoh, Sadayoshi; Hirabayashi, Yasuhiko; Sasaki, Takeshi; Stoh, T

doi:10.2169/naika.91.3282

“…Approximately 0.3% (9 out of 251) ESRD in SLE patients is attributed to nonlupus nephritis [ 20 ]. IgA nephropathy in SLE patients has been previously described in a total of 10 patients from 8 reports [ 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 ]. One patient had 2 sequential kidney biopsies, lupus nephritis class II on the initial biopsy, and IgA nephropathy on the follow-up biopsy after treatment [ 9 ].…”

Section: Discussionmentioning

confidence: 99%

“…IgA nephropathy has been reported in patients with SLE, although rare [ 5 , 6 , 7 , 8 , 9 , 10 , 11 , 12 ]. IgA nephropathy, the most common primary glomerulonephritis globally, is characterized by glomerular deposition of polymeric IgA1 with aberrant glycosylation at the hinge region O glycans (Gd-IgA1) and the autoantibody formation against this region.…”

Section: Introductionmentioning

confidence: 99%

Diagnostic Utility of Galactose-Deficient Immunoglobulin A1 Immunostaining in the Differentiation of Lupus Nephritis and Immunoglobulin A Nephropathy

Bu

¹

,

Ye

²

,

Kouri³

et al. 2021

Glomerular Dis

0

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Background: Renal biopsy plays an important role in the establishment of the diagnosis and the management of patients with lupus nephritis. Immunoglobulin A (IgA) nephropathy rarely has been reported in kidney biopsy of lupus patients. Lupus nephritis and IgA nephropathy can be readily diagnosed on renal biopsy when the classic patterns are present. However, atypical patterns can become a diagnostic challenge. Galactose-deficient IgA1 (Gd-IgA1) is a key element in the pathogenesis of primary IgA nephropathy. Glomerular Gd-IgA1 deposits, detected by immunofluorescent staining of KM-55 (a Gd-IgA1-specific monoclonal antibody), are consistently identified in the mesangium of IgA nephropathy but are significantly less or absent in lupus nephritis accompanied by significant IgA deposition. Case Presentation: Here we report the case of an 11-year-old girl who was recently diagnosed with systemic lupus erythematosus (SLE) and was found to have hematuria and proteinuria. Renal biopsy showed focal mesangial hypercellularity with IgA dominant, “full house” like pattern of mesangial deposition. The biopsy findings present a diagnostic dilemma with the differential diagnosis of IgA nephropathy versus lupus nephritis with atypical immunofluorescence, and IgA nephropathy is favored, in the absence of strong straining of C1q or C3, extraglomerular deposits, tissue antinuclear antibodies, and endothelial tubuloreticular inclusions. However, no detectable glomerular KM-55 staining was seen in the kidney biopsy. Conclusions: We demonstrate the unique diagnostic utility of immunostaining for KM-55 in a challenging kidney biopsy of an SLE patient with features suggestive of IgA nephropathy. The absence of KM-55 staining excludes IgA nephropathy, supporting a diagnosis of lupus nephritis with atypical immunofluorescence in this patient with SLE.

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Nefropatia por IgA em paciente portadora de lúpus eritematoso sistêmico: relato de caso e revisão de literatura

Silva

¹

,

Almeida

²

,

Melo

³

et al. 2016

Revista Brasileira de Reumatologia

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Systemic erythematosus lupus (SLE) is a multisystemic autoimmune disease which has nephritis as one of the most striking manifestations. Although it can coexist with other autoimmune diseases, and determine the predisposition to various infectious complications, SLE is rarely described in association with non-lupus nephropathies etiologies. We report the rare association of SLE and primary IgA nephropathy (IgAN), the most frequent primary glomerulopathy in the world population. The patient was diagnosed with SLE due to the occurrence of malar rash, alopecia, pleural effusion, proteinuria, ANA 1: 1280, nuclear fine speckled pattern, and anticardiolipin IgM and 280U/mL. Renal biopsy revealed mesangial hypercellularity with isolated IgA deposits, consistent with primary IgAN. It was treated with antimalarial drug, prednisone and inhibitor of angiotensin converting enzyme, showing good progress. Since they are relatively common diseases, the coexistence of SLE and IgAN may in fact be an uncommon finding for unknown reasons or an underdiagnosed condition. This report focus on the importance of the distinction between the activity of renal disease in SLE and non-SLE nephropathy, especially IgAN, a definition that has important implications on renal prognosis and therapeutic regimens to be adopted in both the short and long terms.

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IgA nephropathy in systemic lupus erythematosus patients: case report and literature review

Silva¹,

Almeida²,

Melo³

et al. 2016

Revista Brasileira de Reumatologia (English Edition)

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Systemic erythematosus lupus (SLE) is a multisystemic autoimmune disease which has nephritis as one of the most striking manifestations. Although it can coexist with other autoimmune diseases, and determine the predisposition to various infectious complications, SLE is rarely described in association with non-lupus nephropathies etiologies. We report the rare association of SLE and primary IgA nephropathy (IgAN), the most frequent primary glomerulopathy in the world population. The patient was diagnosed with SLE due to the occurrence of malar rash, alopecia, pleural effusion, proteinuria, ANA 1: 1280, nuclear fine speckled pattern, and anticardiolipin IgM and 280U/mL. Renal biopsy revealed mesangial hypercellularity with isolated IgA deposits, consistent with primary IgAN. It was treated with antimalarial drug, prednisone and inhibitor of angiotensin converting enzyme, showing good progress. Since they are relatively common diseases, the coexistence of SLE and IgAN may in fact be an uncommon finding for unknown reasons or an underdiagnosed condition. This report focus on the importance of the distinction between the activity of renal disease in SLE and non-SLE nephropathy, especially IgAN, a definition that has important implications on renal prognosis and therapeutic regimens to be adopted in both the short and long terms.

show abstract

IgA nephropathy in the patient with systemic lupus erythematosus in remission

Cited by 7 publications

References 0 publications

Diagnostic Utility of Galactose-Deficient Immunoglobulin A1 Immunostaining in the Differentiation of Lupus Nephritis and Immunoglobulin A Nephropathy

Diagnostic Utility of Galactose-Deficient Immunoglobulin A1 Immunostaining in the Differentiation of Lupus Nephritis and Immunoglobulin A Nephropathy

Nefropatia por IgA em paciente portadora de lúpus eritematoso sistêmico: relato de caso e revisão de literatura

IgA nephropathy in systemic lupus erythematosus patients: case report and literature review

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