The Efficacy of Rituximab in the Treatment of Membranous Nephropathy

Maharjan, Ruby; Wang, Jin Wen; Shrestha, Indra Kumar

doi:10.33314/jnhrc.v18i4.2481

Cited by 3 publications

(2 citation statements)

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“…Autoantibodies in PMN may be mostly derived from CD20 or CD38. Lowering CD20/38 levels in patients with high anti-PLA2R antibody titres may be a bene cial strategy [34,35]. Furthermore, CD38 is an excellent target for the therapy of AL amyloidosis [36].…”

Section: Discussionmentioning

confidence: 99%

Primary membranous nephropathy combined with ALECT-2 renal amyloidosis：a case report

Yang,

Yan,

Liu

2023

Preprint

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Background Primary membranous nephropathy (PMN) is an autoimmune kidney disease and the leading cause of nephrotic syndrome in adults. It is characterized by the accumulation of immune deposits leading to glomerular basement membrane dysfunction caused by the deposition of subepithelial immune complexes. Amyloidosis is a rare group of diseases characterized by abnormal protein folding and extracellular deposition of insoluble protofibrils. It can be limited to one organ system or affect the entire body. In systemic amyloidosis, the kidney is the most commonly affected organ, often leading to renal failure and nephrotic syndrome. PMN combined with renal amyloidosis without secondary factors is rare. In this study, we report a case of PMN combined with amyloid nephropathy with only interstitial immunoglobulin light chain deposits. Case reportThis article reports a case of membranous nephropathy associated with ALECT-2 amyloidosis with nephrotic syndrome. A 62-year-old woman with the nephrotic syndrome had positive antiphospholipase A2 receptor (PLA2R) antibodies and a renal biopsy suggesting stage II membranous nephropathy, but a few focal deposits of faintly stained material were seen in the interstitium with positive expression of PAS and Congo red. It is rare for membranous nephropathy to be complicated by ALECT-2 protein deposition, and even rarer for it to be deposited only in the interstitium. Mass spectrometry can be used clinically as an aid to diagnosis and treatment is based on the treatment of primary glomerular disease with supportive therapy for ALECT-2 renal amyloidosis. Conclusion The combination of PMN and amyloidosis is rare and attention should be paid to the subtype of the disease and the exclusion of secondary factors. A thorough clinical and pathological examination will help in the classification and staging of the disease. Detection of serum anti-PLA2R antibodies and glomerular PLA2R antigen is helpful in the diagnosis of PMN. ALECT-2 amyloidosis has a relatively benign progression and renal biopsy is helpful in the diagnosis. For amyloidosis with unknown typing, further typing can be refined with genetic testing or mass spectrometry. We look forward to finding novel therapeutic options that can target both PMN and ALECT-2 amyloid nephropathy in the future.

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Section: Discussionmentioning

confidence: 99%

Primary membranous nephropathy combined with ALECT-2 renal amyloidosis：a case report

Yang,

Yan,

Liu

2023

Preprint

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“…CD20 or CD38 may be the main source of autoantibodies in IMN. For patients with high anti-PLA2R antibody titers, reducing CD20/38 may be an effective intervention ( 34 , 35 ). At the same time, CD38 is also an ideal target for the treatment of amyloidosis ( 36 ).…”

Section: Discussionmentioning

confidence: 99%

Idiopathic membranous nephropathy with renal amyloidosis: A case report

Wang

Wang²,

Yu³

et al. 2022

Front. Med.

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BackgroundImmunoglobulin light chain amyloidosis is a clonal, non-proliferative plasma cell disorder, in which fragments of immunoglobulin light chain are deposited in tissues. Clinical features depend on organs involved but can include restrictive cardiomyopathy, nephrotic syndrome, hepatic failure, peripheral/autonomic neuropathy, and atypical multiple myeloma. Membranous nephropathy (MN) is a group of diseases characterized by deposition of immune complexes under the epithelial cells of glomerular basement and diffuse thickening of the basement membrane. Most patients with idiopathic MN (IMN) have been exposed to phospholipase A2 receptor (PLA2R) antigen, and anti-PLA2R antibodies that attack podocytes can be detected in their blood. IMN combined with amyloidosis nephropathy without secondary factors is rare. The present study describes a patient with IMN combined with immunoglobulin light chain amyloidosis nephropathy.Case reportA 39-year-old man was admitted to our hospital because of weight loss and edema. His clinical manifestation was nephrotic syndrome. Renal pathology revealed MN. A positive Congo red staining and the pathognomonic apple-green birefringence under cross-polarized light were considered to be associated with amyloid nephropathy. Immunofluorescence showed that λ light chain was positive. Heavy chain deposition disease and amyloid-associated protein amyloidosis were excluded by immunofluorescence and immunohistochemistry, respectively. Subsequent examinations showed that his serum was negative for antibodies against the PLA2R, but PLA2R was present in renal tissue. The final diagnosis was IMN with light chain amyloid nephropathy.ConclusionRenal amyloidosis accompanied by IMN is uncommon. Attention should be paid to the subtype of the disease and the exclusion of secondary factors. Perfect clinical and pathological examination are helpful for the classification and staging of the disease. Congo red staining, light microscopy, immunofluorescence, immunohistochemistry, electron microscopic examination, pathological tissue staining for PLA2R antigen and testing for anti-PLA2R antibody in serum are helpful.

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