NELL1-Positive HIV-Associated Lupus-Like Membranous Nephropathy with Spontaneous Remission

Dinesh, Kumar P.; Charu, Vivek; Troxell, Megan L.; Andeen, Nicole K.

doi:10.1159/000525541

Cited by 5 publications

(4 citation statements)

References 14 publications

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“…An interesting study reported a possible association between mercury and NELL-1-associated MN [4]. NELL-1-associated MN has also been reported in patients with HIV infection [5].…”

Section: Discussionmentioning

confidence: 92%

A Rare Case of Neural Epidermal Growth Factor-Like 1 Protein (NELL-1) Antigen-Associated Membranous Nephropathy

Jiffry¹,

Pitts²,

Munir³

et al. 2023

Cureus

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Membranous nephropathy (MN) is an autoimmune disease resulting in nephrotic syndrome. Neural epidermal growth factor-like 1 protein (NELL-1) has been shown to cause a rare form of MN that is more likely to be associated with malignancy. We present a case of a 73-year-old female who was found to have a NELL-1-associated segmental MN. She presented complaining of generalized weakness, chills, and poor appetite, worsening over a one-week duration. Her kidney functions were noted to be markedly deranged, with a computed tomography scan of the abdomen showing evidence of chronic kidney disease. Further testing confirmed heavy proteinuria, although the etiology was still uncertain. A kidney biopsy revealed granular subepithelial immunoglobulin G deposits with subsequent immunohistochemical staining for NELL-1 antigen being positive. She improved with supportive care over the next few days. Despite an extensive workup, no underlying malignancy was found. NELL-1 is a rare yet recognized antigen target for the development of MN. Up to a third of patients with NELL-1-associated MN have associated cancer, thus requiring evaluation for underlying malignancy in this cohort.

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“…An interesting study reported a possible association between mercury and NELL-1-associated MN [4]. NELL-1-associated MN has also been reported in patients with HIV infection [5].…”

Section: Discussionmentioning

confidence: 92%

A Rare Case of Neural Epidermal Growth Factor-Like 1 Protein (NELL-1) Antigen-Associated Membranous Nephropathy

Jiffry¹,

Pitts²,

Munir³

et al. 2023

Cureus

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“…A third patient had an initial biopsy with PLA2R+ NELL1-MN (no serum available), treated conservatively, with relapse >10 years later and biopsy demonstrating dual PLA2R+ NELL1+ MN with corresponding serum antibodies to both PLA2R and NELL1 (37). In two cases, PLA2R glomerular staining is described as weak with an incomplete capillary loop distribution and was associated with a negative serum anti-PLA2R antibodies at the time of biopsy (35,45,49), which may represent false-positive tissue reactivity.…”

Section: Dual Pla2r and Nell1 Positive Mnmentioning

confidence: 99%

“…Of reported patients, 9 of 12 (75%) with oncologic remission also had remission of proteinuria ( 13 , 42 ), supporting this link. Other conditions include infections – such as human immune deficiency virus (HIV) ( 35 ), hepatitis B and C (HBV, HCV) ( 40 ), tuberculosis ( 43 ). MN in the setting of hematopoietic stem cell transplant and graft-versus-host disease (GVHD) can occur with the NELL1 antigen ( 29 ) although is much more commonly associated with the protocadherin FAT1 antigen ( 18 ).…”

Section: Clinicopathologic Studies Of Nell1 Mnmentioning

confidence: 99%

NELL1 membranous nephropathy: clinical associations provide mechanistic clues

Andeen,

Kung,

Avasare

2024

Front. Nephrol.

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Neural epidermal growth factor-like 1 (NELL1) membranous nephropathy (MN) is notable for its segmental deposit distribution, IgG1 dominant deposits, and comparatively high rate of spontaneous remission. It has been associated with a variety of exposures and secondary conditions, specifically use of thiol-containing medications – including lipoic acid, bucillamine, and tiopronin – as well as traditional indigenous medications (TIM) particularly those with high mercury content, and non-steroid anti-inflammatory drugs (NSAIDs). Malignancies, graft vs. host disease (GVHD), infection, and autoimmune conditions have also been associated with NELL1 MN. Herein, we provide a detailed summary of the clinicopathologic features of NELL1 and associations with underlying conditions, with a focus on treatment and outcomes. Rare cases of dual NELL1 and phospholipase A2 receptor (PLA2R) positive MN are reviewed. Genome-wide association study of NELL1, role of NELL1 in other physiologic and pathologic processes, and connection between NELL1 MN and malignancy with relevance of NELL1 tumor staining are examined. Finally, relationships and potential disease mechanisms of thiol- and mercury- associated NELL1 MN are discussed.

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“… 1 , 2 , 3 In 2019, 2 groups identified the NELL1 podocyte antigen in a subset of MN with distinct histologic and immunologic features, namely segmental or incomplete glomerular capillary loop involvement with predominant IgG1 staining. 4 , 5 NELL1 MN has also been identified in association with thiol-containing medications, namely lipoic acid, 6 , 7 bucillamine, 8 and tiopronin 9 ; traditional indigenous medications, including those with high mercury content 10 ; and with underlying conditions, including malignancy, 5 infections (including HIV 11 ), sarcoidosis, 12 hematopoietic stem cell transplant and graft-versus-host disease, 13 and rheumatoid arthritis. 8 …”

mentioning

confidence: 99%

NELL1-Positive HIV-Associated Lupus-Like Membranous Nephropathy with Spontaneous Remission

Cited by 5 publications

References 14 publications

A Rare Case of Neural Epidermal Growth Factor-Like 1 Protein (NELL-1) Antigen-Associated Membranous Nephropathy

A Rare Case of Neural Epidermal Growth Factor-Like 1 Protein (NELL-1) Antigen-Associated Membranous Nephropathy

NELL1 membranous nephropathy: clinical associations provide mechanistic clues

Characteristics and Outcomes of NELL1 Membranous Nephropathy in Lipoic Acid Users and Nonusers

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