Monoclonal IgG1κ Anti–Glomerular Basement Membrane Disease: A Case Report

Coley, Shana M.; Shirazian, Shayan; D’Agati, Vivette D.

doi:10.1053/j.ajkd.2014.08.022

Cited by 23 publications

(32 citation statements)

References 31 publications

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“…Another important deviation from classic anti-GBM disease is that half of our cases were due to monotypic anti-GBM antibody deposition (Table 3), which in some instances may not be as effective in complement activation as polytypic antibodies typical of classic anti-GBM disease. 17 Although there appear to be clinical and pathological differences between polytypic and monotypic atypical anti-GBM nephritis, including the degree of proteinuria, the frequency of nephrotic syndrome, and the presenting serum creatinine, none of the specific clinical or histopathologic features were statistically different between these two groups, likely because of the small sample size. Still, these 2 variants of atypical anti-GBM nephritis may represent different entities with a different pathogenesis.…”

Section: Discussionmentioning

confidence: 87%

The clinicopathologic characteristics and outcome of atypical anti-glomerular basement membrane nephritis

Nasr

Collins

Alexander

et al. 2016

Kidney International

111

150

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Classic anti-glomerular basement membrane (GBM) disease presents with rapidly progressive glomerulonephritis (GN) with or without pulmonary hemorrhage. On biopsy typical disease displays bright polytypic linear GBM staining for IgG by immunofluorescence and diffuse crescentic/necrotizing GN on light microscopy. Here, we studied 20 patients with atypical anti-GBM nephritis typified by bright linear GBM staining for immunoglobulins but without a diffuse crescentic phenotype. Patients had hematuria, proteinuria, and mild renal insufficiency, without pulmonary hemorrhage. Light microscopy showed endocapillary proliferative GN in 9 patients, mesangial proliferative GN in 6, membranoproliferative GN in 3, and focal segmental glomerulosclerosis with mesangial hypercellularity in 2. Eight of the 20 showed features of microangiopathy. Crescents/necrosis were absent in 12 and were focal in 8 patients. Bright linear GBM staining for IgG was seen in 17 patients, IgM in 2, and IgA in 1 patient, which was polytypic in 10 patients and monotypic in 10 patients. No circulating α3NC1 antibodies were detected by commercial ELISA. The 1-year patient and renal survival rates were 93% and 85%, respectively. Thus, atypical anti-GBM nephritis is a rare variant of anti-GBM disease characterized clinically by an indolent course, no pulmonary involvement, and undetectable circulating α3NC1 antibodies. Further studies are needed to characterize the molecular architecture of GBM autoantigens in these patients and establish optimal therapy.

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Section: Discussionmentioning

confidence: 87%

The clinicopathologic characteristics and outcome of atypical anti-glomerular basement membrane nephritis

Nasr

Collins

Alexander

et al. 2016

Kidney International

111

150

View full text Add to dashboard Cite

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“…There is linear GBM staining for k-and l-light chains with similar intensity. Rarely, linear staining for monoclonal IgG occurs, 47 or linear IgA rather than IgG may be present, indicating an IgA class of anti-GBM antibodies. 48 C3 is often present in a semilinear or granular pattern along the glomerular capillary walls.…”

Section: Key Disease-specific Commentsmentioning

confidence: 99%

Mayo Clinic/Renal Pathology Society Consensus Report on Pathologic Classification, Diagnosis, and Reporting of GN

Sethi

Haas

Markowitz

et al. 2015

JASN

Self Cite

237

153

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Renal pathologists and nephrologists met on February 20, 2015 to establish an etiology/pathogenesis-based system for classification and diagnosis of GN, with a major aim of standardizing the kidney biopsy report of GN. On the basis of etiology/pathogenesis, GN is classified into the following five pathogenic types, each with specific disease entities: immune-complex GN, pauci-immune GN, antiglomerular basement membrane GN, monoclonal Ig GN, and C3 glomerulopathy. The pathogenesis-based classification forms the basis of the kidney biopsy report. To standardize the report, the diagnosis consists of a primary diagnosis and a secondary diagnosis. The primary diagnosis should include the disease entity/pathogenic type (if disease entity is not known) followed in order by pattern of injury (mixed patterns may be present); score/grade/class for disease entities, such as IgA nephropathy, lupus nephritis, and ANCA GN; and additional features as detailed herein. A pattern diagnosis as the sole primary diagnosis is not recommended. Secondary diagnoses should be reported separately and include coexisting lesions that do not form the primary diagnosis. Guidelines for the report format, light microscopy, immunofluorescence microscopy, electron microscopy, and ancillary studies are also provided. In summary, this consensus report emphasizes a pathogenesis-based classification of GN and provides guidelines for the standardized reporting of GN.

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“…In the scenario of focal crescents, it is very important to remember passive adsorption of IgG linear staining as highlighted by Coley et al . [10] We can definitely exclude this possibility in our case by virtue of serum paraprotein and more importantly positive linear pattern for >1 antisera (IgG, IgG1, and kappa).…”

Section: Discussionmentioning

confidence: 69%

“…Bone marrow in our case is similar to those described in previous cases of monoclonal anti-GBM nephritis. [5678910] The percentages of plasma cells were within the normal range (<10% plasma cells). In the scenario of focal crescents, it is very important to remember passive adsorption of IgG linear staining as highlighted by Coley et al .…”

Section: Discussionmentioning

confidence: 89%

“…have recently described a patient with monoclonal IgG1-kappa anti-GBM antibody-related glomerulonephritis without a circulating antibody despite repeat testing by multiplex bead assay. [10] Renal histology was that of diffuse endocapillary and exudative glomerulonephritis without crescents in twenty glomeruli sampled on first instance. Myeloma workup including serum and urine paraprotein, bone marrow biopsy, and serum-free light chain ratio were normal.…”

Section: Discussionmentioning

confidence: 99%

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Circulating monoclonal IgG1-kappa antibodies causing anti-glomerular basement membrane nephritis

Vankalakunti¹,

Nada²,

Kumar³

et al. 2017

Indian J Nephrol

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Anti-glomerular basement membrane (GBM) antibody disease is a rare but well-characterized cause of glomerulonephritis. Patients present with rapidly progressive renal failure with hemoptysis. Early diagnosis is crucial in salvaging the renal damage and life-threatening pulmonary hemorrhage. Plasmapheresis and immunosuppression is the mode of therapy. Anti-GBM antibodies are polyclonal in nature. However, rare monoclonal antibodies can cause similar destruction of glomerular capillary walls. We describe distinct combination of circulating monoclonal and anti-GBM nephritis.

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Monoclonal IgG1κ Anti–Glomerular Basement Membrane Disease: A Case Report

Cited by 23 publications

References 31 publications

The clinicopathologic characteristics and outcome of atypical anti-glomerular basement membrane nephritis

The clinicopathologic characteristics and outcome of atypical anti-glomerular basement membrane nephritis

Mayo Clinic/Renal Pathology Society Consensus Report on Pathologic Classification, Diagnosis, and Reporting of GN

Circulating monoclonal IgG1-kappa antibodies causing anti-glomerular basement membrane nephritis

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