The pathogenesis of renal injury and treatment in light chain deposition disease

Wang, Qi; Jiang, Fuming; Xu, Gaosi

doi:10.1186/s12967-019-02147-4

Cited by 11 publications

(11 citation statements)

References 56 publications

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“…Amyloid nephropathy is easily confused with other renal diseases like as broid glomerulonephritis (25), immune vibrissae glomerulopathy (26), collagen type III glomerular disease (27), and light chain deposition disease (28), all of which present with proteinuria and edema, and there is broid or granular material deposition under electron microscopy, which has been poorly recognized by doctors in the early years, leading to easy misdiagnosis in clinical practice. These diseases can be differentiated by Congo red staining and electron microscopy.…”

Section: Discussionmentioning

confidence: 99%

Pathological, Clinical Characteristics and Prognostic Analysis of Amyloid Nephropathy

Zhao

Wang

2020

Preprint

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Background: The number of patients diagnosed with renal amyloidosis is increasing year by year, and the pathological grade and prognostic factors are not clear. This study attempts to investigate the pathological features of renal amyloidosis and the prognostic factors in patients with amyloidosis. Method：To evaluate the pathological sections of 184 patients with renal amyloidosis in the Department of Pathology, Shandong University, and to retrospectively analyze the clinical data of 175 patients with amyloidosis in our center and to follow up them. Results: There was a positive correlation between different pathological grades and creatinine levels (rs = 0.275, P = 0.38), the higher the pathological grade, the higher the incidence of renal insufficiency (χ2 = 8.44, P = 0.015); the more severe the tubulointerstitial damage, the higher the incidence of renal insufficiency (χ2 = 14.15, P = 0.007); different degrees of tubulointerstitial damage and glomerulosclerosis affected the urinary protein quantification, and the difference was statistically significant (P < 0.05). The treatment effect of older is worse (P=0.030，OR=1.040，95%CI=1.004，1.078) and the effect of selecting an effective treatment is better (P = 0.002, OR = 0.618, 95% CI = 0.456, 0.839). Hypotension, cardiac involvement, decreased serum albumin, and increased TnT were independent risk factors for survival; while receiving effective treatment modalities was an independent protective factor.Conclusion: The pathological grade of renal amyloidosis is associated with the incidence of renal insufficiency, in which the degree of tubulointerstitial chronic lesions significantly affects renal function.

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Section: Discussionmentioning

confidence: 99%

Pathological, Clinical Characteristics and Prognostic Analysis of Amyloid Nephropathy

Zhao

Wang

2020

Preprint

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“…Renal failure is a hallmark of LCDD, with the majority of LCDD patients developing progressive renal failure from deposition of light chains in tissue 7. This patient had pre-existing chronic kidney disease stage 3, and careful thought was given to performing a renal biopsy.…”

Section: Differential Diagnosismentioning

confidence: 99%

Sjögren’s syndrome as a cause of both lymphoid interstitial pneumonia and light chain deposition disease in a single patient

Steward

Gibbons

2022

BMJ Case Rep

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A man in his 70s presents with 12 months of progressive dyspnoea, sicca symptoms and Raynaud’s phenomenon. Serological testing and tear duct biopsy confirm Sjögren’s syndrome (SS). Bilateral nodular-cystic appearances highly suggestive of lymphoid interstitial pneumonia (LIP) are noted on high-resolution computed tomography (HRCT), supported by a 40% lymphocytosis on bronchoalveolar lavage.Biopsy of a non-characteristic additional pulmonary nodule diagnoses light chain deposition disease (LCDD). Extrapulmonary organ involvement is excluded. Pulmonary function tests are well-preserved, and the patient is kept under active surveillance without requiring immunomodulatory treatment.LIP and LCDD both have a strong association with SS. Identification of these disease associations is crucial as they may result in multiorgan involvement or progression to haematological malignancy. This is the first case published in the literature and highlights that a pragmatic approach to investigations can avoid unnecessary procedures, and that treatment may be guided by symptomology.

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“…Waldenstrom macroglobulinemia, and other B-cell lymphomas also produce excess free light chains in addition to intact immunoglobulin's. AL amyloidosis and light chain deposition disorders are associated with variable excess monoclonal light chains detectable in serum and urine [10,16,17].…”

Section: Identification and Measurement Of Monoclonal Serum Free Ligh...mentioning

confidence: 99%

Monoclonal Light Chains in Multiple Myeloma: The Sinister Immunoglobulin

Gurmukh¹,

Xu²,

J³

2022

Int J Pathol Clin Res

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Multiple myelomas are the commonest hematological malignancy in adults, next to the heterogeneous group non-Hodgkin lymphomas, and account for about 10% of such tumors. About 21% of the multiple myelomas are associated with higher levels of free monoclonal light chains. This subgroup of patients exhibits high incidence of renal disease manifested by significantly lower estimated glomerular filtration rate, higher incidence of dialysis and significantly shorter survival.We have defined criteria for identification of high risk patients with light chain only as well as intact immunoglobulin myelomas. Kappa and lambda light chain specific criteria have been enunciated for intact immunoglobulin multiple myelomas and the need for development of similar criteria for light chain only myelomas has been identified.

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The pathogenesis of renal injury and treatment in light chain deposition disease

Cited by 11 publications

References 56 publications

Pathological, Clinical Characteristics and Prognostic Analysis of Amyloid Nephropathy

Pathological, Clinical Characteristics and Prognostic Analysis of Amyloid Nephropathy

Sjögren’s syndrome as a cause of both lymphoid interstitial pneumonia and light chain deposition disease in a single patient

Monoclonal Light Chains in Multiple Myeloma: The Sinister Immunoglobulin

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