Diffuse large B cell lymphoma in a preceding IgG4-related disease with kidney restricted lambda light chain expression: case report and literature review

Wang, Hui; Su, Tao; Liu, Kang; Yang, Li

doi:10.1186/s12882-020-01975-7

Cited by 9 publications

(8 citation statements)

References 31 publications

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“…Indeed, on the one hand, THS has been described in association with IgG4-RD (Lindfield et al 2012). On the other hand, evidence is accumulating that this chronic inflammatory state is associated with high incidence to lymphoma (Wang et al 2020). In particular, an association has been reported between DLBCL and IgG4-RD, notably with autoimmune pancreatitis or orbital inflammation (Wang et al 2020;Takahashi et al 2009;Peng et al 2020).…”

Section: Discussionmentioning

confidence: 99%

“…On the other hand, evidence is accumulating that this chronic inflammatory state is associated with high incidence to lymphoma (Wang et al 2020). In particular, an association has been reported between DLBCL and IgG4-RD, notably with autoimmune pancreatitis or orbital inflammation (Wang et al 2020;Takahashi et al 2009;Peng et al 2020). In the majority of reported cases, IgG4-RD occurred precedently or concurrently to the development of malignant lymphoma.…”

Section: Discussionmentioning

confidence: 99%

“…Therefore, we raised the hypothesis of an IgG4-RD. Indeed, IgG4-RD may cause an orbital inflammatory disease and an etiological link is suspected between IgG4-RD and lymphoma (Lindfield et al 2012;Wang et al 2020;Bledsoe et al 2018;Takahashi et al 2009;Peng et al 2020;Wallace et al 2016;Yamamoto et al 2012). Serological dosage of IgG4 was therefore performed and returned negative.…”

Section: Investigationsmentioning

confidence: 99%

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Painful ophthalmoplegia in a patient with a history of marginal zone lymphoma

Bogaert

Mathey

Vierasu

et al. 2021

European J Hybrid Imaging

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A 73-year-old man with a history of marginal zone lymphoma was admitted to the emergency room for diplopia and ipsilateral headache. The Fluorine-18-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) demonstrated intense and symmetrical hypermetabolism of the cavernous sinuses, and hypermetabolic lesions diffusely in the lymph nodes and bones. The diagnosis of high-grade relapse of lymphomatous disease was made. In this context, the homogenous and symmetric lesion of the cavernous sinuses, without any other encephalic or meningeal lesions, raised the hypothesis of a paraneoplastic origin. A plausible paraneoplastic link between the neuro-ophthalmological lesion and the malignant disorder is IgG4-related disease, a condition that may be associated with lymphoma. As in our case, this diagnosis is often presumptive because histopathological confirmation is difficult to obtain.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Investigationsmentioning

confidence: 99%

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Painful ophthalmoplegia in a patient with a history of marginal zone lymphoma

Bogaert

Mathey

Vierasu

et al. 2021

European J Hybrid Imaging

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“…Among patients with crescent formation, IgG1, IgG2, and IgG3 were positive in 37.5% of the patients, and IgG4 was positive in 45.0% of the patients. Examination of IF combined with electron microscopy revealed restricted λ-light chain expression in two patients [one in the glomeruli by IF, and the other in the interstitium (17) by EM] and κ-light chain in one. Renal pathology revealed that 19 patients (47.5%) had TBM deposits and 52.6% of them had glomerular lesions.…”

Section: General Pathological Featuresmentioning

confidence: 99%

“…Four patients had a history of carcinoma, and one each had a thymoma, carcinoma of the ureter, clear cell renal cell carcinoma, and tubular adenocarcinoma. One patient was diagnosed with extranodal marginal zone lymphoma (17). Five patients had monoclonal immunoglobulinemia, namely, IgG-κ (n = 3) and IgG-λ (n = 2).…”

Section: Clinical Featuresmentioning

confidence: 99%

Clinicopathological Patterns and Predictors of the Functional Restoration of Immunoglobulin G4-Related Kidney Disease: A Chinese Single-Center Cohort Study

Wang

Yang

2021

Front. Med.

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Background: Immunoglobulin G4-related disease (IgG4-RD) is a systemic immunoreactivity-based fibro-inflammatory disease. Immunoglobulin G4-related kidney disease (IgG4-RKD) is a frequently overlooked diagnosis. This study aimed to describe IgG4-RKD and examine the factors relevant to the renal outcomes of IgG4-RD.Methods: We studied a prospective IgG4-RKD cohort between January 2012 and December 2020 with close follow-up. Clinicopathologic data at kidney biopsy were collected and analyzed. We aimed to explore independent risk factors for long-term renal outcome and disease relapse. Patients with an eGFR<45 ml/min per 1.73m2 at 12 months were defined as having poor outcomes.Results: The included 42 patients with IgG4-RKD had a mean age of 58.5 ± 8.7 years (male-to-female ratio = 5:1). The IgG4-RD responder index (RI) was 12.2 ± 3.3. A total of 66.7% of the patients presented with acute on kidney disease or acute on chronic kidney disease. Eight patients (19.0%) showed nephrotic-range proteinuria, and nine (21.4%) had high-titer IgG4-autoantibodies, including antineutrophil cytoplasmic antibody and anti-phospholipase A2 receptor. A kidney biopsy was conducted in 40 patients. Thirty-seven (90.0%) patients were diagnosed with IgG4-related tubulointerstitial nephritis, and 19 (47.5%) of them had concurrent glomerular diseases (membranous nephropathy [MN], n = 3; crescentic glomerulonephritis [CrGN], n = 11; diabetic kidney disease, n = 3; and both MN and CrGN, n = 2). IgG4-RD RI had a close relationship with serum C3 (R = −0.509, P = 0.001), C4 (R = −0.314, P = 0.049) levels, and peripheral blood eosinophil count (PBEC; R = 0.377, P = 0.024), factors that were not included in RI scores. Correlation analysis disclosed that IgG4-RD RI (R = 0.422, P = 0.007), organs involved (R = 0.452, P = 0.003), and C3 (R = −0.487, R = 0.002) were correlated with the percentage decrease of serum creatinine at 1 month. However, multivariate regression analysis failed to identify any clinicopathological parameters that could predict short-term renal restoration and IgG4-RKD relapse. Ten out of 29 variables, of most importance, were identified by the least absolute shrinkage and selection operator (LASSO) regression analysis. By multivariate logistic regression a higher serum IgG4 (OR = 0.671, P = 0.010), IgG1 (OR = 1.396, P = 0.049), IgG3 (OR = 19.154, P = 0.039), and erythrocyte sedimentation rate (ESR; OR = 1.042, P = 0.032) were found to be independent factors for poor long-term outcome. Conventional immunosuppressive medications and/or rituximab were prescribed, and in 83.3% of the patients, the kidney function improved. Repeat kidney biopsies confirmed the remission of interstitial inflammation in two patients under immunosuppressive therapy. However, the disease relapse rate was as high as 31.0%.Conclusions: We strongly recommend a kidney biopsy in active IgG4-RD, especially when there is proteinuria and renal dysfunction, because concurrent glomerular involvement and active interstitial inflammation should be assessed. A higher serum IgG1, IgG3, and ESR were independent factors for the poor long-term renal outcome; however, elevated IgG4 predicted a good renal prognosis, and appropriate and timely immunosuppressive therapy can help achieve a better prognosis.

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