Which should be the correct treatment for monoclonal gammopathy of renal significance with complement alternative pathway dysregulation (C3 glomerulopathy and atypical hemolytic uremic syndrome): clone-directed or anticomplement therapy?

Ciocchini, Mariana; Musso, Carlos Guido

doi:10.1007/s11255-018-1896-8

Cited by 6 publications

(4 citation statements)

References 11 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Recent reports described an unusually high prevalence of MG in C3GN and TMA patients compared with the age-matched general population (Table 2). 11,12,44,45 Hypothetically, the monoclonal protein acts as an autoantibody against complement regulatory proteins, especially complement factor H, or as a stabilizer C3 convertase (C3 nephritic factor), resulting in sustained C3 activation. These mechanisms can coexist in the same patient.…”

Section: Indirect Mechanismsmentioning

confidence: 99%

“…These mechanisms can coexist in the same patient. 44,45 POEMS syndrome. Microangiopathic renal lesions seen in this syndrome are thought to be mediated by vascular endothelial growth factor released by the clonal plasma cells.…”

Section: Indirect Mechanismsmentioning

confidence: 99%

“…clinical information should be available because not all MGRS lesions demonstrate monoclonal protein deposition in kidney (for instance, C3G and TMA) and vice versa. 3,12,44,45 To this end, we will discuss the step-wise diagnostic approach to MGRS, pitfalls in currently available tests, and challenges faced by clinicians while dealing with a suspected case of MGRS.…”

Section: Clinical Presentationmentioning

confidence: 99%

See 2 more Smart Citations

Pathophysiology and management of monoclonal gammopathy of renal significance

et al. 2019

View full text Add to dashboard Cite

Recent years have witnessed a rapid growth in our understanding of the pathogenic property of monoclonal proteins. It is evident that some of these small monoclonal proteins are capable of inducing end-organ damage as a result of their intrinsic physicochemical properties. Hence, an umbrella term, monoclonal gammopathy of clinical significance (MGCS), has been coined to include myriad conditions attributed to these pathogenic proteins. Because kidneys are the most commonly affected organ (but skin, peripheral nerves, and heart can also be involved), we discuss MGRS exclusively in this review. Mechanisms of renal damage may involve direct or indirect effects. Renal biopsy is mandatory and demonstration of monoclonal immunoglobulin in kidney, along with the corresponding immunoglobulin in serum or urine, is key to establish the diagnosis. Pitfalls exist at each diagnostic step, and a high degree of clinical suspicion is required to diagnose MGRS. Recognition of MGRS by hematologists and nephrologists is important, because timely clone-directed therapy improves renal outcomes. Autologous stem cell transplant may benefit selected patients.

show abstract

Section: Indirect Mechanismsmentioning

confidence: 99%

Section: Indirect Mechanismsmentioning

confidence: 99%

Section: Clinical Presentationmentioning

confidence: 99%

See 1 more Smart Citation

Pathophysiology and management of monoclonal gammopathy of renal significance

et al. 2019

View full text Add to dashboard Cite

show abstract

“…C3 glomerulonephritis is a typical MGRS-related disease primarily caused by dysregulation of the alternative complement pathway. Recent studies have reported an unusually high prevalence of MIg and TMA in patients with C3 glomerulonephritis compared to the age-matched general population ( 29 , 30 ). In our study, we observed one patient with MGRS had typical renal pathology of C3 glomerulonephritis combined with TMA.…”

Section: Discussionmentioning

confidence: 97%

Clinicopathological features and individualized treatment of kidney involvement in B-cell lymphoproliferative disorder

et al. 2022

View full text Add to dashboard Cite

BackgroundDue to the various clinical and pathological manifestations of kidney involvement in lymphoproliferative disorder (LPD), the whole spectrum of kidney disease in LPD is still unclear, and data on kidney prognosis is scarce.MethodsWe retrospectively reviewed the renal pathology profiles from January 2010 to December 2021, and 28 patients with B-cell LPD combined with intact renal biopsy data were included.ResultsThere were 20 men and eight women aging 41 to 79 years at the time of renal biopsy (median age 62 years). According to hematological diagnosis, patients were classified into four groups: chronic lymphocytic leukemia (CLL) (group1, n=7), Waldenström macroglobulinemia/lymphoplasmacytic lymphoma (WM/LPL) (group 2, n=8; WM, n=6; LPL, n=2), Other non-Hodgkin’s lymphomas (NHL) (group3, n=7; diffuse large B-cell lymphoma (DLBCL), n=2; mucosa-associated lymphoid tissue (MALT) lymphoma, n=4; Low grade B-cell lymphoma, n=1), and monoclonal gammopathy of undetermined significance/monoclonal gammopathy of renal significance (MGUS/MGRS) (group 4, n=6). Median serum creatinine (Scr) level was 129 (range,59-956) umol/L. Eight patients (29%) were presented with acute kidney injury (AKI), and five patients (18%) required hemodialysis upon admission. Twenty-three patients (82%) presented with proteinuria (median protein excretion, 2.14 g/d), 11(39%) of whom had the nephrotic syndrome. Interstitial malignant infiltration was the most frequent renal lesion (n=6). Eight patients underwent immunohistochemistry of renal tissues, of which three patients (CLL, n=1; LPL, n=1; WM, n=1) had confirmed lymphoma infiltrates, and the infiltrating cells in the remaining five patients (CLL, n=1; MALT lymphoma, n=2; MGUS, n=2) were considered unrelated to lymphoma. The most common glomerular diseases were renal amyloidosis (n=4) and membranous nephropathy (n=4). Only 20 patients were treated, 13 of whom were treated with rituximab separately or in combination. The median follow-up time was 11 months. Of these, six had achieved hematological response, complete response in five cases. Eight had achieved renal response. At the end-of-study visit, four patients died and two progressed to end stage kidney disease (ESKD).ConclusionIn conclusion, the clinicopathological spectrum of renal involvement in BLPD is diverse. Renal biopsy and immunohistochemistry are required for early diagnosis and prognostic assessment.

show abstract