In Russia, PMN is a common type of glomerulopathy, the specific features of which should include the low rates of spontaneous remissions and detection of anti-PLA2R Abs. For renal protection, the majority of patients with PMN require timely diagnosis and treatment; individualization of the choice of treatment and its enhanced efficiency call for further investigations.
cases of non-AL MGRS were presented with combination of light chain deposition disease and thrombotic microangiopathy. The majority of patients (80.4%) were treated with clone-specific agents. Non-IgM cases of MGRS were managed with bortezomib/melphalan/lenolidamidebased regimen. In IgM-MGRS group (n=9) rituximab-based chemotherapy protocols were used. Autologous stem cell transplantation was performed in 13 cases. For 23 AL and 14 non-AL MGRS patients assessment of HR/RR was not available due to death before treatment initiation (n=6) or missed follow-up (n=31). HR was achieved in 74% and 80% of the treated in AL and non-AL MGRS groups, respectively (Fig. 1b). RR was obtained in 42% of AL patients and in 62% of non-AL MGRS cases. Stable proteinuria without kidney function deterioration was registered in 8% of AL and in 14% of non-AL MRGS patients. The five-year cumulative renal survival did not differ significantly between the groups (Fig. 1c). The cumulative renal survival in patients who did not achieve HR was significantly lower than in cases with complete HR, p=0.0014 (Fig. 1d).Conclusions: MGRS is a clinically and morphologically heterogeneous nosological entity characterized by a poor renal prognosis, especially in the absence of clone-specific therapy. Treatment in MGRS should be carried out in a timely manner with the participation of hematologist and nephrologist in order to prevent loss of kidney function and increase life expectancy.
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