2 CASES WITH IGM NEPHROPATHY

8 Feb 2025 12 a.m. 12 a.m.
WCN25-AB-2987, Poster Board= SAT-127

Introduction:

The reported frequency of IgMN in the literature has varied from 1.8% to 18.5% in native biopsies. IgMN appears to have different clinical outcomes at different ages. It mainly presents proteinuria or haematuria in young adults or children. Proteinuria in IgMN can range from asymptomatic proteinuria to nephrotic syndrome. IgM deposits may be found in patients with MCD, FSGS, and mesangial proliferative glomerulonephritis. The presence of IgM deposits in a patient with MCD signifies a poorer prognosis compared with MCD without deposits, as fewer than 50 % of such patients respond to glucocorticoids.

Methods:

Case 1

19-year-old male patient, known to have nephrotic syndrome since the age of 3 with frequent relapses. He received multiple courses of immunosuppression medications, including multiple courses of steroids, tacrolimus, 5 doses of rituximab, and cyclophosphamide for 3months in 2010. He was admitted to our facility with a relapse of his nephrotic syndrome in the form of generalized body edema and weight gain of 9kgs, his urinary protein was >6g/L, started on oral pulse steroids in the form of prednisolone at 1 mg/kg/day.

Case2 

37-year-old male known case of mesangioproliferative GN proved in a biopsy done 2 years ago following his Covid infection. He was on mycophenolate mofetil, but it was stopped for financial reasons. He presented with generalized and facial edema and acute kidney injury with proteinuria of 6.49 g. his serum albumin was 6 g/. he was started on pulse steroids with a kidney biopsy done and showed IgM (++) on immunofluorescence and effacement of the podocyte food process of more than 80 % which is consistent with severe podocytopathy. He started mycophenolate mofetil and prednisolone with tapering dose and his urine protein dropped to 129mg/L in 2 months' time. 

Results:

Kidney biopsy images for the first patient showing  mild mesangial expansion, Unremarkable tubules, and interstitium with IgM (2+) and C1q (1+), kappa (1+) and lambda (1+)  light chains . The glomeruli show no immunoreactivity to IgA, IgG, C3, fibrinogen, and albumin. Tubular protein reabsorption droplets are immunoreactive for IgA, IgG,C3, albumin, kappa, and lambda light chains. Electron Microscopic study showed  focal small mesangial electron-dense deposits. The podocytes are vacuolated and swollen. The podocytes’ foot processes are severely effaced and show diffuse effacement with an overall involvement of approximately 90% of the capillary surface. The features are consistent with IgM nephropathy

Conclusions:

IgM Nephropathy is considered a distinctive pathological entity with a lot of controversy and may not be distinguished from Minimal change disease or FSGS, it carries variable response to steroids. 

I have no potential conflict of interest to disclose.

I did not use generative AI and AI-assisted technologies in the writing process.