Glomerulonephritis, mesangioproliferative

The most common form of mesangioproliferative glomerulonephritis, IgA nephritis, was first described by J. Berger in 1968 (Schweisfurth 2010).

Mesangioproliferative glomerulonephritis (MESPGN [Escher 2022] or MesPGN [Mokhtar 2014]) or MesP (Axelsen 2014) is an immune-mediated lesion of the kidneys (Kasper 2015).

MESPGN belongs to the secondary glomerulonephritides and is divided into 6 subgroups according to the WHO classification:

- I. minimal to absent glomerular changes.

- II. mesangioproliferative glomerulonephritis

- III. focal segmental glomerulonephritis

- IV. diffuse segmental or global glomerulonephritis

- V. membranous glomerulonephritis

- VI. advanced sclerosing glomerulonephritis (Renz 2009).

MESPGN represents a common morphological pattern and includes a whole group of renal diseases such as IgA- nephropathy, IgM- nephropathy, lupus nephritis, C1q- nephropathy, etc (Mokhtar 2014).

The incidence of MESPGN in Denmark is approximately 10.8 million cases per year, making it the most commonly diagnosed glomerulonephritis (Axelsen 2014).

In renal biopsies, MESPGN is found in approximately 10-25% of all renal biopsies worldwide. Affected individuals in the study by Mokhtar (2014) were primarily young people with an average age of 19 years. A gender prevalence was not found here. In other studies, however, the majority of patients were middle-aged with male predominance (Mokhtar 2014).

MESPGN most frequently occurs idiopathically. Otherwise, it can occur as a secondary form in liver diseases, chronic inflammatory bowel diseases and rheumatic diseases (Benzing 2022).

In the context of IgA vasculitis (Purpura- Schönlein- Henoch) it is found clinically in 30% and bioptically in up to 80% (Herold 2022).

It can also occur in

- Plasmodium falciparum malaria

- post-infectious glomerulonephritis in the decaying stage

- a class II lupus nephritis (Kasper 2015).

Probably also by the following viruses:

- Parvo

- mumps

- leprosy

- Malaria caused by Plasmodium falciparum, Plasmodium vivax or Plasmodium ovale (Kasper 2015).

In MESPGN, there is expansion of the mesangium, sometimes associated with mesangial hypercellularity, thin, simply contoured walls of capillaries, and mesangial immune deposits (Kasper 2015).

The clinical course is variable (Kasper 2015).

Micro- or macrohematuria (Herold 2022), as well as proteinuria (Kasper 2015) can be found.

The most common presentations are:

- nephrotic syndrome, defined as a proteinuria of 3.5 g / 24 h (found in 71% - 82%)

- hematuria

- combined proteinuria and hematuria (Mokhtar 2014).

Diagnosis of MESPGN is made by histological examination a biopsy (Mokhtar 2014).

Common features of the various glomerular diseases are mesangial cell proliferation and mesangial matrix accumulation. There is a diffuse or focal increase in the number of mesangial cells and expansion of extracellular matrix in the glomerular mesangium with or without immunoglobulin or complement deposition (Mokhtar 2014).

Histologically, mesangial IgA deposits are most commonly (Mokhtar 2014) detectable in MESPGN (Herold 2022).

There is little agreement to date on the form of therapy. Some clinical reports suggest that the following agents are beneficial:

- Inhibitors of the renin- angiotensin system

- steroids

- cytotoxic agents (Kasper 2015).

Given the prognostically favorable course, all supportive measures should be exhausted initially in MESPGN. If this fails to reduce proteinuria < 0.5 - 1 g / d or demonstrates progressive loss of renal function, steroid treatment is recommended. Immunosuppressants should only be considered as the last therapeutic option if all supportive measures have been unsuccessful (Benzing 2022). For more details, see "Internal therapy" below.

Prednisolone

If the GFR is still > 30 ml / min, the following scheme of steroid therapy has proven effective:

Dosage recommendation: 0.8 - 1 mg / kg bw / d prednisolone p. o. for 2 months. Subsequent reduction of the dose monthly by 0.2 mg / kg bw / d (Benzing 2022).

Or

A combination with methylprednisolone pulses at a dose of 1 g for 3 days a month (1, 3, 5) plus prednisone on the remaining days at a dose of 0.5 mg / kg bw every 2nd day for a period of 6 months.

If GFR < 30 min / min, corticosteroid therapy should be discontinued (Benzing 2022).

Immunosuppressants

Immunosuppressants such as azathioprine, cyclophosphamide, mycophenolate mofetil, or rituximab are not recommended for Caucasian patients unless there is a rapid progressive course with glomerular crescents. From a GFR < 30 ml / min, immunosuppressants are no longer indicated (Benzing 2022).

From a GFR < 30 min / min, therapy with immunosuppressants should no longer be given (Benzing 2022).

Overall, the prognosis of MESPGN is good (Axelsen 2014).

Patients with severe proteinuria occasionally develop renal failure, whereas patients with isolated hematuria usually have a benign course (Kasper 2015).

MESPGN caused by Plasmodium vivax or Plasmodium ovale usually shows a benign course (Kasper 2015).

The 3-year renal survival rate has been reported to be as high as 93% in several studies (Mokhtar 2014). Overall, renal survival at 30 years is significantly better for women at 70% than for men at 40% (Axelsen 2014).

If there is a temporal relationship between the occurrence of macrohematuria and infection, tonsillectomy may be indicated. However, this should by no means be performed routinely (Benzing 2022).

1. Axelsen M, Pedersen R S, Heaf J G, Ellingsen T (2014) Mesangioproliferative glomerulonephritis: a 30-year prognosis study. Nephron Extra. 4 (1) 26 - 32
2. Benzing T (2022) Therapy handbook nephrology. Elsevier Urban and Fischer Publishers 8
3. Escher (2022) Mesangioproliferative glomerulonephritis. Pschyrembel online DOI: https://www.pschyrembel.de/Mesangioproliferative%20Glomerulonephritis/K0RAK
4. Herold G et al (2022) Internal medicine. Herold Publishers 694
5. Kasper D L, Fauci A S, Hauser S L, Longo D L, Jameson J L, Loscalzo J et al (2015) Harrison's Principles of Internal Medicine. Mc Graw Hill Education 1841, 1849.
6. Mokhtar G A, Jalalah S, Sultana S (2014) Pathological patterns of mesangioproliferative glomerulonephritis seen at a tertiary care center. J Nephropharmacol. 3 (1) 33 - 37.
7. Renz H (2009) Practical laboratory diagnostics. Walter de Gruyter Verlag Berlin 266
8. Schweisfurth A (2010) Epidemiology and prognosis of glomerulonephritis under evidence-based therapy in West Mecklenburg. Inaugural dissertation for the degree of Doctor of Medicine of the Medical Faculty of the University of Rostock.