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Membranoproliferative Glomerulonephritis

MPGN

Membranoproliferative glomerulonephritis, also called mesangiocapillary glomerulonephritis, is an uncommon cause of glomerular injury with characteristic light microscopic changes.


Presentation

Membranoproliferative glomerulonephritis (MPGN) is primarily, though not exclusively, a disease of children [1]. The age range is known to extend into young adulthood with the disease being occasionally diagnosed in older adults. MPGN appears to affect both sexes equally [2].

The clinical onset of MPGN may be preceded by upper respiratory infections. Up to one-third of affected patients may be hypertensive at the time of diagnosis. Renal dysfunction is known to occur in more than 50% of children. Some patients may suffer from severe anemia, owing to the effects of kidney failure and complement-mediated destruction of red blood cells.

All patients with MPGN have proteinuria and/or hematuria. The degree of proteinuria is varied with a majority of individuals presenting in the nephrotic range (40-70%). Some may also present with acute nephritic syndrome (20-30%), isolated macroscopic hematuria, or microscopic hematuria with subnephrotic range proteinuria.

It is not possible to differentiate the various types of MPGN clinically (there are three types of MPGN). However, type II MPGN is characterized by certain extra-renal manifestations. Notable amongst these are deposits termed drusen in the Bruch’s membrane of the retina. As compared to the drusen seen in age-related macular degeneration, the drusen of MPGN II develops at a much younger age. Partial lipodystrophy is also noted in these patients, years before the onset of clinical disease [3].

In contrast to adults, children with MPGN have an acute presentation but a slower progression of the disease. Spontaneous remission is rare and the disease varies in severity over its clinical course. MPGN is also known to recur in kidney transplant recipients [4].

Rapidly Progressive Glomerulonephritis
  • Rapidly progressive glomerulonephritis (numerous crescent cells on renal biopsy).[lecturio.com]
  • Called also membranous nephropathy. rapidly progressive glomerulonephritis acute glomerulonephritis marked by a rapid progression to end-stage renal disease and histologically by profuse epithelial proliferation, often with epithelial crescents; principal[medical-dictionary.thefreedictionary.com]
Rectal Bleeding
  • We present here the case of a man with rectal bleeding and rapidly progressive renal failure, who had MCL in the gastrointestinal tract and paraneoplastic membranoproliferative glomerulonephritis, and whose renal dysfunction normalised after chemotherapy[ncbi.nlm.nih.gov]
Hypertension
  • A renal biopsy revealed he suffered from focal segmental glomerulosclerosis (FSGS), possibly due to obesity and hypertension, not CVID - associated MPGN. This is the first case report of FSGS in a CVID patient.[ncbi.nlm.nih.gov]
Mononeuropathy
  • However, 3 weeks after the start of treatment, nephrotic syndrome developed again and was accompanied by lower-extremity mononeuropathy and renal dysfunction.[ncbi.nlm.nih.gov]
Cerebral Calcification
  • Idiopathic basal ganglia calcification (IBGC) is a syndrome in which bilateral cerebral calcification occurs despite the absence of abnormal calcium metabolism.[ncbi.nlm.nih.gov]
Dark Urine
  • urine (smoke, cola, or tea colored) Decrease in urine volume Swelling of any part of the body Exams and Tests The health care provider will examine you and ask about your symptoms.[printer-friendly.adam.com]
  • Symptoms Symptoms may include any of the following: Blood in the urine Changes in mental status such as decreased alertness or decreased concentration Cloudy urine Dark urine (smoke, cola, or tea colored) Decrease in urine volume Swelling of any part[account.allinahealth.org]

Workup

The diagnosis of MPGN is suggested by the presence of hematuria with dysmorphic red blood cells. The degree of proteinuria is variable and the serum creatinine may be normal or raised. The diagnosis is confirmed via a renal biopsy.

Light microscopy may reveal a classic membranoproliferative pattern that includes lobular accentuation of the tuft of glomeruli and duplication of the basement membrane (termed the tram-track appearance).

Immunofluorescence microscopy may reveal the deposition of immune complexes and complement factors. Hypocomplementemia is common in all types of MPGN. In type I disease or immune complex-mediated MPGN, complement activation occurs via the classic pathway, thereby, resulting in low serum C4 concentration and normal/low C3 levels. Type II disease or complement-mediated MPGN show a low serum C3 and normal serum C4 levels due to the activation of the alternative pathway. MPGN type III involves the activation of the alternative and terminal pathways with low levels of C3 and C5-C9 [5]. C3 nephritic factor levels may also be tested.

Electron microscopy may reveal deposits in the mesangium, basement membranes of the glomeruli, and occasionally within Bowman’s capsule [6] [7].

Genetic testing for factors involved in the complement regulation may also be performed [8].

Abnormal Renal Function
  • Recommendations from the KDIGO guideline for treatment of MCGN are limited to cases with abnormal renal function and are drawn mainly from small observational studies [17].[journals.plos.org]
Human Parvovirus B19
  • Abstract We report a case in which renal biopsies were performed 4 years apart in a woman with a prolonged human parvovirus B19 (HPB19) infection.[ncbi.nlm.nih.gov]
Lymphocytic Infiltrate
  • Salivary gland biopsy demonstrated mild lymphocytic infiltration, indicating chronic GVHD. Improvement of the proteinuria and recovery of renal function were achieved within 11 months of treatment with oral prednisolone and azathioprine.[ncbi.nlm.nih.gov]
Foam Cell
  • Renal biopsy showed diffuse global endocapillary proliferative lesions with infiltration of mononuclear cells and occasional foam cells.[ncbi.nlm.nih.gov]

Treatment

  • If the patient receives high doses of steroid therapy in the early stages, their treatment is more likely to be successful. The effect of immunosuppressive treatment on MPGN is not clear.[ncbi.nlm.nih.gov]

Prognosis

  • CONCLUSIONS: Our results suggest that the treatment response and prognosis of patients with C3GN are worse than those of patients with IC-mediated MPGN.[ncbi.nlm.nih.gov]
  • The causative agents, clinical features, laboratory data, and prognosis were determined.[adc.bmj.com]

Etiology

  • These new classification was satisfactory for the finding of etiology in one part of the cases.[ncbi.nlm.nih.gov]

Epidemiology

  • BACKGROUND: Data on pediatric membranoproliferative glomerulonephritis (MPGN) epidemiology, complications, and healthcare costs are critical to our understanding of MPGN's economic burden and of how best to direct clinical care and research efforts in[ncbi.nlm.nih.gov]
  • OBJECTIVES Introduction before we drive on the road to etiology:  Epidemiology  Pathogenesis & Types  Renal Presentation  Pathology Diagnostic Road for Etiology of MPGN:  Step 1: Pathology Tips  Step 2: Age & Sex  Step 3: History &Examination3[de.slideshare.net]
  • Epidemiology MPGN is a collection of morphologically related but pathogenically distinct disorders. As a result, true epidemologic information is difficult to come by.[wikispaces.psu.edu]
  • Renal diseases associated with human immunodeficiency virus infection: Epidemiology, clinical course, and management. Clin Infect Dis 2001;33:115-9.[indianjnephrol.org]
Sex distribution
Age distribution

Pathophysiology

  • Only 2 (9%) of these patients (4 eyes) had retinal pathologic features that potentially could be related to the same underlying pathophysiologic process as their renal disease, and neither had vision loss.[ncbi.nlm.nih.gov]

Prevention

  • […] lupus may help prevent MPGN.[nlm.nih.gov]
  • For the latter patients, plasma replacement therapy prevents renal failure, but for the majority of patients, there is no proven effective treatment.[ncbi.nlm.nih.gov]
  • Preventing infections such as hepatitis or managing diseases such as lupus may help prevent MPGN.[printer-friendly.adam.com]
  • Possible Complications Prevention Preventing infections such as hepatitis or managing diseases such as lupus may help prevent MPGN.[account.allinahealth.org]

References

Article

  1. Galle P, Mahieu P. Electron dense alteration of kidney basement membranes. A renal lesion specific of a systemic disease. Am J Med. 1975;58:749–764.
  2. Barbour TD, Pickering MC, Terence Cook H. Dense deposit disease and C3 glomerulopathy. Semin Nephrol. 2013;33:493–507.
  3. Duvall-Young J, MacDonald MK, McKechnie NM. Fundus changes in (type II) mesangiocapillary glomerulonephritis simulating drusen: a histopathological report. Br J Ophthalmol. 1989;73:297–302.
  4. Braun MC, Stablein DM, Hamiwka LA, Bell L, Bartosh SM, Strife CF. Recurrence of membranoproliferative glomerulonephritis type II in renal allografts: the North American pediatric renal transplant cooperative study experience. J Am Soc Nephrol. 2005;16:2225–2233.
  5. Varade WS, Forristal J, West CD. Patterns of complement activation in idiopathic membranoproliferative glomerulonephritis, types I, II, and III. Am J Kidney Dis. 1990;16:196–206.
  6. Levy M, Gubler MC, Sich M, Beziau A, Habib R. Immunopathology of membranoproliferative glomerulonephritis with subendothelial deposits (Type I MPGN) Clin Immunol Immunopathol. 1978;10:477–492.
  7. Habib R, Gubler MC, Loirat C, Mäiz HB, Levy M. Dense deposit disease: a variant of membranoproliferative glomerulonephritis. Kidney Int. 1975;7:204–215.
  8. Bomback AS, Appel GB: Pathogenesis of the C3 glomerulopathies and reclassification of MPGN. Nat Rev Nephrol 2012;8: 634–642.

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Last updated: 2019-07-11 19:57