Systemic lupus erythematosus (SLE) is an autoimmune disease that affects various organs including the skin, brain, kidneys, and joints. Kidney inflammation triggered by the autoimmune illness SLE can lead to hematuria and proteinuria, as well as end-stage renal failure that can be fatal.
There are six subtypes or stages of lupus nephritis. Class IV disease is named diffuse lupus nephritis. Diffuse lupus nephritis, also referred to as diffuse proliferative glomerulonephritis (DPGN), is the most severe type of lupus nephritis. It can cause scarring in the kidneys. Scars are permanent, and as additional scars grow, kidney function deteriorates.
Early detection and treatment may help prevent long-term harm and improve the quality of life.
What is diffuse lupus nephritis?
Diffuse lupus nephritis is also referred to as diffuse proliferative glomerulonephritis (DPGN), which is a distinct histologic form of glomerulonephritis. DPGN occurs in a variety of systemic inflammatory diseases, such as:
- Autoimmune disorders such as systemic lupus erythematosus
- Vasculitis syndrome (an autoimmune reaction to the proteins in blood vessels) such as granulomatosis with polyangiitis
- Infectious processes such as hepatitis B or C or acquired immunodeficiency syndrome
More than half of the glomeruli in DPGN had an increase in mesangial, epithelial, and endothelial cells caused by proliferation and inflammation in the cells, hence the name glomerulonephritis.
The majority of DPGN cases are caused by immune complex deposition in various cells of the kidney such as:
- Glomerular basement membrane
- Subepithelial sites
How to diagnose diffuse lupus nephritis
- Urinary examination:
- Seventy percent of people with blood tests suggestive of lupus are found to have active urine sediments in the urinary examination. Due to immunological attacks on the glomerulus, the permeability of proteins, red blood cells (RBCs), and white blood cells (WBCs) is increased.
- Urinalysis during active inflammation (or glomerulonephritis) typically reveals active urine sediment, including RBCs or casts, WBCs or casts, and varying degrees of proteinuria.
- Microscopic studies:
- Microscopic studies of the kidney tissue indicate more than 50 percent of glomeruli are involved in active and/or chronic endocapillary and/or extracapillary glomerulonephritis.
- In various combinations, widespread endocapillary hypercellularity, extensive subendothelial deposits producing wire-loop lesions, hyaline thrombi, necrotizing lesions, and crescents may be seen.
- There are mesangial proliferative characteristics.
- Chronic glomerulosclerosis may progress to segmental or global glomerulosclerosis.
- Acute and chronic tubulointerstitial damage of varying severity is evident and linked with the severity of the acute and chronic glomerular lesion.
- Immunofluorescence studies:
- Staining patterns with coarse granular deposits in the segmental distribution along capillary walls, primarily in the subendothelial site, and mesangial deposits are observed under a microscope.
- Scattered subepithelial deposits are seen.
- Immune deposits in the tubules and arteries are present.
- All these are seen with class III lupus nephritis but are more diffuse in diffuse lupus nephritis.
What are the treatment options for diffuse lupus nephritis?
The prognosis of diffuse proliferative glomerulonephritis (WHO class IV) is very poor among other types of lupus nephritis and results in 11 to 48 percent of individuals having end-stage renal disease after five years.
Cyclophosphamide (CYC) has been shown to aid in long-term renal function preservation in severe lupus nephritis. Studies consistently report that CYC-containing regimens preserve renal function better than corticosteroids alone. Long-term follow-up (median 11 years) revealed that pulse CYC is still more effective than pulse methylprednisolone (MP) alone. However, other research reports that a combination of pulse MP and pulse CYC is superior to pulse MP alone in inducing remission.
Therefore, a corticosteroid with a longer term of intravenous pulse CYC has become the usual regimen for the first treatment of aggressive lupus nephritis.
The renal transplant offers a final hope to people who have severely impaired kidneys and cannot manage their daily affairs without severe restrictions.
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AJKD Atlas of Renal Pathology: Focal and Diffuse Lupus Nephritis (ISN/RPS Class III and IV): https://www.ajkd.org/article/S0272-6386(17)30745-X/fulltext
Diffuse Proliferative Glomerulonephritis: https://emedicine.medscape.com/article/239646-overview#a2
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