Rapidly Progressive Glomerulonephritis (RPGN)

Rapidly Progressive Glomerulonephritis (RPGN) is a medical condition that results in rapid deterioration of kidney function. Kidney dysfunction causes progressive deterioration of Glomerular Filtration Rate (GFR) within an extremely short period of time. In this article, we will discuss in detail about the various causes, symptoms, and treatments for Rapidly Progressive Glomerulonephritis (RPGN).

Rapidly Progressive Glomerulonephritis (RPGN)

Progression Of Rapidly Progressive Glomerulonephritis (RPGN)

  • Rapidly Progressive Glomerulonephritis (RPGN)- The disease causes rapid deterioration of renal function.
  • Kidney Function- Glomerulonephritis disease progresses to 50% loss of function of kidney within 3 months.
  • Immune Complex Activated Disease- Immune complex and antibodies triggers autoimmune response, which causes vasculitis and glomerular tissue damage.
  • Glomerular Vasculitis1- Immune activation causes vasculitis, which results in glomerular crescent formation and kidney ischemic changes. Glomerular capillary rupture is often observed in biopsy study.
  • Glomerular Crescent- Glomerular crescents are observed in kidney biopsy, which is a diagnostic feature of the rapidly progressing glomerulonephritis.
  • Autoimmune Diseases- The progressive glomerulonephritis is often seen in in association with other autoimmune disease like Goodpasture Syndrome, Systemic Lupus Erythematosus and Wagner Granulomatosis. Glomerular antibodies and vasculitis are the result of autoantibody disease.
  • Glomerular Filtration Rate- Rapidly Progressive Glomerulonephritis if not treated may result in end stage kidney disease. Kidney failure results in lower glomerular filtration rate.

Causes Of Rapidly Progressive Glomerulonephritis (RPGN)

a. Genetic Abnormality Causing Rapidly Progressive Glomerulonephritis (RPGN)-

  • Gene Mutation- Research has suggested COL4A3/COL4A4 gene mutations associated with Glomerulosclerosis resulting in renal failure.2
  • Genetic Susceptible Individual- Rapidly progressive glomerulonephritis is associated with antiglomerular basement membrane (anti- GBM) antibodies.

b. Autoimmune Disease Causing Rapidly Progressive Glomerulonephritis (RPGN)

  • Antiglomerular Basement Membrane (Anti-GBM) Antibody-
    • Lab study has identified deposits of Anti-GBM antibodies over basement membrane.2
    • Bacterial and viral infection triggers the abnormal autoimmune response resulting in anti-GBM antibodies deposits in basement membrane, vasculitis and crescentic glomerulonephritis.
  • ANCA Associated Disease-
    • The patient diagnosed of kidney failure may show increase of Anti-Neutrophil Cytoplasmic Antibody (ANCA) in blood serum.
    • Elevated serum (blood) ANCA is associated with autoimmune disease resulting in vasculitis.
    • Vasculitis is caused by activated white blood cells attacking the blood vessels in kidney and other organs.
    • The secretion of ANCA is triggered by viral or bacterial infection. Multiple studies have demonstrated that ANCA-activated neutrophils attack vascular endothelial cells.
    • The antibody or immune complex also activates white blood cell, which results in autoimmune disease.

c. Side Effects of Medications Causing Rapidly Progressive Glomerulonephritis (RPGN)-

  • Hydralazine
  • Rifampin
  • Allopurinol

d. Vasculitis Causing Rapidly Progressive Glomerulonephritis (RPGN)-

  • Ninety percent of vasculitis or microscopic polyangitis is associated with renal disease.
  • Wegener Granulomatosis is caused by vasculitis and 80% of cases results in glomerulonephritis.
  • Churg-Strauss disease when caused by vasculitis results in progressive glomerulonephritis in 40% of the cases.

Risk Factors of Rapidly Progressive Glomerulonephritis (RPGN)-

  • Disease Resulting In Increased Anti-GBM-
    • Goodpasture syndrome4 (lung and kidney involvement)
  • Diseases Associated With Immune Complex Diseases-
    • Collagen-vascular disease
    • Lupus nephritis
    • Henoch-Schönlein purpura (immunoglobulin A and systemic vasculitis)
    • Mixed Cryoglobulinemia
    • Primary renal disease
    • Wegener granulomatosis (WG)
    • Churg-Strauss syndrome

Symptoms Of Rapidly Progressive Glomerulonephritis (RPGN)

Non-Specific Symptoms of Rapidly Progressive Glomerulonephritis (RPGN)-

  • Flu like symptoms-
    • Fever
    • Joint pain
    • Lethargy
  • Muscle ache
  • Fatigue
  • Weight loss

Specific Symptoms of Rapidly Progressive Glomerulonephritis (RPGN)-

  • Skin-
    • Painful vascular nodule
    • Cutaneous ischemic necrosis of skin of the extremities
    • Ulceration of skin
  • Nail-
    • Ischemia of nail causes discoloration of nail as a result of infarct or ischemic changes of nail.
  • Eyes-
    • Vasculitis of blood vessels of eye causes following inflammatory condition and ischemic changes
      • Uveitis
      • Conjunctivitis
  • Kidney Disease
    • Rapid deterioration of kidney function (3 to 6 months) is observed.
    • Chronic Pain-
      • Pain mostly localized over flank
      • Dull to severe pain
    • Oliguria-
      • Low output of urine in 24 hours is observed.
    • Microscopic Hematuria-
      • Blood in urine is observed in urine examination.
    • Proteinuria-
      • Protein in urine is observed in urine examination.
    • Electrolyte Abnormalities-
      • Low sodium and high potassium were observed
      • High potassium can cause life-threatening complications.
  • Central Nervous System
    • Mononeuritis multiplex-
      • Ischemic changes observed in multiple bundles of nerve fibers.
      • Numbness and muscle weakness secondary to neuritis is observed along the distribution of nerve.
    • Seizures or Epilepsy- Vasculitis of meningeal and cerebral vessels causes ischemic changes of brain tissue resulting in epilepsy.
  • Gastrointestinal System
    • Gastric ulcer and bleeding from ulcer results from ischemia of mucosal membrane and vasculitis of arteries supplying blood to gastric mucosa.
    • Pancreatitis may result in severe abdominal pain and elevated pancreatic enzymes.
  • Pulmonary Symptoms-
    • Cough with hemoptysis (coughing blood)
    • Sinusitis
    • Ulcer and bleeding from nasal mucosa
  • Hypertension
    • Inflammation of arteries causes increased resistance to blood flow at the peripheral tissue and results in hypertension or high blood pressure.
  • Polyarthopathy and Muscle Pain-
    • This is caused by vasculitis of arterial blood vessels in joint and muscles.
    • Arthritis is symmetrical and migratory symptoms.

Diagnosis Of Rapidly Progressive Glomerulonephritis (RPGN)

Urine Examination

  • Urine Volume- Decreased
  • Hematuria- Blood in urine
  • Proteinuria- Protein in urine

Blood Examination

  • Hemoglobin- Low hemoglobin resulting in anemia
  • Complete Blood Count (CBC)- Low red blood cell count
  • Electrolytes- Low sodium or high potassium
  • Blood Urea Nitrogen- Elevated
  • Creatinine- Elevated
  • Antiglomerular Basement Membrane Antibody Test- Positive
  • Test for ANCA- Positive
  • Creatinine Clearance

Renal Biopsy-

  • Glomerular crescent are seen in biopsy
  • Capillary bleeding observed

Treatment For Rapidly Progressive Glomerulonephritis (RPGN)

  • Immunosuppressant-
    • Corticosteroid
      • Large dosage of corticosteroid treatment prevents rapid deterioration and frequent relapse.5
    • Cyclophosphamide
      • Low to medium dosage of cyclophosphamide helps to prevent deterioration and complications.
      • Cyclophosphamide is used for short period and often treatment is combined with corticosteroid.
    • Azathioprine
      • Azathioprine is used instead cyclophosphamide
      • Azathioprine is prescribed with corticosteroids.
    • Methotrexate
      • Methotrexate is prescribed in few cases instead cyclophophamide.
      • Methotrexate is used with corticosteroid.
  • Plasmaphoresis
    • Treatment helps to remove most of the Anti-GBM and ANCA protein (Globulin) from blood serum.5
  • Kidney Dialysis
    • Kidney dialysis is advised if renal function is deteriorated to level the metabolites and if biological wastes are not adequately eliminated.
  • Kidney Transplant
    • Kidney transplant is the last choice of treatment when symptoms and signs of kidney failure are observed.

Overall Prognosis For Rapidly Progressive Glomerulonephritis (RPGN)

  • End Stage Renal Disease-
    • In the absence of treatment, Rapidly Progressive Glomerulonephritis results in end stage renal disease.
  • Complete or Partial Recovery of Kidney Function-
    • Kidney function returns to normal when adequately treated during early phase.
    • Recovery of kidney is totally dependent on the state of the function of the kidney at the time of diagnosis
  • Microscopic Types of Progressive Glomerulonephritis-
    • Non-Circumferential Crescent Seen In Biopsy-
      • Less than 50% of glomeruli are not functional.
      • Responds to medications
      • Remissions is often less severe.
    • Circumferential Crescents Seen in Biopsy-
      • Biopsy shows more than 80% glomeruli are non functional
      • Advanced renal failure observed in 3 to 6 months
      • Disease does not respond to medications.

Complications Of Rapidly Progressive Glomerulonephritis (RPGN)

Complications Of Rapidly Progressive Glomerulonephritis (RPGN) Are As Follows:

  • CHF
  • Pulmonary Edema
  • Hyperkalemia
  • Acute Kidney Failure
  • ESRD

References:

1. Granulomatosis with polyangiitis: rapidly progressive necrotizing glomerulonephritis in a pediatric patient

Luna M1, Bocanegra V2, Vallés PG3

Int J Nephrol Renovasc Dis. 2014 Apr 23;7:153-6.

2. COL4A3/COL4A4 mutations producing focal segmental glomerulosclerosis and renal failure in thin
basement membrane nephropathy.Voskarides K1, Damianou L, Neocleous V, Zouvani I, Christodoulidou S, Hadjiconstantinou V, Ioannou K, Athanasiou Y, Patsias C, Alexopoulos E, Pierides A, Kyriacou K, Deltas C.

J Am Soc Nephrol. 2007 Nov;18(11):3004-16.

3. Clinicopathologic spectrum of crescentic glomerulonephritis: a hospital-based study.

Choudhury TA1, Singh RG; Usha, Singh S, Singh TB, Rathore SS; Prabhakar.

Saudi J Kidney Dis Transpl. 2014 May;25(3):689-96.

4. Diagnosis and classification of Goodpasture's disease (anti-GBM). Hellmark T1, Segelmark M2.

J Autoimmun. 2014 Feb-Mar;48-49:108-12.

5. Rapidly progressive crescentic glomerulonephritis: Early treatment is a must.

Moroni G1, Ponticelli C2.

Autoimmun Rev. 2014 Jul;13(7):723-9.

Written, Edited or Reviewed By:

, MD, FFARCSI

Last Modified On: June 27, 2014

Pain Assist Inc.

Pramod Kerkar
  Note: Information provided is not a substitute for physician, hospital or any form of medical care. Examination and Investigation is necessary for correct diagnosis.

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