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Genetic and Rare Diseases Information Center (GARD)

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Megalocytic interstitial nephritis

Other Names for this Disease
  • Interstitial megalocytic nephritis
  • Transplant megalocytic interstitial nephritis (type)
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I'd like to learn more about megalocytic interstitial nephritis with malakoplakia.

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What is interstitial nephritis?

Interstitial nephritis is a kidney disorder in which the spaces between the kidney tubules become swollen (inflamed). The inflammation can affect the kidneys' ability to filter waste. Interstitial nephritis can be caused by many different things, and may be temporary (acute) or it may be chronic and get worse over time.[1]
Last updated: 4/2/2009

What are the symptoms of interstitial nephritis?

Symptoms of interstitial nephritis may include blood in the urine, fever, increased or decreased urine output, mental status changes (drowsiness, confusion, coma), nausea, vomiting, rash, swelling of the body, and weight gain (from retaining fluid).[1]
Last updated: 4/2/2009

What is malakoplakia?

Malakoplakia is a rare chronic inflammatory disease. It commonly involves the urinary tract, but may also involve the prostate, ureter, pelvis, bones, lungs, testes, gastrointestinal tract, skin, and kidney.  Malakoplakia of the kidney is often associated with chronic kidney infection and obstruction. E. coli  is the most common organism found in urine samples.[2] Careful studies of the involved tissues can help to distinguish malakoplakia of the kidney from similar conditions, namely xanthogranulomatous pyelonephritis and megalocytic interstitial nephritis.[2][3][4]
Last updated: 4/2/2009

What are the symptoms of malakoplakia of the kidney?

Common symptoms of malakoplakia of the kidney include flank pain and signs of active kidney infection. It may affect one or both kidneys and can cause symptoms similar to that of kidney failure.[2]
Last updated: 4/2/2009

What causes malakoplakia?

The cause of malakoplakia is unknown, but is thought to be associated with immunodeficiency or autoimmune disorders, such as hypogammaglobinlinemia, therapies that suppress the immune system, cancer, a chronic debilitating disorder, rheumatoid arthritis, and AIDS.[2]
Last updated: 4/2/2009

What is megalocytic interstitial nephritis?

Megalocytic interstitial nephritis is a rare chronic kidney disease that is characterized by inflammation of the kidney. It is similar to malakoplakia of the kidney. In this condition the inflammation is caused by various infiltrate, particularly histiocytes. A histiocyte is a type of immune cell that eats foreign substances in an effort to protect the body from infection.
Last updated: 4/2/2009

  • Interstitial nephritis. MedlinePlus. 2007; Accessed 4/2/2009.
  • Tolkoff-Rubin NE, Rubin RH, Contran RS. Urinary Tract Infection, Pyelonephritis, and Reflux Nephropathy. In: Brenner BM, Levine SA. Brenner & Rector's THE KIDNEY 8th ed. Philadelphia, PA: 2008;
  • Kapasi H, Roberstson S, Futter N. Diagnosis of renal malacoplakia by fine needle aspiration cytology. A case report. Acta Cytol. 1998 Nov-Dec;
  • Krupp G et al. Tumefactive megalocytic interstitial nephritis in a patient with Escherichia coli bacteremia. Am J Kidney Dis. 1995 Jun;