National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Megalocytic interstitial nephritis



I'd like to learn more about megalocytic interstitial nephritis with malakoplakia.


What is megalocytic interstitial nephritis?

Megalocytic interstitial nephritis is a rare chronic kidney disease that is characterized by inflammation of the kidney. The inflammation is mainly found in an area of the kidney called the renal cortex, which is in the outer portion of the kidney. The exact cause of megalocytic interstitial nephritis is not clear; however, it is associated with an impaired function of histiocytes (immune cell that eats foreign substances to protect the body from infection), particularly in patients that have a lowered immune system. Alcohol abuse may also be a risk factor.[1] Generalized symptoms of kidney inflammation (interstitial nephritis) may include: blood in the urine, fever, increased or decreased urine output, mental status changes, rash, and swelling of the body. Treatment is dependent on the underlying cause (if identified) and the stage of the disease.[2]
Last updated: 8/17/2016

What is malakoplakia?

Malakoplakia is a rare chronic inflammatory disease. It most commonly involves the urogenital system (reproductive organs and urinary system), but may also be found in other regions of the body, including the pelvis, bones, lungs, thyroid gland, gastrointestinal (digestive) tract, skin, and kidneys. Symptoms of malakoplakia differ depending on the involved area. For example, when the skin is affected the malakoplakia may appear rash-like with small areas of itchy, reddened skin that may be painful and/or fluid filled.[3][4]

The cause of malakoplakia is not well understood. It is thought to be related to an issue with the function of one's macrophages, which are one type of cell within the immune system that respond to foreign invaders (bacteria). E.coli is the most common type of bacteria that leads to malakoplakia. Individuals with a compromised immune system have an elevated risk to develop malakoplakia. Treatment typically begins with antibiotics.[3][4]
Last updated: 8/17/2016

What are the signs and symptoms of malakoplakia of the kidney?

Symptoms of malakoplakia of the kidney are similar to symptoms present during a kidney infection including fever, back side (flank) or groin pain, abdominal pain, frequent urination, pus or blood in the urine, and a burning sensation with urination.[5][6] Malakoplakia can lead to kidney failure. It may affect one or both kidneys and often appears as a mass or tumor which can be misdiagnosed as renal cancer.[5][7]
Last updated: 8/17/2016

What are the signs and symptoms of megalocytic interstitial nephritis?

Symptoms of generalized megalocytic 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).[2]
Last updated: 8/18/2016

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.[5]
Last updated: 8/17/2016

Are malakoplakia of the kidney and megalocytic interstitial nephritis related?

There is no clear clinical distinction between megalocytic interstitial nephritis and malakoplakia. Some researchers suspect that megalocytic interstitial nephritis might be an early stage of malakoplakia or might represent a variation of malakoplakia. Some studies indicate that a kidney biopsy can be useful in differentiating malakoplakia, megalocytic interstitial nephritis, and xanthogranulomatous pyelonephritis. All three of these conditions are chronic inflammatory disorders with similar clinical signs and symptoms.[1]
Last updated: 8/18/2016

We hope this information is helpful. We strongly recommend you discuss this information with your doctor. If you still have questions, please contact us.

Warm regards,
GARD Information Specialist

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  1. Hee Jin Kwon, Kwai Han Yoo, In Young Kim, Seulkee Lee, Hye Ryoun Jang, Ghee Young Kwon. Megalocytic Interstitial Nephritis Following Acute Pyelonephritis with Escherichia coli Bacteremia: A Case Report. J Korean Med Sci. Jan 2015; 30(1):110-114. http://www.ncbi.nlm.nih.gov/pubmed/?term=25552891.
  2. Interstitial nephritis. MedlinePlus. 9/22/2015; https://medlineplus.gov/ency/article/000464.htm.
  3. Amira M Elbendary. Malakoplakia. Medscape. Jan 19, 2016; http://emedicine.medscape.com/article/1055606-overview.
  4. João Paulo Junqueira Magalhães Afonso, Patricia Naomi Ando, Maria Helena Valle de Queiroz Padilha, Nilceo Schwery Michalany, Adriana Maria Porro. Cutaneous malakoplakia: case report and review. An Bras Dermatol. 2013 May-Jun; 88(3):432-437. http://www.ncbi.nlm.nih.gov/pubmed/23793204.
  5. 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;
  6. Kidney Infection. Mayo Clinic. August 16, 2014; http://www.mayoclinic.org/diseases-conditions/kidney-infection/basics/definition/CON-20032448?p=1.
  7. Maryam Abolhasani, Azam Mohammad Jafari, Mojgan Asgari, Hormoz Salimi. Renal malakoplakia presenting as a renal mass in a 55-year-old man: a case report. J Med Case Rep. 2012; 6:379. http://www.ncbi.nlm.nih.gov/pubmed/23130868.