Rare Hematology News

Disease Profile

Megalocytic interstitial nephritis

Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.


US Estimated

Europe Estimated

Age of onset





Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease.


Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype.


dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.


recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.


Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.


Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.


Not applicable


Other names (AKA)

Interstitial megalocytic nephritis; Transplant megalocytic interstitial nephritis (type)


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]


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]


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.[3]


Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Providing General Support

    Learn more

    These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

    Where to Start

      In-Depth Information

      • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
      • PubMed is a searchable database of medical literature and lists journal articles that discuss Megalocytic interstitial nephritis. Click on the link to view a sample search on this topic.


        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. https://www.ncbi.nlm.nih.gov/pubmed/?term=25552891.
        2. Interstitial nephritis. MedlinePlus. 9/22/2015; https://medlineplus.gov/ency/article/000464.htm.
        3. 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;

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