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Last updated: 6/12/2025
Years published: 2021, 2025
NORD gratefully acknowledges Gioconda Alyea, MD (FMG), MS, National Organization for Rare Disorders, Greg Cichon, MD, Creighton School of Medicine and Khalid Bashir, MD, Assistant Professor of Nephrology, Creighton School of Medicine, for the preparation of this report.
Summary
Immunotactoid glomerulopathy belongs to a group of rare kidney conditions where abnormal proteins build up in the glomeruli, the filtering units of the kidneys. These conditions are called “non-amyloid fibrillary glomerular diseases,” meaning the protein buildup forms in a specific organized pattern but is not the same as amyloid (a different kind of protein deposit seen in other diseases). The abnormal protein structures are called microtubules and are formed from immune system proteins known as immunoglobulins. The microtubules can block or damage the glomeruli, which leads to a decline in kidney function.
Common signs of this condition include protein in the urine (proteinuria) which may make urine look foamy, and blood in the urine (hematuria) which may cause it to appear red or tea colored. Other symptoms can include swelling in the legs or feet (edema), high blood pressure (arterial hypertension) and decreased kidney function over time.
A diagnosis is made by examining a kidney tissue sample (kidney biopsy) under the microscope.
The exact cause of this disease is still being studied. However, it is frequently found in people with blood-related cancers such as chronic lymphocytic leukemia, B-cell lymphomas and multiple myeloma. It may also be linked to immune system disorders, hepatitis C infection, or a condition called monoclonal gammopathy of undetermined significance (MGUS), where abnormal proteins are produced by certain immune cells.
There are two main forms of immunotactoid glomerulopathy:
Currently, there is no widely approved treatment that works for everyone with this condition. Standard therapies like steroids, chemotherapy drugs, or procedures to remove antibodies from the blood have had mixed results. If the disease is linked to a specific cancer or immune disorder, treatment usually focuses on that underlying condition.
In some people, medications such as rituximab, which targets certain immune cells, have helped improve kidney function, especially when given over an extended period.
Over time, many people with immunotactoid glomerulopathy will have worsening kidney function. Between 40%-50% of cases progress to kidney failure within two to six years. When this happens, dialysis or a kidney transplant may become necessary. The disease can return after transplant but usually in a milder form.
Research is ongoing to better understand why this disease occurs and to develop more effective treatments.
Introduction
Immunotactoid glomerulopathy and fibrillary glomerulonephritis, a similar but slightly more common condition, are two forms of non-amyloid fibrillary glomerular deposition diseases. These conditions are sometimes grouped together. Microtubules and antibodies (immunoglobulins) plug the glomerulus, the body’s filter in the kidneys. Under normal conditions, the glomerulus filters the blood to form urine and keeps the antibodies to help fight infections and microtubules to maintain cell structure. The word “immunotactoid” refers to these rod-like structures (“-tactoid”) produced by the immune system (“immuno”). “Glomerulopathy” refers to the abnormal buildup of these proteins, which causes swelling and eventually irreversible kidney damage. Scientists do not fully understand why these antibodies begin depositing in the glomerulus but most suspect that certain underlying diseases play a role.
Immunotactoid glomerulopathy can affect the kidneys in several ways and symptoms often develop gradually. Some of the most common signs include:
The cause of immunotactoid glomerulopathy is not yet fully understood. However, research has shown strong links to certain conditions involving the immune system. In many people it is associated with:
These conditions may cause the body to produce abnormal immune proteins which may then form microtubule deposits in the kidneys. In some people, no underlying disease is found despite extensive testing.
The overall prevalence of immunotactoid glomerulopathy is very low, and found in only 0.5% to 1.4% of kidney biopsies.
Adults with a history of leukemia, lymphoma, multiple myeloma or another monoclonal gammopathy are most at risk for immunotactoid glomerulopathy. Some evidence also shows an association between a previous hepatitis C viral infection and immunotactoid glomerulopathy.
Diagnosing immunotactoid glomerulopathy requires a detailed look at the kidneys. Blood and urine tests may show early signs but the only way to confirm the diagnosis is through a kidney biopsy. Blood and urine tests may show:
A kidney biopsy is essential to confirm the diagnosis. Under a microscope, doctors can see the microtubular deposits and determine whether the disease is monoclonal or polyclonal.
In a biopsy, a small piece of kidney tissue is removed and examined under a microscope. Special types of microscopes are used to see the exact structure of the deposits in the glomeruli. Electron microscopy shows the microtubular shape of the deposits, while immunofluorescence tests can identify the types of proteins involved. These findings help determine whether the deposits come from a single group of immune cells (monoclonal) or multiple groups (polyclonal).
Electron microscopes are a special type of microscope that uses a beam of electrons, instead of light, to produce detailed images of extremely small objects that are not seen with regular microscopes. Immunofluorescence is a light microscopy-based technique that relies on the use of antibodies chemically labeled with fluorescent dyes to visualize small tissue structures.
After a kidney biopsy confirms the diagnosis of immunotactoid glomerulopathy, a doctor will likely order more blood tests and imaging. These tests are important to find any underlying cancer, hepatitis or autoimmune disease which may be associated with the glomerulonephritis.
The additional tests to search for an underlying cause might include:
In some people, finding and treating the underlying condition can decrease the severity of the kidney disease. When an underlying condition is not found, treatment focuses on controlling the hypertension, protein in the urine and progression of the kidney disease.
Treatment
There is no single treatment proven to work for everyone who is affected with immunotactoid glomerulopathy. Because the condition is rare and complex, treatment needs to be individualized, depending on whether an underlying disease is present and what type of protein deposits are found.
In cases where the disease is associated with a blood cancer or abnormal protein production, treatment focuses on controlling that condition. This is known as clone-directed therapy, and it may include drugs like rituximab, bortezomib, or other chemotherapy agents. When successful, these treatments can improve both kidney and overall health.
For people with polyclonal disease or no identified underlying illness, treatment is less clear. Medications like steroids, immunosuppressants, or rituximab may be tried to reduce inflammation and slow the damage. While results vary, some people with this form of the disease have had long-term improvement or stability with these medications.
Supportive care is also important. This includes managing high blood pressure, controlling protein loss in the urine, and protecting the remaining kidney function. These measures can slow the disease even when a specific treatment is not available.
Unfortunately, many people with immunotactoid glomerulopathy will eventually lose most kidney function. About 40%-50% will develop end-stage kidney disease within two to six years. At that stage, dialysis or a kidney transplant may be needed. In some people, the disease may come back after a transplant, but it usually causes less damage and is easier to manage.
Ongoing research continues to look for better ways to treat this rare condition, especially for patients without a clear underlying cause.
Information on current clinical trials is posted on the Internet at https://clinicaltrials.gov/ All studies receiving U.S. Government funding, and some supported by private industry, are posted on this government web site.
For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:
Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: [email protected]
Some current clinical trials also are posted on the following page on the NORD website:
https://rarediseases.org/living-with-a-rare-disease/find-clinical-trials/
For information about clinical trials sponsored by private sources, contact:
https://www.centerwatch.com/
For information about clinical trials conducted in Europe, contact:
https://www.clinicaltrialsregister.eu/
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Ali K, Agrawal A, Karan A, et al. Immunotactoid Glomerulopathy Masquerading as Heart Failure. Cureus. 2024;16(7):e63687. Published 2024 Jul 2. doi:10.7759/cureus.63687
Saito A, Kameoka Y, Ubukawa K & cols. Successful Treatment of Monoclonal Immunotactoid Glomerulopathy Associated with Chronic Lymphocytic Leukemia Using Ibrutinib. Intern Med. 2025 May 1;64(9):1388-1392. doi: 10.2169/internalmedicine.3902-24. Epub 2024 Oct 4. PMID: 39370258.
Neukirchen W, Oesterling A, Wennmann DO, et al. Polyclonal Immunotactoid Glomerulopathy Associated with Monoclonal Gammopathy of IgM Type and Underlying Plasmacellular Disease: Successful Treatment with Rituximab Alone. Case Rep Nephrol Dial. 2022;12(1):63-72. Published 2022 Apr 25. doi:10.1159/000524131
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Rood IM, Lieverse LG, Steenbergen EJ, et al. Spontaneous remission of immunotactoid glomerulopathy. Neth J Med. 2011; 69:341.
Sathyan S, Khan FN, Ranga KV. A case of recurrent immunotactoid glomerulopathy in an allograft treated with rituximab. Transplant Proc. 2009; 41:3953.
Vilayur E, Trevillian P, Walsh M. Monoclonal gammopathy and glomerulopathy associated with chronic lymphocytic leukemia. Nat Clin Pract Nephrol. 2009; 5:54.
Rosenstock JL, Markowitz GS, Valeri AM, et al. Fibrillary and immunotactoid glomerulonephritis: Distinct entities with different clinical and pathologic features. Kidney Int..2003; 63:1450.
Schwartz MM, Korbet SM, Lewis EJ. Immunotactoid glomerulopathy. J Am Soc Nephrol. 2002; 13:1390.
Carles X, Rostaing L, Modesto A, et al. Successful treatment of recurrence of immunotactoid glomerulopathy in a kidney allograft recipient. Nephrol Dial Transplant. 2000; 15:897.
Markowitz GS, Cheng JT, Colvin RB, et al. Hepatitis C viral infection is associated with fibrillary glomerulonephritis and immunotactoid glomerulopathy. J Am Soc Nephrol. 1998; 9:2244.
Alpers CE. Immunotactoid (microtubular) glomerulopathy: an entity distinct from fibrillary glomerulonephritis? Am J Kidney Dis. 1992; 19:185.
Moulin B, Ronco PM, Mougenot B, et al. Glomerulonephritis in chronic lymphocytic leukemia and related B-cell lymphomas. Kidney Int. 1992; 42:127.
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Learn more https://rarediseases.org/patient-assistance-programs/caregiver-respite/No patient organizations found related to this disease state.
The information provided on this page is for informational purposes only. The National Organization for Rare Disorders (NORD) does not endorse the information presented. The content has been gathered in partnership with the MONDO Disease Ontology. Please consult with a healthcare professional for medical advice and treatment.
The Genetic and Rare Diseases Information Center (GARD) has information and resources for patients, caregivers, and families that may be helpful before and after diagnosis of this condition. GARD is a program of the National Center for Advancing Translational Sciences (NCATS), part of the National Institutes of Health (NIH).
View reportOrphanet has a summary about this condition that may include information on the diagnosis, care, and treatment as well as other resources. Some of the information and resources are available in languages other than English. The summary may include medical terms, so we encourage you to share and discuss this information with your doctor. Orphanet is the French National Institute for Health and Medical Research and the Health Programme of the European Union.
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