Introduction
Histologically, the lesion is characterized by highly structured ultrastructural deposits that are negative for amyloid by Congo red stain and appear composed of immunoglobulin and complement. Patients do not exhibit any signs of paraproteinemia, amyloidosis, systemic lupus erythematosus, cryoglobulinemia, or any other illnesses linked to glomerular deposits, which also have a highly ordered tactoidal or fibrillar characteristic. This is the case both clinically and serologically. Immunotactoid glomerulopathy may be a primary glomerulopathy since it is not a multisystemic disease process.
Proteinuria (nephrotic range in over 60 % of patients) and renal insufficiency are present in more than half of patients with immunotactoid glomerulopathy patients. Over 40 % of the individuals who have been reported to date have progressed to End-Stage Renal Failure. There is little and unimpressive experience treating this illness with immunosuppression and/or prednisone. Renal transplantation has been successful in four patients; however, in two of them, proteinuria returned and was linked to the return of immunotactoid glomerulopathy in the renal allograft.
What is Immunotactoid Glomerulopathy?
The disorder called immunotactoid glomerulopathy prevents the kidneys from functioning normally. The term "tactoid" refers to a collection of structures resembling rods, and "immuno" indicates that they are connected to the immune system. Antibodies are typically produced by immune system cells and aid in the defense against infections. These antibodies are unusually deposited in the kidneys in this condition. As a result, distinct rod-like arrays—known as tactoids—form. Specialized microscopes can be used to see these. The term "glomerulopathy" refers to a disease that affects the kidney's tiny glomeruli, which act as filters. Urine is produced by the glomeruli filtering the blood. This is made up of the body's surplus water and waste products. This is comparable to a water filter that creates clean water by capturing pollutants in the water. On the other hand, waste items can pass through the kidneys' filters and into the urine, which is how they "clean" the blood. The most beneficial and significant chemicals in the blood cannot get past these filters. They stay in the circulation. Cells and proteins are necessary for the kidney filters to establish a normal filtration barrier.
Normally, proteins cannot enter the urine due to the filtration barrier. The blood's barrier maintains the proteins there. When the filter is broken, proteins can cross the filter and enter the urine. This is comparable to when there's a hole in a coffee filter that lets coffee grinds get through and into the coffee. Nephrotic syndrome and symptoms like ankle and foot edema may result from a protein deficiency in the urine.
How Immunotactoid Glomerulopathy Appears Under A Microscope?
Kidney biopsy is necessary for this disease's diagnosis. A kidney sample is removed, and pathologists—specialist medical professionals—examine it. These medical professionals can see the various kidney cells and structures using microscopes. The kidney is magnified 10 to 100 times with light microscopy. It makes the different cell types and kidney filters visible. Under a microscope, biopsies from people with this illness can look different. It may appear quite similar to other renal illnesses.
Light microscopy is a useful diagnostic tool for many kidney disorders. However, an electron microscope, which is far more powerful, is needed to diagnose this illness. Usually, it has a magnification of 10,000 times or more. This disease has a very distinctive pattern of groups of rod-like features using electron microscopy. Like a stack of pipes or logs, they are arranged in lines. The rod-like structures are called microtubular structures. In the middle, they seem "hollow." There is evidence to show that immunoglobulins are present in these rod-like structures.
What Are the Causative Approaches for Immunotactoid Glomerulopathy?
This disease's precise etiology is still unknown. Most people are older than fifty. A lymphoproliferative illness is also present in many of these patients. This is an aberrant proliferation of specific immune system cells. Patients with this illness have been discovered to have both lymphomas and leukemias. The production of immunoglobulins is frequently elevated in response to aberrant lymphocyte proliferation. The kidney filters are harmed as a result of these being deposited there. In the end, renal failure can happen. Consequently, to determine whether one has lymphoproliferative disorder, the doctor could prescribe more testing.
What Are the Signs and Symptoms Present In Immunotactoid Glomerulopathy?
When a patient is first diagnosed, their urine typically contains protein. Their nephrotic syndrome is common. The protein may cause leg and ankle edema in the urine. Renal impairment is typically present to some extent in patients. At diagnosis, hypertension or high blood pressure may also be present. A microscope can detect some patients' tiny amounts of red blood cells in their urine. Additional systemic symptoms associated with the disease may also be present in patients with lymphoproliferative disorders. Fatigue, weight loss, or easy bleeding or bruises are some of these symptoms.
What Are the Treatment Options for Immunotactoid Glomerulopathy?
As of yet, there isn't a single effective treatment. An expert in blood cells and malignancies typically treats patients with an underlying lymphoproliferative illness, referred to as a hematologist/oncologist. Chemotherapy is typically used to treat these people, and kidney function may be enhanced by treating the underlying illness. Patients who do not have a lymphoproliferative illness are frequently prescribed immune system-suppressive drugs. Moreover, drugs known as cytotoxic agents—which destroy immune cells—are used to treat them. With these treatments, many people do not get better, though. Kidney failure may progress over time. Doctors are still researching novel ways to treat patients with this illness.
Conclusion
The kidneys' capacity to eliminate waste materials and extra fluid from the body is compromised if the filters are damaged. At the time of diagnosis, the majority of individuals with this condition have some degree of renal impairment is referred to as a chronic renal disease. When most filters sustain injury, the kidneys' ability to efficiently filter waste materials is compromised. End-Stage Kidney Disease (ESKD) is developed in patients. Although the exact course of the illness is unknown, some research indicates that by the time five years pass following the diagnosis, 50% of patients may have ESKD.
Kidney replacement therapy is necessary for ESKD patients. Dialysis or kidney transplantation are the options. Successful transplants have been performed on patients with this illness. The illness can return to the kidney that was transplanted. However, because this disease is so uncommon, it is still unknown to what extent and how frequently it recurs.
