Heyman's nephritis is most probably a disease that may persist for an extended period without cure. Close surveillance is required to anticipate new symptoms of flare-ups of an already present renal disease.
Nephritis can be treated with medications or immunosuppressive treatment. Most patients need a combination of different treatments. However, the combination of corticosteroid, mycophenolate mofetil, anakinra or rituximab is superior. There is no difference in efficacy of rituximab or tacrolimus in relapse-free, progression-free, or patient survivals. All patients with kidney transplants need long-term immunosuppressants in combination with medications and biologic agents. In most cases, patients need treatment for life.
Heymann nephritis, described by the French medical term in 1889, affects a group of persons of European descent with hereditary antineutrophil cytoplasmic autoimmunity usually associated with congenital heart defects, malformations of the kidneys (kidney abnormalities), mental retardation and other developmental abnormalities. The disorder was first described by the pathologist Karl Heine Heymann (1847-1909). Heymann nephritis and granulomatous or fibrino-follicular (necro-)kidney disease share similar clinical presentations and is a manifestation of the autoimmune syndrome.
While there are many specific signs and symptoms of HN, there is currently no single sign that will indicate an autoimmune disease. Instead, HN can be diagnosed if abnormalities of urinalysis, blood and kidney biopsy (in HN) and renal biopsy are consistent with antibody-mediated vasculitis and vasculitis with glomerulopathy is confirmed.
The cause of Heymann nephritis is a mixture of factors, rather than a single agent or cause. A major factor was certainly related to genetics. Some other factors are thought to have been due to immunologic dysfunction (inflammation) in the kidney. A genetic predisposition may help explain the increased incidence among people with HLA-DR3.
Between 1994 and 2007, the reported incidence of HN varied widely. Although an increase in the rate of HN has been documented in some population-based registries, others report no discernible increase. A variety of factors may explain this phenomenon, including subtle changes in diagnostic criteria, changes in the frequency of HN, or changes in medical management of patients with renal disease. Further studies are needed to elucidate these important issues.
A study of a limited cohort of patients with HKD showed no correlation between demographic parameters, histological findings, severity, or clinical presentation, and enrollment into a clinical trial, or the response to treatment. The main reasons for proceeding to RCT were patients' subjective complaints and the need to prevent progressive renal destruction. The response to treatment was limited to a small group of patients in whom renal function remained stable and, more importantly, to the patients who benefited from intensive therapy. Clinical trials should be considered only for patients with severely progressive AKD, or for whom RCT may be of benefit.
This article reviews current research on the treatment of Heymann nephritis. We hope that it will help you understand and treatment this condition and avoid many complications. (https://www.dartmouth.edu/fractures-pediatrics/heymannns/what-is-heymns/).
There are several new discoveries for treating HN. There are several types of immunomodulatory agents such as monoclonal antibodies that are being tested. As of now there is no conclusive evidence on whether these compounds are a cure or improvement to the disease severity. However, they do show great potential for new treatments for the disease. Immunoglobulin (Ig) is a compound that is being studied as a medication for treating HN. There are specific antibody that are being studied as a therapy in people's life-spans and ages such as anti-platelet receptors and Fc receptors on macrophages. These different types of antibody are very safe and can only help with the HN disease.
Heymann nephritis does have an impact on family life and this should be taken into account when assessing the value of familial studies as a means of identifying susceptibility genes.
Untreated Heymann nephritis can cause progressive [kidney dysfunction, kidney failure, and death], but treatment with the right immunosuppressive drug at the right time can prevent death.
Obinutuzumab had previously been investigated in the treatment of patients with chronic active steroid-refractory rheumatic diseases and severe active/active lupus nephritis. This drug was originally designated CUBIC-001 in early phase III clinical trials with patients with severe active/active lupus nephritis. In one of these trials, patients on therapy with obinutuzumab (obinutuzumab) had a 50% reduction in the necessity for renal transplantation (from 21% to 11%) compared with those on placebo therapy (from 25% to 26%).