WHAT IS NEW IN MEDICINE – GOODPASTURE SYNDROME
Clinical Management of Pregnancy in Women with Goodpasture Syndrome
Goodpasture’s syndrome, although a rare, but also a well-known autoimmune disease, affects mainly the kidneys and lungs of a person. This pathology is also found in pregnant women.
The article by Czech scientists published in January 2015 in the international peer-reviewed journal Gynecologic and Obstetric Investigations refer to cases of Good pesture syndrome occurring during pregnancy that can pose a serious threat to the life of the mother and fetus. The article summarizes the modern experience of the clinical diagnosis and treatment of Goodpasture’s syndrome during pregnancy.
Researchers, the authors of the article conducting an in-depth literature search, an overview of all published articles or case studies highlighting Goodpasture’s syndrome during pregnancy. The following data were extracted from each specific case of the syndrome during the woman’s pregnancy: the age of the patient, the gestational age, the good-for-pregnancy syndrome, the pregnancy of the child, the neonatal history of the child, and the patient’s kidney condition.
A review of the available literature showed 4 cases of Goodpasture’s syndrome during pregnancy. The mean age of the patients was 29.3 ± 2.5 years, and most of them were pervious, with an average ratio of 1.3 ± 1.5. The mean gestational age at the time of diagnosis was 12.5 ± 5.9 weeks. Therapy of Goodpasture’s syndrome. This fact is not consistent with international recommendations regarding the treatment of that syndrome. In addition, neonatal outcomes in neonates were also very individual, among all observed cases.
It is worth noting that the appearance of a good-for-life syndrome during pregnancy, after all, is very rare. This is an unusual complication of pregnancy, which is associated with significant harm to both the mother and the fetus. Pregnancy management in the case of pregnancy requires intensive care, close observation, monitoring of patient and fetus conditions, counseling of a number of specialists and interdisciplinary cooperation of doctors. However, to date, there are no unified protocols for administering goodpasture’s syndrome.
Glomerulopathy, induced by immunization with a peptide derived from the α3IV-NC1 antigen found in the Goodpasture’s syndrome.
Mice with experimental autoimmune glomerulonephritis represent a model of human antiglomerular disease of the renal basement membrane, which depends on the responses of antibodies and cells -chain domain of type IV collagen (α3IV-NC1). The purpose of this study was to further characterize the T-cell immune response in this syndrome.
Repeated immunization of mice with α3IV-NC1 antigen caused fatal development of glomerulonephritis in DBA1 mice. Although two grafts were sufficient to create high α3IV-NC1-specific IgG antibody titres, and also to deposit them along the glomeruli of the basal membranes of the kidneys and develop the nephrotic syndrome, two additional inoculations were necrotic or sickle-shaped glomerulonephritis.
Ten days after the first immunization, α3IV-HC1-specific CD4 + cell-producing TNF-α, IFN-γ or IL-17A were detected in the spleen of mice. With the advent of necrotic or sickle-shaped glomerulonephritis, about 0.15% of renal CD4 + cells were specific for the α3IV-NC1 antigen. Using the peptides covering the entire domain of the α3IV-NC1 antigen, three immunodominant T-cell epitopes were identified. Immunization with these peptides did not lead to the clinical signs of experimental autoimmune glomerulonephritis, nor did it lead to the development of necrotic or sickle-shaped glomerulonephritis. However, in mice immunized with one of the peptides (STVKAGDLEKIISRC), circulating antibodies against the murine antigen α3IV-NC1, which were first detected at week 8, were developed.
Collectively, the results obtained that autoreactive T cells are capable of inducing the formation of pathological autoantibodies. The quality and quantity of α3IV-NC1-specific antibodies and T-cells in the blood are critical to the phenotype of developing glomerulonephritis.
