In this light, an additional question arises about the safe reduction of standard immunosuppressive therapy under the protective permanent IVIG administration, with the ultimate goal of reducing calcineurin inhibitor (CNI) toxicity on the one hand and preventing episodes of infection around the other

In this light, an additional question arises about the safe reduction of standard immunosuppressive therapy under the protective permanent IVIG administration, with the ultimate goal of reducing calcineurin inhibitor (CNI) toxicity on the one hand and preventing episodes of infection around the other. Appropriate management of our patient’s acquired immunodeficiency was initiated with the administration of IVIG 500 mg/kg every 2 weeks targeting at IgG levels > 500 mg/dl. received a cadaveric kidney transplant. IVIG was continued along with standard immunosuppression so that both recurrent infections and allograft rejection are avoided. Patient is closely monitored, and her post-transplant course is usually amazingly satisfying so far. ESKD Lomustine (CeeNU) patients with immunodeficiency syndromes should not be excluded by definition from kidney transplantation. Keywords: kidney transplantation, immunodeficiency, systemic lupus erythematosus, hypogammaglobulinemia, intravenous immunoglobulin Introduction Systemic Lupus Erythematosus and Autoimmunity Lupus nephritis MAP3K3 remains a significant cause of end-stage kidney disease (ESKD). Despite the improvement in renal survival during the past decades due to new therapies, the 5-12 months incidence of ESKD in patients with lupus nephritis is usually 11% (1). Lupus clinical course is characterized by periods of remissions and unpredicted flareups that dictate the need and even the increase of immunosuppressive treatment in order to control symptoms. The cumulative impact of immunosuppression on patients is reflected around the incidence of infections (2) and even neoplasms (3) around the long-term, making clear the need for minimization of toxicity. Nevertheless, the dysregulations of immunity that are present are related to the pathophysiology of lupus with emphasis on coexisting genetic variations, lymphopenia and hypocomplementemia aggravating further the susceptibility to infections (4). Systemic lupus erythematosus (SLE) is usually a systemic multi-organ chronic disease with genetic predisposition and environmental triggering that lead to the production of autoantibodies against nuclear antigens which are responsible for the disease manifestations. Shared genetic pathways determine the complex interplay between SLE and immunity disorders (4, 5). The underlying deficiency in match components, the defective immunoglobulin synthesis (partial deficiencies in IgA and IgM mostly) and/or the co-existence of granulomatous or other autoimmunity disorders (i.e., Wiskott-Aldrich syndrome, autoimmune lymphoproliferative syndrome etc) are the main parameters of clinically expressed immunodeficiency and SLE (6). Under these circumstances, there is a constant activation of the immune system against self-antigens, a dysregulated immune complex formation and degradation that lead to unpredicted tissue damage enhancing further any pre-existing tendency toward autoimmunity and SLE as well (4, 5). Immunodeficiency Syndromes The range of immunodeficiency syndromes (Is usually) is usually wide and consists of main forms with genetic predisposition and secondarily induced immunity dysregulations that lead to hypogammaglobulinemia and frequent infections (7). Lomustine (CeeNU) Diagnosis demands broad investigation and exclusion of secondary causes. Despite efforts and consensus reports on diagnostic criteria for IS there are overlapping cases that do not fall into one category in specific, termed unspecified hypogammaglobulinemia (8) and mirror the complexity in diagnosing and classifying Is usually (8, 9). The exact prevalence of immunodeficiency syndromes is not known as the field is currently under study but secondary forms of Is usually are adding up making the interest for the establishment of a classification even greater (10). Secondary immunodeficiency has been described in patients suffering from hematologic malignancies (lymphoma, multiple myeloma) as well as those under immunosuppressants. A special group of patients are solid organ transplant patients who are likely to develop hypogammaglobulinemia post transplantation but especially Lomustine (CeeNU) those with pre-existing gamma globulin dysregulations and an intention to receive an allograft plus immunosuppressants. As these patients are highly prone to infections and the aetiologic treatment of secondary IS with immunosuppressants’ withdrawal is not an option, close monitoring and proper translation of laboratory parameter results is usually a possible strategy. For example, low levels of match components indicate an increased risk for bacterial infections Lomustine (CeeNU) while enzyme-linked immune absorbent spot (ELISPOT) and circulation cytometry low response of anti-CD8 lymphocytes to cytomegalovirus (CMV) antigens reflect increased probability for CMV contamination (11). B-cell depleting therapies are established as a highly effective treatment option not only in lymphoproliferative disorders but also in collagen inflammatory diseases. However, one cannot avoid realizing the induced rise in the rate of infections that complicate patients’ course that may even be fatal (12), in the context of secondary Is usually. The main axis of treatment in patients with Is usually is the management of infections and their prevention through prophylactic antibiotics and antivirals. The substitution of gamma globulins.