Complement and antibody primary immunodeficiency in juvenile systemic lupus erythematosus patients

Abstract

Objective: To evaluate the frequency of primary immunodeficiencies (PID) in juvenile systemic lupus erythematosus (JSLE) patients. Methods: Some 72 JSLE patients were analyzed for levels of immunoglobulin classes and IgG subclasses and early components of the classical complement pathway. Determination of C4 gene copy number (GCN) and detection of type I C2 deficiency (D) were also performed. Results: PID was identified in 16 patients (22%): C2D in three, C4D in three, C1qD in two, IgG2D (<20 mg/dl) in four, IgAD (<7 mg/dl) in three, and IgMD (<35 mg/dl) in three; one of these patients presented IgA, C2 and C4D. Two patients had low C4 GCN and two had type I C2D. Demographic data, family history of autoimmune disease and PID, JSLE clinical findings, occurrence of infections, disease activity and therapies were similar in patients with and without PID (p > 0.05). Remarkably, the median of Systemic Lupus International Collaborating Clinics/ACR-damage index (SLICC/ ACR-DI) was significantly higher in JSLE patients with PID compared with patients without these abnormalities (p ¼ 0.0033), likewise the high frequency of SLICC/ACR-DI > 1 (p ¼ 0.023). Conclusions: A high frequency of PID was observed in JSLE patients, suggesting that these defects may contribute to lupus development. Our findings indicate that these two groups of PID should be investigated in severe pediatric lupus. Lupus (2011) 20, 1275-1284.

Complications of autologous hematopoietic stem cell transplantation for patients with autoimmune diseases

“…Patients with ADs who are eligible for HSCT have often received several lines of immunosuppressive therapies before HSCT, and some ADs are per se accompanied by defects of the immune system (e.g., SLE and complement deficiencies) (8). These factors contribute to the risk of infection after HSCT.…”

Autoimmune diseases and autoantibodies in pediatric patients and their first-degree relatives with immunoglobulin A deficiency

“…[9][10][11][12][13]26 Recently, IgAD has also been evidenced in 4% of our c-SLE population. 27 Additionally, hematologic autoimmune disorders are very frequent in PID patients, especially antibody deficiencies, such as common variable immunodeficiency and IgAD, particularly idiopathic thrombocytopenic purpura and autoimmune haemolytic anemia, as evidenced in two of our IgAD patients. 28 Importantly, the diagnosis of CD in IgAD patients may be very difficult, since the majority of the tests are based on specific IgA antibody.…”

Associação de imunodeficiência primária com lúpus eritematoso sistêmico: revisão da literatura e as lições aprendidas pela Divisão de Reumatologia de um hospital universitário terciário em São Paulo

“…Também observou--se nessa coorte uma grande quantidade de pacientes com LES com deficiência de subclasses de IgG, enquanto a literatura relata apenas alguns casos de deficiência isolada de IgG2 e IgG4. 18,41,71,72 No presente estudo, todos os pacientes com deficiência de IgG4 e 75% daqueles com deficiência de IgG3 tinham nefropatia lúpica, o que corresponde a uma frequência de aproximadamente 50% de todos os casos de nefrite lúpica observados nessa coorte. Além disso, os pacientes com DIgM apresentaram menor frequência de úlceras orais.…”

“…17 Nosso grupo relatou que uma grande fração de pacientes com LES juvenil apresentava uma das várias formas de IDP. 18 Mais recetemente, documentou-se que 28% de uma coorte consecutiva com 300 pacientes adultos com LES apresentavam algum tipo de IDP, principalmente relacionada com a deficiên-cia de imunoglobulina. Nesta revisão, descrevem-se as diversas associaç ões entre as IDP e o LES ou manifestaç ões semelhantes ao LES, além da nossa experiência no ambulatório…”