Common Variable Immunodeficiency in Systemic Lupus Erythematosus

Fernández‐Castro, Mónica; Mellor‐Pita, Susana; Cítores, M.J.; Muñoz, Pilar; Tutor-Ureta, Pablo; Silva, Lucía; Vargas, Juan A.; Yebra-Bango, M; Andreu, José Luís

doi:10.1016/j.semarthrit.2006.09.005

Cited by 58 publications

(45 citation statements)

References 20 publications

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“…Both cyclophosphamide and azathioprine have been reported to cause reversible hypogammaglobulinemia in patients with SLE. 14,40,41 Yet, such secondary hypogammaglobulinemic states present as panhypogammaglobulinemia, involving decreased levels of IgG and other immunoglobulin isotypes. 14,40 Remarkably, all 10 cases presently reported as immunoglobulin PID had consistently very low levels of one immunoglobulin class or subclass with normal or high levels of the other immunoglobulin classes, including total IgG and subclasses ( Figure 2).…”

Section: Discussionmentioning

confidence: 99%

Complement and antibody primary immunodeficiency in juvenile systemic lupus erythematosus patients

Jesús

Liphaus

Silva

et al. 2011

Lupus

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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.

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Section: Discussionmentioning

confidence: 99%

Complement and antibody primary immunodeficiency in juvenile systemic lupus erythematosus patients

Jesús

Liphaus

Silva

et al. 2011

Lupus

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“…In patients with SLE, hypergammaglobulinemia is often present; paradoxically, however, hypogammaglobulinemia similar to common variable immunodeficiency occasionally occurs and may relate to the presence of lymphocytotoxic autoantibodies 12, 13. Selective IgM and IgA deficiency has also been reported 14, 15.…”

Section: Introductionmentioning

confidence: 99%

Pragmatic Treatment of Patients With Systemic Lupus Erythematosus With Rituximab: Long‐Term Effects on Serum Immunoglobulins

Reddy

Martinez

Isenberg

et al. 2017

Arthritis Care & Research

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ObjectiveB cell–depletion therapy based on rituximab is a therapeutic option for refractory disease in patients with systemic lupus erythematosus (SLE). The aim of this observational study was to document long‐term effects on B cell function by following serum immunoglobulin levels in patients with SLE treated with rituximab in routine clinical practice.MethodsWe included 57 consecutive patients with SLE treated with rituximab and concomitant/sequential immunosuppressants and measured serum total IgG, IgM, and IgA and IgG anti‐dsDNA antibodies, over a median of 48 months most recent followup. Flow cytometry was used prospectively to assess B cell phenotypes in 17 of 57 patients.ResultsTwelve patients (21%) had persistent IgM hypogammaglobulinemia (<0.4 gm/liter), and 4 of 57 (5%) had low IgG (<7 gm/liter) at the most recent followup (range 12–144 months). This was not associated with serious adverse events or high anti–double‐stranded DNA (anti‐dsDNA) antibodies (>1,000 IU/ml; normal <50 IU/ml). Factors predictive of low serum IgM included baseline serum IgM ≤0.8 gm/liter (receiver operator curve analysis) and subsequent therapy with mycophenolate mofetil (MMF; odds ratio 6.8, compared with other immunosuppressants). In patients maintaining normal IgM levels (9 of 17), the frequency of circulating IgD+CD27+ B cells was significantly higher (P = 0.05). At 12 months after rituximab, 7 of 30 SLE patients with baseline anti‐dsDNA ≤1,000 IU/ml had lost seropositivity.ConclusionLower baseline serum IgM levels and sequential therapy with MMF were predictive of IgM hypogammaglobulinemia after rituximab in SLE, but this was not associated with higher levels of anti‐dsDNA antibodies or an increased risk of infections. This provides useful directions for clinicians regarding rituximab and sequential immunosuppressive treatment for patients with SLE.

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“…At the time of the initial diagnosis, patients had normal sera immunoglobulins and it was later that hypogammaglobulinemia developed. While in the majority, the disease activity and immunological markers decreased after the development of CVID, in other patients the disease remained unchanged [3].…”

Section: Discussionmentioning

confidence: 94%

“…Cyclophosphamide, azathioprine, mycophenolate mofetil, rituximab, corticosteroids and other drugs including phenytoin have been reported to cause hypogammaglobulinemia [3,[10][11][12].…”

Section: Discussionmentioning

confidence: 99%

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Systemic lupus erythematosus as a first presentation of common variable immunodeficiency associated with infrequent mannose-binding lectin gene polymorphisms

et al. 2009

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The association of common variable immunodeficiency (CVID) and Systemic Lupus Erythematosus (SLE) is infrequent. Mannose-binding lectin (MBL) has been shown to play a role in CVID and SLE. The purpose of this study is to describe two cases of CVID who presented as SLE and also evaluate the presence of MBL polymorphisms and MBL serum levels in those patients.In both patients, SLE was the first manifestation of CVID. In these patients the SLE immunological markers and disease activity disappeared after the development of CVID. They carried the very infrequent MBL haplotype 4Q-57Glu. One of them had a homozygous genotype, whereas the other patient was heterozygous and also presented the haplotype 4P-57Glu that had never been previously detected. Interestingly, this last patient was presenting frequent respiratory tract infections, developed brochiectasis and had low levels of circulating MBL.These results may support the role of MBL in the development of autoimmunity in CVID. Further genetic studies are needed to clarify the role of the MBL polymorphisms in the development of autoimmunity in CVID.

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Common Variable Immunodeficiency in Systemic Lupus Erythematosus

Cited by 58 publications

References 20 publications

Complement and antibody primary immunodeficiency in juvenile systemic lupus erythematosus patients

Complement and antibody primary immunodeficiency in juvenile systemic lupus erythematosus patients

Pragmatic Treatment of Patients With Systemic Lupus Erythematosus With Rituximab: Long‐Term Effects on Serum Immunoglobulins

Systemic lupus erythematosus as a first presentation of common variable immunodeficiency associated with infrequent mannose-binding lectin gene polymorphisms

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