Systemic Lupus Erythematosus as Manifestation of Common Variable Immunodeficiency Disorder

2021 ◽  
pp. 160-168
Author(s):  
Carmen M. Cabrera
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Common Variable Immunodeficiency ◽  
Lupus Erythematosus ◽  
Systemic Lupus ◽  
Immunodeficiency Disorder ◽  
Common Variable

Common Variable Immunodeficiency in Systemic Lupus Erythematosus

2007 ◽  
Vol 36 (4) ◽  
pp. 238-245 ◽  
Author(s):  
Mónica Fernández-Castro ◽  
Susana Mellor-Pita ◽  
María Jesús Citores ◽  
Pilar Muñoz ◽  
Pablo Tutor-Ureta ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Common Variable Immunodeficiency ◽  
Lupus Erythematosus ◽  
Systemic Lupus ◽  
Common Variable

scholarly journals Prosthetically Driven Therapy for a Patient With Systemic Lupus Erythematosus and Common Variable Immunodeficiency: A Case Report

2018 ◽  
Vol 44 (6) ◽  
pp. 447-455 ◽  
Author(s):  
Alexander Drew ◽  
Nurit Bittner ◽  
Whitney Florin ◽  
Alia Koch
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Case Report ◽  
Common Variable Immunodeficiency ◽  
Lupus Erythematosus ◽  
Dental Treatment ◽  
Comprehensive Treatment ◽  
Systemic Lupus ◽  
Digital Dentistry ◽  
Implant Placement ◽  
Common Variable

Patients who have systemic diseases in conjunction with severely resorbed maxillary and mandibular bone present challenges for dental implant therapy and rehabilitation. This case report describes the interdisciplinary comprehensive treatment completed on a patient with systemic lupus erythematosus (SLE) and common variable immunodeficiency (CVID). Patients with these systemic conditions present a multifactorial challenge for dental treatment due to advanced carious lesions, missing teeth, lack of adequate bone quality and quantity, as well as secondary effects of their medications. The sequence of treatment presented allowed for the necessary case control to ensure successful, predictable reconstruction of the edentulous patient with limited bone available for implant placement. For this patient, we used a combination of autogenous iliac bone graft, bilateral maxillary sinus lifts with BMP-2, transitional implants, and dental endosseous root form implants. Digital dentistry aided in designing the final implant supported fixed restorations. Transitional implants eliminated the need for tissue-borne prostheses, avoiding pressure to the graft and implants. Digital dentistry allowed for prosthetically driven implant placement and a functional, esthetic result. The techniques and staging presented for implant placement and rehabilitation can be used for other patients presenting with similar challenging conditions.

Systemic Lupus Erythematosus and Common Variable Immunodeficiency

2021 ◽  
Vol Publish Ahead of Print ◽  
Author(s):  
Antoine Azar ◽  
Nawras Aldaoud ◽  
Dylan Hardenbergh ◽  
Rebecca Krimins ◽  
James Son ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Common Variable Immunodeficiency ◽  
Lupus Erythematosus ◽  
Systemic Lupus ◽  
Common Variable

scholarly journals Development of common variable immunodeficiency in IgA- and IgG2-deficient patients with systemic lupus erythematosus

2011 ◽  
Vol 27 (3) ◽  
pp. 489-492 ◽  
Author(s):  
Kazuhide Suyama ◽  
Yukihiko Kawasaki ◽  
Yusaku Abe ◽  
Masahiro Watanabe ◽  
Shinichiro Ohara ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Common Variable Immunodeficiency ◽  
Lupus Erythematosus ◽  
Systemic Lupus ◽  
Common Variable

scholarly journals Common Variable Immunodeficiency Associated with Hepatosplenic T-Cell Lymphoma Mimicking Juvenile Systemic Lupus Erythematosus

2011 ◽  
Vol 2011 ◽  
pp. 1-4 ◽  
Author(s):  
A. A. Jesus ◽  
C. M. A. Jacob ◽  
C. A. Silva ◽  
M. Dorna ◽  
A. C. Pastorino ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
T Cell ◽  
Common Variable Immunodeficiency ◽  
Lupus Erythematosus ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Systemic Lupus ◽  
Juvenile Systemic Lupus Erythematosus ◽  
Hepatosplenic T Cell Lymphoma ◽  
Common Variable

Common variable immunodeficiency (CVID) is a heterogeneous disorder with susceptibility to infections, autoimmune manifestations, and cancer. To our knowledge, CIVD with T-cell lymphoma mimicking juvenile systemic lupus erythematosus (JSLE) was not described in the literature, and one case was reported herein. An 8-year-old female was admitted in our Pediatric Immunology Unit with a clinical history of hypogammaglobulinemia, recurrent upper respiratory infections, and pneumonias. She had a marked decrease of three serum immunoglobulin isotypes, and the diagnosis of CVID was established. At the age of 17 years, she presented with oral ulceration, nonerosive arthritis, nephritis, serositis, cytopenia, positive antiphospholipid antibodies, and positive antinuclear antibody fulfilling the American College of Rheumatology (ACR) criteria for SLE. She was treated with intravenous methylprednisolone for three consecutive days, and intravenous immunoglobulin, and maintenance therapy of chloroquine, azathioprine and prednisone 40 mg/day. Two months later, she died of septic shock secondary to acute pneumonia. The necropsy showed hepatosplenic T-cell lymphoma with diffuse involvement of bone marrow, spleen, liver, and lungs. The lymphoma cells were positive for CD3 immunostaining and negative for CD20 and lysozyme. In conclusion, the association of CVID and hepatosplenic T-cell lymphoma may simulate JSLE diagnosis.

scholarly journals Shared Pathogenetic Features Between Common Variable Immunodeficiency and Sjögren’s Syndrome: Clues for a Personalized Medicine

2021 ◽  
Vol 12 ◽  
Author(s):  
Luca Quartuccio ◽  
Ginevra De Marchi ◽  
Simone Longhino ◽  
Valeria Manfrè ◽  
Maria Teresa Rizzo ◽  
...  
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Sjögren’S Syndrome ◽  
Common Variable Immunodeficiency ◽  
Lupus Erythematosus ◽  
Sjögren's Syndrome ◽  
Sjogren’S Syndrome ◽  
Sjogren's Syndrome ◽  
Systemic Lupus ◽  
Common Variable ◽  
Sjögren‘S Syndrome

Common variable immunodeficiency disorders (CVID) are a group of rare diseases of the immune system and the most common symptomatic primary antibody deficiency in adults. The “variable” aspect of CVID refers to the approximately half of the patients who develop non-infective complications, mainly autoimmune features, in particular organ specific autoimmune diseases including thyroiditis, and cytopenias. Among these associated conditions, the incidence of lymphoma, including mucosal associated lymphoid tissue (MALT) type, is increased. Although these associated autoimmune disorders in CVID are generally attributed to Systemic Lupus Erythematosus (SLE), we propose that Sjogren’s syndrome (SS) is perhaps a better candidate for the associated disease. SS is an autoimmune disorder characterized by the lymphocytic infiltrates of lacrimal and salivary glands, leading to dryness of the eyes and mouth. Thus, it is a lymphocyte aggressive disorder, in contrast to SLE where pathology is generally attributed to auto-antibody and complement activation. Although systemic lupus erythematosus (SLE) shares these features with SS, a much higher frequency of MALT lymphoma distinguishes SS from SLE. Also, the higher frequency of germ line encoded paraproteins such as the monoclonal rheumatoid factor found in SS patients would be more consistent with the failure of B-cell VDJ switching found in CVID; and in contrast to the hypermutation that characterizes SLE autoantibodies. Thus, we suggest that SS may fit as a better “autoimmune” association with CVID. Examining the common underlying biologic mechanisms that promote lymphoid infiltration by dysregulated lymphocytes and lymphoma in CVID may provide new avenues for treatment in both the diseases. Since the diagnosis of SLE or rheumatoid arthritis is usually based on specific autoantibodies, the associated autoimmune features of CVID patients may not be recognized in the absence of autoantibodies.

Concurrent systemic lupus erythematosus and common variable hypogammaglobulinemia

1985 ◽  
Vol 28 (4) ◽  
pp. 462-465 ◽  
Author(s):  
Anthony Stein ◽  
Alan Winkelstein ◽  
Amrit Agarwal
Keyword(s):  
Systemic Lupus Erythematosus ◽  
Lupus Erythematosus ◽  
Systemic Lupus ◽  
Common Variable
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