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Association of common variable immunodeficiency and rare and complex connective tissue and musculoskeletal diseases. A systematic literature review
J. Mucke1, A. Cornet2, T. Witte3, M. Schneider4
- Policlinic and Hiller Research Unit for Rheumatology, ERN-ReCONNET member, University Clinic Duesseldorf, Heinrich-Heine-University Duesseldorf, Germany. johanna.mucke@med.uni-duesseldorf.de
- ERN-ReCONNET Patient Advocacy Group Advocate, and Lupus Europe, Brussels, Belgium.
- Department of Immunology and Rheumatology, Medical School Hannover, Germany.
- Policlinic and Hiller Research Unit for Rheumatology, ERN-ReCONNET member, University Clinic Duesseldorf, Heinrich-Heine-University Duesseldorf, Germany.
CER15324
2022 Vol.40, N°5 ,Suppl.134
PI 0040, PF 0045
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PMID: 35349404 [PubMed]
Received: 10/11/2021
Accepted : 21/03/2022
In Press: 28/03/2022
Published: 18/05/2022
Abstract
OBJECTIVES:
To perform a systematic literature review (SLR) on the association of common variable immunodeficiency (CVID) and rare and complex connective tissue and musculoskeletal diseases, namely systemic lupus erythematosus (SLE), Sjögren’s syndrome (SS), idiopathic inflammatory myopathies (IIM), systemic sclerosis (SSc), relapsing polychondritis, antiphospholipid syndrome, immunoglobulin (Ig) G4-related disease, as well as undifferentiated and mixed connective tissue disease.
METHODS:
An SLR on studies and cases about the association of CVID and rare and complex connective tissue and musculoskeletal diseases was performed. Animal studies were excluded.
RESULTS:
170 publications fulfilled the inclusion criteria. Sjögren’s syndrome was the most frequent connective tissue disease in CVID-patients. Most case reports exist on SLE and CVID with SLE mostly preceding the manifestation of CVID. Multiple cases were published reporting the concurrence of CVID and inclusion body myositis and single cases were found on CVID and antisynthetase syndrome, polymyositis, limited SSc and relapsing polychondritis, respectively. There are no cases of CVID and antiphospholipid syndrome, IgG4-related disease, as well as undifferentiated and mixed connective tissue disease.
CONCLUSIONS:
The concurrence of CVID and complex connective tissue and musculoskeletal diseases, especially SS, IIM, SSc and relapsing polychondritis is rare but relevant. The measurements of Ig-levels should be performed before the initiation of immunosuppressive therapy to allow for the differentiation of primary and secondary Ig-deficiency and substitute IG if necessary.
