Development of systemic sclerosis in a patient with common variable immunodeficiency

Balcı, Mehmet; Pamuk, Gülsüm Emel; Dönmez, Salim; Pamuk, Ömer Nurı

doi:10.1016/j.clim.2015.09.009

Cited by 1 publication

(1 citation statement)

References 12 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…One case of concurrent CVID and limited systemic sclerosis was identified (28). The female patient had the diagnosis of anti-centromere-antibody positive scleroderma at 53 years and received methotrexate.…”

Section: Cvid and Sscmentioning

confidence: 99%

Association of common variable immunodeficiency and rare and complex connective tissue and musculoskeletal diseases. A systematic literature review

Mücke

Cornet

Witte

et al. 2022

Clinical and Experimental Rheumatology

View full text Add to dashboard Cite

Objective. 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. Conclusion. 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.

show abstract

Section: Cvid and Sscmentioning

confidence: 99%