- •CVID-associated neurological symptoms are frequently underreported, with studies showing prevalences of up to 50% in these patients.
- •Infections are the main cause of CVID-associated neurological dysfunction, followed by autoimmune/inflammatory conditions.
- •To date, there is no established standard of care for the treatment of patients with CVID, particularly with GLILD, a recently described subtype of interstitial lung disease, or neurological complications.
- •Treatment with steroids seems to be safe and effective in the control of neurological manifestations.
Abbreviations:ACE (Serum angiotensin-converting-enzyme), ADA (Adenosine deaminase test), ANA (Antinuclear antibody), ANCA (Antineutrophil cytoplasmic antibody), Anti-dsDNA (Anti-double strand DNA antibodies), Anti-MOG (Antibodies against myelin-oligodendrocyte-glycoprotein), Anti-AQ4 (Antibodies against aquaporin 4), Ca (Carbohydrate antigen), CDSS (Disease severity score for CVID and CVID-like disorders), CMV (Cytomegalovirus), CVID (Common variable immunodeficiency disorders), EBV (Epstein-Barr virus), ESID (European Society for Immunodeficiencies), GLILD (Granulomatous and Lymphocytic Interstitial Lung Disease), HIV (Human immunodeficiency virus), HSV (Herpes simplex virus), LIP (Lymphocytic interstitial pneumonia), NR (Normal range), PCR (Polymerase chain reaction), PET (Positron Emission Tomography), TPPA (Treponema pallidum particle agglutination), VZV (Varicella-zoster virus)
Purchase one-time access:Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
One-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:Subscribe to Multiple Sclerosis and Related Disorders
- Central nervous system involvement in common variable immunodeficiency: a case of acute unilateral optic neuritis in a 26 -year-old Italian Patient.Front. Neurol. 2018; 9: 1-7https://doi.org/10.3389/fneur.2018.01031
- Assessing disease severity in common variable immunodeficiency disorders (CVID) and CVID-like disorders.Front. Immunol. 2018; 9: 1-8https://doi.org/10.3389/fimmu.2018.02130
- Perspective: evolving concepts in the diagnosis and understanding of common variable immunodeficiency disorders (CVID).Clin. Rev. Allergy Immunol. 2020; 59: 109-121https://doi.org/10.1007/s12016-019-08765-6
- Granulomatous disease in CVID: retrospective analysis of clinical characteristics and treatment efficacy in a cohort of 59 patients.J. Clin. Immunol. 2013; 33: 84-95https://doi.org/10.1007/s10875-012-9778-9
- Granulomatous lymphocytic interstitial lung disease (GLILD) in common variable immunodeficiency (cvid): a multicenter retrospective study of patients from italian pid referral centers.Front. Immunol. 2021; 12: 1-10https://doi.org/10.3389/fimmu.2021.627423
- Common variable immunodeficiency with granulomatous-lymphocytic interstitial lung disease and preceding neurological involvement: a case-report.BMC Pulm. Med. 2020; 20: 1-8https://doi.org/10.1186/s12890-020-01231-6
- Recurrent myelitis in common variable immunodeficiency successfully managed with high-dose subcutaneous immunoglobulin.BMJ Case Rep. 2012; : 4-7https://doi.org/10.1136/bcr-01-2012-5637
- ESID Registry - Working Definitions for Clinical Diagnosis of PID [Internet].2016 (Available from:)
- The Central nervous system effects and mimicry of common variable immunodeficiency (CVID): a case report with literature review.Case Rep Rheumatol. 2019; 2019: 1-6https://doi.org/10.1155/2019/7623643
- Atypical epilepsy in common variable immunodeficiency: a single institution case series (P1.2-044).Neurology. 2019; 92
- Longitudinally extensive transverse myelitis: a rare association with common variable immunodeficiency.Pract. Neurol. 2015; 15: 49-52https://doi.org/10.1136/practneurol-2014-000953
- Common variable immunodeficiency-associated myelitis: report of treatment with infliximab.J. Neurol. 2008; 255: 1821-1824https://doi.org/10.1007/s00415-008-0898-3
- Neurologic conditions and symptoms reported among common variable immunodeficiency patients in the USIDNET.J. Clin. Immunol. 2020; 40: 1181-1183https://doi.org/10.1007/s10875-020-00861-z
- Recurrent intra-cranial granulomata presenting as space-occupying lesions in a patient with common variable immunodeficiency.Postgrad. Med. J. 1992; 68: 359-362https://doi.org/10.1136/pgmj.68.799.359
- Intra cranial granulomatous disease in common variable immunodeficiency: case series and review of the literature.Semin. Arthritis Rheum. 2018; 47: 890-896https://doi.org/10.1016/j.semarthrit.2017.10.014
- Sarcoidosis: pitfalls and challenging mimickers.Front. Med. 2021; 7: 1-15https://doi.org/10.3389/fmed.2020.594275
- Neurologic complications of common variable immunodeficiency.J. Clin. Immunol. 2016; 36: 793-800https://doi.org/10.1007/s10875-016-0336-8
- Immunosuppressive therapy with rituximab in common variable immunodeficiency.Clin. Mol. Allergy. 2019; 17: 1-11https://doi.org/10.1186/s12948-019-0113-3
- Unexpectedly high prevalence of common variable immunodeficiency in Finland.Front. Immunol. 2017; 8: 1-10https://doi.org/10.3389/fimmu.2017.01190
- Bilateral optic neuritis in a 26-year-old man with common variable immunodeficiency: a case report.J. Med. Case Rep. 2011; 5: 5-6https://doi.org/10.1186/1752-1947-5-319
- Encephalomyelitis with retinopathy in common variable immunodeficiency (CVID).Neuro-Ophthalmology. 2020; 44: 38-40https://doi.org/10.1080/01658107.2018.1542008
- Rituximab for the treatment of common variable immunodeficiency (CVID) with pulmonary and central nervous system involvement.Open Rheumatol. J. 2021; 15: 9-15https://doi.org/10.2174/1874312902115010009
- Structural noninfectious manifestations of the central nervous system in common variable immunodeficiency disorders.J. Allergy Clin. Immunol. Pract. 2020; 8 (1047-1062.e6)https://doi.org/10.1016/j.jaip.2019.11.039