Humoral Immunity Status in Pediatric Patients with Familial Mediterranean Fever: Exploring the Role of IgA Familial Mediterranean Fever: What about IgA
Journal of Pediatric Nephrology,
Vol. 11 No. 2 (2023),
7 February 2024
https://doi.org/10.22037/jpn.v11i3.43855
Abstract
Background and Aim:Research on the role of the adaptive immune system in familial Mediterranean fever (FMF) is limited. The aim of this study was to investigate the levels of immunoglobulins (Ig) IgG, IgM, IgA and absolute lymphocyte count (ALC) in FMF patients compared to normal subjects.
Methods: Patients diagnosed with FMF were included in the study. At the time of data entry, we recorded demographic information, mutation type, ALC, eosinophils and levels of IgA, IgG, IgM and IgE. We compared these variables with those of the control group.
Results: Thirty-five FMF patients (M/F=9/26) were included in this study. There was no significant difference between the mean quantitative levels of the immunoglobulins (IgA, IgG and IgM) and Ig E between the patient and control groups (P>0.05). Compared with the biallelic and monoallelic mutations, we found higher Ig A levels in the biallelic group (1.5±0.7 vs 0.9±0.3 mg/dL, respectively P=0.008). An interesting finding was that three patients with homozygous M694 mutation had an Ig A level above the upper limit while.
Conclusion: No significant difference was found in terms of humoral immunity between FMF patients and healthy individuals. However, we believe that IgA levels should be examined in patients with severe FMF manifestations; the finding of high IgA levels in three patients with a homozygous M694V mutation supports our hypothesis.
- Familial Mediterranean fever
- Adaptive immunity
- Immunoglobulin A (IgA)
How to Cite
References
Sarı İ, Birlik M, Kasifoğlu T. Familial Mediterranean fever:
An updated review. Eur J Rheumatol. 2014; 1(1):21-33.
[DOI:10.5152/eurjrheum.2014.006] [PMID] [PMCID]
Yüksel S, Ayvazyan L, Gasparyan AY. Familial Mediterranean
fever as an emerging clinical model of atherogenesis
associated with low-grade inflammation. Open Cardiovasc
Med J. 2010; 4:51-6 [DOI:10.2174/1874192401004010051]
[PMID] [PMCID]
Fonnesu C, Cerquaglia C, Giovinale M, Curigliano V, Verrecchia
E, de Socio G, et al. Familial Mediterranean fever:
A review for clinical management. J Joint Bone Spine. 2009;
(3):227-33. [DOI:10.1016/j.jbspin.2008.08.004] [PMID]
Manna R, Cerquaglia C, Curigliano V, Fonnesu C, Giovinale
M, Verrecchia E, et al. Clinical features of familial Mediterranean
fever: An Italian overview. Eur Rev Med Pharmacol Sci.
; 13(Suppl 1):51-3. [PMID]
Schroder K, Tschopp J. The inflammasomes. Cell. 2010;
(6):821-32. [DOI:10.1016/j.cell.2010.01.040] [PMID]
Vivier E, Raulet DH, Moretta A, Caligiuri MA, Zitvogel
L, Lanier LL, et al. Innate or adaptive immunity? The example
of natural killer cells. Science. 2011; 331(6013):44-9.
[DOI:10.1126/science.1198687] [PMID] [PMCID]
Chae JJ, Aksentijevich I, Kastner DL. Advances in the understanding
of familial Mediterranean fever and possibilities
for targeted therapy. Br J Haematol. 2009; 146(5):467-78.
[DOI:10.1111/j.1365-2141.2009.07733.x] [PMID] [PMCID]
Livneh A, Langevitz P. Diagnostic and treatment concerns
in familial Mediterranean fever. Best Pract Res Clin Rheumatol.
; 14(3):477-98. [DOI:10.1053/berh.2000.0089]
Wang DQH, Bonfrate L, de Bari O, Wang TY, Portincasa P.
Familial Mediterranean fever: From pathogenesis to treatment.
J Genet Syndr Gene Ther. 2014; 5:1-11. [Link]
Schuetz C, Niehues T, Friedrich W, Schwarz K. Autoimmunity,
autoinflammation and lymphoma in combined immunodeficiency
(CID). Autoimmun Rev. 2010; 9(7):477-82.
[DOI:10.1016/j.autrev.2010.02.005] [PMID]
Kholoussi S, Kholoussi N, Zaki ME, El-Bassyouni HT, Elnady
H, Morcos B, et al. Immunological evaluation in patients
with familial Mediterranean fever. Open Access Maced J Med
Sci. 2018; 6:310-3 [DOI:10.3889/oamjms.2018.079] [PMID]
[PMCID]
Özer S, Yılmaz R, Sönmezgöz E, Karaaslan E, Taşkın S,
Bütün İ, et al. Simple markers for subclinical inflammation in
patients with Familial Mediterranean Fever. Med Sci Monit.
; 21:298-303. [DOI:10.12659/MSM.892289] [PMID] [PMCID]
Duksal F, Alaygut D, Güven AS, Ekici M, Oflaz MB, Tuncer
R, et al. Neutrophil- lymphocyte ratio in children with familial
Mediterranean fever. Eur J Rheumatol. 2015; 2(1):20-3.
[DOI:10.5152/eurjrheumatol.2015.0093] [PMID] [PMCID]
Güneş-Yılmaz S, Kasap-Demir B, Soyaltın E, Erfidan G,
Özdemir-Şimşek Ö, Arslansoyu-Çamlar S, et al. Prediction
of more severe MEFV gene mutations in childhood. Turk
Arch Pediatr. 2021; 56(6):610-7. [DOI:10.5152/TurkArchPediatr.
21147] [PMID] [PMCID]
Schlesinger M, Rubinow A, Vardy PA. Henoch-Schönlein
purpura and familial Mediterranean fever. Isr J Med Sci. 1985;
(1):83-5. [PMID]
Flatau E, Kohn D, Schiller D, Lurie M, Levy E. Schönlein-
Henoch syndrome in patients with familial Mediterranean
fever. Arthritis Rheum. 1982; 25(1):42-7. [DOI:10.1002/
art.1780250107] [PMID]
Ozdogan H, Arisoy N, Kasapçapur O, Sever L, Calişkan S,
Tuzuner N, et al. Vasculitis in familial Mediterranean fever. J
Rheumatol. 1997; 24(2):323-7. [PMID]
Tekin M, Yalçinkaya F, Tümer N, Akar N, Misirlioğlu M,
Cakar N. Clinical, laboratory and molecular characteristics of
children with Familial Mediterranean Fever-associated vasculitis.
Acta Paediatr. 2000; 89(2):177-82. [PMID]
Saulsbury FT. Clinical update: Henoch-Schönlein purpura.
Lancet. 2007; 369(9566):976-8. [DOI:10.1016/S0140-
(07)60474-7] [PMID]
He X, Yu C, Zhao P, Ding Y, Liang X, Zhao Y, et al. The
genetics of Henoch-Schönlein purpura: A systematic review
and meta-analysis. Rheumatol Int.2013; 33(6):1387-95.
[DOI:10.1007/s00296-012-2661-4] [PMID]
Ekinci RMK, Balci S, Bisgin A, Atmis B, Dogruel D, Altintas
DU, et al. MEFV gene variants in children with Henoch-
Schönlein purpura and association with clinical manifestations:
A single-center Mediterranean experience. Postgrad
Med. 2019; 131(1):68-72. [DOI:10.1080/00325481.2019.155247
[PMID]
Gullu BE, Çelik S, Dagel T, Dogan İ, Kahvecioglu S , Durak
H, et al. IgA Nephritis in a patient with familial Mediterranean
Fever: 5 years-follow-up. Turkish J Nephrol. 2010; 19:224-
[DOI:10.5262/tndt.2010.1003.15]
Rigante D, Federico G, Ferrara P, Maggiano N, Avallone
L, Pugliese AL, et al. IgA nephropathy in an Italian child
with familial Mediterranean fever. Pediatr Nephrol. 2005;
(11):1642-4. [DOI:10.1007/s00467-005-2023-5] [PMID]
Said R, Nasrallah N, Hamzah Y, Tarawneh M, al-Khatib M.
IgA nephropathy in patients with familial Mediterranean fever.
Am J Nephrol. 1988; 8(5):417-20. [DOI:10.1159/000167628]
[PMID]
İlgen U, Nergizoğlu G. Less IgA deposits with more severe
disease: The immunoclinical paradox in Henoch-Schönlein
Purpura with MEFV mutations. Clin Rheumatol. 2019;
(11):3311-3. [DOI:10.1007/s10067-019-04677-0] [PMID]
Abbara S, Grateau G, Ducharme-Bénard S, Saadoun D,
Georgin-Lavialle S. Association of vasculitis and familial Mediterranean
fever. Front Immunol. 2019; 10:763. [DOI:10.3389/
fimmu.2019.00763] [PMID] [PMCID]
Chbihi M, Dumaine C, Deschênes G, Couderc A, Monteiro
RC, Hogan J, et al. A pediatric case of IgA nephropathy associated
with familial Mediterranean fever. Arch Clin Med Case
Rep. 2020; 4:218-5. [DOI:10.26502/acmcr.96550189]
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