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Pediatr Rheumatol Online J. 2015 Nov 21;13:51. doi: 10.1186/s12969-015-0050-8.

Primary complement and antibody deficiencies in autoimmune rheumatologic diseases with juvenile onset: a prospective study at two centers.

Author information

1
2nd Department of Paediatrics, Iuliu Hatieganu University of Medicine and Pharmacy, 3-5 Crisan Street, Cluj-Napoca, 400177, Romania. mihaelaspirchez@gmail.com.
2
Emergency Children's Hospital, Cluj-Napoca, Romania. mihaelaspirchez@gmail.com.
3
Molecular Biology Center, Interdisciplinary Research Institute on Bio-Nano-Sciences, Babes-Bolyai University Cluj-Napoca, Cluj-Napoca, Romania.
4
Emergency Children's Hospital, Cluj-Napoca, Romania.
5
2nd Department of Paediatrics, Iuliu Hatieganu University of Medicine and Pharmacy, 3-5 Crisan Street, Cluj-Napoca, 400177, Romania.
6
Rheumatology Department, Emergency Clinical County Hospital, Cluj-Napoca, Romania.
7
Department of Rheumatology, Iuliu Hatieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania.
8
3rd Department of Internal Medicine, Iuliu Hatieganu University of Medicine and Pharmacy, Cluj-Napoca, Romania.

Abstract

BACKGROUND:

Our aim was to investigate the prevalence and clinical relevance of inherited complement and antibody deficiency states in a large series of patients with various autoimmune rheumatologic diseases (ARD) with juvenile onset.

METHODS:

A total number of 117 consecutive patients from 2 tertiary referral hospitals were included in the study. All patients underwent genetic screening for type I C2 deficiency and C4 allotyping. Serum levels of immunoglobulin classes measured systematically throughout their regular medical care were recorded retrospectively.

RESULTS:

Our cohort of patients included 84 with juvenile idiopathic arthritis (JIA), 21 with systemic lupus erythematosus (SLE), 6 with systemic vasculitis, 2 with juvenile scleroderma, 2 with idiopathic uveitis, 1 with mixed connective tissue disease and 1 with SLE/scleroderma overlap syndrome. We have found 16 patients with evidence of primary immunodeficiency in our series (13.7%), including 7 with C4 deficiency, 5 with selective IgA deficiency, 3 with C2 deficiency and 2 with unclassified hypogammaglobulinemia (one also presented C4D). Of the 84 patients with JIA, 4 (4.8%) had a complement deficiency, which was less prevalent than in the SLE cohort (23.8%), but all of them have exhibited an aggressive disease. Most of our patients with primary antibody deficiencies showed a more complicated and severe disease course and even the co-occurrence of two associated autoimmune diseases (SLE/scleroderma overlap syndrome and SLE/autoimmune hepatitis type 1 overlap).

CONCLUSIONS:

Our findings among others demonstrate that complement and immunoglobulin immunodeficiencies need careful consideration in patients with ARD, as they are common and might contribute to a more severe clinical course of the disease.

PMID:
26590091
PMCID:
PMC4654875
DOI:
10.1186/s12969-015-0050-8
[Indexed for MEDLINE]
Free PMC Article

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