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.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117 J+Lab+Physicians
2018 ; 10
(2
): 232-236
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Complement deficiency in pediatric-onset systemic lupus erythematosus
#MMPMID29692593
Afzali P
; Isaeian A
; Sadeghi P
; Moazzami B
; Parvaneh N
; Robatjazi M
; Ziaee V
J Lab Physicians
2018[Apr]; 10
(2
): 232-236
PMID29692593
show ga
BACKGROUND: Pediatric-onset systemic lupus erythematosus (pSLE) accounts for
about 10%-20% of all patients with SLE. Deficiencies in early complement
components of the classical pathway are the strong genetic risk factor for the
development of SLE. In this study, clinical and laboratory manifestations of both
complement-deficient and normal complement pSLE patients were compared. MATERIALS
AND METHODS: To investigate clinical and immunological manifestations of pSLE in
Iran, 36 consecutive pSLE patients (onset before 18 years) who were followed up
over a period of 2 years, were studied. Complement C1q and C2 levels were
measured using radial immunodifusion assay and complement C3 and C4 levels were
measured using nephelometry. Medical records were retrospectively evaluated from
patient database of Children Medical Center Hospital. Data were assessed through
descriptive analysis (confidence interval = 95%), paired t-test, and Pearson
correlation test. RESULTS: Twenty-one patients (58%) had at least one component
of complement deficiency. Ten patients (27%) had low C1q level, 11 patients
(30.5%) had low C2, nine patients (25%) had low C3, and four patients (11%) had
low C4 level. Serum level of complement in pSLE was significantly lower than the
control group, except C4 (P = 0.005). The low C1q patients had an earlier age of
onset of disease (P < 0.0001). The cutaneous manifestations were more frequent
and much more severe in pSLE with low complement (100% vs. 73%). The frequency of
renal and musculoskeletal symptoms was equal, but renal morbidity was more common
in pSLE with low complement. Positivity for anti-ds-DNA was less common in pSLE
with low complement (71% vs. 86%). CONCLUSION: In pSLE patients with early
disease onset and more aggressive SLE manifestations and negative anti-ds-DNA
test, complement deficiency should be considered.