Article Text
Statistics from Altmetric.com
A 13 year old Turkish boy with a known C1q deficiency and SLE-like disease developed recurrent parotitis. Investigations confirmed a secondary Sjögren’s syndrome (SS). As far as we know, the association between C1q deficiency and SS has not been described before. The potential role of C1q in the pathogenesis of SS and systemic lupus erythematosus (SLE) might stimulate further research in understanding the pathogenesis of these and other autoimmune diseases. Screening patients with SS for complement deficiencies, including C1q deficiency, seems indicated.
Deficiency of the complement component C1q is a rare genetic disorder with susceptibility to recurrent infections with polysaccharide encapsulated micro-organisms and a high prevalence of autoimmune phenomena, most often SLE.
CASE REPORT
At the age of 4 years a boy of consanguineous Turkish descent had meningitis of unknown origin. At the age of 8 years he …