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Letter
Hereditary C1q deficiency and secondary Sjögren’s syndrome
  1. E P A H Hoppenreijs1,
  2. P J van Dijken2,
  3. P J Kabel3,
  4. J M Th Draaisma4
  1. 1Department of Paediatrics, University Medical Centre Utrecht, The Netherlands
  2. 2Department of Paediatrics, St Elisabeth Hospital Tilburg, The Netherlands
  3. 3Department of Medical Microbiology, St Elisabeth Hospital Tilburg, The Netherlands
  4. 4Department of Paediatrics, University Medical Centre Nijmegen, The Netherlands
  1. Correspondence to:
    Dr E P A H Hoppenreijs
    Department of Paediatrics, University Medical Centre Utrecht, Wilhelmina Kinderziekenhuis, PO Box 85090, 3508 AB Utrecht, the Netherlands; e.p.a.h.hoppenreijswkz.azu.nl

https://doi.org/10.1136/ard.2003.016592

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    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.

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