Affiliations: Department of Pediatrics, University Medical Center
Hamburg-Eppendorf, Germany | Department of Neuroradiology, University Medical
Center Hamburg-Eppendorf, Germany
Note: [] Correspondence: Enke Grabhorn, MD, Division of Pediatric
Immunology, Department of Pediatrics, University Medical Center
Hamburg-Eppendorf, Martinistrasse 52, 20246 Hamburg, Germany. Tel.: +49 40
42803 9976; Fax: +49 40 42803 9444; E-mail: [email protected]
Abstract: A 5-month-old girl developed intractable cerebral seizures and a
global mental and motor retardation after a scheduled hexavalent vaccination.
In the following years, recurrent fever, hepatosplenomegaly, and a pancytopenia
indicated other organ system involvement. At the age of 4 years the diagnosis
of a severe systemic autoimmune disorder was made, defined by highly elevated
antinuclear antibody (ANA), smooth muscle antigen (SMA), extractable nuclear
antigen, and decreased complement factors C3c and C4. Moreover, a low B cell
count and an IgA and IgG2 subclass deficiency were found. After initiation of
an immunosuppressive therapy as well as monthly intravenous immunoglobulin
application, the clinical situation improved dramatically. Despite therapy and
clinical improvement, there is still an intermittent elevation of ANA and SMA
as well as decreased complement C4, a persistent low B cell count and an IgA
deficiency. We propose the possibility of an underlying primary
immunodeficiency with subsequent development of a severe systemic autoimmune
disease possibly triggered by routine vaccination. The clinical features and
laboratory tests are consistent with a systemic lupus erythematosus or
lupus-like syndrome.
