Background: IgA vasculitis/Henoch-Schoenlein purpura (IgAV/HSP) is a systemic small
vessel vasculitis of unknown pathogenesis predominantly affecting children. While skin,
GI tract, joints, and kidneys are frequently affected and considered, central nervous
system (CNS) involvement of this disease is underestimated.
Methods: We provide a case report and systematically review the literature on IgAV,
collecting data on the spectrum of neurological manifestations.
Results: We report on a 7-year-old girl with IgAV who presented with diplopia
and afebrile focal seizures, which preceded the onset of purpura. Cranial magnetic
resonance imaging was consistent with posterior reversible encephalopathy syndrome
(PRES), showing typical focal bilateral parietal swelling and cortical and subcortical high
signal intensities on T2-fluid attenuated inversion recovery (FLAIR) images predominantly
without diffusion restriction. Cerebrospinal fluid analysis and blood tests excluded
systemic inflammation or vasculitis. Interestingly, hypertension was not a hallmark of
the developing disease in the initial phase of PRES manifestation. Renal disease and
other secondary causes for PRES were also excluded. Supportive- and steroid treatment
resulted in restitution ad integrum. Reviewing the literature, we identified 28 other cases of
IgAV with CNS involvement. Severe CNS involvement includes seizures, cerebral edema,
or hemorrhage, as well as PRES. Thirteen patients fulfilled all diagnostic criteria of PRES.
The mean age was 11.2 years (median 8.0, range 5-42 years), with no reported bias
toward gender or ethnic background. Treatment regimens varied from watchful waiting
to oral and intravenously steroids up to plasmapheresis. Three cases showed permanent
CNS impairment.
Conclusion: Collectively, our data demonstrate that (I) severe CNS involvement such
as PRES is an underappreciated feature of IgAV, (II) CNS symptoms may precede other
features of IgAV, (III) PRES can occur in IgAV, and differentiation from CNS vasculitis is
challenging, (IV) pathogenesis of PRES in the context of IgAV remains elusive, which
hampers treatment decisions. We, therefore, conclude that clinical awareness and
the collection of structured data are necessary to elucidate the pathophysiological
connection of IgAV and PRES.
| Identifer | oai:union.ndltd.org:DRESDEN/oai:qucosa:de:qucosa:84343 |
| Date | 27 March 2023 |
| Creators | Funken, Dominik, Götz, Friedrich, Bültmann, Eva, Hennies, Imke, Gburek-Augustat, Janina, Hempel, Julya, Dressler, Frank, Baumann, Ulrich, Klemann, Christian |
| Publisher | Frontiers Research Foundation |
| Source Sets | Hochschulschriftenserver (HSSS) der SLUB Dresden |
| Language | English |
| Detected Language | English |
| Type | info:eu-repo/semantics/publishedVersion, doc-type:article, info:eu-repo/semantics/article, doc-type:Text |
| Rights | info:eu-repo/semantics/openAccess |
| Relation | 1664-2295, 759386 |
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