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Case ReportImages in Rheumatology

Choroid Plexitis as a Unique Neurological Manifestation in Granulomatosis with Polyangiitis (Wegener’s Disease)

SALVADOR SIERRA, NATASHA LUQUIN, MANUEL TARDÁGUILA and ALEJANDRO OLIVÉ
The Journal of Rheumatology June 2014, 41 (6) 1192-1193; DOI: https://doi.org/10.3899/jrheum.131302
SALVADOR SIERRA
Division of Neurosciences, Center for Applied Medical Research, University of Navarra, Pamplona
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  • For correspondence: ssierra@alumni.unav.es
NATASHA LUQUIN
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MANUEL TARDÁGUILA
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ALEJANDRO OLIVÉ
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A 70-year-old man presented with a 3-day history of nausea, vomiting and constitutional symptoms (fatigue and anorexia). During hospitalization he developed diplopia. Cranial magnetic resonance imaging with gadolinium (Figure 1a) showed inflammation of the entire choroid plexus. A diagnosis of granulomatosis with polyangiitis (GPA) was made based on: presence of perinuclear antineutrophil cytoplasmic antibody at titer 1/40 with elevated anti-proteinase 3 antibodies; sinus radiography showing a mucosal thickening of the left maxillary sinus; and a lung biopsy demonstrating chronic granulomatous inflammation with multinucleated giant cells. The patient was treated with methylprednisolone [1 g/day intravenously (IV) for 3 days], cyclophosphamide (0.6 mg/m2/dose IV, monthly pulses for 6 mos) and mycophenolate mofetil (2 g/day PO, maintenance therapy). He made a full recovery. A followup MRI showed partial improvement of the inflammation (Figure 1B).

Figure 1.
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Figure 1.

Axial contrast-enhanced T1-weighted images. Homogenous marked enhancement of enlarged choroid plexus within the 2 temporal horns (a); and followup imaging 3 months after immunosuppression therapy showing a prominent reduction in size and enhancement (b).

Although not commonly the initial symptom1,2, the most frequent pattern of central nervous system involvement is granulomatous inflammation that leads to compression of usually cranial nerves II–III, V–VIII, meningitis and pituitary gland inflammation3. Our literature review of choroid plexus involvement in GPA cases yielded no reports.

Acknowledgments

The authors thank Mireia Tomás for assistance in acquiring patient data for this report.

REFERENCES

  1. 1.↵
    1. Nishino H,
    2. Rubino FA,
    3. Parisi JE
    . The spectrum of neurologic involvement in Wegener’s granulomatosis. Neurology 1993;43:1334–7.
    OpenUrlCrossRef
  2. 2.↵
    1. de Groot K,
    2. Schmidt DK,
    3. Arlt AC,
    4. et al.
    Standardized neurologic evaluations of 128 patients with Wegener granulomatosis. Arch Neurol 2001;58:1215–21.
    OpenUrlCrossRefPubMed
  3. 3.↵
    1. Holle JU,
    2. Gross WL
    . Neurological involvement in Wegener′s granulomatosis. Curr Opin Rheumatol 2011;23:7–11.
    OpenUrlCrossRefPubMed
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1 Jun 2014
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Choroid Plexitis as a Unique Neurological Manifestation in Granulomatosis with Polyangiitis (Wegener’s Disease)
SALVADOR SIERRA, NATASHA LUQUIN, MANUEL TARDÁGUILA, ALEJANDRO OLIVÉ
The Journal of Rheumatology Jun 2014, 41 (6) 1192-1193; DOI: 10.3899/jrheum.131302

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Choroid Plexitis as a Unique Neurological Manifestation in Granulomatosis with Polyangiitis (Wegener’s Disease)
SALVADOR SIERRA, NATASHA LUQUIN, MANUEL TARDÁGUILA, ALEJANDRO OLIVÉ
The Journal of Rheumatology Jun 2014, 41 (6) 1192-1193; DOI: 10.3899/jrheum.131302
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