granulomatosis with polyangiitis (GPA, formerly Wegener's) is a systemic necrotizing
vasculitis that predominantly involves respiratory tract and kidneys [
[1]
,
[2]
]. GPA usually presents with nervous system involvement in the later course, mainly
affecting the peripheral nervous system [
[3]
]. Vasculitis has been reported up to 6% of GPA cases with central nervous system
(CNS) involvement [
[2]
,
[4]
]. Detection of either anti-proteinase 3 (PR3)-ANCA or myeloperoxidase (MPO)-ANCA
can support the diagnosis, but in some cases ANCA are not detected [
[3]
]. Overall, 82–94% of patients with GPA are ANCA positive [
[5]
], leaving approximately 10% ANCA negative. A large-scale study has demonstrated that
83% of ANCA-negative GPA patients show severe CNS involvement, whereas only 10% of
all the GPA patients have CNS involvement [
[6]
]. To diagnose GPA, histologic review via biopsy is one of the major criteria and
imaging studies are helpful [
- Reinhold-Keller E.
- de Groot K.
- Holl-Ulrich K.
- et al.
Severe CNS manifestations as the clinical hallmark in generalized Wegener's granulomatosis
consistently negative for anti- neutrophil cytoplasmic antibodies (ANCA). A report
of 3 cases and a review of the literature.
Clin. Exp. Rheumatol. 2001; 19: 541-549
[3]
]. Characteristic histopathologic findings are necrotizing vasculitis that affects
small- and medium-size vessels and extravascular granulomas.To read this article in full you will need to make a payment
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References
- Wegener granulomatosis: an analysis of 158 patients.Ann. Intern. Med. 1992; 116: 488-498
- Neurological involvement in Wegener's granulomatosis: an analysis of 324 consecutive patients at the Mayo Clinic.Ann. Neurol. 1993; 33: 4-9
- Seronegative granulomatosis with polyangiitis presenting with multiple cranial nerve palsies.Neuropathology. 2018; 38: 192-197
- Neurological involvement in Wegener's granulomatosis.Curr. Opin. Rheumatol. 2011; 23: 7-11
- ANCA small-vessel vasculitis.J. Am. Soc. Nephrol. 1997; 8 (314–122)
- Severe CNS manifestations as the clinical hallmark in generalized Wegener's granulomatosis consistently negative for anti- neutrophil cytoplasmic antibodies (ANCA). A report of 3 cases and a review of the literature.Clin. Exp. Rheumatol. 2001; 19: 541-549
- Spectrum of choroid plexus lesions in children.Am. J. Radiol. 2009; 192: 32-40
- 2012 revised international Chapel Hill consensus conference nomenclature of vasculitides.Arthritis Rheum. 2013; 65: 1-11
- Choroid plexitis as a unique neurological manifestation in granulomatosis with polyangitiis (Wegener's disease).J. Rheumatol. 2014; 41: 6
- Granulomatosis with polyangiitis: recurrence presenting as ependimoplexitis.Clin. Exp. Rheumatol. 2014; 32: S70-S72
Article info
Publication history
Published online: January 26, 2019
Accepted:
January 25,
2019
Received in revised form:
January 3,
2019
Received:
December 7,
2018
Identification
Copyright
© 2019 Elsevier B.V. All rights reserved.
