Abstract
Granulomatosis with polyangiitis (GPA) is a pauci-immune small vessel vasculitis characterised by neutrophil-mediated vasculitis and granuloma. The presence of intracranial parenchymal space-occupying lesions is rarely seen in GPA patients. In this manuscript, we report a case of GPA with granuloma of the fourth ventricle accompanied by obstructive hydrocephalus. Treatment with glucocorticoids (GCs) and multiple immunosuppressants cyclophosphamide (CYC), mycophenolate mofetil (MMF), and rituximab (RTX) showed poor efficacy in this case. After removal of the granuloma by craniotomy, GPA relapsed within 3 months. Under the premise of GC and MMF treatment combined with intrathecal injection of dexamethasone (DXM) and methotrexate (MTX), the intracranial granuloma gradually shrank, and the patient’s general condition was alleviated, showing that this is an effective treatment method.
Key Points • To date, there are few reports of granulomatous vasculitis combined with granuloma of the fourth ventricle, and our case is the second. • In this case, multiple immunosuppressants and rituximab were ineffective treatments, and the intracranial granuloma was effectively controlled by intrathecal injection of dexamethasone (DXM) and methotrexate (MTX). • Based on this report, it can be suggested that intrathecal injection is effective in treating patients with GPA and central nervous system involvement, but large-scale sample studies are needed. |
Similar content being viewed by others
References
Jennette JC, Falk RJ, Bacon PA et al (2013) 2012 revised International Chapel Hill consensus conference nomenclature of vasculitides. Arthritis Rheum 65(1):1–11. https://doi.org/10.1002/art.37715
Zhang W, Zhou G, Shi Q et al (2009) Clinical analysis of nervous system involvement in ANCA-associated systemic vasculitides. Clin Experim Rheumatol 27(1):S65. https://doi.org/10.1186/1471-2474-10-1
Zheng Y, Zhang Y, Cai M et al (2019) Central nervous system involvement in ANCA-associated vasculitis: what neurologists need to know. Front Neurol 10(9):1166. https://doi.org/10.3389/fneur.2018.01166
Robson JC, Grayson PC, Ponte C et al (2022) 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology classification criteria for granulomatosis with polyangiitis. Ann Rheum Dis 81(3):315–320. https://doi.org/10.1136/annrheumdis-2021-221794
Holle JU, Gross WL et al (2011) Neurological involvement in Wegener’s granulomatosis. Curr Opin Rheumatol 23(1):7–11. https://doi.org/10.1097/BOR.0b013e32834115f9
Yokoseki A, Saji E, Arakawa M et al (2014) Hypertrophic pachymeningitis: significance of myeloperoxidase anti-neutrophil cytoplasmic antibody. Brain 137(Pt 2):520–536. https://doi.org/10.1093/brain/awt314
Berlis A, Petschner F, Bötefür IC et al (2003) Wegener granuloma in the fourth ventricle. AJNR Am J Neuroradiol 24(3):523–5. https://doi.org/10.1055/s-2003-37569
Spísek R, Kolouchová E et al (2006) Combined CNS and pituitary involvement as a primary manifestation of Wegener granulomatosis. Clin Rheumatol 25(5):739–742. https://doi.org/10.1007/s10067-005-0065-5
Ser or R, Mahr A, Ramanoelina J et al (2006) Central nervous system involvement in Wegener granulomatosis. Medicine (Baltimore) 85(1):53–65. https://doi.org/10.1097/01.md.0000200166.90373.41
Azuma N, Katada Y et al (2008) A case of granuloma in the occipital lobe of a patient with Wegener’s granulomatosis. Mod Rheumatol 18(4):411–415. https://doi.org/10.1007/s10165-008-0070-z
Hughes J, Barkhoudarian G et al (2013) Refractory pituitary granulomatosis with polyangiitis (Wegener’s) treated with rituximab. Endocr Pract 19(1):e1-7. https://doi.org/10.4158/EP12181.CR
Nicolosi F, Nodari G et al (2013) Cerebral Wegener’s granuloma: surgery mandatory for diagnosis and treatment. Case Rep Neurol Med 2013:750391. https://doi.org/10.1155/2013/750391
Kara O, Demirel F, Acar BC et al (2013) Wegener granulomatosis as an uncommon cause of panhypopituitarism in childhood. J Pediatr Endocrinol Metab 26(9–10):959–962. https://doi.org/10.1515/jpem-2013-0033
Pereira EA, Plaha P, Hofer M et al (2013) Hypophyseal Wegener’s granulomatosis presenting by visual field constriction without hypopituitarism. Clin Neurol Neurosurg 115(6):762–764. https://doi.org/10.1016/j.clineuro.2012.06.041
Al-Fakhouri A, Manadan A, Gan J et al (2014) Central diabetes insipidus as the presenting symptom of granulomatosis with polyangiitis. J Clin Rheumatol 20(3):151–154. https://doi.org/10.1097/RHU.0000000000000093
Baird SM, Pratap U, McLean C et al (2017) Rare presentation of Wegener’s granulomatosis in the pituitary gland: case report and literature review. Int J Surg Case Rep 33:24–26. https://doi.org/10.1016/j.ijscr.2017.02.014
Costa C, Santiago T, Espirito-Santo J et al (2017) Pachymeningitis and cerebral granuloma in granulomatosis with polyangiitis: is rituximab a promising treatment option? Acta Reumatol Port 42(1):82–87
Vega-Beyhart A, Medina-Rangel IR, Hinojosa-Azaola A et al (2020) Pituitary dysfunction in granulomatosis with polyangiitis. Clin Rheumatol 39(2):595–606. https://doi.org/10.1007/s10067-019-04735-7
Zhang X, Xing B et al (2018) Severe ophthalmic manifestation in pituitary-involved granulomatosis with polyangiitis: a case report and literature review. BMC Ophthalmol 18(1):299. https://doi.org/10.1186/s12886-018-0966-0
Peters JE, Gupta V, Saeed IT et al (2018) Severe localised granulomatosis with polyangiitis (Wegener’s granulomatosis) manifesting with extensive cranial nerve palsies and cranial diabetes insipidus: a case report and literature review. BMC Neurol 18(1):59. https://doi.org/10.1186/s12883-018-1058-8
Celik O, Kalfa M, Celebi ARC et al (2019) Granulomatosis with polyangiitis as an uncommon cause of panhypopituitarism. J Clin Rheumatol 25(8):e145–e146. https://doi.org/10.1097/RHU.0000000000000766
Ramakrishna MP, Arun CS, Pavithran PV et al (2020) Hypophysitis in granulomatosis with polyangiitis: rare presentation of a multisystem disease. J R Coll Physicians Edinb 50(3):287–290. https://doi.org/10.4997/JRCPE.2020.315
Triano MJ, Haberstroh WD, Lenka A et al (2021) Relapsed granulomatosis with polyangiitis with panhypopituitarism. BMJ Case Rep 14(1):e237774. https://doi.org/10.1136/bcr-2020-237774
Yates M, Watts RA, Bajema IM et al (2016) EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis. Ann Rheum Dis 75(9):1583–1594. https://doi.org/10.1136/annrheumdis-2016-209133
Gapud EJ (2017) ANCA-associated vasculitis pathogenesis: a commentary. Curr Rheumatol Rep 19(4):15. https://doi.org/10.1007/s11926-017-0641-0
Craft JE (2012) Follicular helper T cells in immunity and systemic autoimmunity. Nat Rev Rheumatol 8(6):337–347. https://doi.org/10.1038/nrrheum.2012.58
Stone JH, Merkel PA, Spiera R et al (2010) Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med 363(3):221–232. https://doi.org/10.1056/NEJMoa0909905
Takada K, Illei GG, Boumpas DT et al (2001) Cyclophosphamide for the treatment of systemic lupus erythematosus. Lupus 10(3):154–61. https://doi.org/10.1191/096120301671376017
Rubenstein JL, Combs D, Rosenberg J et al (2003) Rituximab therapy for CNS lymphomas: targeting the leptomeningeal compartment. Blood 101(2):466–468. https://doi.org/10.1182/blood-2002-06-1636
Liu S, Guo L et al (2021) Clinical features of central nervous system involvement in patients with eosinophilic granulomatosis with polyangiitis: a retrospective cohort study in China. Orphanet J Rare Dis 16(1):152. https://doi.org/10.1186/s13023-021-01780-x
Nie Y, Sun B, He X et al (2023) The influence of intrathecal injection of methotrexate and dexamethasone on neuropsychiatric systemic lupus erythematosus (NPSLE): a retrospective cohort study of 386 patients with NPSLE. Arthritis Res Ther 25(1):50. https://doi.org/10.1186/s13075-023-03030-w
Zhou HQ, Leng XM et al (2006) Neuropsychiatric manifestations in systemic lupus erythematosus and the treatment of intrathecal methotrexate plus dexamethasone. Zhonghua Yi Xue Za Zhi 86(11):771–774. https://doi.org/10.3760/j:issn:0376-2491.2006.11.013
Surapaneni UR, Cortes JE, Thomas D et al (2002) Central nervous system relapse in adults with acute lymphoblastic leukemia. Cancer 94(3):773–779. https://doi.org/10.1002/cncr.10265
Funding
No specific funding was received from any bodies in the public, commercial or not for profit sectors to carry out the work described in this manuscript.
Author information
Authors and Affiliations
Contributions
CL and YW contributed equally to this work and should be considered co-first authors.
Corresponding author
Ethics declarations
Ethics approval
This study followed the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments; written informed consent was obtained; it was explained to the patient; she understood it, and she subsequently signed and was approved by the ethics committee of the institution. Details about the identity of the patient under study were omitted.
Disclosures
None.
Additional information
Publisher's Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
Chunyan Li and Yu Wang contributed equally to this work and listed as first coauthors.
Supplementary Information
Below is the link to the electronic supplementary material.
Rights and permissions
Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law.
About this article
Cite this article
Li, C., Wang, Y., Lin, Y. et al. Intrathecal injection of methotrexate and dexamethasone for vasculitis granuloma of the fourth ventricle: a case report and literature review. Clin Rheumatol 43, 1217–1226 (2024). https://doi.org/10.1007/s10067-023-06777-4
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10067-023-06777-4