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Characteristics of hypertrophic pachymeningitis in patients with granulomatosis with polyangiitis

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Abstract

Hypertrophic pachymeningitis (HP) is an important neurologic complication of granulomatosis with polyangiitis (GPA, formerly Wegener’s granulomatosis). The aim of this study is to investigate the clinical features, radiological findings, and diagnostic pitfalls of GPA-related HP. A retrospective chart review was performed to screen patients diagnosed with GPA at Samsung Medical Center between 1997 and 2016. Neurologic manifestation, laboratory findings, neuroimaging data, and clinical course were evaluated in all patients. Characteristics of patients with HP were compared to those of patients without HP. Sixty-five patients with GPA were identified. Twenty-five of these patients had central nervous system involvement. HP (N = 9, 36%) was the second most common radiologic finding. Other neurologic findings included stroke (N = 7, 28%) and granulomatous disease (N = 10, 40%). Patients with HP had lower incidences of systemic manifestations (N = 2, 22.2% vs. N = 38, 67.9%, p = 0.013 in the lung and N = 1, 11.1% vs. N = 28, 50.0%, p = 0.030 in the kidney) than those without HP. Six patients with GPA-related HP were MPO-ANCA positive (66.7%) and two had PR3-ANCA (22.2%). Most of the patients with HP presented with headache (N = 8, 88.9%) at a rate that is similar to those of primary headache disorders (migraine, tension-type, and stabbing) and other secondary headache disorders (postural type and meningitis). Patients with HP rarely had neurologic deficits (N = 3, 37.5%). Different clinical or radiologic features may be observed in GPA-related HP. Early recognition and accurate diagnosis of GPA-related HP are needed in addition to neuroimaging findings.

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References

  1. Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F, Flores-Suarez LF, Gross WL, Guillevin L, Hagen EC, Hoffman GS, Jayne DR, Kallenberg CG, Lamprecht P, Langford CA, Luqmani RA, Mahr AD, Matteson EL, Merkel PA, Ozen S, Pusey CD, Rasmussen N, Rees AJ, Scott DG, Specks U, Stone JH, Takahashi K, Watts RA (2013) 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 65(1):1–11. doi:10.1002/art.37715

    Article  CAS  PubMed  Google Scholar 

  2. De Luna G, Terrier B, Kaminsky P, Quellec AL, Maurier F, Solans R, Godmer P, Costedoat-Chalumeau N, Seror R, Charles P, Cohen P, Puechal X, Mouthon L, Guillevin L (2015) Central nervous system involvement of granulomatosis with polyangiitis: clinical-radiological presentation distinguishes different outcomes. Rheumatology (Oxford, England) 54 (3):424–432. doi:10.1093/rheumatology/keu336

  3. Anderson JM, Jamieson DG, Jefferson JM (1975) Non-healing granuloma and the nervous system. Q J Med 44(174):309–323

    CAS  PubMed  Google Scholar 

  4. de Groot K, Schmidt DK, Arlt AC, Gross WL, Reinhold-Keller E (2001) Standardized neurologic evaluations of 128 patients with Wegener granulomatosis. Arch Neurol 58(8):1215–1221

    Article  PubMed  Google Scholar 

  5. Fauci AS, Haynes BF, Katz P, Wolff SM (1983) Wegener’s granulomatosis: prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med 98(1):76–85

    Article  CAS  PubMed  Google Scholar 

  6. Hoffman GS, Kerr GS, Leavitt RY, Hallahan CW, Lebovics RS, Travis WD, Rottem M, Fauci AS (1992) Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med 116(6):488–498

    Article  CAS  PubMed  Google Scholar 

  7. Nishino H, Rubino FA, DeRemee RA, Swanson JW, Parisi JE (1993) Neurological involvement in Wegener’s granulomatosis: an analysis of 324 consecutive patients at the Mayo Clinic. Ann Neurol 33(1):4–9. doi:10.1002/ana.410330103

    Article  CAS  PubMed  Google Scholar 

  8. Reinhold-Keller E, Beuge N, Latza U, de Groot K, Rudert H, Nolle B, Heller M, Gross WL (2000) An interdisciplinary approach to the care of patients with Wegener’s granulomatosis: long-term outcome in 155 patients. Arthritis Rheum 43(5):1021–1032. doi:10.1002/1529-0131(200005)43:5<1021:aid-anr10>3.0.co;2-j

    Article  CAS  PubMed  Google Scholar 

  9. Huang YH, Ro LS, Lyu RK, Chang HS, Wu YR, Chang KH, Kuo HC (2015) Wegener’s granulomatosis with nervous system involvement: a hospital-based study. Eur Neurol 73(3–4):197–204. doi:10.1159/000375492

    Article  PubMed  Google Scholar 

  10. Drachman DA (1963) NEurological complications of wegener’s granulomatosis. Arch Neurol 8(2):145–155. doi:10.1001/archneur.1963.00460020045003

    Article  Google Scholar 

  11. Holle JU, Gross WL (2011) Neurological involvement in Wegener’s granulomatosis. Curr Opin Rheumatol 23(1):7–11. doi:10.1097/BOR.0b013e32834115f9

    Article  PubMed  Google Scholar 

  12. Seror R, Mahr A, Ramanoelina J, Pagnoux C, Cohen P, Guillevin L (2006) Central nervous system involvement in Wegener granulomatosis. Medicine 85(1):54–65. doi:10.1097/01.md.0000200166.90373.41

    Article  PubMed  Google Scholar 

  13. Murphy JM, Gomez-Anson B, Gillard JH, Antoun NM, Cross J, Elliott JD, Lockwood M (1999) Wegener granulomatosis: MR imaging findings in brain and meninges. Radiology 213(3):794–799. doi:10.1148/radiology.213.3.r99dc11794

    Article  CAS  PubMed  Google Scholar 

  14. Leavitt RY, Fauci AS, Bloch DA, Michel BA, Hunder GG, Arend WP, Calabrese LH, Fries JF, Lie JT, Lightfoot RW Jr et al (1990) The American College of Rheumatology 1990 criteria for the classification of Wegener’s granulomatosis. Arthritis Rheum 33(8):1101–1107

    Article  CAS  PubMed  Google Scholar 

  15. Martin N, Masson C, Henin D, Mompoint D, Marsault C, Nahum H (1989) Hypertrophic cranial pachymeningitis: assessment with CT and MR imaging. AJNR Am J Neuroradiol 10(3):477–484

    CAS  PubMed  Google Scholar 

  16. Kupersmith MJ, Martin V, Heller G, Shah A, Mitnick HJ (2004) Idiopathic hypertrophic pachymeningitis. Neurology 62(5):686–694

    Article  CAS  PubMed  Google Scholar 

  17. Dash GK, Thomas B, Nair M, Radhakrishnan A (2015) Clinico-radiological spectrum and outcome in idiopathic hypertrophic pachymeningitis. J Neurol Sci 350(1–2):51–60. doi:10.1016/j.jns.2015.02.013

    Article  PubMed  Google Scholar 

  18. Provenzale JM, Allen NB (1996) Wegener granulomatosis: CT and MR findings. AJNR Am J Neuroradiol 17(4):785–792

    CAS  PubMed  Google Scholar 

  19. Mukhtyar C, Guillevin L, Cid MC, Dasgupta B, de Groot K, Gross W, Hauser T, Hellmich B, Jayne D, Kallenberg CG, Merkel PA, Raspe H, Salvarani C, Scott DG, Stegeman C, Watts R, Westman K, Witter J, Yazici H, Luqmani R (2009) EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis 68(3):310–317. doi:10.1136/ard.2008.088096

    Article  CAS  PubMed  Google Scholar 

  20. Stone JH, Merkel PA, Spiera R, Seo P, Langford CA, Hoffman GS, Kallenberg CG, St Clair EW, Turkiewicz A, Tchao NK, Webber L, Ding L, Sejismundo LP, Mieras K, Weitzenkamp D, Ikle D, Seyfert-Margolis V, Mueller M, Brunetta P, Allen NB, Fervenza FC, Geetha D, Keogh KA, Kissin EY, Monach PA, Peikert T, Stegeman C, Ytterberg SR, Specks U (2010) Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med 363(3):221–232. doi:10.1056/NEJMoa0909905

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  21. Holle JU, Gross WL, Holl-Ulrich K, Ambrosch P, Noelle B, Both M, Csernok E, Moosig F, Schinke S, Reinhold-Keller E (2010) Prospective long-term follow-up of patients with localised Wegener’s granulomatosis: does it occur as persistent disease stage? Ann Rheum Dis 69(11):1934–1939. doi:10.1136/ard.2010.130203

    Article  PubMed  Google Scholar 

  22. Jayne D (2009) The diagnosis of vasculitis. Best Pract Res Clin Rheumatol 23(3):445–453. doi:10.1016/j.berh.2009.03.001

    Article  PubMed  Google Scholar 

  23. Tumiati B, Zuccoli G, Pavone L, Buzio C (2005) ENT Wegener’s granulomatosis can hide severe central nervous system involvement. Clin Rheumatol 24(3):290–293. doi:10.1007/s10067-004-1025-1

    Article  CAS  PubMed  Google Scholar 

  24. Reinhold-Keller E, de Groot K, Holl-Ulrich K, Arlt AC, Heller M, Feller AC, Gross WL (2001) Severe CNS manifestations as the clinical hallmark in generalized Wegener’s granulomatosis consistently negative for antineutrophil cytoplasmic antibodies (ANCA). A report of 3 cases and a review of the literature. Clin Exp Rheumatol 19(5):541–549

    CAS  PubMed  Google Scholar 

  25. Di Comite G, Bozzolo EP, Praderio L, Tresoldi M, Sabbadini MG (2006) Meningeal involvement in Wegener’s granulomatosis is associated with localized disease. Clin Exp Rheumatol 24(2 suppl 41):S60–64

    PubMed  Google Scholar 

  26. Falk RJ, Jennette JC (1997) ANCA small-vessel vasculitis. J Am Soc Nephrol 8(2):314–322

    CAS  PubMed  Google Scholar 

  27. Wang YJ, Fuh JL, Lirng JF, Lu SR, Wang SJ (2004) Headache profile in patients with idiopathic hypertrophic cranial pachymeningitis. Headache 44(9):916–923. doi:10.1111/j.1526-4610.2004.04175.x

    Article  PubMed  Google Scholar 

  28. Nagahori T, Nishijima M, Takaku A (1993) Histological study of the outer membrane of chronic subdural hematoma: possible mechanism for expansion of hematoma cavity. No shinkei geka Neurol Surg 21(8):697–701

    CAS  Google Scholar 

  29. Kitazono M, Yokota H, Satoh H, Onda H, Matsumoto G, Fuse A, Teramoto A (2012) Measurement of inflammatory cytokines and thrombomodulin in chronic subdural hematoma. Neurol Med Chir 52(11):810–815

    Article  Google Scholar 

  30. Tanaka Y, Ohno K (2013) Chronic subdural hematoma—an up-to-date concept. Journal of medical and dental sciences 60(2):55–61

    PubMed  Google Scholar 

  31. Yokoseki A, Saji E, Arakawa M, Kosaka T, Hokari M, Toyoshima Y, Okamoto K, Takeda S, Sanpei K, Kikuchi H, Hirohata S, Akazawa K, Kakita A, Takahashi H, Nishizawa M, Kawachi I (2014) Hypertrophic pachymeningitis: significance of myeloperoxidase anti-neutrophil cytoplasmic antibody. Brain 137(Pt 2):520–536. doi:10.1093/brain/awt314

    Article  PubMed  Google Scholar 

  32. Yonekawa T, Murai H, Utsuki S, Matsushita T, Masaki K, Isobe N, Yamasaki R, Yoshida M, Kusunoki S, Sakata K, Fujii K, Kira J (2014) A nationwide survey of hypertrophic pachymeningitis in Japan. J Neurol Neurosurg Psychiatry 85(7):732–739. doi:10.1136/jnnp-2013-306410

    Article  PubMed  Google Scholar 

  33. Csernok E, Trabandt A, Muller A, Wang GC, Moosig F, Paulsen J, Schnabel A, Gross WL (1999) Cytokine profiles in Wegener’s granulomatosis: predominance of type 1 (Th1) in the granulomatous inflammation. Arthritis Rheum 42(4):742–750. doi:10.1002/1529-0131(199904)42:4<742:aid-anr18>3.0.co;2-i

    Article  CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Neurology, Neuroscience Center, Samsung Medical Center, Sungkyunkwan University School of Medicine, 81 Irwon-Ro, Gangnam-gu, Seoul, 135-710, Korea

    Hyun Ah Choi, Mi Ji Lee & Chin-Sang Chung

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  1. Hyun Ah Choi
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Correspondence to Chin-Sang Chung.

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Choi, H.A., Lee, M.J. & Chung, CS. Characteristics of hypertrophic pachymeningitis in patients with granulomatosis with polyangiitis. J Neurol 264, 724–732 (2017). https://doi.org/10.1007/s00415-017-8416-0

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