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Table of Contents    
Year : 2019  |  Volume : 67  |  Issue : 4  |  Page : 1048-1049

Neurological Manifestations of ANCA-associated Vasculitides: Not Good but Not Bad or Ugly Either

1 Department of Neurology, Dayanand Medical College, Ludhiana, Punjab, India
2 Research and Development Unit, Dayanand Medical College, Ludhiana, Punjab, India

Date of Web Publication10-Sep-2019

Correspondence Address:
Dr. Gagandeep Singh
Department of Neurology, Dayanand Medical College, Ludhiana - 141 001, Punjab
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.266269

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How to cite this article:
Singh G, Singh AN. Neurological Manifestations of ANCA-associated Vasculitides: Not Good but Not Bad or Ugly Either. Neurol India 2019;67:1048-9

How to cite this URL:
Singh G, Singh AN. Neurological Manifestations of ANCA-associated Vasculitides: Not Good but Not Bad or Ugly Either. Neurol India [serial online] 2019 [cited 2021 Jan 17];67:1048-9. Available from:

The antineutrophilic cytoplasmic antibody (ANCA)-associated vasculitides are a group of small-vessel vasculitic disorders characterized by antibodies reacting to the cytoplasm of ethanol-fixed peripheral blood granulocytes and monocytes. There are two main types of antibodies: (1)p (perinuclear)-ANCA, which targets myeloperoxidase in the granulocytes, and (2) c (cytoplasmic)-ANCA, which targets proteinase-3.[1] From the clinical standpoint, the following conditions are included: (1) granulomatosis with polyangiitis (GPA) (previously known as Wegner's granulomatosis), (2) microscopic polyangiitis, and (3) Churg–Strauss syndrome.

Early and even more recent descriptions of ANCA-vasculitides (then eponymously known as Wegner's granulomatosis) emphasized the characteristic triad of granuloma of the respiratory tract, widespread arteritis, and nephritis.[2],[3],[4] Neurological manifestations have all along been neglected. These were perhaps first methodically investigated and described by (later Lord) Walton in 1958.[5] Later, Drachman in 1963 drew attention to the neurological manifestations, which in his analysis comprised over half of the cases reviewed.[6] The largest subgroup (28%) comprised peripheral nerve involvement of the pattern of mononeuritis multiplex. Neurological manifestations thus constitute an important element in the clinical spectrum of ANCA-vasculitides. The prognosis, specifically associated with neurological manifestations is thus of interest, only again largely ignored.

Sharma et al., in this issue, report the prognosis specifically associated with neurological manifestations ANCA-associated vasculitides.[7] Nearly three-fourths of the patients in this single-center series comprised those with GPA. When followed up for a median period of 31 months, some 46% of the patient population developed neurological manifestations. In a tenth, moreover, neurological disorders were the presenting manifestation. About half of those with neurological manifestations demonstrated evidence of peripheral nerve involvement. Mononeuritis multiplex is the most common peripheral neuropathy in subjects with ANCA-positive vasculitides. A smaller subgroup comprises cranial neuropathies, predominantly, the seventh and eighth cranial nerves.

All clinicians should be mindful of the figures reported in this article.[7] It is, however, the prognosis associated with neurological manifestations in ANCA-vasculitides that the authors wish to allude upon. Their analysis sets out the lack of association between neurological involvement and cumulative survival. There could be several reasons for this observation. One is that the cohort comprised a large proportion (23% of the entire cohort and nearly half of those with neurological manifestations) with peripheral nerve involvement. Peripheral nerve involvement in comparison to other system's involvement, for example, renal, respiratory and cardiac is likely to be associated with a better prognosis in terms of mortality. Among the neurological manifestations, it is the occurrence of central nervous system disorders, which are difficult to treat and hence more ominous. The central nervous system manifestations of ANCA-positive vasculitides include stroke, pachymeningits, and pseudotumor. In the series reported, there were only 10 patients with central nervous system involvement. Furthermore, those with neurological involvement were less likely to have renal involvement. There were also baseline differences in terms of etiology among those with and without neurological manifestations. Finally, limited forms of vasculitides have been described, and out of these those with isolated peripheral nerve vasculitides seem to be associated with a good prognosis.[8]

Right from the beginning, nervous system manifestations of the ANCA-vasculitides have been largely ignored, and as a result, poorly understood.[2],[3],[4] Some of these aspects still remain obscure. Perhaps, longer duration of follow-up from multiple centers might provide a better view of the long-term prognosis associated with neurological manifestations of the ANCA-vasculitides. In addition, clinical researchers should focus collectively on the central nervous system manifestations of the vasculitides.

  References Top

Woude FJV, Rasmussen N, Lobatto S, Wiik A, Permin H, Van Es LA, et al. Autoantibodies against neutrophiles and monocytes: Tool for diagnosis and marker of disease activity in Wegener's granulomatosis. N Engl J Med 1966;274:1428-30.  Back to cited text no. 1
Wegener F. Uber eine eigenartige rhinogena granulomatose mit besonderer Beteiligung des arteriensystems und der nieren. Beitr Path Anat 1939;102:36.  Back to cited text no. 2
Churg J, Strauss L. Allergic granulomatosis, allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol 1951;27:277.  Back to cited text no. 3
Godman GC, Churg J. Wegener's granulomatosis. Pathology and review of the literature. Arch Pathol 1954;58:533.  Back to cited text no. 4
Walton EW. Giant-cell granuloma of the respiratory tract (Wegener's granulomatosis). Brit Med J 1958;2:265.  Back to cited text no. 5
Drachman DA. Neurological complications of Wegner's granulomatosis. Arch Neurol 1963;8:145-55.  Back to cited text no. 6
Sharma A, Rajan R, Modi M, Pinto B, Dhooria A, Rathi M, et al. Neurological manifestations do not affect cumulative survival in Indian patients with antineutrophil cytoplasmic antibody associated vasculitis. Neurol India 2019;67:1043-7.  Back to cited text no. 7
  [Full text]  
Cassan SM, Coles DT, Harrison EG. The concept of limited forms of Wegener's granulomatosis. Am J Med 1970;49:366-79.  Back to cited text no. 8


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