Treatment of Mononeuritis Multiplex

Diagnosis and Underlying Causes

Mononeuritis multiplex is a peripheral neuropathy characterized by asymmetric involvement of multiple peripheral nerves, presenting with altered sensation, pain, muscle weakness or atrophy, according to the European League Against Rheumatism 1
Eosinophilic granulomatosis with polyangiitis (EGPA) is a common underlying cause of mononeuritis multiplex, as stated by the American College of Rheumatology 2
Diagnosis should include nerve conduction studies (NCS) and electromyography to identify mononeuropathies and differentiate multiple mononeuropathy from polyneuropathy, as recommended by the European Federation of Neurological Societies 1
CSF analysis is recommended when inflammatory demyelinating polyradiculoneuropathy is suspected, according to the American Academy of Neurology 1
Deep skin biopsy reaching medium-sized vessels is recommended when polyarteritis nodosa (PAN) is suspected, as stated by the European League Against Rheumatism 3, 4
Testing for ANCA is recommended when vasculitis is suspected, according to the American College of Rheumatology 5

The American College of Rheumatology recommends high-dose glucocorticoids combined with cyclophosphamide for remission induction in severe vasculitis-associated mononeuritis multiplex, with a strength of evidence rated as high 3, 5
The European League Against Rheumatism recommends transitioning to maintenance therapy with methotrexate, azathioprine, or mycophenolate mofetil after achieving remission with cyclophosphamide, with a duration of approximately 18 months 3, 4, 6, 7
The American College of Rheumatology recommends glucocorticoids combined with non-cyclophosphamide immunosuppressants (methotrexate, azathioprine, or mycophenolate mofetil) as first-line treatment for non-severe vasculitis-associated mononeuritis multiplex, with a strength of evidence rated as moderate 3, 4, 6
The European League Against Rheumatism recommends considering mepolizumab with glucocorticoids for EGPA-associated cases, with a strength of evidence rated as low 6, 7
The American Academy of Neurology recommends considering rituximab, particularly in ANCA-positive cases, with a strength of evidence rated as moderate 1, 6, 7
The European Federation of Neurological Societies recommends considering intravenous immunoglobulin or plasma exchange in severe or treatment-resistant cases, with a strength of evidence rated as low 1

The American College of Rheumatology recommends physical therapy for patients with nerve and/or muscle involvement, with a strength of evidence rated as high 3, 4
The European League Against Rheumatism recommends tumor necrosis factor inhibitors over glucocorticoids alone for patients with deficiency of adenosine deaminase 2 (DADA2), with a strength of evidence rated as moderate 3, 4

The American Academy of Neurology recommends monitoring for clinical improvement in neurological symptoms and function, with a strength of evidence rated as high 1
The European Federation of Neurological Societies recommends following nerve conduction studies to assess improvement, though parameters may remain unchanged in most patients over time, with a strength of evidence rated as moderate 1
The American College of Rheumatology recommends being aware that peripheral neuropathy has been reported to be a significant predictor of damage in SLE, with a strength of evidence rated as high 1

The European League Against Rheumatism recommends early initiation of therapy, as delay is associated with severe neurological deficits, with a strength of evidence rated as high 8

The European League Against Rheumatism (EULAR) recommends immunosuppressive agents, including mycophenolate mofetil (MMF), as first-line steroid-sparing or adjunctive agents for systemic manifestations of Sjögren's syndrome, although they acknowledge the lack of head-to-head comparison studies 9

MMF has demonstrated efficacy in Sjögren's systemic manifestations in open-label studies, with a favorable side-effect profile compared to cyclophosphamide or calcineurin inhibitors 9