Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ACUTE

primary systemic or non-systemic vasculitic neuropathies: initial presentation

1st line – corticosteroid

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ACUTE
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primary systemic or non-systemic vasculitic neuropathies: initial presentation
1st line – 

corticosteroid

Primary systemic vasculitic neuropathies associated with MNM are: classic polyarteritis nodosa (PAN); eosinophilic granulomatosis with polyangiitis (EGPA; formerly known as Churg-Strauss syndrome); granulomatosis with polyangiitis (formerly known as Wegener's granulomatosis); and microscopic polyarteritis. Corticosteroid treatment is always part of first-line therapy for MNM associated with these conditions.[6][65]​​​​

Treatment for MNM associated with classic PAN or EGPA begins with a risk assessment for an adverse prognosis. Indicators for poor prognosis are creatinine >1.58 mg/dL (>140 micromol/L); proteinuria >1 g/day; and central nervous system, gastrointestinal, or cardiac involvement.[1][66]​​​ MNM is not an indicator of a poor prognosis for these conditions.

For patients with mild classic PAN or EGPA without indicators of poor prognosis, prednisolone monotherapy alone may be sufficient.[1][60]

For patients with classic PAN or EGPA and any factors indicating a poor prognosis, with granulomatosis with polyangiitis, or with microscopic polyarteritis (which are generally severe), or with non-systemic vasculitic neuropathy, standard corticosteroid therapy is intravenous methylprednisolone at diagnosis (for 3-5 days), followed by oral prednisolone until remission is achieved.[7][23]​​

See also Polyarteritis nodosa, Eosinophilic granulomatosis with polyangiitis, and Granulomatosis with polyangiitis.

Primary options

prednisolone: 1 mg/kg/day orally, adjust dose according to response and taper gradually

OR

methylprednisolone sodium succinate: 1 g intravenously once daily for 3-5 days, followed by oral prednisolone

and

prednisolone: 1 mg/kg/day orally following methylprednisolone course, adjust dose according to response and taper gradually

Consider – immunosuppressant

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ACUTE
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primary systemic or non-systemic vasculitic neuropathies: initial presentation
Consider – 

immunosuppressant

Additional treatment recommended for SOME patients in selected patient group

Oral cyclophosphamide is part of standard therapy at diagnosis (in addition to corticosteroids) for patients with MNM associated with granulomatosis with polyangiitis or microscopic polyarteritis.[65] For patients with classic PAN or EGPA, oral cyclophosphamide is part of standard therapy at diagnosis for patients with factors indicating a poor prognosis, and may be added for patients with an initially good prognosis if their vasculitic neuropathy is not controlled by prednisolone alone.[65] For patients with non-systemic vasculitic neuropathy, combination immunosuppression seems more effective at inducing remission and reducing disability than corticosteroids alone.[23] However, many neuromuscular neurologists begin therapy with corticosteroids alone, and add cyclophosphamide only if the neuropathy is progressive despite corticosteroid therapy.[63]

Options for treating vasculitic neuropathy with an insufficient response to first-line treatment include replacing oral cyclophosphamide with either intravenous pulse cyclophosphamide (administered with mesna and fluids to prevent haemorrhagic cystitis) or methotrexate.[7][23][65]

Rituximab may be considered for treating primary systemic vasculitic neuropathies that are resistant to cyclophosphamide and methotrexate, or for patients who are intolerant of these treatments.[65] Primary non-systemic vasculitic neuropathies usually respond well to cyclophosphamide or methotrexate, but rituximab is a further option for patients refractory to these treatments.[23]

Recommended doses vary between guidelines; consult your local guidelines for more information.

Primary options

cyclophosphamide: consult specialist for guidance on dose

Secondary options

methotrexate: consult specialist for guidance on dose

Tertiary options

rituximab: consult specialist for guidance on dose

Consider – plasma exchange or intravenous immunoglobulin (IVIG)

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ACUTE
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primary systemic or non-systemic vasculitic neuropathies: initial presentation
Consider – 

plasma exchange or intravenous immunoglobulin (IVIG)

Additional treatment recommended for SOME patients in selected patient group

Plasma exchange or IVIG may be given as adjunctive treatment for refractory vasculitis.[1][7][23][63][65]

Reasonable plasma exchange protocols are: for granulomatosis with polyangiitis, microscopic polyarteritis, and non-systemic vasculitic neuropathies: 5-7 sessions over 2 weeks; for classic PAN and EGPA: 1-3 treatments per week for 6-10 weeks (exact approach will depend on disease severity).

Primary options

normal immunoglobulin human: consult specialist for guidance on dose

secondary vasculitic neuropathies: initial presentation

1st line – prednisolone + antiviral therapy

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ACUTE
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secondary vasculitic neuropathies: initial presentation
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hepatitis B-associated polyarteritis nodosa
1st line – 

prednisolone + antiviral therapy

A hepatologist, an infectious disease specialist, and a neuromuscular neurologist should all be involved in treatment decisions.

Immunosuppressive therapy alone can lead to activation of hepatitis B replication.[67]​ Therefore, patients with hepatitis B-associated polyarteritis nodosa (PAN) should have an initial course of therapy comprising prednisolone for immunosuppression and an antiviral agent for treating hepatitis B.[6]

For patients with vasculitic neuropathy with an insufficient response to first-line treatment, antiviral therapy should be amended and prednisolone continued.

For current recommendations for antiviral therapy, see Hepatitis B. See also Polyarteritis nodosa.

Primary options

prednisolone: 1 mg/kg/day orally, adjust dose according to response and taper gradually

Consider – plasma exchange or intravenous immunoglobulin (IVIG)

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ACUTE
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secondary vasculitic neuropathies: initial presentation
|
hepatitis B-associated polyarteritis nodosa
Consider – 

plasma exchange or intravenous immunoglobulin (IVIG)

Additional treatment recommended for SOME patients in selected patient group

Plasma exchange (1-3 treatments per week for 6-10 weeks, depending on disease severity) or IVIG may form part of therapy for fulminant cases at presentation or for refractory vasculitis.[1]

Primary options

normal immunoglobulin human: consult specialist for guidance on dose

1st line – prednisolone

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ACUTE
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secondary vasculitic neuropathies: initial presentation
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hepatitis C-associated cryoglobulinaemic vasculitic neuropathy
1st line – 

prednisolone

A hepatologist, an infectious disease specialist, and a neuromuscular neurologist should all be involved in treatment decisions.

The goal of therapy is to achieve remission of the vasculitis without causing a sustained increase in the activity of the underlying hepatitis C.

Initial immunosuppressive therapy with prednisolone plus an immunosuppressant is often used.[1]

Primary options

prednisolone: 1 mg/kg/day orally, adjust dose according to response and taper gradually

Plus – immunosuppressant

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ACUTE
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secondary vasculitic neuropathies: initial presentation
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hepatitis C-associated cryoglobulinaemic vasculitic neuropathy
Plus – 

immunosuppressant

Treatment recommended for ALL patients in selected patient group

Initial immunosuppressive therapy with prednisolone plus oral cyclophosphamide is often used.[1]

For vasculitic neuropathy with an insufficient response to first-line treatment, prednisolone is combined with an alternative immunosuppressant such as intravenous pulse-dose cyclophosphamide (administered with mesna and fluids to reduce risk of haemorrhagic cystitis) or methotrexate.[7]

Rituximab may be considered for treating vasculitic neuropathy that is resistant to cyclophosphamide and methotrexate, or for patients who are intolerant of these treatments. Some clinicians may try prednisolone plus rituximab (with antiviral therapy) as initial treatment for severe advanced hepatitis C-associated cryoglobulinaemic vasculitis disease.[7]

Recommended doses vary between guidelines; consult your local guidelines for more information.

Primary options

cyclophosphamide: consult specialist for guidance on dose

Secondary options

methotrexate: consult specialist for guidance on dose

Tertiary options

rituximab: consult specialist for guidance on dose

Plus – antiviral therapy

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ACUTE
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secondary vasculitic neuropathies: initial presentation
|
hepatitis C-associated cryoglobulinaemic vasculitic neuropathy
Plus – 

antiviral therapy

Treatment recommended for ALL patients in selected patient group

Corticosteroids may stimulate viral replication and increase viraemia, so antiviral therapy (with a direct-acting antiviral for hepatitis C) should be started once vasculitic neuropathy is under control.[68]​ For current recommendations for direct-acting antiviral therapy, see Hepatitis C.

Consider – plasma exchange or intravenous immunoglobulin (IVIG)

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ACUTE
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secondary vasculitic neuropathies: initial presentation
|
hepatitis C-associated cryoglobulinaemic vasculitic neuropathy
Consider – 

plasma exchange or intravenous immunoglobulin (IVIG)

Additional treatment recommended for SOME patients in selected patient group

Plasma exchange (1-3 treatments per week for 6-10 weeks, depending on disease severity) may form part of first-line therapy in fulminant cases.[1] Plasma exchange or IVIG may be given as adjunctive treatment for refractory vasculitis.[1][7]

Primary options

normal immunoglobulin human: consult specialist for guidance on dose

1st line – antiretroviral therapy

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ACUTE
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secondary vasculitic neuropathies: initial presentation
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HIV-associated vasculitic neuropathy: cytomegalovirus (CMV)-negative
1st line – 

antiretroviral therapy

Specialist consultation is recommended.

There are few reliable data on the treatment of HIV-associated vasculitis.

The most important determinant is whether the MNM is CMV-positive or CMV-negative.

For patients who are negative for CMV, optimised antiretroviral therapy directed at HIV is the most important intervention. HIV-associated, CMV-negative vasculitic neuropathies often remit spontaneously.[69]

For details of antiretroviral therapy, see HIV infection.

Consider – prednisolone

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ACUTE
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secondary vasculitic neuropathies: initial presentation
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HIV-associated vasculitic neuropathy: cytomegalovirus (CMV)-negative
Consider – 

prednisolone

Additional treatment recommended for SOME patients in selected patient group

A short course of oral prednisolone may be required to abrogate severe HIV-associated painful MNM.[40]

Primary options

prednisolone: 1 mg/kg/day orally, adjust dose according to response and taper gradually

1st line – antiretroviral therapy

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ACUTE
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secondary vasculitic neuropathies: initial presentation
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HIV-associated vasculitic neuropathy: CMV-positive
1st line – 

antiretroviral therapy

Specialist consultation is recommended.

There are few reliable data on the treatment of CMV-associated vasculitis in HIV seropositive patients. These patients usually have advanced AIDS, with very high mortality if treatment is delayed.[1]

For details of antiretroviral therapy, see HIV infection.

Plus – antiviral therapy

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ACUTE
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secondary vasculitic neuropathies: initial presentation
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HIV-associated vasculitic neuropathy: CMV-positive
Plus – 

antiviral therapy

Treatment recommended for ALL patients in selected patient group

Antiviral therapy to address CMV infection is also essential.[1]

For details of antiviral therapy for CMV, see HIV-related opportunistic infections and Cytomegalovirus infection.

1st line – treatment of underlying malignancy

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ACUTE
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secondary vasculitic neuropathies: initial presentation
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cancer-associated vasculitic neuropathy
1st line – 

treatment of underlying malignancy

Management in consultation with an oncologist and a neuromuscular neurologist is recommended.

The first-line treatment for cancer-associated vasculitis is to treat the underlying malignancy.

Consider – corticosteroid

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ACUTE
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secondary vasculitic neuropathies: initial presentation
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cancer-associated vasculitic neuropathy
Consider – 

corticosteroid

Additional treatment recommended for SOME patients in selected patient group

If the neuropathy remains after treating malignancy, corticosteroids can be used.[1][7]

Initial treatment is with intravenous methylprednisolone for 3-5 days, followed by oral prednisolone.

Primary options

methylprednisolone sodium succinate: 1 g intravenously once daily for 3-5 days, followed by oral prednisolone

and

prednisolone: 1 mg/kg/day orally following methylprednisolone course, adjust dose according to response and taper gradually

Consider – cyclophosphamide

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ACUTE
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secondary vasculitic neuropathies: initial presentation
|
cancer-associated vasculitic neuropathy
Consider – 

cyclophosphamide

Additional treatment recommended for SOME patients in selected patient group

Oral cyclophosphamide may be added to corticosteroid treatment.[1]

Primary options

cyclophosphamide: consult specialist for guidance on dose

Consider – intravenous immunoglobulin (IVIG)

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ACUTE
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secondary vasculitic neuropathies: initial presentation
|
cancer-associated vasculitic neuropathy
Consider – 

intravenous immunoglobulin (IVIG)

Additional treatment recommended for SOME patients in selected patient group

IVIG may be given as adjunctive treatment.[1][7]

Primary options

normal immunoglobulin human: consult specialist for guidance on dose

1st line – corticosteroid

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ACUTE
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secondary vasculitic neuropathies: initial presentation
|
secondary vasculitis associated with connective tissue disease
1st line – 

corticosteroid

MNM may result from a secondary vasculitis associated with a variety of connective tissue diseases: commonly rheumatoid arthritis and systemic lupus erythematosus, but vasculitic neuropathy may also present in Sjogren syndrome, mixed connective tissue disease, scleroderma, or relapsing polychondritis. The goal of therapy is the remission of the vasculitic process and MNM.

Initial therapy is intravenous methylprednisolone for 3-5 days, followed by oral prednisolone.

Primary options

methylprednisolone sodium succinate: 1 g intravenously once daily for 3-5 days, followed by oral prednisolone

and

prednisolone: 1 mg/kg/day orally following methylprednisolone course, adjust dose according to response and taper gradually

Plus – treatment of underlying condition

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ACUTE
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secondary vasculitic neuropathies: initial presentation
|
secondary vasculitis associated with connective tissue disease
Plus – 

treatment of underlying condition

Treatment recommended for ALL patients in selected patient group

A rheumatologist and neuromuscular neurologist should be involved, as treatment will vary with the underlying connective tissue disease and involvement of other organ systems.

For details of treatments for the underlying condition, see Rheumatoid arthritis, Systemic lupus erythematosus, Sjogren syndrome, Overlap syndromes, and Systemic sclerosis (scleroderma)

1st line – immunosuppressant therapy for sarcoidosis

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ACUTE
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secondary vasculitic neuropathies: initial presentation
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neurosarcoidosis
1st line – 

immunosuppressant therapy for sarcoidosis

Neurosarcoidosis presenting with MNM is rare, and usually responds well to standard immunosuppressant therapy for sarcoidosis.[70]​ See Sarcoidosis.

ONGOING

vasculitis in remission

1st line – continue immunosuppressant therapy

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ONGOING
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vasculitis in remission
1st line – 

continue immunosuppressant therapy

After remission is achieved, the goal is to maintain remission while minimising medication toxicities. Due to high relapse rates when treatment is discontinued within 12 months, it is reasonable to maintain therapy for at least 24 months for a patient in remission.[1][23][61]

For patients whose MNM was controlled with prednisolone monotherapy that was gradually tapered over 6-12 months once remission was achieved, no additional treatments other than prednisolone are needed. For patients who required additional treatment to induce remission, maintenance therapy should also include one of the following in addition to prednisolone: continuing oral cyclophosphamide for 12 months, then tapering; or (for patients with cyclophosphamide intolerance or toxicity) switching from oral cyclophosphamide to oral or intravenous methotrexate or to oral azathioprine for 18-24 months.[1]

Intravenous immunoglobulin (IVIG) may be used as a maintenance treatment over 6-12 months for patients who did not respond to or were intolerant of other immunosuppressant treatments.[1]

Immunosuppressant regimens may vary; consult your local guidelines for more information on choice of regimen and doses.

Consider – continue treatment for underlying condition

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ONGOING
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vasculitis in remission
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secondary vasculitic neuropathies
Consider – 

continue treatment for underlying condition

Additional treatment recommended for SOME patients in selected patient group

Treatment for the condition underlying the vasculitis may need to be continued. See the ‘Acute’ section of the algorithm for further details of treatments for particular conditions.

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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