Vasculitic neuropathy
| Vasculitic neuropathy | |
|---|---|
| Specialty | Neurology |
Vasculitic neuropathy is a peripheral neuropathic disease.[1][2][3] In a vasculitic neuropathy there is damage to the vessels that supply blood to the nerves.[1][2][3] It can be as part of a systemic problem or can exist as a single-organ issue only affecting the peripheral nervous system (PNS).[1][2][3] It is diagnosed with the use of electrophysiological testing, blood tests, nerve biopsy and clinical examination. It is a serious medical condition that can cause prolonged morbidity and disability and generally requires treatment. Treatment depends on the type but it is mostly with corticosteroids or immunomodulating therapies.
Types[edit]
There are three main categories of vasculitic neuropathies: primary, secondary and non-systemic.[2]
Primary systemic vasculitic neuropathy[edit]
Some patients with systemic vasculitis will have their multi-organ disease spread to the peripheral nervous system; this is primary vasculitic neuropathy. Some examples of systemic vasculitic disease are: IgA vasculitis, Hypocomplementemic urticarial vasculitis, polyarteritis nodosa (PAN) and anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitides such as granulomatosis with polyangiitis (GPA), eosinophilic granulomatosis with polyangiitis (EGPA) and microscopic polyangiitis (MPA).[1][3]
Vasculitic neuropathy secondary to other disease[edit]
Some patients with a non-vasculitic systemic disease or another illness such as infection or malignancy can subsequently develop vasculitic neuropathy as a direct consequence of the former illness; this is secondary vasculitic neuropathy. Some examples of such illness which can cause vasculitic neuropathy are:[citation needed]
- Connective tissue diseases: rheumatoid arthritis, systemic lupus erythematosus, primary sjögren's, dermatomyositis.
- Infectious diseases: hepatitis B, hepatitis C, human immunodeficiency virus (HIV), cytomegalovirus, lyme disease, human T-cell-lymphotrophic virus-I, parvovirus B19.
- Malignancy.
- Drugs (amphetamines, sympathomimetics, cocaine, etc.)
- Vaccinations.
Non-systemic vasculitic neuropathy[edit]
Non-systemic vasculitic neuropathy (NSVN) is a diagnosis of elimination. When no systemic illness can be found, yet evidence of a vasculitic neuropathy exists, a diagnosis of non-systemic vasculitic neuropathy is made. It is a single-organ problem. A nerve biopsy is required in order to make the diagnosis of non-systemic vasculitic neuropathy.
There are distinct subtypes of NSVN with evolving categorisation in the literature. Currently accepted subtypes are:[2][3]
- 'Classical' distal-predominant NSVN
- Wartenberg migratory sensory neuropathy
- Post-surgical inflammatory neuropathy
- Diabetic radiculoplexus neuropathy (lumbosacral, thoracic or cervical predominant)
- Neuralgic amyotrophy
- Non-systemic skin/nerve vasculitis (for example, cutaneous PAN)
'Classical' distal-predominant NSVN[edit]
There is an ongoing debate over this categorisation, particularly its overlap with the condition non-diabetic radiculoplexus neuropathy. This neuropathy involves a clinical picture where the nerve damage is distally predominant as demonstrated in a nerve biopsy.[3]
Warternberg migratory sensory neuropathy[edit]
Warternberg migratory sensory neuropathy is typically a multi-focal neuropathy where there is pure sensory deficits. It is characterised by sudden-onset and chronicity as well as having a propensity for relapse. It generally resolves slowly with time.[3]
Postsurgical inflammatory neuropathy[edit]
Postsurgical inflammatory neuropathy is typically a multi-focal neuropathy which manifests thirty days after a surgical procedure. It mostly presents with motor and sensory symptoms. It is generally a self-limiting condition that has resolved with and without treatment.[3]
Diagnosis[edit]
Diagnosis of a vasculitic neuropathy depends on whether the patient first presents with multiple symptoms pointing at a systemic disorder or else primary neuropathic complaints. In the former case the patient is more likely to be assessed first by a rheumatologist and in the latter a neurologist or neurosurgeon.[citation needed]
Treatments[edit]
Treatment of vasculitic neuropathy depends on the type.[citation needed]
References[edit]
- ^ a b c d Graf, Jonathan; Imboden, John (January 2019). "Vasculitis and peripheral neuropathy". Current Opinion in Rheumatology. 31 (1): 40–45. doi:10.1097/BOR.0000000000000559. ISSN 1040-8711. PMID 30461543. S2CID 53943955.
- ^ a b c d e Collins, Michael P.; Dyck, P. James B.; Hadden, Robert D.M. (October 2019). "Update on classification, epidemiology, clinical phenotype and imaging of the nonsystemic vasculitic neuropathies". Current Opinion in Neurology. 32 (5): 684–695. doi:10.1097/WCO.0000000000000727. ISSN 1350-7540. PMID 31313704. S2CID 197423473.
- ^ a b c d e f g h Collins, Michael P.; Hadden, Robert D. (May 2017). "The nonsystemic vasculitic neuropathies". Nature Reviews Neurology. 13 (5): 302–316. doi:10.1038/nrneurol.2017.42. ISSN 1759-4758. PMID 28447661.