**2.6 Vasculitic peripheral neuropathy**

Vasculitic neuropathy is routinely considered as a vasculitis associated with neuropathy. The consensus definition of pathologically definite vasculitic neuropathy requires that vessel wall inflammation is accompanied by vascular damage. A case definition of clinically probable vasculitic neuropathy in patients lacking biopsy proof incorporates clinical features typical of vasculitic neuropathy: sensory or sensory-motor involvement, asymmetric/multifocal pattern, lower-limb predominance, distal-predominance, pain, acute relapsing course, and non-demyelinating electrodiagnostic features (Good Practice Points from class II/III evidence). (Collins et al, 2010). There are reports of the efficacy of IVIg in vasculitic peripheral neuropathy (Hughes et al, 2009). Recommendations:


#### **2.7 Painful sensory neuropathy of Sjogren's syndrome**

Primary Sjogren's syndrome is associated with seven forms of neuropathy: sensory ataxic neuropathy, painful sensory neuropathy without sensory ataxia, multiple mononeuropathy, multiple cranial neuropathy, trigeminal neuropathy, autonomic neuropathy and radiculoneuropathy, based on the predominant neuropathic symptoms. The majority of patients are diagnosed with Sjogren's syndrome after neuropathic symptoms appearance. Painful sensory neuropathy without sensory ataxia is the second more frequent form of neuropathy associated with Sjogren's syndrome. It is characterised by chronic progression of sensory symptoms without substantial motor involvement, although the affected sensory modalities and distribution pattern vary. Autonomic symptoms, like abnormal pupils and orthostatic hypotension are often seen. Unelicited somatosensory evoked potentials and spinal cord posterior column abnormalities in MRI are observed. Sural nerve biopsy specimens reveal variable degrees of axon loss, predominantly small fibre loss (Mori et al, 2005). Patients usually suffer from severe neuropathic pain, with small-fiber neuropathy causing lancinating or burning pain which can disproportionately affect the proximal torso or extremities, and the face (ie, in a "nonlength-dependent distribution") (Birnbaum, 2010).

There are reports and small open studies of the efficacy of IVIg in painful sensory neuropathy associated with Sjogren's syndrome (Hughes et al, 2009).

Recommendations:

342 Autoimmune Disorders – Current Concepts and Advances from Bedside to Mechanistic Insights

used for CIDP (including IVIg) as compared with 20% of those with axonal neuropathy (class IV). A Cochrane review states that observational or open trial data provides limited support for the use of immunotherapy, including IVIg, in patients with IgG and IgA




Lumbosacral radiculoplexus neuropathy (LRPN) originally described in diabetic patients as diabetic amyotrophy is a distinct clinical condition characterized by debilitating pain, weakness and atrophy most commonly affecting the proximal thigh muscles asymmetrically. The syndrome is usually monophasic and preceded by significant weight loss (at least more than 10 lbs). Though a self-limited condition, recovery is gradual with



Vasculitic neuropathy is routinely considered as a vasculitis associated with neuropathy. The consensus definition of pathologically definite vasculitic neuropathy requires that vessel wall inflammation is accompanied by vascular damage. A case definition of clinically probable vasculitic neuropathy in patients lacking biopsy proof incorporates clinical features typical of vasculitic neuropathy: sensory or sensory-motor involvement, asymmetric/multifocal pattern, lower-limb predominance, distal-predominance, pain, acute relapsing course, and non-demyelinating electrodiagnostic features (Good Practice Points from class II/III evidence). (Collins et al, 2010). There are reports of the efficacy of IVIg in


Primary Sjogren's syndrome is associated with seven forms of neuropathy: sensory ataxic neuropathy, painful sensory neuropathy without sensory ataxia, multiple mononeuropathy, multiple cranial neuropathy, trigeminal neuropathy, autonomic

paraproteinaemic neuropathy (Allen et al, 2007).

neuropathy (level B recommendation).

some residual weakness (Bhanushai & Muley, 2008).

vasculitic peripheral neuropathy (Hughes et al, 2009).

**2.7 Painful sensory neuropathy of Sjogren's syndrome** 

although its efficacy is not proven (good practice point).

Recommendations:

force, 2008).

**2.5 Diabetic amyotrophy** 

(Hughes et al, 2009).

**2.6 Vasculitic peripheral neuropathy** 

Recommendations:

use of IVIg.

Recommendations:

use of IVIg.

