Non-systemic vasculitic neuropathy
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:
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
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. Warternberg migratory sensory neuropathy
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. Postsurgical inflammatory neuropathy
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. Diagnosis
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.
Disruption to action potential propagation can affect the AV and SA nodes which are part of the conduction system of the heart.Fetal susceptibility and environmental factors could also play a role in pathogenesis since not all infants develop congenital heart block even when exposed to antibodies. Clinical manifestations
Neonatal lupus can present with several signs and symptoms. The most common manifestations involve the heart and skin. Problems involving the liver, gallbladder, brain, and blood can be seen but are usually transient. Heart
Cardiac manifestations present more commonly in utero, but can also present after birth. The most common complications are varying degrees of heart block and endocardial fibroelastosis. A large percentage of infants diagnosed with isolated congenital heart block are associated to neonatal lupus. Heart block occurs when there is dysfunction in the conduction system, preventing impulses from traveling from the atria to the ventricles. Heart block can initially present with bradycardia in the prenatal period, usually around the second trimester. Infants with lower grades of heart block can progress to higher grades, but they can also present with rapid onset of complete heart block. Endocardial fibroelastosis is considered a type of cardiomyopathy that occurs in response to heart cell injury and can be seen with or without conduction system dysfunction.Common complications

First degree heart block
Second degree heart block
Third degree heart block (Complete heart block)
Endocardial fibroelastosisOther complications seen with neonatal lupus

Patent ductus arteriosus
Patent foramen ovale
Pulmonic stenosis
Pulmonary valvular dysplasia
Fusion of chordae tendineae of the tricuspid valve
Ostium secundum type atrial septal defects

Skin
A rash can be seen upon delivery.