By:-HEMANT RAJ SINGH

MPY(2nd y)
 Peripheral Vascular Disease is usually found to be more common in men
but can also be seen in women.

 The disease is intermittent claudication in the peripheral limbs, meaning the

hands and feet.

 Generally Peripheral Vascular Disease is in association with coronary artery

disease and atherosclerosis.

 Other causes for Peripheral Vascular Disease could include smoking,

diabetes, obesity and hypertension.
 Systemic Vasculidities
– Polyarteritis Nodosa
• Polyarteritis Nodosa associated with Hepatitis B or C
– Wegner’s Granulomatosis
– Allergic Angitis (Churg-Strauss Syndrome)
– Rheumatoid vasculitis
– Sjogren’s Syndrome
– Systemic Lupus Erythematosus
– Cryoglobulinemic Neuropathy
• Mixed Cryoglobulinemia Associated with Hepatitis C

 Non-systemic vasculitis (vasculitis restricted to the peripheral nervous

system)
– Vasculitis Neuropathy Associated with Diabetes Mellitus
 Definite diagnosis of vasculitis is done with the identification of the typical
necrotizing arteritis of the vessel walls.

 The changes are present in the medium-sized arteries of the perineurium and
epineurium,

 Inflammatory infiltrates near the vessel wall without necrotizing vasculitis

can also be observed in support of the diagnosis.

 Indirect changes in the nerve biopsy can also be helpful in the diagnosis,
these include: 1) fiber loss and Wallerian degeneration,
– 2) necrosis of the perineurium, and
– 3) new vessel formation indicative of re-canalization of an occluded
pathway. Segmental

 demyelization can also be found accounting for conduction block or

segmental nerve conduction slowing.
 Mononeuritis multiplex is the some what exotic term has been used to
describe the classical and most frequent pattern of vasculitic neuropathy.

 It implies the sequential involvement of individual nerves or trunks usually

in a distal to proximal pattern in an asymmetrical fashion.

 The neuropathy is often abrupt, preceded by pain in the field of the affected
nerve, showing involvement of both motor and sensory modalities.

 Long nerves of the lower extremities are affected more frequently than
those in the arms
 Over the years careful studies have lead to a consensus regarding the
usefulness of biopsy for the diagnosis of vasculitis with peripheral nerve
involvement.

 It seems that:
– 1) Only a percentage of diagnosed patients have a positive nerve biopsy
– 2) Combined Nerve and muscle biopsy adds to the overall diagnostic
yield than either alone
– 3) The absence of a positive tissue biopsy does not exclude the disorder
– 4) Biopsy of “symptomatic sites” seems to improve the diagnostic
yield.
– 5) Electromyography (EMG) and Nerve Conduction Studies (NCS)
help in the selection of the biopsy site and
– 6) whole nerve biopsy more useful than fascicular biopsy. (1,4,6,8,)
 The findings of NCV and EMG in the vasculidities with peripheral nerve
involvement reflect its pathology.

 The findings are those of an axonal neuropathy involving both motor and
sensory nerves at all levels.

 Conduction block can be seen from nerve ischemia.

 Asymmetries in the compound action potential of different nerves can also

substantiate a mononeuritic axonal pattern. (1,6,)
 Perhaps the most classical of the vasculitis with peripheral nerve
involvement

 A rare disorder.

 Estimates of the incidence in the general population range from 4.6 to 9.0
per million.

 It affects men and women of all racial groups with predominance.

 Ages 40 to 60.

 Its etiology remains unknown despite a clear association with viral illnesses
in many cases. (HIV, Hep B & C, etc)
 PAN affects mainly medium sized arteries.

 Nerve and muscle seem to be the most involved tissues.

 Peripheral neuropathy has been recognized as one of the most frequent

clinical manifestation.

 Initial presenting symptom together with asthenia, weight loss, malaise and
fever.

 Fifty to seventy (50 to 70%) percent of the diagnosed cases have

neuropathy.

 The neuropathy is asymmetric involving sensory and motor functions,

affecting most frequently the lower extremities.

 Two percent (2%) of the patients showed cranial nerve involvement.

 Sensory complaints can vary from dysesthesia to pain. Motor deficits

usually present abruptly.
 The CSF is usually normal.

 Nerve biopsy, usually taken from the sural nerve, will usually show the
necrotizing arteritis in medium sized vessels (fibrinoid necrosis of all three
coats of the vessel walls).

 Muscle biopsy may also show perivascular inflammation and necrosis but
the diagnostic yield is less than for biopsy of an affected nerve.

 Rapidly progressive glomerulonephritis and lung hemorrhage are the

additional features of the latter disease, neuropathy occurring somewhat less
frequently than in typical polyarteritis.
• (Lhote et al )
 Similar in clinical and neurologic manifestations to PAN.

 this disorder is characterized by pulmonary involvement, systemic small

vessel vasculitis, extravascular granulomas, and hypereosinophillia.

 It occurs in individuals with allergic rhinitis and asthma.

 The typical diagnostic pathology shows angitis and extravascular

granulomas with eosinophillic infiltrates.

 A prodromal period with rhinitis may last for years then followed by the
development of eosinophillia and later asthma which precedes the vasculitis.

 Peripheral nerve involvement is recognized in 65-to 75% of the patients

 In patients with the triad of vasculitis, granulomas of the respiratory tract,
and glomerulonephritis,

 The disease, however, can present without this obvious clinical picture.

 The most valuable serological test is the anti-neutrophil cytoplasmic

autoantibody (ANCA) which is present in over 90% of the active cases with
an specificity of 98% in the initial phases of the disease.This activity
decreases to 65% in inactive disease.

 The vasculitic neuropathy presents with mononeuritis often with cranial

nerve involvement.

 Granulomatous lesions can also produce single or multiple cranial palsies.

Cerebral infarction and hemorrhage can occur.
 Mononeuritis multiplex due to vasculitis in patients with
RheumatoidArthritis (RA) is a rare occurrence.

 Some 1 to 5 percent of patients with rheumatoid arthritis have vasculitic

involvement of one or more nerves at some time in the course of their
disease.

 The arteritis is of small-vessel fibrinoid type and immune globulins are

demonstrable in the walls of vessels.

 In addition to the neuropathy, such patients often have rheumatoid nodules,

skin vasculitis, weight loss, fever, a high titer of rheumatoid factor, and low
serum complement.
 Approximately 10 percent of patients with lupus will exhibit symptoms and
signs of peripheral nerve involvement.

 progressive sensorimotor paralysis, beginning in the feet and legs and

extending to the arms,

 In a few, weakness and areflexia were more prominent than the sensory
loss; the latter involved mainly vibratory and position senses.

 An elevation of CSF protein in some cases suggests nerve root involvement.

 Sural nerve biopsies may show vascular changes consisting of endothelial

thickening and mononuclear inflammatory infiltrates in and around the
small vessels for which reason the disease is included with the other
vasculitic neuropathies
 Axonal degeneration is the most common change, but a chronic
demyelinating pathology has also been described (Rechthand et al)

 Vascular injury from deposition of immune complexes is the proposed

mechanism of nerve damage.
 Hepatitis B and C have been associated with PAN.

 Immune complexes to viral proteins are detected in these patients.

 A mononeuritis multiplex, or even symmetrical neuropathy in patients with

abnormal liver tests should lead to the investigation of hepatitis B and C.

 Hepatitis can also be associated with a mixed cryoglobulinemia which can

produce both a symmetrical as well as a mononeuritic polyneuropathy.

 These patients have a PAN type of vasculitic neuropathy with systemic

 Involvement.
 Necrotizing vasculitis has been described in patients with HIV.

 The clinical syndromes include a distal symmetrical neuropathy,

mononeuritis multiplex and less commonly an inflammatory demyelinating
neuropathy.

 Distal neuropathy, the most common manifestation, is usually painful and

associated with weight loss and myalgia.

 The neuropathy affects .1 to 3% of the patients with AIDS

 These patients show definite, biopsy-proven vasculitis, without evidence of
any underlying diseases, central nervous system vasculitis or evidence of
vasculitis in any other part of their bodies.

 This syndrome has good prognosis after treatment, particularly if no

vasculitic involvement of other tissues appears during the year following
the diagnosis

 Diabetic Amyotrophy (Proximal Diabetic Neuropathy), diabetic

syndrome has been lately associated with an inflammatory angitis.