Treatment

Many treatment strategies for peripheral neuropathy are symptomatic. Some current research in
animal models has shown that neurotrophin-3 can oppose the demyelination present in some
peripheral neuropathies.[10]

A range of drugs that act on the central nervous system such as drugs originally intended as
antidepressants and antiepileptic drugs have been found to be useful in managing neuropathic
pain. Commonly used treatments include using a tricyclic antidepressant (such as amitriptyline)
and antiepileptic therapies such as gabapentin or sodium valproate. These have the advantage
that besides being effective in many cases they are relatively low cost.

A great deal of research has been done between 2005 and 2010 which indicates that synthetic
cannabinoids and inhaled cannabis are effective treatments for a range of neuropathic disorders.
[11]
Research has demonstrated that the synthetic oral cannabinoid Nabilone is an effective
adjunct treatment option for neuropathic conditions, especially for people who are resistant,
intolerant, or allergic to common medications.[12] Orally, opiate derivatives were found to be
more effective than cannabis for most people.[13] Smoked cannabis has been found to provide
relief from HIV-associated sensory neuropathy. [14] Smoked cannabis was also found to relieve
neuropathy associated with CRPS type I, spinal cord injury, peripheral neuropathy, and nerve
injury. [15]

Pregabalin (INN, pronounced /prɨˈɡæbəlɨn/) is an anticonvulsant drug used for neuropathic

pain. It has also been found effective for generalized anxiety disorder. It was designed as a more
potent successor to gabapentin but is significantly more expensive, especially now the patent on
gabapentin has expired and gabapentin is available as a generic drug. Pregabalin is marketed by
Pfizer under the trade name Lyrica.

TENS (Transcutaneous Electrical Nerve Stimulation) therapy may be effective and safe in the
treatment of diabetic peripheral neuropathy. A recent review of three trials involving 78 patients
found some improvement in pain scores after 4 and 6 but not 12 weeks of treatment, and an
overall improvement in neuropathic symptoms at 12 weeks.[16] A second review of four trials
found significant improvement in pain and overall symptoms, with 38% of patients in one trial
becoming asymptomatic. The treatment remains effective even after prolonged use, but
symptoms return to baseline within a month of treatment cessation.[17]

Guillain–Barré syndrome (GBS) (French pronunciation: [ɡiˈjɛ̃ baˈʁe];[1][2] in English, pronounced /

ˈɡiːlæn ˈbɑreɪ/,[3] /ɡiːˈlæn bəˈreɪ/,[4] etc.[5]) is an acute inflammatory demyelinating
polyneuropathy (AIDP), an autoimmune disorder affecting the peripheral nervous system,
usually triggered by an acute infectious process. The syndrome was named after the French
physicians Guillain, Barré and Strohl, who were the first to describe it in 1916. It is sometimes
called Landry's paralysis, after the French physician who first described a variant of it in 1859. It
is included in the wider group of peripheral neuropathies. There are several types of GBS, but
unless otherwise stated, GBS refers to the most common form, AIDP. GBS is rare and has an
incidence of 1 or 2 people per 100,000.[6] It is frequently severe and usually exhibits as an
ascending paralysis noted by weakness in the legs that spreads to the upper limbs and the face
along with complete loss of deep tendon reflexes. With prompt treatment by plasmapheresis or
intravenous immunoglobulins and supportive care, the majority of patients will regain full
functional capacity. However, death may occur if severe pulmonary complications and
autonomic nervous system problems are present.[7] Guillain-Barré is one of the leading causes of
non-trauma-induced paralysis in the world.[citation needed]

American actor Andy Griffith developed Guillain-Barré syndrome in 1983. Griffith is seen here receiving
an award at the U.S. White House in 2005

Contents
[hide]

 1 Classification
 2 Signs and symptoms
 3 Cause
o 3.1 Influenza vaccine
 4 Diagnosis
o 4.1 Diagnostic criteria
 4.1.1 Required
 4.1.2 Supportive
o 4.2 Differential diagnosis
 5 Management
 6 Prognosis
 7 Epidemiology
 8 History
 9 Notable sufferers
 10 References
  11 External links

[edit] Classification
Six different subtypes of Guillain–Barré syndrome (GBS) exist:

 Acute inflammatory demyelinating polyneuropathy (AIDP) is the most common form of GBS,
and the term is often used synonymously with GBS. It is caused by an auto-immune response
directed against Schwann cell membranes.
 Miller Fisher syndrome (MFS) is a rare variant of GBS and manifests as a descending paralysis,
proceeding in the reverse order of the more common form of GBS. It usually affects the eye
muscles first and presents with the triad of ophthalmoplegia, ataxia, and areflexia. Anti-GQ1b
antibodies are present in 90% of cases.
 Acute motor axonal neuropathy (AMAN),[8] aka Chinese Paralytic Syndrome, attacks motor
nodes of Ranvier and is prevalent in China and Mexico. It is probably due to an auto-immune
response directed against the axoplasm of peripheral nerves. The disease may be seasonal and
recovery can be rapid. Anti-GD1a antibodies[9] are present. Anti-GD3 antibodies are found more
frequently in AMAN.
 Acute motor sensory axonal neuropathy (AMSAN) is similar to AMAN but also affects sensory
nerves with severe axonal damage. Like AMAN, it is probably due to an auto-immune response
directed against the axoplasm of peripheral nerves. Recovery is slow and often incomplete. [10]
 Acute panautonomic neuropathy is the most rare variant of GBS, sometimes accompanied by
encephalopathy. It is associated with a high mortality rate, owing to cardiovascular involvement,
and associated dysrhythmias. Impaired sweating, lack of tear formation, photophobia, dryness
of nasal and oral mucosa, itching and peeling of skin, nausea, dysphagia, constipation unrelieved
by laxatives or alternating with diarrhea occur frequently in this patient group. Initial nonspecific
symptoms of lethargy, fatigue, headache, and decreased initiative are followed by autonomic
symptoms including orthostatic lightheadedness, blurring of vision, abdominal pain, diarrhea,
dryness of eyes, and disturbed micturition. The most common symptoms at onset are related to
orthostatic intolerance, as well as gastrointestinal and sudomotor dysfunction (Suarez et al.
1994). Parasympathetic impairment (abdominal pain, vomiting, obstipation, ileus, urinary
retention, dilated unreactive pupils, loss of accommodation) may also be observed.
 Bickerstaff’s brainstem encephalitis (BBE), is a further variant of Guillain–Barré syndrome. It is
characterized by acute onset of ophthalmoplegia, ataxia, disturbance of consciousness,
hyperreflexia or Babinski’s sign (Bickerstaff, 1957; Al-Din et al.,1982). The course of the disease
can be monophasic or remitting-relapsing. Large, irregular hyperintense lesions located mainly
in the brainstem, especially in the pons, midbrain and medulla are described in the literature.
BBE despite severe initial presentation usually has a good prognosis. Magnetic resonance
imaging (MRI) plays a critical role in the diagnosis of BBE.

A considerable number of BBE patients have associated axonal Guillain–Barré syndrome,

indicative that the two disorders are closely related and form a continuous spectrum.

[edit] Signs and symptoms

The disorder is characterized by symmetrical weakness which usually affects the lower limbs
first, and rapidly progresses in an ascending fashion. Patients generally notice weakness in their
legs, manifesting as "rubbery legs" or legs that tend to buckle, with or without dysesthesias
(numbness or tingling). As the weakness progresses upward, usually over periods of hours to
days, the arms and facial muscles also become affected. Frequently, the lower cranial nerves may
be affected, leading to bulbar weakness, oropharyngeal dysphagia (drooling, or difficulty
swallowing and/or maintaining an open airway) and respiratory difficulties. Most patients require
hospitalization and about 30% require ventilatory assistance.[11] Facial weakness is also
commonly a feature, but eye movement abnormalities are not commonly seen in ascending GBS,
but are a prominent feature in the Miller-Fisher variant (see below.) Sensory loss, if present,
usually takes the form of loss of proprioception (position sense) and areflexia (complete loss of
deep tendon reflexes), an important feature of GBS. Loss of pain and temperature sensation is
usually mild. In fact, pain is a common symptom in GBS, presenting as deep aching pain, usually
in the weakened muscles, which patients compare to the pain from overexercising. These pains
are self-limited and should be treated with standard analgesics. Bladder dysfunction may occur in
severe cases but should be transient. If severe, spinal cord disorder should be suspected.

Fever should not be present, and if it is, another cause should be suspected.

In severe cases of GBS, loss of autonomic function is common, manifesting as wide fluctuations
in blood pressure, orthostatic hypotension, and cardiac arrhythmias.

Acute paralysis in Guillain–Barré syndrome may be related to sodium channel blocking factor in
the cerebrospinal fluid (CSF). Significant issues involving intravenous salt and water
administration may occur unpredictably in this patient group, resulting in SIADH. SIADH is one
of the causes of hyponatremia and can be accompanied with various conditions such as
malignancies, infections and nervous system diseases. Symptoms of Guillain- Barre syndrome
such as general weakness, decreased consciousness, and seizure are similar to those of
hyponatremia

The symptoms of Guillain–Barré syndrome are also similar to those for progressive
inflammatory neuropathy.[12]

[edit] Cause
Structure of a typical neuron

Neuron
 Dendrite

Soma

Axon

Nucleus

Node of
Ranvier

Axon terminal

Schwann cell

Myelin sheath

All forms of Guillain–Barré syndrome are due to an immune response to foreign antigens (such
as infectious agents) that are mistargeted at host nerve tissues instead. The targets of such
immune attack are thought to be gangliosides, compounds naturally present in large quantities in
human nerve tissues. The most common antecedent infection is the bacterium Campylobacter
jejuni.[13][14] However, 60% of cases do not have a known cause; one study suggests that some
cases are triggered by the influenza virus, or by an immune reaction to the influenza virus.[15]

The end result of such autoimmune attack on the peripheral nerves is damage to the myelin, the
fatty insulating layer of the nerve, and a nerve conduction block, leading to a muscle paralysis
that may be accompanied by sensory or autonomic disturbances.

However, in mild cases, nerve axon (the long slender conducting portion of a nerve) function
remains intact and recovery can be rapid if remyelination occurs. In severe cases, axonal damage
occurs, and recovery depends on the regeneration of this important tissue. Recent studies on the
disorder have demonstrated that approximately 80% of the patients have myelin loss, whereas, in
the remaining 20%, the pathologic hallmark of the disorder is indeed axon loss.
Guillain-Barré, unlike disorders such as multiple sclerosis (MS) and Lou Gehrig's disease (ALS),
is a peripheral nerve disorder and does not generally cause nerve damage to the brain or spinal
cord.

[edit] Influenza vaccine

GBS may be a rare side-effect of influenza vaccines; a study of the Vaccine Adverse Event
Reporting System (VAERS) indicates that it is reported as an adverse event potentially
associated with the vaccine at a rate of an excess of 1 per million vaccines (over the normal risk).
[16]
There were reports of GBS affecting an excess of 10 per million who had received swine flu
immunizations in the 1976 U.S. outbreak of swine flu—25 of which resulted in death from
severe pulmonary complications, leading the government to end that immunization campaign.[17]
However, the role of the vaccine in these cases has remained unclear, partly because GBS had an
unknown but very low incidence rate in the general population making it difficult to assess
whether the vaccine was really increasing the risk for GBS. Later research has pointed to the
absence of or only a very small increase in the GBS risk due to the 1976 swine flu vaccine.[18]
Furthermore, the GBS may not have been directly due to the vaccine but to a bacterial
contamination of the vaccine.[19]

Since 1976, no other influenza vaccines have been linked to GBS, though as a precautionary
principle, caution is advised for certain individuals, particularly those with a history of GBS.[20][21]
On the other hand, getting infected by the flu increases the risk of developing GBS to a much
higher level (approx. 10 times higher by recent estimates[22]) and, all in all, the flu vaccination
contributes protection against the risk of GBS.[23]

From October 6 to November 24, 2009, the U.S. CDC, through the VAERS reporting system,
received ten reports of Guillain-Barre syndrome cases associated with the H1N1 vaccine and
identified two additional probable cases from VAERS reports (46.2 million doses were
distributed within the U.S. during this time). Only four cases, however, meet the Brighton
Collaboration Criteria for Guillain–Barré syndrome, while four do not meet the criteria and four
remain under review.[24] A preliminary report by the CDC's Emerging Infections Programs (EIP)
calculates the rate of GBS observed in patients who previously received the 2009 H1N1
influenza vaccination is an excess of 0.8 per million cases, which is on par with the rate seen
with the seasonal trivalent influenze vaccine. [25]

[edit] Diagnosis
The diagnosis of GBS usually depends on findings such as rapid development of muscle
paralysis, areflexia, absence of fever, and a likely inciting event. Cerebrospinal fluid analysis
(through a lumbar spinal puncture) and electrodiagnostic tests of nerves and muscles (such as
nerve conduction studies) are common tests ordered in the diagnosis of GBS.

 cerebrospinal fluid

Typical CSF findings include albumino-cytological dissociation. As opposed to infectious causes,

this is an elevated protein level (100–1000 mg/dL), without an accompanying increased cell
 count pleocytosis. A sustained increased white blood cell count may indicate an alternative
diagnosis such as infection.

 Electrodiagnostics

Electromyography (EMG) and nerve conduction study (NCS) may show prolonged distal
latencies, conduction slowing, conduction block, and temporal dispersion of compound action
potential in demyelinating cases. In primary axonal damage, the findings include reduced
amplitude of the action potentials without conduction slowing.

[edit] Diagnostic criteria

[edit] Required

 Progressive, relatively symmetrical weakness of two or more limbs due to neuropathy

 Areflexia
 Disorder course < 4 weeks
 Exclusion of other causes (see below)

[edit] Supportive

 relatively symmetric weakness accompanied by numbness and/or tingling

 mild sensory involvement
 facial nerve or other cranial nerve involvement
 absence of fever
 typical CSF findings obtained from lumbar puncture
 electrophysiologic evidence of demyelination from electromyogram

[edit] Differential diagnosis

 acute myelopathies with chronic back pain and sphincter dysfunction

 botulism with early loss of pupillary reactivity and descending paralysis
 diphtheria with early oropharyngeal dysfunction
 Lyme disease polyradiculitis and other tick-borne paralyses
 porphyria with abdominal pain, seizures, psychosis
 vasculitis neuropathy
 poliomyelitis with fever and meningeal signs
 CMV polyradiculitis in immunocompromised patients
 critical illness neuropathy
 myasthenia gravis
 poisonings with organophosphate, poison hemlock, thallium, or arsenic
 paresis caused by West Nile virus
 spinal astrocytoma
 Motor Neurone Disease
 West Nile virus can cause severe, potentially fatal neurological illnesses, which include
encephalitis, meningitis, Guillain-Barre syndrome, and anterior myelitis.
 Myalgic Encephalomyelitis/Chronic Fatigue Syndrome.
[edit] Management
Supportive care with monitoring of all vital functions is the cornerstone of successful
management in the acute patient. Of greatest concern is respiratory failure due to paralysis of the
diaphragm. Early intubation should be considered in any patient with a vital capacity (VC) <20
ml/kg, a negative inspiratory force (NIF) <-25 cmH2O, more than 30% decrease in either VC or
NIF within 24 hours, rapid progression of disorder, or autonomic instability.

Once the patient is stabilized, treatment of the underlying condition should be initiated as soon as
possible. Either high-dose intravenous immunoglobulins (IVIg) at 400 mg/kg for 5 days or
plasmapheresis can be administered,[26][27] as they are equally effective and a combination of the
two is not significantly better than either alone. Therapy is no longer effective two weeks after
the first motor symptoms appear, so treatment should be instituted as soon as possible. IVIg is
usually used first because of its ease of administration and safety profile, with a total of five daily
infusions for a total dose of 2 g/kg body weight (400 mg/kg each day). The use of intravenous
immunoglobulins is not without risk, occasionally causing hepatitis, or in rare cases, renal failure
if used for longer than five days. Glucocorticoids have not been found to be effective in GBS. If
plasmapheresis is chosen, a dose of 40-50 mL/kg plasma exchange (PE) can be administered
four times over a week.

Following the acute phase, the patient may also need rehabilitation to regain lost functions. This
treatment will focus on improving ADL (activities of daily living) functions such as brushing
teeth, washing, and getting dressed. Depending on the local structuring on health care, a team of
different therapists and nurses will be established according to patient needs. An occupational
therapist can offer equipment (such as wheelchair and special cutlery) to help the patient achieve
ADL independence. A physiotherapist would plan a progressive training program and guide the
patient to correct, functional movement, avoiding harmful compensations which might have a
negative effect in the long run. A speech and language therapist would be essential in the patient
regaining speaking and swallowing ability if they were intubated and received a tracheostomy.
The speech and language therapist would also offer advice to the medical team regarding the
swallowing abilities of the patient and would help the patient regain their communication ability
pre-dysarthria. There would also be a doctor, nurse and other team members involved, depending
on the needs of the patient. This team contribute their knowledge to guide the patient towards his
or her goals, and it is important that all goals set by the separate team members are relevant for
the patient's own priorities. After rehabilitation the patient should be able to function in his or her
own home and attend necessary training as needed.

[edit] Prognosis
Most of the time recovery starts after the fourth week from the onset of the disorder.
Approximately 80% of patients have a complete recovery within a few months to a year,
although minor findings may persist, such as areflexia. About 5–10% recover with severe
disability, with most of such cases involving severe proximal motor and sensory axonal damage
with inability of axonal regeneration. However, this is a grave disorder and despite all
improvements in treatment and supportive care, the death rate among patients with this disorder
is still about 2–3% even in the best intensive care units. Worldwide, the death rate runs slightly
higher (4%), mostly from a lack of availability of life support equipment during the lengthy
plateau lasting four to six weeks, and in some cases up to one year, when a ventilator is needed
in the worst cases. About 5–10% of patients have one or more late relapses, in which case they
are then classified as having chronic inflammatory demyelinating polyneuropathy (CIDP).

Poor prognostic factors include: 1) age >40 years, 2) history of preceding diarrheal illness, 3)
requiring ventilator support, 4) high anti-GM1 titre and 5) poor upper limb muscle strength.

Case reports do exist of rapid patient recovery.

[edit] Epidemiology
The incidence of GBS during pregnancy is 1.7 cases per 100,000 of the population.[28] The
mother will generally improve with treatment but death of the fetus is a risk. The risk of
Guillain–Barré syndrome increases after delivery, particularly during the first two weeks
postpartum. There is evidence of Campylobacter jejuni as an antecedent infection in
approximately 26% of disease cases, requiring special care in the preparation and handling of
food. Congenital and neonatal Guillain–Barré syndrome have also been reported.[29]

[edit] History
The disorder was first described by the French physician Jean Landry in 1859. In 1916, Georges
Guillain, Jean Alexandre Barré, and André Strohl diagnosed two soldiers with the illness and
discovered the key diagnostic abnormality of increased spinal fluid protein production, but
normal cell count.[30]

GBS is also known as acute inflammatory demyelinating polyneuropathy, acute idiopathic

polyradiculoneuritis, acute idiopathic polyneuritis, French Polio, Landry's ascending paralysis
and Landry Guillain Barré syndrome.

[edit] Notable sufferers

 Andy Griffith, American actor on Andy Griffith Show, and Matlock. He developed Guillain–Barré
in 1983.[31]
 Rachel Chagall, actress, contracted GBS in 1982. In 1987 she portrayed Gabriela Brimmer, a
notable disabilities activist.[32]
 Joseph Heller, author, contracted GBS in 1981. This episode in his life is recounted in the
autobiographical No Laughing Matter, which contains alternating chapters by Heller and his
good friend Speed Vogel.[33]
 Franklin D. Roosevelt, U.S. president. In 2003, a peer-reviewed study [34] found that it was more
likely that Roosevelt's paralysis—long attributed to poliomyelitis—was actually Guillain–Barré
syndrome.
 Markus Babbel, former international footballer, contracted GBS in 2001, following a period
suffering from the Epstein–Barr virus. He lost almost an entire year of his footballing career
 between the two illnesses and never again demonstrated the same level of ability that won him
over 50 caps for Germany.[35]
 Serge Payer, Canadian-born professional hockey player. After battling and overcoming the
syndrome, he set up the Serge Payer Foundation, which is dedicated to raising money for
research into new treatments and cures for Guillain–Barré syndrome. [36]
 Hans Vonk, Dutch conductor.[37]
 Lucky Oceans, Grammy Award winning musician with Asleep at the Wheel was diagnosed with
GBS in 2008.[38]
 William “The Refrigerator” Perry , former professional American football player with the Chicago
Bears was diagnosed with GBS in 2008.[39]
 Tony Benn, British politician.[40]
 Len Pasquarelli, sports writer and analyst for ESPN and resident of the Pro Football Writers of
America, diagnosed in 2008.[41]
 Hiroko Mima, Miss Universe Japan 2008, was diagnosed with GBS at the age of 13. [42]
 Norton Simon[43]
 Hugh McElhenny, former Hall-of-Fame, professional American football player with the San
Francisco 49ers.[44]
 Luci Baines Johnson, daughter of President Lyndon Johnson and Lady Bird Johnson. Diagnosed
and under treatment for Guillain–Barré in April 2010. [45]
 Zeituni Onyango, paternal aunt of U.S. President Barack Obama.[46]

[edit] References
Polyarteritis nodosa (also known as "Panarteritis nodosa,"[1] and "Periarteritis nodosa"[1]) is a
vasculitis of medium-sized arteries, which become swollen and damaged from attack by rogue
immune cells. Polyarteritis nodosa is also called Kussmaul disease or Kussmaul-Maier
disease.[2] Infantile polyarteritis nodosa is a type of PAN restricted to infants.[3]

Contents
[hide]

 1 Causes and risk factors

 2 Incidence
 3 Symptoms
 4 Diagnosis
 5 Treatment and Prognosis
 6 Complications
 7 Prevention
 8 See also
 9 References
 10 External links

[edit] Causes and risk factors

Polyarteritis nodosa is a disease of unknown cause that affects arteries, the blood vessels that
carry blood from the heart to organs and tissues. It occurs when certain immune cells attack the
affected arteries. It is caused by immune complex deposition in the walls of blood vessels, which
makes it a type III hypersensitivity reaction.

Incidence
The condition affects adults more frequently than children and males more frequently than
females. It damages the tissues supplied by the affected arteries because they don't receive
enough oxygen and nourishment without a proper blood supply.

Polyarteritis nodosa is more common in people with hepatitis B infection.[4]

[edit] Symptoms
In this disease, symptoms result from ischaemic damage to affected organs, often the skin, heart,
kidneys, and nervous system.

Generalised symptoms include fever, fatigue, weakness, loss of appetite, and weight loss. Muscle
and joint aches are common. The skin may show rashes, swelling, ulcers, and lumps.[citation needed]

Nerve involvement may cause sensory changes with numbness, pain, burning, and weakness.
Central nervous system involvement may cause strokes or seizures. Kidney involvement can
produce varying degrees of renal failure.[citation needed]

Involvement of the arteries of the heart may cause a heart attack, heart failure, and inflammation
of the sac around the heart (pericarditis).

 Fatigue
 Weakness
 Fever
 Abdominal pain
 Decreased appetite
 Unintentional weight loss
 Muscle aches
 Joint aches[citation needed]

[edit] Diagnosis
There are no specific lab tests for diagnosing polyarteritis nodosa. Diagnosis is generally based
upon the physical examination and a few laboratory studies that help to confirm the diagnosis:

 CBC (may demonstrate an elevated white blood count)

 ESR (elevated)
  Perinuclear pattern of antineutrophil cytoplasmic antibodies (p-ANCA) - not
associated with "classic" polyarteritis nodosa, but is present in a form of the
disease affecting smaller blood vessels, known as microscopic polyangiitis or
leukocytoclastic angiitis.
 Tissue biopsy (reveals inflammation in small arteries, called arteritis)
 Elevated C-reactive protein

A patient is said to have polyarteritis nodosa if he or she has 3 of the 10 following signs known
as the 1990 ACR (American College of Rheumatology)[5] criteria:

 Weight loss greater than/equal to 4.5 kg.

 Livedo reticularis (a mottled purplish skin discoloration over the extremities or
torso).
 Testicular pain or tenderness. (occasionally, a site biopsied for diagnosis).
 Muscle pain, weakness, or leg tenderness.
 Nerve disease (either single or multiple).
 Diastolic blood pressure greater than 90mmHg (high blood pressure).
 Elevated kidney blood tests (BUN greater than 40 mg/dl or creatinine greater than
1.5 mg/dl).
 Hepatitis B virus tests positive (for surface antigen or antibody).
 Arteriogram (angiogram) showing the arteries that are dilated (aneurysms) or
constricted by the blood vessel inflammation.
 Biopsy of tissue showing the arteritis (typically inflamed arteries).[6]

It should be underlined that the 1990 ACR criteria were designed for classification purposes
only. Nevertheless their good discriminatory performances, indicated by the initial ACR
analysis, suggested their potential usefulness for diagnostic purposes also. Subsequent studies
did not confirm their diagnostic utility, demonstrating a significant dependence of their
discriminant abilities on the prevalence of the various vasculitides in the analyzed populations.
Recently an original study, combining the analysis of more than 100 items used to describe
patients characteristics in a large sample of vasculitides with a computer simulation technique
designed to test the potential diagnostic utility of the various criteria, proposed a set of eight
positively or negatively PAN discriminating items to be used a screening tool for PAN diagnosis
in patients suspected of systemic vasculitis.[7]

[edit] Treatment and Prognosis

Treatment involves medications to suppress the immune system, including prednisone and
cyclophosphamide. Therapy results in remissions or cures in 90% of cases. Untreated, the
disease is fatal in most cases. The most serious associated conditions generally involve the
kidneys and gastrointestinal tract.A fatal course usually involves gastrointestinal hemorrhage,
infection, myocardial infarction and/or renal failure.[8]

[edit] Complications
  Stroke[citation needed]
 Kidney failure[citation needed]
 Heart attack[citation needed]
 Intestinal necrosis and perforation[citation needed]

[edit] Prevention
This disease cannot be currently prevented, but early treatment can prevent some damage and
symptoms.[citation needed]

[edit] See also

 List of cutaneous conditions

Churg–Strauss syndrome (also known as "Allergic granulomatosis"[1]) is a medium and small

vessel autoimmune vasculitis, leading to necrosis. It involves mainly the blood vessels of the
lungs (it begins as a severe type of asthma), gastrointestinal system, and peripheral nerves, but
also affects the heart, skin and kidneys. It is a rare disease that is non-inheritable, non-
transmissible. Churg-Strauss syndrome was once considered a type of polyarteritis nodosa due to
their similar morphologies.

The syndrome was first described by Drs. Jacob Churg and Lotte Strauss at Mount Sinai Hospital
in New York City in 1951.[2][3]

Contents
[hide]

 1 Diagnosis
 2 Disease stages
 3 Risk stratification
 4 Treatment
 5 Montelukast
 6 Famous patients
 7 References
 8 External links

[edit] Diagnosis
Diagnostic markers include eosinophil granulocytes and granulomas in affected tissue and anti-
neutrophil cytoplasmic antibodies (ANCA) against neutrophil granulocytes. Differentiation from
Wegener's granulomatosis can be difficult, though the increasing use of ANCA assays has made
the distinction more routine. Wegener's is closely associated with c-ANCA, unlike Churg-
Strauss, which shows elevations of p-ANCA.

[edit] Disease stages

This disease has three distinct stages.

 The first stage often involves the sinuses and the onset of allergies not previously had or
the worsening of pre-existing allergies.
 The second stage involves the onset of acute asthma. Normally, the person would not
have had asthma previously.
 The third and final stage involves the various organ systems. Stage three is by far the
most life threatening and painful. Often the person will develop severe nerve pain in their
legs, arms and hands. Purple marks will appear on the skin and often sores will appear in
the mouth or nose. The disease can affect the heart, lungs, kidneys and liver.

People can live for many years in the first two stages before progressing to stage three.

[edit] Risk stratification

The French Vasculitis Study Group has developed a five-point score ("five-factor score" or FFS)
that predicts the risk of death in Churg-Strauss syndrome. These are (1) reduced renal function
(creatinine >1.58 mg/dL or 140 μmol/l), (2) proteinuria (>1 g/24h), (3) gastrointestinal
hemorrhage, infarction or pancreatitis, (4) involvement of the central nervous system or (5)
cardiomyopathy. Presence of 1 of these indicates severe disease (5-year mortality 26%) and 2 or
more very severe disease (mortality 46%), while absence of any of these 5 indicates a milder
case (mortality 11.9%).[4]

[edit] Treatment
Treatment for Churg-Strauss syndrome includes glucocorticoids such as prednisolone and other
immunosuppressive drugs such as azathioprine and cyclophosphamide. In many cases the
disease can be put into a type of chemical remission through drug therapy, but the disease is
chronic and lifelong.

A systematic review conducted in 2007 indicated that all patients should be treated with high-
dose steroids, but that in patients with an FFS of 1 or higher cyclophosphamide pulse therapy
should be commenced, with 12 pulses leading to fewer relapses than 6. Remission can be
maintained with a less toxic drug, such as azathioprine or methotrexate.[5]

[edit] Montelukast
A case study in 2000 noticed a "marked and severe inflammatory reaction characterized by
eosinophilia and glomerulonephritis" and wondered whether this "might have been triggered by
the leukotriene receptor antagonist, montelukast."[6] Researchers have searched for links between
drugs such as montelukast (Singulair)[7] and Churg-Strauss syndrome; in another study in 2000,
researchers did not find a cause-and-effect relationship and wrote: "The occurrence of Churg-
Strauss syndrome in asthmatic patients receiving leukotriene modifiers appears to be related to
unmasking of an underlying vasculitic syndrome that is initially clinically recognized as
moderate to severe asthma and treated with corticosteroids. Montelukast does not appear to
directly cause the syndrome in these patients."[8] A case study in 2006 was inconclusive but
suggested the need for further research.[9]

[edit] Famous patients

The memoir Patient, by the musician Ben Watt, deals with Watt's mid-1990s experience with
Churg-Strauss syndrome, and his recovery. Watt's case was unusual in that it mainly affected his
gastrointestinal tract, leaving his lungs largely unaffected; this unusual presentation contributed
to a delay in proper diagnosis. His treatment required the removal of large sections of necrotized
intestine, leaving Watt on a permanently restricted diet.

Umaru Musa Yar'Adua, the president of Nigeria from 2007-2010, reportedly suffered from
Churg-Strauss syndrome and died in office of complications of the disease.[10]

The DJ and author Charlie Gillett was diagnosed with Churg-Strauss in 2006; he died four years
later.