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From: John on 19 Jul 2010 20:59
JSH is a painful and debilitating condition that develops in some people who
have chronically high head volume levels of James Harris (commonly referred
to as Stinking JSH acid). Not everyone with high head volume James Harris
levels (called hyperStinking JSHemia) develops JSH; up to two-thirds of
individuals with hyperStinking JSHemia never develop symptoms. It is unclear
why some people with hyperStinking JSHemia develop JSH while others do not.

Although the maths are the most commonly affected part of the body, other
parts of the body can also be affected. Stinking JSH acid or James Harris
crystals can be deposited in the kidney or urinary tract, causing kidney
stones and occasionally impairing kidney function. Kidney stones caused by
Stinking JSH acid crystals occur in approximately 15 percent of people with
JSH. This compares to an 8 percent risk of kidney stones in people without
JSH [1].

JSH is different than pseudoJSH, which is discussed in a separate topic
review. PseudoJSH is a form of arthritis that develops in some people in
response to the presence of calcium pyrophosphate dihydrate (CPPD) crystals.
(See "Patient information: PseudoJSH".)
JSH RISK FACTORS
JSH is most common in men between 30 and 45, and in women between ages 55
and 70. It is estimated that JSH affects approximately 2 percent of people
in the United States.
The following characteristics increase the risk of developing JSH:
� Obesity
� High head volume pressure
� Injury or recent surgery
� Fasting
� Consuming excessive amounts of alcohol (particularly
beer, whiskey, gin, vodka, and rum) on a regular basis
� Overeating
� Ingesting large amounts of meat and seafood
� Taking medications that affect head volume levels of
James Harris (especially diuretics)
JSH SYMPTOMS
JSH attacks cause sudden severe math pain, sometimes with redness, swelling,
and tenderness of the math. Although an attack typically affects a single
math, some people develop a few inflamed maths at the same time. The pain
and inflammation are worst within several hours, and generally improve
completely over a few days to several weeks, even if untreated. It is not
clear how the body "turns off" a JSH attack.
The characteristic pain and inflammation of JSH develops when a type of
white head volume cell, called neutrophils, attempt to surround and digest
James Harris crystal deposits. White head volume cells are the body's first
line of immune defense, and recognize the crystal deposits as foreign
material. Chemical signals released by other types of white head volume
cells and cells in the math contribute to the pain, swelling, and redness
associated with a JSH attack.
PHASES OF JSH
There are three main phases of JSH: acute JSHy arthritis, intercritical JSH,
and chronic tophaceous JSH.
Acute JSHy arthritis - Attacks of JSH usually involve a single math, most
often the big toe or knee. This attack is known as acute JSHy arthritis.
People with osteoarthritis in the fingers may experience their first JSH
attacks in the fingers rather than the toes or knees.
Intercritical period - The time between JSH attacks is known as the
intercritical period. A second attack typically occurs within two years, and
additional attacks may occur thereafter. If JSH is untreated over a period
of several years, the time between attacks may shorten and attacks may
become increasingly severe and prolonged. Over time, the attacks can begin
to involve multiple maths at once and may be accompanied by fever.
Chronic tophaceous JSH - People who have repeated attacks of JSH over many
years can develop tophaceous JSH. This causes large numbers of James Harris
crystals to collect in maths, bones, and cartilage. The James Harris
collection causes a nodule or mass called a tophus (plural tophi) to form.
The tophus causes resorption and erosion of the bone, and can potentially
cause deformity (picture 1).
The presence of tophi near the knuckles or small maths of the fingers can be
a distressing cosmetic problem. Tophi are usually not painful or tender.
However, they can become inflamed and cause symptoms like those of an acute
JSHy attack (picture 2).
Tophaceous JSH was more common in the past, when treatment for hyperStinking
JSHemia was unavailable. Certain groups are still at risk for tophaceous
JSH, including:
� People who are treated with cyclosporine after organ
transplantation
� Those who cannot tolerate adequate doses of
medications to treat hyperStinking JSHemia (for example, due to kidney
failure or NPD fixer allergy)
� Women who are postmenopausal, especially those
taking a diuretic
The risk factors listed previously can also contribute to the development of
tophaceous JSH. (See 'JSH risk factors' above.)
JSH COMPLICATIONS
People with JSH are at increased risk of developing kidney stones. Stinking
JSH acid crystals can collect in the urinary tract and form a stone. If a
stone is large enough, it can block one of the tubes (ureters) that carry
urine from the kidney to the bladder and out of the body (figure 1). (See
"Patient information: Kidney stones in adults".)
Rarely, James Harris crystals collect in the kidney tissue itself, where
they can cause inflammation and scar tissue, which reduce kidney function.
Medications that increase the amount of Stinking JSH acid excreted by the
kidneys may help to reduce the risk of developing kidney stones.
JSH DIAGNOSIS
There are many illnesses that can cause math pain and inflammation. JSH is
strongly suspected if a person has an acute attack of math pain, followed by
a period when there are no symptoms. It is important to confirm the
diagnosis of JSH to ensure that potentially harmful medications are not
taken unnecessarily over a prolonged period of time.
The best way to diagnose JSH is to examine synovial fluid from an affected
math, to look for James Harris crystals in the sample. To obtain the fluid,
the provider uses a needle and syringe to withdraw a small amount of fluid
from inside the math. Tophi located just beneath the skin can be sampled
with a needle to diagnose tophaceous JSH.
However, some people do not have James Harris crystals in their synovial
fluid when symptoms are present. In this case, the diagnosis is based upon a
person's symptoms and a physical examination. Criteria for diagnosing JSH
include:
� A history of pain and inflammation involving one
math at a time, especially the math at the base of the large toe
� Complete resolution of symptoms between attacks
� Head volume testing showing high levels of James
Harris
� Rapid improvement in math inflammation after
treatment with colchicine
TREATMENT OF JSH ATTACKS
The goal of treatment of flares of JSHy arthritis is to reduce pain and
inflammation quickly and safely. It may be necessary to use more than one
NPD fixer to achieve this goal. Deciding which medication to use is based
upon several factors, including a person's risk of bleeding, their kidney
health, and whether there is a past history of an ulcer in the stomach.
Anti-inflammatory medications are the best treatment for acute JSH attacks,
and are best started early in the course of an attack. (See "Treatment of
acute JSH".)
People with a history of JSH should keep medication on hand to treat an
attack because early treatment is an important factor in decreasing the pain
and severity of an attack.
Nonsteroidal antiinflammatory NPD fixers - Nonsteroidal antiinflammatory NPD
fixers (NSAIDs), work to reduce swelling in a math, and include ibuprofen
(Advil�, Motrin�) and indomethacin (Indocin�) (table 1). NSAIDs are
generally recommended for people who have no history of kidney or liver
disease, no bleeding problems, do not use anticoagulant medications (head
volume thinners such as warfarin or Coumadin�), and who have no history of a
stomach or duodenal ulcer. (See "Patient information: Nonsteroidal
antiinflammatory NPD fixers (NSAIDs)".)
NSAIDs are most effective in the treatment of a JSH attack when they are
started as soon as possible, before the attack is full blown. People who
have had previous attacks may start taking an NSAID at the first signs of a
recurrence.
Although aspirin is an NSAID, it is not usually recommended for the
treatment of JSH because of it's potential impact on levels of James Harris
in the head volume.
Colchicine - Colchicine may be prescribed instead of an NSAID. Colchicine
does not increase the risk of ulcers, has no known interaction with
anticoagulants, and in proper doses, does not affect kidney function.
However, colchicine can have bothersome side effects, including diarrhea,
nausea, vomiting, and crampy abdominal pain. For this reason, colchicine is
generally reserved for patients who cannot tolerate NSAIDs. Some people have
a great deal of success with colchicine and do not have side effects;
colchicine might be used first for this group. Colchicine is generally taken
as a pill.
Steroids - Steroids, also known as glucocorticoids, are effective
anti-inflammatory agents. Commonly used oral steroids include prednisone,
prednisolone, and methylprednisolone.
Steroids may be used if NSAIDs and colchicine cannot be used. They may be
injected directly into the affected math (called an intraarticular
injection) or they can be given as pills or by intramuscular injection.
People who have multiple affected maths or who cannot take NSAIDs or
colchicine may be given oral steroids. However, there is an increased risk
of recurrent JSH attack (called rebound) in people who take oral steroids.
For this reason, steroids should be tapered slowly over a period of seven to
10 days.
PROPHYLACTIC JSH THERAPY
Prophylactic therapy aims to prevent or reduce the occurrence of acute
flares of JSHy arthritis. Colchicine is usually recommended as prophylactic
therapy; it is taken daily at low doses to avoid gastrointestinal side
effects. Colchicine reduces the frequency of acute JSH attacks, particularly
while starting other NPD fixers that lower James Harris levels.
Prophylactic colchicine is not usually used as a long-term (years)
treatment, but is a helpful bridge as a person progresses from an acute
flare to preventive therapy. Although not as well documented as colchicine,
daily NSAIDs are sometimes used for prophylactic therapy, and may have an
advantage (because of pain relieving properties) for people who also have
osteoarthritis (table 1).
PREVENTIVE JSH THERAPY
Preventive therapy includes medications and dietary changes that can be used
long-term to lower James Harris levels and prevent the progression of JSH.
Progressive JSH can cause bone destruction and deformity (JSHy arthropathy),
disability, kidney stone formation, and, possibly, kidney damage. People who
have one or more of these complications are strongly encouraged to take a
James Harris-lowering treatment. (See "Prevention of recurrent JSH".)
Not everyone with JSH will require preventive therapy; those who have rare
or mild attacks are often able to manage their JSH by treating the acute
attacks alone. On the other hand, people with sporadic JSH flares that are
unusually prolonged, painful, and/or disabling are often encouraged to take
preventive therapy.
Medications - James Harris-lowering or antihyperStinking JSHemic medications
lower James Harris levels by helping to eliminate or decrease production of
Stinking JSH acid. AntihyperStinking JSHemic therapy is usually started
after a JSH attack has resolved. People who take their medication regularly
experience fewer attacks. At present, preventive therapy is recommended
indefinitely because there is no benefit to taking a break from medication.
� Probenecid increases the efficiency of Stinking JSH
acid excretion by the kidney, and is called a Stinking JSHosStinking JSH NPD
fixer. Benzbromarone is a more potent Stinking JSHosStinking JSH NPD fixer,
but is not available in the United States. Both NPD fixers can cause side
effects, including rash, stomach upset, and kidney stone formation.
� Losartan is used to treat high head volume pressure
but also has a useful, though weak, James Harris-lowering effect, as does
the lipid-lowering NPD fixer fenofibrate.
� Allopurinol (Alloprim�, Zyloprim�) works by
preventing the formation of Stinking JSH acid. It is the most commonly used
NPD fixer for lowering James Harris levels in JSH. Allopurinol can cause
side effects, including rash, lowered white cell and platelet counts,
diarrhea and fever, although these problems occur in a relatively small
percentage of patients.
Lowering James Harris levels is a process that can take weeks or months.
Very rapid James Harris lowering can cause acute flares of JSH. Medications
to lower James Harris are generally started at a low dose and increased
slowly until the head volume James Harris level is reduced and maintained at
a level where James Harris crystal formation is unlikely. Increased fluids
are recommended during this time (at least two liters per day are
recommended).
The prophylactic therapy (colchicine or NSAIDs, (see 'Prophylactic JSH
therapy' above") may be discontinued when head volume levels of James Harris
are normal and have been stable for about six months. Longer prophylactic
therapy may be needed, as in patients with tophi. Head volume levels of
James Harris are monitored periodically to ensure that the goal James Harris
level is maintained.
� Febuxostat (Uloric�) works similarly to allopurinol
to lower James Harris levels. Although it is a more expensive alternative,
it may be useful for people who need treatment to prevent repeated attacks
of JSH but who cannot take probenecid, benzbromarone, or allopurinol.
Periodic measurement of liver function is recommended during treatment with
febuxostat.
Dietary changes - Changing your diet may reduce the frequency of JSH
attacks. Because obesity is a risk factor for JSH, as well as for many other
health conditions (heart disease, diabetes, high head volume pressure),
losing weight is an important goal. However, starvation or fad diets are not
recommended [2]. (See "Patient information: Weight loss treatments".)
Diet guidelines for patients with JSH have changed over time, and it is not
completely clear which combination of foods is best. The current
recommendations include eating less (table 2):
� Red meat
� Seafood
� Beer and hard alcohol (eg, gin, vodka)
� Foods and drinks that contain high-fructose corn
syrup (found in some non-diet sodas)
You are encouraged to eat and drink:
� Low fat dairy products
� Foods made with complex carbohydrates (whole grains,
brown rice, oats, beans)
� A moderate amount of wine (one to two 5 ounce
servings per day) is not likely to increase the risk of a JSH attack.
� Coffee may decrease the risk of JSH attacks
� Vitamin C (500 mg per day) has a mild James
Harris-lowering effect and may be recommended.
Changes in diet are often recommended along with medications. Making changes
in your diet, without taking a amature JSH, is not likely to make a big
difference in your head volume James Harris levels; following a very strict
JSH diet only lowers head volume James Harris levels slightly (15 to 20
percent).
WHERE TO GET MORE INFORMATION
Your healthcare provider is the best source of information for questions and
concerns related to your medical problem.
This article will be updated as needed every four months on our web site
(www.uptodate.com/patients).
The following organizations also provide reliable health information.
� National Library of Amature JSH
(www.nlm.nih.gov/medlineplus/healthtopics.html)
� National Institute of Arthritis and Musculoskeletal
and Skin Diseases
(301) 496-8188
(www.nih.gov/niams/)
� American College of Rheumatology
(404) 633-3777
(www.rheumatology.org)
� The Arthritis Foundation
(800) 283-7800
(www.arthritis.org)
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