Common Secondary Conditions in Lupus

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Common Secondary Conditions

in Lupus

This is an over-view of some of the

common conditions, which often accompany Systemic Lupus, but is by no means

intended to be a complete listing of all such conditions. As SLE produces the

greatest number and widest variety of symptoms found in any disease that type

of listing would be near impossible to accomplish.

Fibromyalgia Syndrome

Sjogren's Syndrome

Raynaud's Phenomenon

C.R.E.S.T.

Phenomenon

Systemic

Vasculitis

Central

Nervous System (CNS) Vasculitis

Menstrual

Problems and Menopause

Conditions

of the Eye

Cognitive

Dysfunction (Brain Fog)

Lupus

Headache

Seizures

Psychosis

Lupus

Myositis

Fibromyalgia Syndrome

Fibromyalgia Syndrome,

also called simply Fibromyalgia, FM, or FMS is a

condition, but not a disease. FM afflicts some 6 million Americans, 90% of whom are women. About 20% of all lupus

patients also have FM, and the two are sometimes confused. It has been

estimated that at least 10% of patients diagnosed with Systemic Lupus actually

have only Fibromyalgia. While FM can be extremely

painful with debilitating fatigue, it is not life-threatening

nor crippling, and does not damage organs.

There are 18 designated tender points in all four

quadrants of the body (right side, left side, above the waist, below the

waist), and a person must show pronounced tenderness in at least eleven of

these eighteen points to be diagnosed with FM. If a patient has tender points

in less than the four quadrants, they have a regional myofascial

syndrome.

The cause of FM is unknown, but it is triggered by

trauma, viral infection, an inflammatory disorder, or can happen as a reaction

to physical or emotional stress. The most common symptoms of Fibromyalgia are joint and muscle pain throughout the body,

especially in the jaw, neck, and shoulders; painful muscle cramps or spasms;

muscle weakness; sleep disturbances or non-restorative sleep (waking up after 8

hours of sleep and not feeling refreshed); functional bowel symptoms such as

abdominal cramping, bloating, and swelling; numbness, tingling, and burning

sensations in the limbs and extremities; profound fatigue; headaches; and

cognitive dysfunctions, such as memory loss, difficulty concentrating, and

anxiety.

About 10% of FM patients have a positive ANA. Since these

symptoms are also common to Systemic Lupus, many physicians make the mistake of

taking the symptoms at face value, especially if a positive ANA is present, and

diagnosing the patient with SLE without further laboratory findings to validate

the diagnosis. While these symptoms in lupus patients are responsive to

steroids and narcotic pain relievers, the same drugs cause these symptoms in Fibromyalgia to worsen. This is particularly a problem to

patients who do actually have both, SLE and FM.

Fibromyalgia is best treated with NSAIDs (Non-Steroid Anti- Inflammatory Drugs) and muscle

relaxants for pain; various types of medications that promote restful sleep;

mild, non-strenuous exercises, and deep-muscle massage therapy. FM is a chronic

process that worsens and lessens, but most patients respond to treatments.

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Sjogren's Syndrome

Sjogren's Syndrome,

named after the man who discovered it, Henrik Sjogren, (pronounced show-gren)

is usually thought of as the " dry-eye " syndrome. Those individuals,

whose mouth and eyes are exclusively affected, are said to have " Sicca Syndrome, " a milder presentation of Sjogren's.

However, Sjogren's can be much more inclusive than

this. Mucous membranes throughout the body, such as eyes, ears, nose, mouth, salvitory glands, larynx, lungs, stomach, and vagina can

all become extremely dry, along with the skin, hair, and nails.

Sjogren's Syndrome

can cause blurred vision, ringing in the ears, crops of mouth sores, constant

thirst, and bouts of laryngitis. Sjogren's can be a

secondary condition to other autoimmune diseases such as SLE, or it can exist

by itself.

Possibly as many as one-third of lupus patients have

secondary Sjogren's Syndrome.

It should be noted that most people do not experience all the above listed

symptoms, but any combination of these.

Primary Sjogren's is, in

itself, an autoimmune disease that can affect joints and damage organs. In

severe cases, loss of vision can occur due to cornea damage, dry lungs can lead

to pneumonia and lung disease, severe gastritis can result from dry stomach, pericarditis can result from inflammation in the sac

surrounding the heart, and nerves may be damaged, particularly in the face. The

liver, pancreas, spleen, kidneys, and lymph nodes may all be affected.

Sjogren's Syndrome

is the only autoimmune disease that has a potential for malignant

transformation. Up to 5% of Sjogren's patients

develop a blood disorder, particularly lymphoma. It is easily confused with

SLE, as the signs and symptoms are shared by both conditions. Sjogren's patients often have a positive ANA and rheumatoid

factor.

Sjogren's is usually a benign process and is

treated symptomatically. The problems associated with Sjogren's

are highly responsive to medications and can usually be managed quite

successfully.

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Raynaud's Phenomenon

Primary Raynaud's, which occurs independently from

an autoimmune disorder, is called Raynaud's Disease. When it occurs as a

secondary condition to an underlying disease, it is known as Raynaud's Phenomenon.

However, the symptoms are identical in primary and secondary. Between 60 and

90% of those with Raynaud's are young women.

The condition is the result of small arteries, usually in

the fingers and toes, going into spasms, causing the skin to become pale or a

patchy red to blue. These spasms come on quickly and may last minutes to hours.

Anything that stimulates the sympathetic nervous system, such as exposure to

cold or intense emotion, can cause arterial spasms. Vascular migraine headaches

are also associated with Raynaud's.

Symptoms include the affected area turning a patchy red

and white, usually in a spotted fashion. During an episode, the individual

usually experiences numbness, tingling, pins and needles, and a burning

sensation.

When the episode ends, the affected skin may be pinker

than usual or bluish. If exposure to cold triggered the symptoms, warming the

hands or feet restores normal color and sensation. Sores sometimes develop on

the affected areas and in severe cases, can cause peeling of the skin.

There are specific drugs used to treat Raynaud's disease, as well as using

preventative measures, such as protecting their trunk, arms, and legs from cold

and by taking mild sedatives. In the case of Raynaud's Phenomenon,

the symptoms improve with the treatment of the underlying disorder.

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C.R.E.S.T. Phenomenon

As you can see, the term CREST is an

acronym. It is a syndrome made up of five different disorders presenting

together. CREST Syndrome is a less severe form of an autoimmune disease called,

Scleroderma.

CREST is named for its symptoms:

· C - Calcium deposits

throughout the body;

· R - Raynaud's Phenomenon;

· E - Esophageal dysfunction;

· S - Sclerodactyly

(skin damage on the fingers);

· T - Telangiectasia

(spider veins).

Although this syndrome is usually less severe than Scleroderma, CREST can cause calcium bumps to form on bony

areas or at the joints; severe acid reflux, heartburn, and difficulty

swallowing; intestinal infections; and severe muscle and joint pain and

weakness. It can also lead to high blood pressure, causing heart and

respiratory failure. CREST is treated symptomatically.

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Systemic Vasculitis

Systemic (meaning throughout various

body systems) Vasculitis (meaning inflammation of

blood vessels), is not a disease, but rather a disease process that occurs in a

number of autoimmune, connective tissue diseases. Vasculitis

can also occur without connective tissue involvement.

Lupus is the result of the body's immune system producing

too many antibodies, and these turn against the body, attacking healthy cells

and resulting in inflammation. This same process occurs in vasculitis.

The auto-antibodies attack blood vessels, causing inflammation that damages the

vessels and the tissues they supply.

The blood vessels can become either leaky or clogged, and

both conditions disrupt blood flow to nerves, organs, and other parts of the

body. These affected areas can be damaged permanently.

Symptoms may result directly from the damaged blood

vessels or from the damaged tissues or nerves whose blood supply is restricted.

Any vessels can be affected, and symptoms depend on whether small or medium

veins or large arteries are affected. It has been suggested that a large number

of Lupus symptoms result from vasculitis.

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Central Nervous System (CNS) Vasculitis

Vasculitis of the CNS is an inflammation of

the brain's blood vessels due to lupus activity. The most serious of the CNS

syndromes associated with lupus, it was the first to be described and is one of

only two CNS syndromes (the other being psychosis) that fulfill the ACR

criteria for defining lupus. Vasculitis of the

CNS usually occurs early in the disease course; over 80% of episodes take place

within 5 years of diagnosis.

The typical patient experiences high fevers, seizures,

meningitis-like stiffness of the neck, and may manifest psychotic or bizarre

behavior. 10% of lupus patients develop CNS vasculitis.

Untreated, their course rapidly deteriorates into stupor and ultimately coma.

CNS vasculitis is diagnosed

either by a conventional angiogram, a magnetic resonance angiogram, lumbar

puncture, or tests to detect high levels of antineuronal

antibodies in the serum. It is treated with high doses of corticosteroids, or cytotoxic drugs, & can be treated successfully.

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Menstrual Problems and

Menopause

Scientists are not certain what role

hormones play in Systemic Lupus, but there does seem to be an obvious

correlation. Thus, the menstrual cycle can be affected in some women with SLE.

Those taking birth control pills (made up of the female hormone, estrogen), sometimes experience a decrease in disease activity, as do

some women who have passed menopause. Lupus is much less common in men, yet

males with lupus tend to experience more serious complications of the disease.

About 15% of women with SLE develop menorrhagia

(excessive blood flow during menstrual periods) which may be due to clotting

problems. Some lupus patients may also experience amenorrhea (lack of menstrual

periods). Early menopause has also been noted in women with lupus.

It has been estimated that the symptoms of menopause

(mood swings, hot flashes, night sweats, irregular and/or unusual periods)

actually begin some 3 to 5 years before the woman is pronounced as being in the

process of menopause. Each of these conditions can be a direct result of

Systemic Lupus, or a side effect of steroid therapy.

All lupus pregnancies should be considered high-risk and

closely monitored, yet there is no reason for a lupus patient to avoid

conception, unless she has moderate to severe organ involvement.

However, for those whose organ involvement had been

controlled prior to conception, an increase in organ disease occurs in only 10%

who become pregnant. All women with lupus are at a higher risk of miscarriage,

but especially those with the Lupus Anticoagulant Syndrome.

In 40% of lupus patients, no change in disease activity

occurs during pregnancy, disease exacerbation in seen in another 40%, and

improvement of symptoms occurs in only 20%.

Since the fetus produces additional cortisone, the

withdrawal of this increased hormone after childbirth can cause a flare in the

mother's disease. Such exacerbation is usually mild in patients who have been

treated prior to and during pregnancy, but can be quite severe in those who

hadn't yet been diagnosed and treated.

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Conditions of the Eye

Whether due to Lupus itself, certain

lupus medications, or secondary conditions such as Sjogren's,

Raynaud's, or Vasculitis,

the eyes are often affected in a variety of ways.

Cataracts and Glaucoma are often seen in lupus patients,

but the disease is not associated with either of these eye conditions. However,

extended use of steroids, such as Prednisone, can cause Glaucoma and almost

always produces cataracts.

Since lupus patients are more susceptible to infections,

Conjunctivitis (more commonly known as " pink eye " ) commonly occurs,

but is easily treated with antibiotic eye drops.

Should you ever be given antibiotic eye drops, which

cause the affected eye to burn, itch, or worsen, discontinue use and contact

your doctor immediately. These are indications of an allergic reaction to the

particular solution, and there is more than one type of antibiotic drop

available to treat eye infections.

Inflammation of the iris, known as Iritis,

is a recurrent problem in about 2% of lupus patients. It has been observed in

almost every autoimmune disease and can also exist by itself. It is usually

managed successfully with steroid eye drops. The symptoms of iritis include hazy vision or floating black spots. Severe

pain, redness in the white of the eye, and sensitivity to light are especially

common.

Blood vessel disorders of the eye, called Retinal Vasculitis cause bleeding behind

the eye, inadequate blood supply, and blood clots causing blockage to the

vessels. These disorders lead to decreased vision and sometimes blindness. They

also indicate that the person is at a high risk of other problems, such as stroke.

This condition is usually a complication of the Lupus Anticoagulant Syndrome.

The decreased ability of the eyes to produce tears in

persons with Sjogren's Syndrome

prevents the cornea (outer covering of the eyeball) from being adequately

lubricated. Left untreated, this excessive dryness results in the development

of pits, leaving scarred areas on the cornea surface. This can be effectively

treated with artificial tears.

Optic Neuritis, inflammation of the optic nerve, can also

occur in SLE. The optic nerve is the primary nerve leading from the brain to

the back of the eye, through which all sight responses are transmitted and

received. This condition is not as common in lupus as it is in Multiple

Sclerosis (MS). However, lupus mimics the symptoms of many other diseases,

including MS.

Optic Neuritis can be painful or painless, depending on

which section of the optic nerve is affected. It can cause blurred or cloudy

vision or blindness, depending upon if the inflammation has actually damaged

the nerve. Optic Neuritis is usually limited to one eye, but not always. The

blindness can be temporary or permanent, and is treated with steroids.

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Cognitive Dysfunction

Known by lupus patients as " brain

fog " '! Symptoms include confusion, profound fatigue, and difficulty

in articulating thoughts, memory impairment and difficulty concentrating.

Cognitive dysfunction may come and go on its own, but antimalarial

drugs, tricyclic antidepressants or serotonin

blockers, or DHEA may help.

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Lupus headache

Compared with the general population,

lupus patients are perhaps twice as likely to suffer from migraine-like

headaches. Many patients also have antiphospholipid

antibodies, while others display Raynaud's phenomenon.

Lupus headache is managed much like conventional migraine, using painkillers,

anti-inflammatories, vasoconstrictors, and sometimes

beta-blockers, or tricyclic antidepressants. Lupus

headache is different to most migraines, since patients may respond to a one

week trial of 20-60 milligrams of prednisone daily, which is rarely useful to

migraine sufferers.

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Seizures

Seizures can result from acute brain

inflammation, scarring from prior vasculitis, acute

strokes, or reactions to medications used to treat the disease, such as

corticosteroids or high-dose antimalarials.

There are different forms of seizures - 'grand-mal' seizures are characterized

by total body shaking, loss of consciousness, incontinence, and after seizure

lethargy. Temporal lobe/partial-complex seizures are characterized by

perceptual distortions, odd smells, altered emotional states, staring, and no

loss of consciousness.

Electroencephalography (EEG) can be used to diagnose, CT and MRI scans are used

to rule out other causes. Anticonvulsant medications are normally used to treat

seizures.

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Psychosis

Some lupus patients may demonstrate

symptoms of psychosis. Psychosis is defined as an inability to judge reality,

marked by disordered thinking and bizarre ideas, often including delusions and

hallucinations. It usually results in an inability to carry out the ordinary

demands of living.

About 10 to 15% of lupus patients will display acute psychosis during the

course of their disease. Most psychotic episodes occur with CNS vasculitis, but others occur as a result of steroid

therapy, water intoxication with low blood levels of sodium, seizures, central

hyperventilation, or antimalarial therapy. It is

managed with corticosteroids, and antipsychotic preparations.

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Lupus Myositis

Unlike the joints, the muscles can be seriously damaged

by SLE. This damage may result in muscle weakness and loss of strength unless

early, appropriate treatment is given. Inflamed muscles may not only be

painful, but may also be tender to the touch. Muscle weakness is the most

common symptom of lupus myositis

Characteristically, the muscles of the trunk of the body

are affected (i.e., neck, pelvic girdle and thighs, shoulder girdle and upper

arms). Pain in the small muscles of the hand or weakness of

the grip are not symptoms of SLE myositis.

However, nerves as well as muscle fibers can be caught up in the inflammatory

process and, occasionally, some weakness of the wrists and hands or the ankles

and feet may occur as a result of nerve damage.

Diagnosis

The diagnosis of SLE myositis is relatively straightforward. There are chemical

enzymes (e.g., CPK, SGOT, SGPT, aldolase)

which are normally concentrated within muscle fibers and which escape into the

blood circulation when muscle fibers are being damaged by inflammation.

Thus, tests for these chemicals in the blood are abnormal

in SLE myositis. These tests can also be used to

determine the severity of muscle involvement: more severe myositis

results in a higher level of these enzymes in the blood. Such tests are

therefore useful in the diagnosis of SLE myositis,

and in following the course of the disease and its response to therapy.

Just as the electrocardiogram (EKG) reflects damage to

heart muscle, the electromyogram (EMG) can be used to

determine the character of muscle damage in lupus myositis.

When inflammation is present, the EMG shows a

characteristic pattern of electrical response. A microscopic examination of a

sample of muscle tissue a biopsy) may also be taken from a painful muscle to

confirm the presence of inflammation and to help identify the severity of the

inflammation.

Treatment

Corticosteroids (Prednisone) are necessarily

prescribed for the treatment of SLE myositis. High

doses (50 mg. per day or more of Prednisone or equivalent) are initially given

for prompt suppression and control of the inflammation. The steroid dose is

gradually reduced as the inflammation subsides, as determined by the patient's

symptoms and enzyme levels in the blood.

The vast majority of people with lupus respond promptly

and well to corticosteroids. It is seldom necessary to augment treatment with cytotoxic or immunosuppressive medications.

Once the acute, inflammatory phase is past, a well

directed exercise program should be started to help the patient regain normal

muscle strength and function.

Lupus Awareness

Do Your Part to STOP THE SICKNESS!

Lupus is a

disease that destroys the muscles, joints,

major organs, and the lives of millions of Americans.

So why is so little being

done about it?

Maybe

because most of its victims are women.

1-888-38LUPUS

Contact the Lupus Foundation of America or the local

Chapter that serves your area for more information about lupus, or the programs

and services the LFA offers including support group information and physician

referral.

Hugs,

Deanna

LUPUS Serenity

Prayer...

Lord, grant me the serenity to accept the things I cannot

change, the courage to change the things I can, and the wisdom to hide the

bodies of doctors I shot when they said, You're perfectly healthy, it's all in

your head "