The Lupus Book: A Guide for Patients and Their Families, Third Edition (23 page)

the eyelids. The eye muscles may become swollen or inflamed, which is called

orbital myositis
, or temporarily paralyzed due to a
cranial neuropathy
, since 3

of the 12 cranial nerves (peripheral nerves) control the movement of eye mus-

cles. If diabetes and thyroid conditions are ruled out as the cause of orbital

pathology, corticosteroids can relieve these conditions.

Conjunctivitis
, or inflammation of tissues around the eyeball, is more common among lupus patients since they are more susceptible to infection. Antibiotic

solutions can keep conjunctivitis in check, but the presence of conjunctivitis

does not indicate more active disease. Patients with Sjo¨gren’s syndrome (see

below) have decreased ability to manufacture tears. When the cornea (the outer

covering of the eyeball) cannot be adequately lubricated, it becomes dry and

develops pits, leaving scarred areas on its surface. Artificial tears are usually helpful in such cases.

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Where and How Can the Body Be Affected by Lupus?

DOES LUPUS AFFECT SIGHT?

What About Cataracts, Glaucoma, or Iritis?

The
uveal tract
or middle layer of the eye includes the iris, lens, and ciliary muscles. Lupus does not directly induce
cataracts
, or the clouding of the lens, but corticosteroids can. Similarly, these agents are associated with a greater risk of developing
glaucoma
, which is caused by elevated pressures in the eye. Cataracts can be removed surgically, and eyedrops lower increased ocular pressures.

Inflammation of the iris, or
iritis
, is a recurrent problem in 1 to 2 percent of lupus patients. It has been observed in almost every autoimmune disease and

exists as an autoimmune disorder by itself. Iritis is managed with steroid eye-

drops, but at times oral corticosteroids, immunosuppressives, or intraocular injections of steroid are needed.

The Retina

Rachel woke up blind in her right eye. She had a history of mild SLE but

had generally felt well. Rachel was married to a stockbroker on the West

Coast who was already at work at 6 A.M.; she felt helpless. Michael and

she had been trying to have children, and these efforts culminated in two

heartbreaking miscarriages. Rachel placed a call to her internist. The inter-

nist referred her to an eye doctor. She saw him that day. He performed a

fluorescein angiogram that documented a clot in her retinal artery. Rachel

was admitted to the hospital, where she was anticoagulated with intrave-

nous heparin followed by oral warfarin (Coumadin). A rheumatologist was

called in to see Rachel, and her workup documented evidence for anti-

phospholipid antibodies. Rachel was advised to take Coumadin for 3

months followed by lifelong low-dose aspirin. Gradually, her sight im-

proved, and within 3 months she had only a small blind spot that did not

bother her.

The back layer of the eye is known as the
retina
. It is the only part of the body where physicians can actually see internal blood vessels in their entirety.

Although antimalarial drugs or infectious agents infrequently damage the retina, 10 percent of lupus patients develop visible retinal pathology.
Retinal vasculitis
, or inflammation of the retinal vessels, can be painful and cause visual changes.

Usually seen with active disease, it is treated with corticosteroids. On the other hand, sometimes areas of ‘‘infarction’’ or dead tissue are noted in the retina.

These
cytoid bodies
, as they are called, indicate either old, healed retinal vasculitis or signify that a clot has traveled to the eye as a complication of the antiphospholipid syndrome (Chapter 21). Steroids will not benefit these patients; blood thinning or anticoagulation is the treatment of choice.

When another cranial nerve known as the optic nerve is inflamed,
optic neu-

The Head, Neck, and Sjo¨gren’s Syndrome

[123]

ritis
results, causing varying degrees of visual impairment. This must be differentiated from blurred vision induced by corticosteroids.

DOES LUPUS CAUSE HEARING OR EAR PROBLEMS?

In general, lupus patients have no unusual or specific hearing deficits. They may complain of
tinnitus
, or ringing in the ears, due to anti-inflammatory medications such as antimalarials or NSAIDs. This is especially common in those over the

age of 60.

One lupus patient in 500 has a rare complication known as
autoimmune ves-

tibulitis
, characterized by sudden hearing loss with or without visual changes or dizziness. Corticosteroids reverse this deafness.

On rare occasions, the cartilage lining the outer part of the ear becomes in-

flamed in active SLE, producing a condition known as
chondritis
. It appears identical to another autoimmune disease called
relapsing chondritis
, where cartilage in the trachea, nose, and valves of the heart can also be inflamed. In SLE, however, chondritis is usually limited to the ear and responds to steroids.

To sum up, ear problems are very rare in SLE and, if present, usually stem

from nonimmunologic sources.

THE NOSE

Some of my patients have been wrongly accused of using cocaine when they

have nasal septal perforations. The nasal septum—the cartilage membrane that

divides the nose into two nostrils—can perforate, or develop holes, in 1 to 2

percent of lupus patients.
Ulcerations
may also be found on the nasal mucosa.

I usually prescribe petroleum jelly (e.g., Vaseline) or a steroid preparation (e.g., Kenalog in Orabase) for these. Recurrent
sinus
infections in a patient who does not improve with antibiotics, decongestants, or antiallergy medications and

seems to have lupus warrants a blood test for a closely related disorder known

as Wegener’s granulomatosis. A blood test for anti-neutrophilic cytoplasmic

antibody (ANCA) can help differentiate these two types of vasculitis, which are treated quite differently.

DENTAL CONSIDERATIONS

Very few patients realize the importance of good oral hygiene as an adjunct in

managing SLE. Since the mouth is teeming with bacteria, some rheumatologists

believe that all dental procedures should be accompanied by prophylactic anti-

biotics to protect patients with lupus from developing infected heart valve vegetations (Chapter 14) or other types of infections. Those who have temporo-

mandibular joint (TMJ) involvement from SLE or oral scleroderma-like

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Where and How Can the Body Be Affected by Lupus?

tightening of the lips with decreased opening of the jaws find chewing more

difficult and may develop cavities and abscesses. They should see their dentist at least once a year for a checkup.

Sores in the mouth, or oral ulcerations, are seen in 20 percent of lupus patients and are discussed in Chapter 12. Sjo¨gren’s syndrome causes dryness of the

mouth and is discussed below.

WHAT IS THE REASON FOR THE RASPY VOICE?

The voice box (vocal cords) is in the larynx. A synovially lined joint known as the cricoarytenoid joint is found in this area (Chapter 13). Lupus activity sometimes produces synovitis. When the synovium of the cricoarytenoid joint be-

comes inflamed as a result of active disease, a hoarse voice can develop. Ear,

nose, and throat specialists see evidence of this when they perform direct lar-

yngoscopy. Anti-inflammatory medications can relieve the problem, although

sometimes the joint may be sprayed with a steroid aerosol or locally injected.

SJO

¨ GREN’S SYNDROME

Do the Skin and Eyes Feel Dry?

Laura was unable to wear the contact lenses her optometrist had prescribed.

A fashion model, Laura tried her best not to think about her lupus, but

fortunately the condition was limited to her skin (which could be covered

with hypoallergic makeup) and symptoms of occasional aching and fatigue.

The only other medicine she took was Naprosyn for intermittent joint pain.

The optometrist referred her to an ophthalmologist, who diagnosed Sjo¨-

gren’s syndrome. She prescribed artificial tears and told Laura to drink

plenty of fluids. When Laura told this to her rheumatologist, he added

Plaquenil and gave her literature from the Sjo¨gren’s Foundation, which

listed over a hundred over-the-counter moisturizing agents for the ears,

nose, mouth, eyes, and vagina. Laura’s joint aches and fatigue improved,

but she still had to use moisturizing agents.

What Is Sjo¨gren’s Syndrome?

In 1933, Henrik Sjo¨gren (pronounced show-gren) described some symptoms

common to a group of patients: dry eyes (keratoconjunctivitis sicca), dry mouth (xerostomia), and arthritis. In the late 1960s, doctors found that many patients with these symptoms had an autoimmune process. Sjo¨gren’s syndrome, as the

disorder is now known, can be part of many autoimmune diseases or may exist

by itself (termed
primary Sjo¨gren’s
) without fulfilling accepted criteria for any other process. It is estimated that 5 to 10 percent of all Sjo¨gren’s patients have SLE, although the incidence of Sjo¨gren’s in established SLE patients has not

The Head, Neck, and Sjo¨gren’s Syndrome

[125]

been reliably ascertained. We do know that at least 10 percent of the individuals with lupus have obvious Sjo¨gren’s. However, if minimally or asymptomatic

patients with lupus undergo vigorous testing for the syndrome, perhaps as many

as one-third would fulfill accepted Sjo¨gren’s definitions.

How Do We Diagnose Sjo¨gren’s and Why Is It Important?

Some of the procedures used to diagnose Sjo¨gren’s include the
Schirmer’s test
, which measures the amount of tearing, or
Rose Bengal staining
of the cornea, which looks for pitting or areas of scarring. Although simple to perform, these procedures have false-positive and false-negative results. The ultimate and most accurate diagnostic test is a
lip biopsy
, which displays a characteristic inflammatory infiltrate. Fortunately, it is rarely necessary to do this potentially painful procedure. Many Sjo¨gren’s patients have enlarged salivary glands.

Why is it important to diagnose Sjo¨gren’s? First of all, the syndrome is as-

sociated with disease outside the salivary glands and tear ducts. For example, a persistent, nonproductive hacking cough is a manifestation of dry lungs or
bronchitis sicca
, which predisposes one to interstitial lung disease (Chapter 14).

Other related disorders include atrophic gastritis (dry stomach), hyperviscosity syndrome (thick blood), cryoglobulinemia, and subacute cutaneous lupus rashes.

The anti-Ro (SSA) antibody (Chapter 11) is noted in 30 percent of those with

SLE but 70 percent of those with primary Sjo¨gren’s. This antibody crosses the

placenta and has the capability of inducing neonatal lupus and congenital heart block (Chapter 22). Of all rheumatic diseases, Sjo¨gren’s correlates with the

highest levels of autoantibodies. It is not unusual for Sjo¨gren’s patients to have ANA tests or rheumatoid factors with levels well over several thousand. Sjo¨-

gren’s is associated with the HLA-DR3 marker (Chapter 7). Also, Sjo¨gren’s

syndrome is the only autoimmune condition that has a potential for malignant

transformation. Up to 8 percent of Sjo¨gren’s patients develop a blood disorder, particularly lymphoma. I obtain a serum protein electrophoresis, an inexpensive blood test, at least once a year to partially screen for this.

How Should Sjo¨gren’s Be Managed?

In spite of all that has been said, Sjo¨gren’s syndrome is usually a relatively benign process and its treatment is symptomatic. For instance, dry eyes usually respond to artificial tears and dry mouth to everything from plugging tear ducts to Lifesavers to sparkling water with a touch of lemon, lime, orange, grapefruit, or any other citrus fruit (although frequently used, Lifesavers are discouraged because of their high sugar content). A humidified environment or room hu-midifiers can be helpful. Chicken soup moisturizes the lungs, as do several drugs that can promote humidification. Recently, some evidence has suggested that

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Where and How Can the Body Be Affected by Lupus?

hydroxychloroquine (Plaquenil) helps to mitigate the underlying immune pro-

cess of Sjo¨gren’s. Pilocarpine and cevimeline (Evoxac) (Salagen) promote in-

creased salivary secretions. Cyclosporin eye drops (Restasis) ameliorate dry eye symptoms and signs. Most experiences with prescribing corticosteroids for Sjo¨-

gren’s have been disappointing except when they are used for a short time in a

rare subset of Sjo¨gren’s called
Mikulicz’s syndrome
where the parotid or salivary glands become greatly enlarged from acute inflammation. This condition is readily identified, since patients appear to have the mumps, and the salivary glands are extremely tender to the touch.

Summing Up

The most common cause of head and neck involvement in SLE is Sjo¨gren’s

syndrome. Eye complaints are often due to medication, especially steroids, but

10 to 15 percent of patients with lupus develop complications from disease

activity or the antiphospholipid antibody. Ulcers of the mouth or nose are seen in 20 percent of these patients. Involvement of the ear or larynx is rare. Patients and their physicians must carefully evaluate head and neck complaints, since

failure to intervene promptly with autoimmune inflammation of the optic nerve

or the ear can result in permanent blindness or deafness.