Greg Black and Julie Tyler
881 Camellia Court
Plantation, FL 33317
Cell (954) 815 2020
Ocular Manifestations of Rheumatoid Diseases
I. Rheumatic diseases- Characterized by inflammation and loss of
function of one or more connecting or supporting structures of the
body
A. Joints, tendons, muscles, ligaments and bones typically
affected with extra-articular manifestations
B. Pain, swelling and stiffness are common symptoms
C. Over 100 rheumatic diseases some with ocular
manifestations
1. Rheumatoid arthritis
2. Systemic lupus erythematosus
3. Scleroderma
4. Juvenile rheumatoid arthritis
5. Spondyloarthropathies
II. Rheumatoid Arthritis- a chronic, progressive inflammatory
disease with several ocular associations
A. Periods of exacerbations and remissions
B. Incidence
1. In the United States, RA is found in approximately 1%
of the adult population
2. Peak incidence between the fourth and sixth decades
3. Prevalence rate about 2.5x greater in women than men
C. Etiology
1. Specific etiology of RA is unidentified
2. Current theory suggests a genetic predisposition
involving multiple genes that are initiated by an unknown
trigger(s)
3. An association with female sex hormones has been
postulated
D. Disease Process
1. Onset of RA is usually insidious over a period of
weeks to months
2. Primary (but not solitary) target of the abnormal
immune response in RA is the synovial membranes and
articular structures
3. RA progresses from swelling of the synovial lining to
synovial thickening and finally resulting in deformity of the
joints
4. Common extra-articular manifestations include
rheumatoid nodules, ocular disease, Sjögren’s syndrome,
rheumatoid vasculitis and neurologic disease
E. Common symptoms
1. Symmetrical stiffness in multiple joints
2. Morning stiffness greater in duration than one hour
3. Generalized fatigue
F. Clinical Signs
1. Joint swelling
2. Tenderness
G. Ocular Manifestations
1. Approximately 25% of patients with RA will report
ocular manifestations
2. Keratoconjunctivitis sicca (KCS)
a. Combination of inflammation of the lacrimal
gland and meibomian gland dysfunction
b. Ocular findings include conjunctival staining,
punctate epithelial keratitis (PEK), diminished tear
meniscus and decreased tear break-up time.
3. Episcleritis - Inflammation of the tissue overlying the
sclera
4. Scleritis - Inflammation of the sclera including the
presence of scleral edema
5. Keratitis - Peripheral corneal ulceration
H. Making the Diagnosis
1. Diagnosis is made based on the 1987 criteria for the
classification of RA
2. Based on medical history, physical examination,
serology, and imaging studies
3. Serologic testing consists of a complete blood count
(CBC), erythrocyte sedimentation rate (ESR), C-reactive
protein and rheumatoid factor (RF)
I. Treatment
1. Dependent on the individual and severity of disease
2. First-line medications, NSAIDS and corticosteroids
3. Second-line medications, disease-modifying antirheumatic drugs (DMARDs) suppress the immune system
and inhibit the loss of cartilage and bone, while biologic
response modifiers (BRMs) influence the activity of
cytokines.
4. Consideration of Hydroxychloroquine
III. Systemic Lupus Erythematosus (SLE)
A. SLE Qualities
1. Considered an “imitator” disorder because its
symptoms and clinical course vary widely and mimic other
conditions
2. Chronic autoimmune inflammatory disease can affect
the skin, kidneys, joints, nervous system, blood, eyes and
other organs
B. Incidence, Prevalence and Mortality
1. In the U. S., SLE prevalence is approximately 1 in
2,000, and frequency varies by ethnicity.
2. SLE is also two to three times more prevalent in nonwhites, including blacks, Asians, Hispanics and Native
Americans
3. An estimated 1.5 million Americans have some form of
lupus.
4. Peak incidence: 80% develop SLE between ages 15
and 45
5. Predominant among females (90% are female)
6. Five-year survival rate of patients has increased from
50% in the 1950s to between 91% to 97% today
7. Mortalitiy rates are higher among SLE patients who
develop infectious complications, seizures, lupus nephritis
and renal failure.
C. Etiology
1. Complex disease with an unknown etiology a combination of genetic, environmental and hormonal
factors that are initiated by a trigger
a. Trigger may be bacterial infection
b. Trigger may be viral infection
2. Flares are also associated with periods of significant
hormonal changes, such as pregnancy and initiation of
hormone replacement therapy.
D. Disease Process
1. Patients initially experience a flu-like syndrome:
including fatigue, fever, weight loss, and muscle & joint
pain
2. The pathology of SLE consists of vascular
abnormalities and inflammation.
3. Vascular abnormalities and inflammation lead to
immune complex deposition, occlusive vasculopathy and
vasculitis
4. As SLE progresses, complications become more
diverse and severe
E. Common symptoms
1. Initial flu-like syndromePatients initially experience a
flu-like syndrome, including fatigue
2. Fatigue, fever, weight loss, and muscle and joint pain
F. Clinical Signs
1. Malar Rash
2. Alopecia
G. Ocular Manifestations
1. Ocular complications of SLE are variable
2. Ocular findings occur more frequently in patients who
have concurrent systemic disease
3. Conditions:
a. Keratoconjunctivitis Sicca (KCS) – combination
of inflammation of the lacrimal gland and meibomian
gland dysfunction
b. Episcleritis – Inflammation of the tissue overlying
the sclera
c. Scleritis – An indicator of significant, concurrent
SLE
d. Uveitis – Initially tends to present as acute,
bilateral and non-granulomatous iridocyclitis
e. Vacular occlusive disease
1) Retinal vein occlusion
2) Retinal artery occlusion
f. Retinopathy
1) Hemorrhages
2) Candle wax dripping
3) Cotton wool spots
H. Making the Diagnosis
1. Diagnosis is made based on the presence of at least
four of eleven diagnostic criteria established by the
American College of Rheumatology
a. Symptoms DO NOT need to be concurrent
2. Serologic testing may be beneficial but is not
necessary for diagnosing
3. Lab tests may help in the diagnosis and be monitored
for management
I. Treatment
1. Currently there is no cure for SLE - goal of therapy is:
a. Alleviate symptoms
b. Prolong remissions
c. Reverse immune dysregulatoin
d. Prevent organ damage
2. First-line drugs include NSAIDs and corticosteroids
3. Second-line drugs include disease-modifying antirheumatic drugs (DMARDs), and biologic response
modifiers (BRMs)
IV Sjogren’s Syndrome
A. A multi-factoral, chronic inflammatory condition resulting in
lymphocytic infiltration of the lacrimal and salivary glands
resulting in xerophthalmia and xerostomia respectively
a. Dry eyes (KCS)
b. Dry mouth
c. Accompanied by associated, specific systemic
conditions, including SLE
B. Other conditions associated with Sjogren’s syndrome autoimmune disorders that compromise lacrimal system
a. Rheumatoid arthritis (RA)
b. Systemic sclerosis
c. Psoriatic arthritis
d. Hashimoto’s thyroiditis
e. Juvenile chronic arthritis
C. Ocular complications and treatment
a. Filamentary keratitis
b. Medications
i. Artificial tears
ii. Restasis
iii. Topical steroids
iv. Neutraceuticals and others
Reference:
Arnett FC, Edworthy SM, Bloch DA, et al. The American Rheumatism Association 1987
revised criteria for the classification of rheumatoid arthritis. Arthritis Rheum 1988
Mar;31(3):315-24.
Tan EM et al. The 1982 Revised Criteria for the Classification of SLE. Arth Rheum
1982;25: 1271-1277.
Hochberg MC. Updating the American College of Rheumatology revised criteria for the
classification of systemic lupus erythematosus [letter]. Arth Rheum 1997;40:1725.
Black GS, Tyler JA. Lessons on Lupus in the Eye. Review of Optometry 2006;
143(4):59-66.
Black GS, Tyler JA, Kabat AG. Arthritis as Seen Through the Eyes. Review of
Optometry 2006; 143(1):39-45.