explaining two collagen vascular diseases (lupus and rheumatoid arthritis)and the effects on eyes(signs symptoms and treatment ) with useful photos

1. Collagen vascular disease
Negin Ghaderi
Sara Imani
Nargess Mirizadeh
BSc students of optometry
Iran University Of Medical Sciences (IUMS)
nargesmirizadeh@gmail.com
n97ghaderi@gmail.com
Sara.imani8034@gmail.com
July 2020

2. Collagen vascular disease
“Collagen vascular disease” is the name of a group of diseases that affect
your connective tissue. Collagen is a protein-based connective tissue that
forms a support system for your skin. Connective tissue holds bones,
ligaments, and muscles together. Collagen vascular disease is sometimes
also called connective tissue disease. Collagen vascular diseases can be
heritable (inherited from one’s parents) or autoimmune (resulting from
activity of the body’s immune system against itself). This article deals with
autoimmune forms of collagen vascular diseases.

4. Types of autoimmune collagen vascular disease include:
lupus
rheumatoid arthritis
scleroderma
temporal arteritis

5. Types of hereditary collagen disease include
Ehlers-Danlos syndrome
Marfan’s syndrome
Osteogenesis imperfect (OI), or brittle bone disease

6. Causes of collagen vascular disease
Collagen vascular disease is an autoimmune disease. This means that
your immune system mistakenly attacks your body’s healthy tissue. No
one knows what causes your immune system to do this. The attacks
usually cause inflammation. If you have a collagen vascular disease,
your immune system causes inflammation in your collagen and nearby
joints.

7. Symptoms of collagen vascular disease
Each type of collagen vascular disease has its own set of symptoms.
However, most forms of collagen vascular disease do share some of the
same general symptoms. People with collagen vascular disorders
typically experience:
fatigue
muscle weakness
fever
body aches
joint pain
skin rash

8. lupus
lupus is a long-term autoimmune disease in which the body’s immune
system becomes hyperactive and attacks normal, healthy tissue. Symptoms
include inflammation, swelling, and damage to the joints, skin, kidneys,
bloDue to its complex nature, people sometimes call lupus the “disease of
1,000 faces.”
In the United States, people report around 16,000 new cases of lupus each
year, and up to 1.5 million people may be living with the condition,
according to the Lupus Foundation of America. od, heart, and lungs.

9. The Foundation say that lupus affects women in particular, and it is
most likely to appear between the ages of 15 and 44 years.
Lupus is not a contagious disease. A person cannot transmit it sexually
or in any other way to another person.

10. Systemic lupus erythematosus
SLE is the most familiar type of lupus. It is a systemic condition. This
means it has an impact throughout the body. The symptoms can range
from mild to severe.
It is more severe than other types of lupus, such as discoid lupus,
because it can affect any of the body’s organs or organ systems. It can
cause inflammation in the skin, joints, lungs, kidneys, blood, heart, or a
combination of these.
This condition typically goes through cycles. At times of remission, the
person will have no symptoms. During a flare-up, the disease is active,
and symptoms appear.

12. Discoid lupus erythematosus
In discoid lupus erythematosus (DLE) or cutaneous lupus symptoms
affect only the skin. A rash appears on the face, neck, and scalp.
The raised areas may become thick and scaly, and scarring may result.
The rash may last from a number of days to several years, and it may
recur.
DLE does not affect the internal organs, but around 10 percent of
people with DLE will go on to develop SLE, according to the Lupus
Foundation of America. It is not clear, however, if these individuals
already had SLE and just showed clinical signs on the skin or if there is
a progression from DLE or SLE

14. Drug-induced lupus
In around 10 percent of people with SLE, symptoms occur because of a
reaction to certain prescription drugs. According to Genetics Home
Reference, some 80 drugs may cause the condition.
These include some of the drugs that people use to treat seizures
and high blood pressure. They also include some thyroid
medications, antibiotics, antifungals, and oral contraceptive pills.

15. Drugs that are commonly associated with this form of lupus
are:
Hydralazine, a hypertension medication
Procainamide, a heart arrhythmia medication
Isoniazid, an antibiotic used to treat tuberculosis (TB)
Drug-induced lupus typically goes away after the person stops taking
the medication

17. Neonatal lupus
Most babies born to mothers with SLE are healthy. However, around 1
percent of women with autoantibodies relating to lupus will have a baby
with neonatal lupus.
The woman may have SLE, Sjögren’s syndrome, or no disease
symptoms at all.

18. Sjögren’s syndrome is another autoimmune condition that often occurs
with lupus. Key symptoms include dry eyes and a dry mouth

19. At birth, babies with neonatal lupus may have a skin rash, liver
problems, and low blood counts. Around 10 percent of them will
have anemia.
The lesions usually go away after a few weeks. However, some infants
have a congenital heart block, in which the heart cannot regulate a
normal and rhythmic pumping action. The infant may need a
pacemaker. This can be a life-threatening condition.
It is important for women with SLE or other related autoimmune
disorders to be under a doctor’s care during pregnancy.

20. Causes
Lupus is an autoimmune condition, but the exact cause is unclear.
What goes wrong?
The immune system protects the body and fights off antigens, such as
viruses, bacteria, and germs.
It does this by producing proteins called antibodies. White blood cells,
or B lymphocytes, produce these antibodies.
When a person has an autoimmune condition, such as lupus, the
immune system cannot differentiate between unwanted substances, or
antigens, and healthy tissue.

21. As a result, the immune system directs antibodies against both the
healthy tissue and the antigens. This causes swelling, pain, and tissue
damage.
The most common type of autoantibody that develops in people with
lupus is an antinuclear antibody (ANA). The ANA reacts with parts of
the cell’s nucleus, the command center of the cell.
These autoantibodies circulate in the blood, but some of the body’s cells
have walls permeable enough to let some autoantibodies through.
The autoantibodies can then attack the DNA in the nucleus of these
cells. This is why lupus affects some organs and not others.

22. Risk factors
Hormones
genes
environment
Lupus may develop in response to a number of factors. These may be
hormonal, genetic, environmental, or a combination of these.

23. 1) Hormones
Hormones are chemical substances that the body produces. They
control and regulate the activity of certain cells or organs.
Hormonal activity could explain the following risk factors:
Sex: The U.S. National Institutes of Health note that females are nine
times more likely to have lupus than males.

24. Age: Symptoms and diagnosis often occur between the ages of 15 and
45 years, during the childbearing years. However, 20 percent of cases
appear after the age of 50 years, according to Genetics Home Reference
As 9 out of 10 occurrences of lupus affect females, researchers have
looked at a possible link between estrogen and lupus. Both men and
women produce estrogen, but women produce more.
In a review published in 2016, scientists observed that estrogen can
affect immune activity and induce lupus antibodies in mice that are
susceptible to lupus.
This may explain why autoimmune diseases are more likely to affect
women than men.
.

25. In 2010, researchers who published a study on self-reported flares in
the journal Rheumatology found that women with lupus report more
severe pain and fatigue during menstruation. This suggests that flares
may be more likely at this time.
There is not enough evidence to confirm that estrogen causes lupus. If
there is a link, estrogen-based treatment could regulate the severity of
lupus. However, more research is necessary before doctors can offer it
as a treatment.

26. 2) Genetic factors
Researchers have not proved that any specific genetic factor causes
lupus, although it is more common in some families.
Genetic factors may be the reason why the following are risk factors for
lupus:
Race: People of any background can develop lupus, but it is two to
three times more common in people of color, compared with the white
population. It is also more common in Hispanic, Asian, and Native
American women.

27. Family history: A person who has a first or second-degree relative
with lupus will have a higher risk of developing it.
Scientists have identified certain genes that may contribute to the
development of lupus, but there is not enough evidence to prove that
they cause the disease.
Lupus can happen in people with no family history of the disease, but
there may be other autoimmune diseases in the family.

28. 3) Environment
Environmental agents such as chemicals or viruses may contribute to
triggering lupus in people who are already genetically susceptible.
Possible environmental triggers include:
Smoking: A rise in the number of cases in recent decades may be due to
higher tobacco exposure.

29. Exposure to sunlight: Some suggest that this may be a trigger.
Medication: Around 10 percent of cases may be drug-related, according
to Genetics Home Reference

30. Viral infections: These may trigger symptoms in people who are prone
to SLE.
Lupus is not contagious, and a person cannot transmit it sexually.
Gut microbiota
Recently, scientists have been looking at gut microbiota as a possible
factor in the development of lupus.
Scientists who published research in Applied and Environmental
Microbiology in 2018 noted that specific changes in gut microbiota
feature in both people and mice with lupus.
They call for more research into this area.
Exposure to sunlight: Some suggest that this may be a trigger.
Medication: Around 10 percent of cases may be drug-related, according
to Genetics Home Reference

31. Symptoms
The symptoms of lupus occur in times of flare-ups. Between flare-ups,
people usually experience times of remission, when there are few or no
symptoms

32. Lupus has a wide range of symptoms, including:
fatigue
a loss of appetite and weight loss
pain or swelling in joints and muscles
swelling in the legs or around the eyes
swollen glands, or lymph nodes
skin rashes, due to bleeding under the skin
mouth ulcers
sensitivity to the sun
fever
headaches
chest pain upon deep breathing
unusual hair loss
pale or purple fingers or toes from cold or stress (Raynaud’s phenomenon)
arthritis

33. Raynaud’s phenomenon

34. Effect on other body systems
Lupus can also affect the following systems:
Kidneys: Inflammation of the kidneys (nephritis) can make it difficult
for the body to remove waste products and other toxins effectively.
Around 1 in 3 people with lupus will have kidney problems.
Lungs: Some people develop pleuritis, an inflammation of the lining of
the chest cavity that causes chest pain, particularly with
breathing. Pneumonia may develop.

35. Central nervous system: Lupus can sometimes affect the brain
or central nervous system. Symptoms include headaches,
dizziness, depression, memory disturbances, vision problems,
seizures, stroke, or changes in behavior.
Blood vessels: Vasculitis, or inflammation of the blood vessels, can
occur. This can affect circulation.
Blood: Lupus can cause anemia, leukopenia (a decreased number of
white blood cells) or thrombocytopenia (a decrease in the number of
platelets in the blood, which assist in clotting).
Heart: If inflammation affects the heart, it can result in myocarditis
and endocarditis. It can also affect the membrane that surrounds the
heart, causing pericarditis. Chest pain or other symptoms may result.
Endocarditis can damage the heart valves, causing the valve surface to
thicken and develop. This can result in growths that can lead to heart
murmurs.

36. Classification: 11 symptoms
The American College of Rheumatology use a standard classification
scheme to confirm a diagnosis.
If a person meets 4 out of 11 criteria, a doctor will consider that they
may have lupus.

37. Malar rash: A butterfly-shaped rash appears across the cheeks and nose.
Discoid rash: Raised red patches develop.
Photosensitivity: A skin rash appears after exposure to sunlight.
Oral or nose ulcers: These are usually painless.
Non-erosive arthritis: This does not destroy the bones around the joints,
but there is tenderness, swelling, or effusion in 2 or more peripheral joints.
Pericarditis or pleuritis: Inflammation affects the lining around the heart
(pericarditis) or lungs (pleuritis).
The 11 criteria are:

38. Kidney disorder: Tests show high levels of protein or cellular casts in the urine if a
person has a kidney problem.
Neurologic disorder: The person has seizures, psychosis, or problems with thinking
and reasoning.
Hematologic (blood) disorder: Hemolytic anemia is present, with a low white blood-cell
count or low platelet count.
Immunologic disorder: Tests show that there are antibodies to double-stranded DNA
(dsDNA), antibodies to Sm, or antibodies to cardiolipin.
Positive ANA: The test for ANA is positive, and the person has not used any drugs that
might induce it.

39. However, even this system sometimes misses early and mild cases.
Underdiagnosis can occur because the signs and symptoms of lupus are
not specific.
On the other hand, some blood tests can lead to overdiagnosis, because
people without lupus can have the same antibodies as those with the
condition.

40. 5. Ocular Manifestations
SLE can affect the periorbita, ocular adnexa, eye, and optic nerve. The
most common association is keratocon junctivitis sicca, while the most
visually devastating sequelae occur secondary to optic nerve
involvement and retinal vaso-occlusion.

41. Orbit
Orbital involvement is a rare manifestation of SLE. Vasculitis, myositis,
and panniculitis have all been described. Signs and symptoms include
proptosis, enophthalmos, orbital pain, blurred vision, chemosis, and
restriction of extraocular movements.
Orbital vasculitis leads to nonperfusion of the globe and extraocular
muscles. This has been shown to cause irreversible vision loss secondary
to ischemic injury to the optic nerve as well as elevated intraocular
pressure from neovascular glaucoma .

42. Orbital myositis is often initially misdiagnosed as bacterial orbital
cellulitis, as it usually presents with significant pain, proptosis,
periorbital swelling, and globe restriction. CT and orbital ultrasound
are both valuable in demonstrating enlargement of one or multiple
extraocular muscles. Creatinine kinase, aldolase, and myoglobin levels
are markedly elevated. Inflammation and symptoms typically respond
to steroids .
Subcutaneous inflammation secondary to SLE was first described by
Kaposi in 1883, and the term “lupus erythematosus panniculitis” was
coined in 1940.

43. Orbital involvement is very rare and has only been reported in a
handful of paper. Histopathology shows perivascular lymphocytic
infiltration . Response to steroids can be quite dramatic in most cases ;
however, few cases have shown a more virulent course with significant
enophthalmos secondary to fat atrophy and even melting of orbital
structures.

44. Periorbita
Periorbital edema is an uncommon
manifestation of systemic and discoid
lupus erythematosus with an overall
incidence of 4.8%

45. . It is most common in patients of African decent . Violaceous swelling
with overlying eczematous changes without any skin necrosis is seen.
Some cases can resemble chronic blepharitis. Etiologies include
nephrosis, increased vascular permeability, dermal mucin deposits, and
angioedema secondary to C1 deficiency. Treatment options include
observation , topical/intradermal/systemic corticosteroids and
antimalarials.

46. Eyelids
Typical lesions of discoid lupus erythematosus are slightly raised, scaly,
and atrophic. Most commonly, they occur on the head, face, neck, and
other sun-exposed areas. Rarely does it affect the eyelids.
Histopathologic study shows a hyperkeratotic epithelium with
liquefactive degeneration of the basal layer and a dense perivascular/
periappendageal lymphocytic infiltration. Diagnosis in most cases is
delayed because lesions are often mistaken for blepharitis and eczema.
Patients most commonly present with chronic erythema,
blepharoconjunctivitis with inflammation of the meibomian glands.
Long-term complications include madarosis, lid scarring, and
cicatricial ectropion/ entropion .

47. Diagnosis in most cases is delayed because lesions are often mistaken
for blepharitis and eczema. Patients most commonly present with
chronic erythema, blepharoconjunctivitis with inflammation of the
meibomian glands. Long-term complications include madarosis, lid
scarring, and cicatricial ectropion/entropion

48. Ocular Surface
The most common ocular manifestation of SLE is keratoconjunctivitis
sicca with the majority of patients endorsing at least one dry eye
symptom
Dryness can occur from multiple etiologies. Most patients with SLE
develop a secondary Sjogren’s syndromeIn their review of 283 SLE
patients, Manoussakis et al. identified 9.2% who had developed
Sjogren’s syndrome (SS).

49. Episclera/Sclera
Episcleritis is generally a benign inflammation of the episclera.
Typically occurring in young women, symptoms include a dull ache,
red eye, and tearing. Decreased visual acuity and severe pain are
uncommon. Systemic associations are extremely rare in adults, and a
systemic workup is not necessary. Incidence in adult patients with
SLE has been reported at 2.4%
. However, in children, episcleritis is much more rare but systemic
associates are much more common. Read et al. found 6 of 9 patients in
their series on pediatric episcleritis to have systemic connective tissue
disease

50. Treatment options include observation or topical/systemic nonsteroidal
anti-inflammatory drugs.
Scleritis Episcleritis

51. Scleritis is a more painful and potentially a vision-threatening condition
that warrants prompt treatment. Anterior scleritis can be nodular or
diffuse and presents with a red, painful eye that is tender to touch.
The injected deep episcleral vessels give a violaceous due to the sclera,
which is best appreciated in natural light. Posterior scleritis on the
other hand may not be associated with a red eye because it affects
sclera posterior to the equator of the globe. Presenting symptoms are
pain, blurred vision, limited eye movements, and proptosis. Blurred
vision is most commonly caused by exudative retinal detachment,
macular distortion due to a large scleral mass, and cystoid macular
edema.

52. Cornea
Corneal epitheliopathy, scarring, ulceration, and filamentary keratitis
can all occur secondary to keratoconjunctivitis sicca. More rare
corneal complications include peripheral ulcerative keratitis which
can be a marker of active systemic vasculitis, interstitial keratitis, and
keratoendothelitis . Spectral microscopy has been used to show
dysfunctional appearing corneal endothelial cells in both patients with
corneal edema and asymptomatic patients [38].

53. Corneal biomechanical properties differ in SLE. Yazici et al. used
Reichert ocular response analyzer measurements to show that corneal
hysteresis and corneal resistance factor were both lower in SLE
patients which can lead to an underestimated IOP and development of
keratoconus .
Dry eye

54. Retina
Lupus retinopathy is one of the most common vision-threatening
complications of systemic lupus erythematosus with an incidence of up
to 29% in patients with active systemic disease. A strong correlation
exists between presence of retinopathy and CNS disease. The most
common pattern of retinopathy is microangiopathy similar to diabetic
and hypertensive retinopathy. The earliest findings are small
intraretinal hemorrhages and cotton wool spots
.

56. Studies using fluorescein angiography describe hyperpermeability of arterioles
and venules as well as capillary nonperfusion
multiple cases have been demonstrating severe vision loss secondary to central
retinal artery/vein occlusions, vitreous hemorrhage, retinal ischemia, and
neovascularization
Immunosuppression has been successful in improving the appearance of the
retinopathy; however, visual recovery has only been reported in few cases.
The permanent loss of visual acuity is likely due to retinal ischemia. Addition
of anticoagulation to immunosuppression helps to stabilize retinal disease and
prevent further vascular events . Other therapies that have been reported for
severe disease include plasmapheresis and plasma exchange . Panretinal
photocoagulation, intravitreal antivascular endothelial growth factor agents,
and vitrectomy may also be considered for the treatment of complications of
ocular ischemia.

57. Fundus photograph demonstrating severe retinal vasculitis. Significant ischemia is present which is
highlighted by the attenuated and sclerotic vasculature. Panretinal photocoagulation was required to treat
ischemic and neovascular complications.

58. Serous retinal detachment in a patient with systemic lupus
erythematosus choroidopathy similar findings
Isolated cotton wool spot in a patient with lupus

59. Choroid
Lupus choroidopathy with exudative retinal detachments is a rare ocular
manifestation with fewer than 40 patients reported in the literature
It is generally seen in patients who have highly active disease including CNS
vasculitis and nephropathy as well as uncontrolled blood pressure

60. Multiple areas of hyperfluorescence seen on
fluorescein angiography caused by increased
vascular permeability of the choroidal circulation.
Large accumulation of subretinal fluid is seen on
optical coherence tomography.

61. Macular serous retinal detachment in a patient with lupus
choroidopathy

62. Optic Nerve/Central Nervous System
Optic nerve disease is a rare manifestation of SLE and consists of optic neuritis
and ischemic optic neuropathy . Presenting visual acuity in SLE-associated
optic neuritis is poor with most patients seeing worse than 20/200 . In the
Optic Neuritis Treatment Trial (ONTT), only 35.9% had a similar vision.
Visual recovery is variable in most patients and can range anywhere from full
recovery to count fingers vision. In a study by Lin et al. only 50% of patients
recovered to better than 20/25, while 37.5% maintained a visual acuity worse
than 20/200. In ONTT, 87% of patients recovered to better than 20/25 at 5
years of followup

63. Laboratory tests
Blood and urine tests may include:
Complete blood count
Erythrocyte sedimentation rate
Kidney and liver assessment
Urinalysis
Antinuclear antibody (ANA) test

64. Treatment
Treatment for lupus depends on your signs and symptoms. Determining
whether your signs and symptoms should be treated and what medications to
use requires a careful discussion of the benefits and risks with your doctor.
As your signs and symptoms flare and subside, you and your doctor may find
that you'll need to change medications or dosages.

65. The medications most commonly used to control lupus
include:
Nonsteroidal anti-inflammatory drugs (NSAIDs): Over-the-counter NSAIDs,
such as naproxen sodium (Aleve) and ibuprofen (Advil, Motrin IB, others),
may be used to treat pain, swelling and fever associated with lupus. Stronger
NSAIDs are available by prescription. Side effects of NSAIDs include stomach
bleeding, kidney problems and an increased risk of heart problems.

66. Antimalarial drugs: Medications commonly used to treat malaria, such as
hydroxychloroquine (Plaquenil), affect the immune system and can help
decrease the risk of lupus flares. Side effects can include stomach upset and,
very rarely, damage to the retina of the eye. Regular eye exams are
recommended when taking these medications.

67. Corticosteroids: Prednisone and other types of corticosteroids can counter the
inflammation of lupus. High doses of steroids such as methylprednisolone (A-
Methapred, Medrol) are often used to control serious disease that involves the
kidneys and brain. Side effects include weight gain, easy bruising, thinning
bones (osteoporosis), high blood pressure, diabetes and increased risk of
infection. The risk of side effects increases with higher doses and longer term
therapy.

68. Immunosuppressants: Drugs that suppress the immune system may be helpful
in serious cases of lupus. Examples include azathioprine (Imuran, Azasan),
mycophenolate mofetil (CellCept) and methotrexate (Trexall). Potential side
effects may include an increased risk of infection, liver damage, decreased
fertility and an increased risk of cancer.

69. Biologics: A different type of medication, belimumab (Benlysta) administered
intravenously, also reduces lupus symptoms in some people. Side effects
include nausea, diarrhea and infections. Rarely, worsening of depression can
occur.
Rituximab (Rituxan) can be beneficial in cases of resistant lupus. Side effects
include allergic reaction to the intravenous infusion and infections.

70. Take steps to care for your body if you have lupus. Simple measures can help you prevent
lupus flares and, should they occur, better cope with the signs and symptoms you experience.
Try to:
See your doctor regularly
Be sun smart
Get regular exercise
Eat a healthy diet.
Don't smoke
Ask your doctor if you need vitamin D and calcium supplements

71. What is rheumatoid arthritis?
Rheumatoid arthritis is a long-term, progressive, and disabling autoimmune
disease. It causes inflammation, swelling, and pain in and around the joints
and other body organs.
Rheumatoid arthritis (RA) usually affects the hands and feet first, but it can
occur in any joint. It usually involves the same joints on both sides of the
body.
Common symptoms include stiff joints, especially upon getting up in the
mornings or after sitting down for a while. Some people often
experience fatigue and a general feeling of being unwell.
The Rheumatoid Arthritis Support Network estimate that RA affects up to 1
percent of the world’s population and over 1.3 million people in AmerRA is an
autoimmune disease

72. . It is also a systemic disease, which means it affects the whole body.
It occurs when a person’s immune system mistakes the body’s healthy tissues
for foreign invaders.
As the immune system responds, inflammation occurs in the target tissue or
organ.
In the case of RA, this can be the joints, lungs, eyes, and heart.

73. Symptoms
Symptoms of RA include:
pain, swelling, and stiffness in more than one joint
symmetrical joint involvement
joint deformity
unsteadiness when walking
a general feeling of being unwell
fever
loss of function and mobility
weight loss
weakness

74. According to the Centers for Disease Control and Prevention (CDC), the
symptoms usually affect the same joints on both sides of the body
Symptoms tend to come and go. During a remission, they can disappear, or they
can be mild. However, during a flare, they can be severe

75. Causes
Nobody knows what causes the immune system to malfunction.
Some people appear to have genetic factors that make it more likely.
One theory is that bacteria or a virus triggers RA in people who have
this genetic feature.
In RA, the immune system’s antibodies attack the synovium, which is
the smooth lining of a joint. When this happens, pain and
inflammation result.

76. Inflammation causes the synovium to thicken. Eventually, if left
untreated, it can invade and destroy cartilage — the connective tissue
that cushions the ends of the bones.
The tendons and ligaments that hold the joint together can also weaken
and stretch. The joint eventually loses its shape and configuration

77. The damage Risk factors
The CDC note that people with a higher risk of developing RA may include
those who:
are aged 60 years or above
are female
have specific genetic traits
have never given birth
have obesity
smoke tobacco or whose parents smoked when they were children

78. People with RA have a higher risk of some other conditions,
including
heart disease
Obesity
diabetes
high blood pressure.

79. The joint damage that occurs with RA can make it difficult to perform
daily activities. RA can also be unpredictable. Often, a person does
not know when a flare will happen.
This uncertainty can lead to:
depression, anxiety, and stress
employment difficulties

80. There is also a higher risk of developing the following conditions:
Carpal tunnel syndrome: This is a type of nerve damage that stems
from compression and irritation of a nerve in the wrist. Symptoms
include aching, numbness, and tingling in the fingers, thumb, and part
of the hand.

81. Tendon rupture: Inflammation in the tendons can lead to rupture,
especially on the backs of the fingers.

82. Inflammation: This can affect the lungs, heart,
blood vessels, eyes and other parts of the body.
Cervical myelopathy: Dislocation of the joints in
the neck or cervical spine can add pressure to
the spinal cord. This can result in decreased
mobility and pain on movement. As RA
progresses, the risk of cervical myelopathy
increases.

83. Vasculitis: Inflammation of the blood vessels can cause them to weaken,
thicken, narrow and scar. This can affect blood flow to tissues and
organ function may be affected.
Susceptibility to infections: There is a higher risk of developing
colds, flu, pneumonia, and other diseases, especially if the person is
taking immunosuppressant medications to manage RA. People with
RA should ensure their vaccinations, such as flu jabs, are up-to-date.

84. Diagnosis
In its early stages, it may be difficult for a doctor to diagnose RA as it can
resemble other conditions. However, early diagnosis and treatment are
essential to slow the progression of the disease.
The CDC recommend diagnosis and an effective treatment strategy to begin
within 6 months of the onset of symptoms.
A doctor will look at the person’s clinical signs of inflammation and ask how
long they have been there and how severe the symptoms are. They will also
carry out a physical examination to check for any swelling, or functional
limitations, or deformity.

85. They may recommend some tests.
Blood tests
Erythrocyte sedimentation rate (ESR or sed rate) : This
test assesses levels of inflammation in the body. It
measures how fast red blood cells in a test tube separate
from blood serum over a set period. If the red blood cells
settle quickly as sediment, inflammation levels are high.
This test is not specific for RA and is a useful test for
other inflammatory conditions or infections.

86. C-reactive protein (CRP): The liver produces CRP. A higher CRP level
suggests that there is inflammation in the body. This test is not specific
for RA and CRP can occur in other inflammatory conditions or
infection.
Anemia: Many people with RA also have anemia. Anemia happens
when there are too few red blood cells in the blood. Red blood cells
carry oxygen to the tissues and organs of the body.

87. Rheumatoid : If an antibody known as rheumatoid factor is present in
the blood, it can indicate that RA is present. However, not everyone
with RA tests positive for this factor.
Imaging scans and X-rays
An X-ray or MRI of a joint can help a doctor identify what type
of arthritis is present and monitor the progress of RA over time

88. Diagnostic criteria
In 2010, the American College of Rheumatology recommended the
following criteria for diagnosing RA:
swelling is present in at least one joint, and it does not have another
cause
results from at least one blood test indicate the presence of RA
symptoms have been present for at least 6 weeks

89. Conditions with similar symptoms
The doctor will need to distinguish RA from other conditions with similar
symptoms, such as:
Gout
Osteoarthritis
lupus

90. Treatment
If a person receives a diagnosis of RA, the doctor may refer them to a
specialist known as a rheumatologist, who will advise on treatment
options.
There is currently no cure for RA, but treatment can help to:
reduce inflammation to the joints
relieve pain
minimize any loss of function caused by pain, joint damage, or deformity
slow down or prevent damage to the joints
Options include medications, physical therapy, occupational therapy,
counseling, and surgery.

91. Medications
Some drugs can help to relieve symptoms and slow disease progression.
Nonsteroidal anti-inflammatory drugs (NSAIDs): These are available over-the-
counter from pharmacies. Examples include Advil, Motrin, and Aleve. Long-
term use and high doses can lead to side effects, such as bruising, gastric
ulcers, high blood pressure, and kidney and liver problems.

92. Corticosteroids: These medications reduce pain and inflammation and
may play a role in slowing down joint damage, but they cannot cure
RA. If NSAIDs do not work, a doctor may inject a steroid into the
joint. Relief is usually rapid, but the effect is variable. It can last a few
weeks or months, depending on the severity of symptoms.
Corticosteroids can help with acute symptoms or short-term flare-ups.
Long-term use of corticosteroids can have serious side effects. These
include cataracts, osteoporosis, glaucoma, diabetes mellitus, and
obesity.

93. Occupational therapy
An occupational therapist can help the individual learn new and effective ways
of carrying out daily tasks. This can minimize stress to painful joints.
For example, a person with painful fingers might learn to use a specially devised
gripping and grabbing tool.
Surgery
If medication and physical therapy do not help, a doctor may recommend
surgery to:
repair damaged joints
correct deformities
reduce pain
The procedures are possible:

94. Diagnosis
Diagnosis of RA is based on joint involvement, symptom duration, and
serum studies, including rheumatoid factor and anti–cyclic
citrullinated protein (the most specific marker). Evaluation may also
include joint radiographs and complete blood count with differential,
C-reactive protein level, and erythrocyte sedimentation rate. Uric acid
level and liver and kidney function tests can help exclude other
etiologies or guide medication dosing.

95. Ocular involvement in rheumatoid arthritis
Dry Eye Syndrome
Dry eye syndrome, also known as
keratoconjunctivitis sicca, is the most com-
mon ocular manifestation of RA. It can occur
as a result of meibomian gland, lacrimal
gland, accessory lacrimal gland, or goblet
cell dysfunction.

96. Characteristics. Symptoms of dry eye syndrome include foreign body
sensation, burning, decreased visual acuity, photophobia, and
pruritus.
Diagnostic signs include less than 5 mm of tear extension on Schirmer’s
test, tear break-up time of 5 seconds or less, decreased lacrimal lake,
and staining with fluorescein, rose bengal, or lissamine green.

97. Up to 25% of patients with RA meet criteria for
secondary Sjögren syndrome, which is less com-
monly associated with xerostomia than is primary
Sjögren syndrome. Notably, different HLA genes
are associated with primary Sjögren syndrome and
Sjögren syndrome secondary to RA.2 Additionally,
the prevalence of positive anti-Ro antibodies and
anti-La antibodies is low in patients with RA, found
in one study to be 12.2% and 11.3%, respectively.3

98. Other considerations. The normal tear film has antimicrobial properties and
contains immunomodulatory factors such as collagenase inhibitors (e.g.,
alpha-2 macroglobulin). Patients with dry eye syndrome are deficient in these
protective mechanisms and have increased proteolytic enzymes such as
plasminogen activator in the tear film.4 Consequentially, dry eye syndrome
can lead to superficial punctate keratitis, filamentary keratitis, corneal ulcer,
and ultimately, corneal melt.1 Accordingly, RA is a relative contraindication
against refractive surgery, especially LASIK, due to its potential to exacerbate
dry eye syndrome.4 (See also “Surgical Considerations in RA Ocular Disease

99. Treatment
Treatment. Therapeutic modalities include lubricating drops and
ointments, topical cyclosporine, and oral pilocarpine. The recent
approval of lifitegrast has added a new medication to our
armamentarium of topical treatments. In-office procedures include
punctal occlusion, use of amniotic membrane, and tarsorrhaphy.

100. Episcleritis
Episcleritis is inflammation of the episclera, which lies
just beneath Tenon’s capsule. Although the disorder is
usually idiopathic, it can also be associated with RA
and other systemic diseases. It is bilateral 40% of the
time, and manifests with salmon-pink eyes and mild
pain. It can occur sectorally in a radial distribution; in
a diffuse pattern; and, less commonly, in a nodular
form. In contrast to scleritis, eye redness resolves with
topical phenylephrine.

101. Treatment
Episcleritis is usually self-limiting. Topical corticosteroids or oral
nonsteroidal anti-inflammatory drugs (NSAIDs) can be used for
symptomatic relief.

102. Scleritis
RA is the most common cause of scleritis, which is an
inflammation of the sclera characterized by vasodilation of
the superficial and deep episcleral vessels. Occasionally,
scleritis may present before the onset of joint symptoms in the
RA patient. It may also occur as a manifestation of systemic
vasculitis. Scleritis occurs bilaterally 40% to 50% of the time
and is classified as anterior or posterior based on its position
relative to the ora serrata.

103. Types of scleritis
. Anterior scleritis can be nodular, diffuse, or necrotizing. It usually
presents with intense pain amplified by eye movements, blurry vision,
photophobia, and tearing .
nodular diffuse

104. ANTERIOR SCLERITIS. Slit-lamp photo displays dilation
of the episcleral vessels in a patient with underlying RA.

105. Scleromalacia perforans describes anterior necrotizing scleritis without
inflammation ,This condition often presents without pain, despite its
ability to lead to visual loss, astigmatism, and globe perforation.
SCLEROMALACIA PERFORANS. Slit-lamp photo of the
left eye shows severe superotemporal scleral thinning with
visible choroid bulging anteriorly, but with minimal
inflammation.

106. Posterior scleritis is characterized by the “T” sign on B-scan. This
classic sign is produced by hyporeflectivity from both the optic nerve
and fluid under Tenon’s capsule.

107. Other considerations. The presence of scleritis in patients with RA is
associated with increased mortality.1 Posterior scleritis can cause
serous retinal detachment or chorioretinal folds. Other sequelae
associated with scleritis include uveitis, cataracts, glaucoma, posterior
segment damage, and peripheral ulcerative keratitis.

108. Treatment
Topical medications are generally not effective in the treatment of scleritis.
Therapy should focus on managing the underlying RA, with a
multidisciplinary team approach involving the patient’s primary care
physician and rheumatologist. Oral NSAIDs are most effective in mild cases
and for nodular anterior scleritis.
For more severe disease, the treatment of choice is oral corticosteroids (1
mg/kg/day of prednisone equivalents), with an optional bolus of
methylprednisolone 1 g daily for 3 days.

109. Depending on response and severity of scleritis at presentation,
additional systemic medications include methotrexate, azathioprine,
mycophenolate mofetil, cyclosporine, and biologic agents such as
infliximab, adalimumab, rituximab, and anakinra. Immunologic
agents, most commonly intravenous or oral cyclophosphamide, can be
added for necrotizing disease and refractory cases.1
For necrotizing scleritis, exploration and debridement with tectonic
patch grafting may be necessary for severe thinning or perforation.4

110. Peripheral Ulcerative Keratitis
Peripheral ulcerative keratitis (PUK) is a type of corneal melt that
occurs when immune complexes infiltrate the vascular arcades in the
0.5-mm corneal periphery. RA is the most common etiology,
accounting for 34% of cases; however, it can also be caused by other
autoimmune, infectious, neurologic, and dermatologic conditions.5 In
the setting of severe RA, PUK is usually accompanied by other ocular
manifestations, and it can occur secondary to scleritis, especially
necrotizing scleritis.1,5

111. PUK. Crescent-shaped ulcers are juxtaposed against the
limbus.

113. Characteristics
PUK presents with stromal thinning and a secondary overlying epithelial defect
and is characterized by neovascularization of the corneal periphery and
crescent-shaped juxtalimbal ulcers .5 It is unilateral 60% of the time.1 It can
present with pain, photophobia, tearing, and decreased visual acuity due to
astigmatism or corneal opacity.
The differential diagnosis includes Mooren ulcer, Terrien marginal de-
generation, herpetic keratitis, corneal ulcer secondary to dry eye, phlyctenular
ulcers, and marginal keratitis. Corneal perforation is a feared complication.

114. Other considerations. Like necrotizing scleritis, PUK is associated with
increased mortality in patients with RA, and it can be a sign of impending
systemic vasculitis.1
Treatment:The treatment strategy should prioritize management of the
underlying RA. Systemic corticosteroids with or without immunologic agents
are the mainstay of treatment. These agents may include oral mycophenolate
mofetil, methotrexate, azathioprine, leflunomide, infliximab, adalimumab, or
rituximab. Oral cyclosporine can be used if there is no underlying systemic
vasculitis. Oral cyclophosphamide can be used in cases unresponsive to treat-
ment, while etanercept is less effective.

115. Ocular support
Additional measures to help protect the cornea include topical
medroxyprogesterone and acetylcysteine, which act as collagenase inhibitors,
and oral tetracycline, which inhibits matrix metalloproteinase.5
Treating coexisting dry eye syndrome can bolster the tear film to conserve
corneal integrity and prevent keratolysis. However, topical steroids can
increase risk of perforation and should be avoided.2 Tissue adhesives such as
cyanoacrylate glue; amniotic membrane; bandage contact lenses; and tectonic,
lamellar, or penetrating keratoplasty can be used to prevent and manage
perforation.
Although controversial, perilimbal conjunctival resection may remove the
immune complexes at their entry point into the cornea.5

116. Surgical Considerations in RA Ocular Disease
Surgical trauma can initiate macrophage and neutrophil infiltration,
resulting in a progressive immunologic response and the deposition of
immune complexes. When deposited in the cornea, these immune
complexes contribute to a keratolytic response. During the initial
inflammatory process, fibroblasts are recruited and activated,
producing complement component C1 and contributing to the
activation of the classical complement cascade. The surgical trauma
itself may compromise ocular immune privilege and incite immune
responses due to molecular mimicry

117. Additionally, the risk of postsurgical sterile corneal melt is higher in the
setting of RA and coexisting dry eye syndrome, so treatment of the RA
and surface disease should be optimized prior to any ocular surgery.
Perioperative oral prednisone should be considered in patients with
known RA. If necrotizing scleritis occurs after ocular surgery without
known underlying RA or other systemic collagen vascular diseases,
these entities should be suspected.1

118. References
1.https://www.hindawi.com/journals/ad/2012/290898/
Ocular Manifestations of Systemic Lupus Erythematosus: A Review of the Literature
Neal V. Palejwala,1 Harpreet S. Walia,1 and Steven Yeh1
2.https://www.mayoclinic.org/diseases-conditions/lupus/diagnosis-treatment/drc-20365790
3.https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4908056/
Ocular findings in systemic lupus erythematosus
Samir S. Shoughya,⁎ and Khalid F. Tabbaraa,b,c
4.https://www.medicalnewstoday.com/articles/323653#alternative-remedies
5.https://www.reviewofophthalmology.com/article/posterior-segment-findings-in-sle
6.www.medicalnewstoday.com
7.www.aao.org

119. Thanks for
your Attention