Connective Tissue Disease

Question 1

Overview- what is Rheumatoid Arthritis (RA)?

Answer 1

Inflammatory arthritis associated with wrist and hands. Inflammation and thickening of the joint capsule. Also may impact bone and cartilage.

Question 2

Difference between Rheumatoid arthritis and osteoarthritis

Answer 2

Rheumatoid- autoimmune disease where self Ab attacks joints.

Osteo- Due to mechanical wear and tear of joints.

Question 3

Who is more likely to have RA?

Answer 3

• Women 3x more likely

- Genetic predisposition

Question 4

Symptoms of RA

Answer 4

pain and swelling in joints. Worsens after rest.

Question 5

How to diagnose RA

Answer 5

Rheumatoid factor, erythrocyte sedimentation rate (ESR), C-reactive protein, Anti CCP

Question 6

Ocular implications of RA

Answer 6

-Dry eye is the most common. (Women are 9x more commonly affected)
-Scleritis: Deep intolerable boring pain, redness, photophobia and tenderness
-Episcleritis: Painless red eye, minimal vision impact.
Peripheral ulcerative keratitis- Thinning of cornea that may lead to ulcers and loss of the eye

Question 7

Treatment of RA

Answer 7

• Steroids to suppress immune system, plaquenil (side effect of building up in cells and causing toxicity to photoreceptor cells)
• Scleral lenses, preservative free artificial tears, restates to help dry eye

Question 8

Sjogren Syndrome (SS) Overview

Answer 8

Excessive dryness of eyes, mouth, and other mucous membranes. Due to WBC damaging glands such as lacrimal, or salivary.

Question 9

SS diagnosis

Answer 9

Labs- antinuclear Ab, Sio (blood work), biopsy of salivary glands

Question 10

Who is most likely to have SS

Answer 10

Middle age women, with lower estrogen levels. Often comes along with other autoimmune diseases.

Question 11

SS symptoms

Answer 11

Vary greatly as can be associated with RA, SLE, more

Question 12

ocular implications of SS

Answer 12

Dry eye syndrome is the most common

-SPK (tight junctions in between cells are unstable) or ulceration also possible.

Question 13

Treatment of SS

Answer 13

-Steroids, artificial tears, restasis, gum to enhance salivary production (no excess water).

Question 14

SLE (lupus) overview

Answer 14

Problems in joints, nervous system, blood, skin, kidneys,Gi tract, lungs.
Other tissues and organs may be impacted

Question 15

Diagnosis of SLE

Answer 15

Antinuclear antibody test (ANA) and blood counts. (low RBC, WBC, and platelets.)

There are 17 criteria for classification of the disease. To be diagnosed, patients must have 4 of the 17 classifiers.

Question 16

Who is mostly likely to have SLE

Answer 16

Women of childbearing ages (20-30). More common in African American and asian women.

Question 17

Treatment of SLE

Answer 17

Steroids (immune suppression) Consequences- weight gain, sugar level increase

Question 18

Symptoms of SLE

Answer 18

Very hard to diagnose. Pt must have 4/17 symptoms.

Question 19

Ocular implications of SLE

Answer 19

Dry eye is most common- Range from SPK to ulceration.
Retina is the 2nd most commonly involved- vasoclusive retinopathy such as cotton wool spots, micro aneurysms, neovascularization.

Question 20

Marfan Syndrome overview

Answer 20

Genetic disease resulting in a decreased number of functional fibril. (Fibrillin typically is composed of elastin in the center and fribrillin protein that forms microfibrils around the core. It is stretchy.

Question 21

Marfan Syndrome characteristics

Answer 21

Tall and thin
long arms, legs, fingers, and toes
flexible joints
Scoliosis (sideways curve to spine)

Question 22

Marfan syndrome impacts

Answer 22

• bones
• eyes: lens dislocation due to weak Zonules. lens most often goes up and out.
• Lungs
• Heart: Mitral valve prolapse, aortic aneurysm due to weakening in the artery wall. Causes rupture.

Question 23

Ehler-Danlos Syndrome (EDS) Overview

Answer 23

Collagen disorder that is a defect in the structure or processing of collagen.

Question 24

Characteristics of EDS

Answer 24

Joint hyper flexibility, stretchy skin, hyper extensibility

This indicates that joints will wear out quicker.

Question 25

EDS impacts to musculoskeletal system, skin, cardiovascular system, and ocular system?

Answer 25

Musculoskeletal- Hyper flexible joints, joint disease, tearing of tendons or muscles, spin deformities

Skin- Fragile, tears easily. Skin folds, easy bruising.

Cardiovascular- Dilation of rupture of ascending aorta, heart murmur

Ocular- High myope, lens dislocation, keratoconus, strabismus, angled streaks (streaks coming out from optic nerve in fungus photo)