Eye and Rheumatologic Diseases

• The presentation is about eye and rheumatologic diseases, by Karen Gil MD, MHSN

Ocular Disorders and Rheumatologic Associations

• Dry eye (keratoconjunctivitis sicca) is associated with rheumatoid arthritis, SLE, scleroderma, primary Sjogren's syndrome, and graft-vs-host disease
  • Symptoms include ocular burning, itching, watering, foreign body sensation, and corneal opacification/ulceration
• Episcleritis is associated with idiopathic conditions (50%), rheumatoid arthritis, vasculitis, and inflammatory bowel disease
  • Symptoms include self-limited mild pain/discomfort, and diffuse or segmental redness; can be nodular
• Scleritis is associated with psoriatic arthritis, Bechet's disease, and Wegener's granulomatosis
  • Causes deep boring pain; injection of deep scleral vessels, may be nodular (violaceous nodules), may be necrotizing and may lead to corneal opacification
• Keratitis (corneal disease) is associated with Sjogren's syndrome, rheumatoid arthritis, vasculitis (Wegener's granulomatosis), SLE, Bechet's syndrome, IBD, psoriatic arthritis, and sarcoidosis
  • Symptoms include corneal opacification, peripheral corneal thinning, decreased vision, pain, injection, and photophobia
• Retinal vasculitis is associated with SLE, polyarteritis nodosa, Bechet's disease, Wegener's granulomatosis, sarcoidosis, Sjogren's A antigen, rheumatoid arthritis, HLA-B27 associated uveitis, Crohn's disease, postvaccination, dermatomyositis, Takayasu's disease, and polymyositis
  • Causes loss of vision
• Optic neuropathy is associated with giant cell arteritis, SLE, and antiphospholipid antibodies syndrome
  • Causes vision loss, central or peripheral

Uveitis

• Uveitis has several rheumatologic associations listed in decreasing incidence
• Anterior uveitis is associated with idiopathic conditions, juvenile rheumatoid arthritis/juvenile idiopathic arthritis (JRA/JIA), ankylosing spondylitis, HLA-B27 positive non-specific arthropathy, sarcoidosis, reactive arthritis (Reiter's syndrome), systemic lupus erythematosus (SLE), inflammatory bowel disease (IBD), psoriatic arthropathy, rheumatoid arthritis (RA), Wegener's granulomatosis, relapsing polychondritis, and Kawasaki syndrome
• Posterior uveitis is associated with idiopathic conditions, SLE, sarcoidosis, Bechet's disease, temporal arteritis, polyarteritis nodosa, and Wegener's granulomatosis
• Panuveitis is associated with idiopathic conditions, sarcoidosis, Behçet's disease (more common in Turkey and China), SLE, Vogt-Koyanagi-Harada syndrome (more common in China), polyarteritis nodosa, dermatomyositis, and progressive systemic sclerosis
• Intermediate uveitis is associated with idiopathic conditions, sarcoidosis, and multiple sclerosis

Anterior Uveitis and Systemic Disease

• Reiter's syndrome is characterized by: "Can't see, can't pee, can't climb a tree"
• Ankylosing spondylitis
  • Affects the lower vertebrae leading to bony fusion
  • Etiology is possibly genetic
  • More common in young, white or Black males aged 20-40
  • Key symptoms are lower back pain
  • Key lab tests are HLA-B27 positives, L-S x-ray showing bamboo spine and sacroiliitis, normal RF, and elevated ESR
  • Treatment: topical steroids, topical cycloplegics, oral NSAIDs, oral steroids, and physical therapy
  • Sequelae include limited mobility of the back and legs
• Reiter's syndrome (reactive arthritis)
  • Affects the bone joints leading to arthritis
  • Associated with possible infection of chlamydia, shigella, salmonella, or campylobacter
  • More common in young, white or Black males aged 20-40
  • Key symptoms are arthritis, urethritis, conjunctivitis and uveitis, painless ulcers, and keratoderma blenorrhagica
  • Key lab tests are HLA-B27 positive, normal of negative ANA and RF, elevated ESR Treatment: topical steroids, topical cycloplegics, and oral steroids, oral tetracycline depending on the cause
  • Sequelae include advanced arthritis, severe corneal keratitis (meltdown)
• Juvenile rheumatoid arthritis
  • Affects the joints, especially the knee area leading to arthritis
  • Etiology is potentially autoimmune
  • More common in white or Black females under 16, especially at 6 y/o
  • Key symptoms are knee pain and joint swelling
  • Key lab tests are X-ray of the knee, negative RF and positive ANA
  • Treatment: topical steroids, topical cycloplegics, oral steroids, oral NSAIDs, and oral immunosuppressants
  • Sequelae include phthisis bulbi and knee joint immobility
• Crohn's Disease
  • The system affected is the GI tract
  • The pathway for this disease is ulcers with multifocal areas
  • Possible genetic association
  • Occurs in white or black, male or female, aged 20-40
  • Look for pain in the gut
  • Testing includes a GI workup, endoscopy, rectal biopsy, and barium enema, often HLA-B27 +
  • Treat with topical steroids, cycloplegics, and oral steroids, along with surgery, diet, and stress reduction
  • Sequelae include severe GI complications
• Sarcoidosis
  • System Affected: Pulmonary
  • Pathway: Granulomas
  • Etiology may be the immune response to airborne antigens
  • Patients are usually African-American males under 50
  • A key symptom is a cough
  • Diagnose with lung biopsy and chest x-ray, lung CT NODULE, elevated serum ACE
  • Treatment includes topical steroid and cycloplegics
  • Sequelae: 5% mortality and respiratory distress
• Bechet's Disease
  • System Affected: Multisystemic
  • Pathway: Ulcers
  • Trigger Antigens: HSV, Strep, Staph, E. Coli
  • Middle Eastern Males, 20-40 y/o
  • Key symptoms are a triad of mouth ulcers, genital ulcers, and uveitis with hypopion
  • Diagnose with the HLA-B5 test
  • Treatment includes topical steroids, topical cycloplegics, Oral steroids and oral immunosuppressants
  • Sequelae: Mortality 5-10 y
• Lyme disease:
  • Affects multi-derm and cardiac-arthritis
  • Inflammation due to bacterial spirochete infection (Borrelia Burgdorferi)
  • Symptoms: rash, fever, arthritis
  • Testing: Check for anti-spirochetal antibody titer (ELISA)
  • Treat with cycloplegics, steroids
  • Use tetracycline, doxycycline, or amoxicillin
  • Sequelae: Advanced cardiopathy and neuropathy
• Syphilis:
  • Affects multi-derm and neuro
  • Inflammatory infection
  • Symptoms: Rash, fever, Chancre
  • Testing: FTA-ABS VDRL
  • Treat with Cycloplegic and Steroids (Systemic Penicillin)
  • Sequelae: Tertiary stage with Neuropathy
• Tuberculosis:
  • Affects the pulmonary system
  • Bacterial infection
  • Symptoms: Cough
  • Testing: PPD Mantoux, Chest X-ray, Sputum Culture
  • Treat with Cycloplegic and Steroids INH Isoniazid
  • Sequelae: Lung Excavations and Cavitation
• Herpetic Disease:
  • Affects the multi-derm
  • Viral infection DNA virus
  • Symptoms: Skin Vesicles
  • Testing: Skin Biopsy, Skin Culture, Consider HIV Testing
  • Treat with Cycloplegic and Steroids and Acyclovir
  • Sequelae: Post herpetic Neuralgia

Treatment for Uveitis

• Corticosteroids are the main treatment for uveitis
• Generally, topical steroid therapy should be started at high doses then slowly tapered
• Mild inflammation: administer q6h
• Moderate to severe inflammation: administer q1-2h
• A loading dose can be considered (e.g. q1min for 5 minutes, then q30min-1h)
• A steroid ophthalmic ointment given at night can also be considered
• A commonly adopted regimen might consist of:
  • One drop q1h for 3 days, then
  • One drop q2h for 3 days, then
  • One drop q6h for 3 days, then
  • One drop q8h for 7 days, then
  • One drop q12h for 7 days, then
  • One drop qday for 7 days, then stop
• Mydriatic and cycloplegic agents are utilized in uveitis to relieve symptoms of pain and photophobia
• Mydriatic agents are used to prevent or break posterior synechiae
• The choice of agent and frequency of use is tailored to the severity and course of the inflammation
• Preparations of short-acting and long-acting agents:
  • Tropicamide 0.5% and 1% last 6 hours
  • Cyclopentolate 0.5% and 1% last 24 hours
  • Phenylephrine 2.5% and 10% lasts 3 hours but no cycloplegia
  • Homatropine 2% lasts up to 2 days
  • Atropine 1% lasts up to 2 weeks

Lab Tests

• HLA-B27 Positive stands for CRAP
  • C for Chron's disease
  • R for Reactive arthritis (Reiter's)
  • A for Ankylosing spondylitis
  • P for Psoriatic arthritis
• HLA-B5 is for Bechet's disease

Systemic Lupus Erythematosus (SLE)

• Autoimmune Diseases
• More common in females; the ratio is F>M 10:1
• Typical age of onset is 2nd or 3rd decade of life
• ANA test +
• Multisystemic affection
  • Skin: Butterfly rash (malar rash) Discoid lupus, photosensitivity
  • Kidneys: Renal disorders
  • Joints: Arthritis (joint pain 90%)
  • Heart: Hemolytic anemia
  • Neurological: Seizures
• Ocular Symptoms
  • Dry eye
  • Recurrent episcleritis
  • Peripheral keratitis (infiltrates)
  • Photophobia
  • Papilledema

Rheumatoid Arthritis (RA)

• Systemic Inflammatory disease
• F>M
• 40-50 y
• Symmetrical arthritis
• Multiple joints
• Destruction of articular cartilage
• Symptoms worse in the morning
• Pain in hands, wrists, feet or small joints
• Lab
  • RF +
• Approximately 25% of the patients present RA ocular manifestations
  • Keratoconjunctivitis sicca 14-25%
  • Scleritis 15%
  • Scleromalacia Perforans (necrotizing scleritis without inflammation)
  • Peripheral corneal ulceration
  • No pain*
  • Choroiditis
  • Retinal vasculitis-Less Common
  • RD
  • Macular Edema

Psoriatic Arthritis

• Asymmetric peripheral small joint pain
• Skin psoriatic lesions
  • sharply demarcated, scaly, erythematous plaques
  • Knees (extensor surface)
  • Elbows (extensor surface)
  • Scalp
• 7% develop anterior uveitis
• HLA-B27 positive
• Treatment
  • UV-B light exposure
  • Methotrexate