ocular manifestations of autoimmune disease.pptx

Autoimmune diseases causing eye problems,
Different types of autoimmune diseases shows different types of eye diseases,
Let’s learn.

1. Ocular Manifestations Of Autoimmune Diseases Dr. SHRIST I AGARWAL J unior Resident 2 Department of Ophthalmology

2. Autoimmune Disorders

3. CONNECTIVE TISSUE DISORDERS (Collagen Vascular Diseases) Dry Eyes are the most common manifestation (keratoconjunctivitis sicca)

4. Sjogren Syndrome Triad- dry eyes, dry mouth and parotid gland enlargement Classified as primary when occur in isolation and secondary when associated with another disease, such as RA or SLE. Specific antibodies- anti-SS-A / anti- SS-B Most common ocular symptoms- feeling of dryness, grittiness and burning that characteristically worsens over the course of the day. Other symptoms- stringy discharge, transient blurring of vision, redness and crusting of lids, lack of emotional or reflex tears Severe tear deficiency may result in corneal ulceration and perforation

5. Ankylosing Spondylitis Acute anterior uveitis is the most common ocular association and occurs in about 25 % of patients with AS. Either eye is frequently affected at different times but bilateral simultaneous involvement is rare. No correlation between the severity and activity of eye and joint involvement. Other ocular features include scleritis, episcleritis, keratitis and mechanical ptosis SPONDYLOARTHROPATHIES Patients with uveitis secondary to the various spondyloarthropathies are often HLA B27 positive

6. Also known as Reiter syndrome, characterized by a triad of non-specific urethritis, conjunctivitis and arthritis. Eye is involved in 50% of cases with a urogenital inciting infection and 75% of enteric ReA syndrome Conjunctivitis is very common, classically follows urethritis but precedes arthritis. Inflammation is usually mild, bilateral, and mucopurulent with a papillary and/or follicular reaction. Spontaneous resolution occurs within 7-10 days AAU occurs in 20 % Episcleritis sometimes occurs. Reactive arthritis Psoriatic arthritis AAU occurs in 7%. Conjunctivitis, marginal corneal infiltrates and secondary Sjogren syndrome may occur

7. Rheumatoid Arthritis It is the most common systemic association of Scleritis and can manifest with any form of immune-mediated scleral inflammation Other ocular manifestations include: Keratoconjunctivitis sicca Peripheral ulcerative keratitis Acquired superior oblique tendon sheath syndrome (very rare)

8. Scleromalacia Perforans

9. Juvenile Idiopathic Arthritis (JIA) It is the most common systemic disease associated with childhood anterior uveitis. Anterior uveitis: common in oligoarticular JIA, asymptomatic. Inflammation is chronic and non-granulomatous. Both eyes are affected in 70% and there is symmetrical severity of inflammation Band keratopathy and cataract are common in severe cases Other serious complications : glaucoma, amblyopia, maculopathy, cyclitic membrane and phthisis

10. Systemic Lupus Erythematosus Keratoconjunctivitis sicca: Most common ocular manifestation Peripheral ulcerative keratitis Episcleritis Scleritis Anterior uveitis Retinal vasculitis Optic neuritis/ neuropathy Orbital inflammation/myositis/proptosis Madarosis Peripheral ulcerative keratitis in the absence of other ocular disease should prompt investigation for a systemic autoimmune disease

11. Polyarteritis nodosa: Ocular manifestation may precede systemic manifestations by several years Dry eyes Peripheral ulcerative keratitis Scleritis (anterior necrotizing scleritis with inflammation) Acute anterior uveitis Occlusive retinal periarteritis Orbital pseudotumor Behcet’s disease Hypopyon uveitis Arthritis Oral ulcers

12. Sarcoidosis Ocular inflammation occurs in 25-70% of sarcoid patients Granulomatous anterior uveitis is the most common manifestation 7 key signs in the diagnosis of intraocular sarcoidosis: Mutton fat KPs Trabecular meshwork nodules Vitreous opacities- snowballs and/or ‘string of pearls’

13. Multiple chorioretinal peripheral lesions Optic disc nodule/ granuloma and/or solitary choroidal nodule

14. Nodular and/or segmental periphlebitis (+ ‘candle wax drippings’) and/or retinal macroaneurysm in an inflamed eye Other ocular manifestations may include conjunctival nodules, lacrimal gland infiltration, dry eye, eyelid nodules and orbital and scleral lesions

15. Giant Cell Arteritis Diplopia AAION ( Arteritic anterior ischaemic optic neuropathy)- affects 30-50% of untreated patients with GCA, of whom one-third develop involvement of the fellow eye, usually within a week Ocular motor palsies, including a pupil involving third nerve palsy

16. Thyroid Eye Disease (TED) Also known as thyroid-associated orbitopathy and Graves ophthalmopathy Most common cause of both bilateral and unilateral proptosis in an adult More common in females Risk factors for ophthalmopathy: once a patient has graves ds, the major clinical risk factor for developing TED is smoking radioactive iodine used to treat hyperthyroidism can worsen TED can also occur in euthyroid and hypothyroid patients

17. Clinical Features: Congestive(inflammatory) stage : eyes are red and painful; usually remit in 1-3 years Fibrotic (quiescent) stage : eyes are white, although a painless motility defect may be present Broadly clinical features can be categorized as: Soft tissue involvement Lid retraction Proptosis Optic neuropathy Restrictive myopathy

18. Soft Tissue involvement: Symptoms: grittiness, red eyes, lacrimation, photophobia, puffy lids and retrobulbar discomfort Signs: Epibulbar hyperaemia Periorbital swelling ( may be associated with chemosis and prolapse of retroseptal fat into the eyelids) Tear insufficiency and instability Corneal signs : punctate epithelial erosions, superior limbic KC

19. Lid Retraction : Retraction of upper and lower lids occurs in about 50% of patients with graves ds Symptoms: staring or bulging eyed appearance, difficulty closing eyes Signs: Dalrymple sign : lid retraction in primary gaze Kocher sign : staring and frightened appearance of the eyes Von graefe sign : retarded descent of the upper lid on downgaze (lid lag)

20. Proptosis: Symptoms are similar to those of lid retraction. Signs: Axial, unilateral or bilateral, symmetrical or asymmetrical and frequently permanent. Severe proptosis may compromise lid closure and along with lid retraction and tear dysfunction can lead to exposure keratopathy, corneal ulceration and infection

21. Assessment of Proptosis on CT Scan: The reference line for measurement of proptosis is the interzygomatic line ( a line is drawn at the anterior portions of the zygomatic bones) The upper limit of normal distance from this line to the anterior surface of the globe is 23 mm, above which indicates proptosis The lower limit of normal distance from this line to the posterior surface of the globe is 5.9mm, below which indicates proptosis.

22. Restrictive myopathy: Symptoms: double vision and often discomfort in some positions of gaze Signs : {in sequence of occurrence} Elevation defect- caused by fibrotic contracture of inferior rectus ; most common motility defect Abduction defect- due to fibrosis of medial rectus Depression defect- secondary to fibrosis of superior rectus Adduction defect- fibrosis of lateral rectus Only the muscle belly is involved and not tendon

23. Optic neuropathy: In TED, compression of the optic nerve secondary to enlarged and congested rectus muscles may occur in the absence of proptosis VA is usually reduced Colour desaturation is a sensitive feature Diminished light brightness appreciation Relative afferent pupillary defect may be present Visual field defects can be central or paracentral and may be combined with nerve fibre bundle defects Optic disc may be normal, swollen or rarely atrophic

24. Immunosuppression in the active inflammatory phase of thyroid eye disease is important to reduce acute inflammation and to prevent long term complications Surgical orbital decompression should be considered when there is sight- threatening compressive optic neuropathy in thyroid eye disease

25. Myasthenia Gravis Myasthenia gravis may be Ocular, Bulbar (affecting cranial nerves arising from the lower brainstem) or Generalized Ocular myasthenia: Ocular involvement occurs in 90% of cases and is the presenting feature in 60%. Ptosis : insidious, bilateral and frequently asymmetrical. Typically, worse at the end of the day Worse on prolonged (60 second) upgaze due to fatigue. Cogan twitch sign is a brief upshoot of the eyelid as the eyes saccade from depression to the primary position If one eyelid is elevated manually as the patient looks up, the fellow eyelid may show fine oscillatory movements

26. Diplopia: frequently vertical, although any or all of the extraocular muscles may be affected. Pseudo-internuclear ophthalmoplegia may be seen Patients with stable deviations may benefit from muscle surgery, botulinum toxin injection or a combination of both Nystagmoid movements: May be present on extremes of gaze.

27. Investigations: Ice pack test: improvement after an ice pack is placed on the ptotic eyelid for 2 minutes , as cold inhibits the breakdown of acetylcholine by acetylcholinesterase. Antibody testing : AchR antibodies : present in around 90% of systemic cases but only 50-70% of patients with ocular myasthenia. MuSK protein antibodies are positive in 50% of those negative for AchR antibodies; positive patients are less likely to have ocular features Striational antibodies Edrophonium ( Tensilon ) test: sensitivity of 85% in ocular and 95% in systemic MG Patients with myasthenia gravis should undergo thoracic imaging to exclude a thymoma

28. Other tests: Electromyography Muscle biopsy : reveals neuromuscular junction antibodies and characteristic electron microscopy features Thoracic imaging { MZR, CT, CT/PET} to detect thymoma Thyroid function testing

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