Ocular manifestations of systemic diseases

1. Ocular Manifestations Of
Systemic Diseases
DR. IFRAD TASNIM
Resident ( phase-A)
Department of ophthalmology

2. INTRODUCTION
 The eyes are frequently involved in diseases
Affecting the rest of the body.
 Ocular manifestations in certain multisystem
Disorders may offer a diagnostic clue.

3. Eye examination
 Vision
 External – Look at eyelids, make sure everything looks normal.
 Pupils
 Relative afferent papillary defect
 See if pupils are reacting normally
 Motility – Have the patient look up, down, right and left.
 Exam of anterior segment
 Dilated ophthalmoscopy – Examine retina, optic nerve, and vessels.
 Visual fields – Place you finger in different places in patient’s visual field and
ask him if he sees it. Test one eye at a time.

4. INVOLVED SYSTEMIC DISEASES
1. Connective tissue diseases
2. Spondyloarthropathies
3. Digestive-inflammatory bowel
disease
4. Multisystem non-infectious
diseases
5. Infectious disease
6. Mucocutaneous disease
7. Cardiovascular disease
8. Endocrine-metabolic disease
9. Myopathies
10. Neurologic diseases
11. Haematological disease

5. Connective Tissue Diseases
A. Rheumatoid arthritis
 Keratoconjunctivitis sicca
 Episcleritis
 Scleritis
 Peripheral ulcerative keratitis
 Uveitis

6.  Rheumatoid Episcleritis  Rheumatoid Scleromalacia Perforans
 Rheumatoid Corneal UlcerationRheumatoid Scleritis

7. Juvenile Idiopathic Arthritis
 Idiopathic Arthritis in
patients younger than 16
years and lasts at least 6
weeks.
 Classification
Oligo or pauciarticular
Polyarticular RF + /
RF-
Psoriatic
Enthesitis
 Risk factor for uveitis
Early onset
female
ANA +
oligoarticular
HLA-DR5

8. Juvenile Idiopathic Arthritis
Ocular feature-
1. AU- chronic and granulomatous
2. Posterior synechia
3. Band keratopathy
4. Cataract
5. Glaucoma

9. C. Systemic Lupus Erythematosus (Lupus)
Ocular feature
Dry eyes
 Scleritis
Peripheral corneal ulcers
 Retinopathy
Optic neuropathy

10. D. Wegener Granulomatosis

11. E. Sjogren Syndrome

12. Sjogren Syndrome

13. F. Giant Cell Arteritis

14. G. Marfan syndrome

15. 2. Spondyloarthropathies
A. Ankylosing spondylitis

16. B. Reiter syndrome/Reactive arthritis
Keratoderma blennorrhagica in reactive arthritis

17. 3. Inflammatory Bowel Disease
a) Crohn’s disease
b)Ulcerative colitis
AAU
Dry eye
Conjunctivitis
Scleritis
Episcleritis

18. 4. Multisystem noninfectious diseases
A. Sarcoidosis  Respiratory symptoms
 Skin lesion
 Lympadenopathy
 Cardiac disease
 Neurologic disease

19. Sarcoidosis
Ocular feature  Granulomatous anterior uveitis
 Mutton fat KP
 Iris nodules (koeppe / Busaca)
 TM nodules
 Vitrious opacities (snow ball)
 Periphlebitis ( candle wax drippings)
 Optic disc nodules
 Dry eye
 Bilaterality
Very large granulomatous ‘mutton fat’ keratic precipitates

20. Sarcoidosis
granulomata and periphlebitis
‘Candle wax drippings’snowballs
Lacrimal gland enlargement in sarcoidosis

21. B. Behçet disease
 Criteria for Behçet's disease:
Mouth sores (oral ulcers) at least 3 times in 12 months
Any 2 of the following:
Recurring genital sores/ulcers
Uveitis
Skin: Pustules or erythema nodosum
Positive pathergy (skin prick test)
Major aphthous ulceration superficial thrombophlebitis

22. Behçet disease
 Ocular feature  AAU
 Vitritis
 Retinitis
 Retinal vasculitis
 Optic disc oedama
 Optic atrophy
Hypopyon in a white eye
occlusive vasculitis

23. C. Vogt Koyanagi Harada syndrome
Vitiligo and poliosis in Vogt–Koyanagi–Harada
syndrome ‘Sunset glow’ fundus

24. 5. Infectious disease
A. Tuberculosis
 ocular feature
 Anterior uveitis – granulomatous
 Vitritis
 Choroidal granuloma
 Choroiditis
 Retnal vasculitis
 Lupus vulgaris
Choroidal granuloma
Occlusive tuberculous periphlebitis. -Superior retinal branch
occlusion

25. B. Syndrome acquired immunodeficiency (SAID)
Kaposi sarcoma HIV wasting syndrome HIV microangiopathy

26. C. Syphilis
 Ocular features  Anterior uveitis
 Chorioretinitis
 Acute syphilitic posterior placoid
chorioretinopathy (ASPPC)
 Retinitis
 Optic neuritis and neuroretinitis
 Argyll Robertson pupils
Maculopapular rash in
secondary disease;
old multifocal chorioretinitis
acute posterior placoid chorioretinitis

27. D. Toxoplasmosis
 Ocular features  ‘Spill-over’ anterior uveitis
 single inflammatory focus of fluffy
white retinitis or retinochoroiditis
(‘satellite lesion’)
 Vitritis
 Vasculitis
 Optic disc oedema
 Visual loss
Typical ‘satellite’
lesion adjacent to an old scar
severe vitreous haze and
‘headlight in the fog’
appearance of lesion
Macular involvement

28. 6. Mucocutaneous disease
A. Pemphigoid
syndromes
 Ocular feature
 Bilateral conjunctivitis
 Symblepharon
 Dry eye
 Trichiasis
 Ankyloblepharon
 Corneal opacities
moderate fibrosis with forniceal
shortening and symblepharon formation
ankyloblepharon
severe skin blistering

29. B. Stevens-Johnson syndrome
Haemorrhagic lid crusting severe acute conjunctivitis pseudomembrane
Corneal keratinization extensive purpuric lesions Haemorrhagic lip crusting

30. 7. Cardiovascular disease
 Grade 1. Mild generalized retinal arteriolar narrowing
 Grade 2. Focal arteriolar narrowing and arteriovenous nipping
(copper wiring)
 Grade 3. Grade 2 plus retinal haemorrhages (dot, blot, flame), hard
exudates (‘macular star’) and cotton wool spots.
 Grade 4. Severe grade 3 plus optic disc swelling.this is a marker of
malignant hypertension.

31. Hypertensive retinopaty
Generalized arteriolar attenuation focal arteriolar attenuation red-free photograph showing
arteriovenous nipping
copper wiring grade 3 retinopathy with macular star grade 4 hypertensive retinopathy

32. B.Carotid stenosis

33. 8. Endocrine-metabolic disease
A. Diabetes mellitus
Microaneurysms Dot & blot VS splinter hemorrhage Hard exudate

34. Diabetic retinopathy
Hard Exudate VS Cotton Wool Spot
Venous beading
Intra-retinal microvascular abnormalities (IRMA)

35. Clinical Significant Macular Edema
(CSME)
1 of 3
Retinal edema
within 500 microns
of centre fovea
Hard exudates within
500 microns of fovea
if ass with adjacent
retinal thickening
Retinal edema > 1 disc
diameter, any part is
within 1 disc diameter
of centre of fovea

36. Diabetic retinopathy
Vitreous hemorrhage
Tractional retinal detachment

37. Rubeosis iridis
(neovascularisation of the iris)
Neovascular glaucoma

38. B. Thyroid eye disease
 CLINICAL MANIFESTATION
 5 main clinical manifestations of TED are:
I. Soft tissue involvement (periorbital & lid swelling, conjuctival
hyperemia).
II. Lid retraction
III. Proptosis (passive or mechanical protrusion of eye ball)
IV. Optic neuropathy (serious complication – compression of optic nerve may
lead to visual impairement)
V. Restrictive myopathy (ocular motilty is reduced initially by inflammatory
edema & later by fibrosis)

39. Thyroid eye disease
moderate bilateral symmetrical lid retraction –Dalrymple sign severe bilateral lid retraction – Kocher sign
right lid lag on downgaze – von Graefe sign Proptosis in thyroid eye disease

40. 9. Myopathies
 A. Myasthenia gravis

41. 10. Neurologic Diseases
A. Multiple Sclerosis

42. B. Neurofibromatosis
Nodular plexiform
neurofibroma of the eyelid
axial CT image
showing right proptosis
with fusiform
enlargement of the
optic nerve due to
glioma;
right proptosis due to optic nerve
glioma
Lisch nodules

43. 11. Haematological Disease
Leukemia
 Ocular feature
 Retinal haemorrhages and cotton
wool spots
 Roth spots
 Peripheral retinal
neovascularization
 Retinal and choroidal infiltrates
 Optic nerve infiltration
 Visual loss
 Orbital involvement
 Iris thickening, iritis and
pseudohypopyon

44. Leukemia
Retinal haemorrhages in
leukaemia. - Numerous
flame haemorrhages with cotton
wool spots and Roth
spots
large retrohyaloid
haemorrhage – a separate white cell
layer is evident

45. Roth spots