1. Eye In Systemic Diseases
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Eye In Systemic Diseases
Prepared By: Moath A. Nassar
© 2007 Microsoft Corporation. All rights reserved. Microsoft, Windows, Windows Vista and other product names are or may be registered trademarks and/or trademarks in the U.S. and/or other countries.
The information herein is for informational purposes only and represents the current view of Microsoft Corporation as of the date of this presentation. Because Microsoft must respond to changing market conditions, it should not be interpreted to be a commitment on the part of Microsoft, and Microsoft cannot guarantee the accuracy of any information provided after the date of this presentation. MICROSOFT MAKES NO WARRANTIES, EXPRESS, IMPLIED OR STATUTORY, AS TO THE INFORMATION IN THIS PRESENTATION.

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Systemic Diseases ?
Systemic diseases are diseases that involve many organs or the whole body.
Many of these diseases also affect the eyes.
In fact, an eye exam sometimes leads to the first diagnosis of a systemic disease
Why is the eye so important in systemic disease?
The eye is composed of many different types of tissues. This unique feature makes the eye susceptible to a wide variety of diseases as well as provides insights into many body systems. 
© 2007 Microsoft Corporation. All rights reserved. Microsoft, Windows, Windows Vista and other product names are or may be registered trademarks and/or trademarks in the U.S. and/or other countries.
The information herein is for informational purposes only and represents the current view of Microsoft Corporation as of the date of this presentation. Because Microsoft must respond to changing market conditions, it should not be interpreted to be a commitment on the part of Microsoft, and Microsoft cannot guarantee the accuracy of any information provided after the date of this presentation. MICROSOFT MAKES NO WARRANTIES, EXPRESS, IMPLIED OR STATUTORY, AS TO THE INFORMATION IN THIS PRESENTATION.

3. Systemic diseases to be discussed :
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Systemic diseases to be discussed :
Collagen vascular disease
Spondyloarthropathies
Sarcoidosis
Systemic vasculitis
Blood disorders
Demyelinating disorders
Hypertention and vascular diseases
Thyroid disorders
General signs & symptoms
Diagnostic tests
General management
Ocular involvement
Signs & symptoms
Treatment and prognosis
© 2007 Microsoft Corporation. All rights reserved. Microsoft, Windows, Windows Vista and other product names are or may be registered trademarks and/or trademarks in the U.S. and/or other countries.
The information herein is for informational purposes only and represents the current view of Microsoft Corporation as of the date of this presentation. Because Microsoft must respond to changing market conditions, it should not be interpreted to be a commitment on the part of Microsoft, and Microsoft cannot guarantee the accuracy of any information provided after the date of this presentation. MICROSOFT MAKES NO WARRANTIES, EXPRESS, IMPLIED OR STATUTORY, AS TO THE INFORMATION IN THIS PRESENTATION.

4. 1.Collagen vascular disease
  problems with the immune system affect collagen
wide category of diseases
Symptoms of collagen vascular disease vary from disease to disease, but may include:
Back pain
Chest pain and shortness of breath
Fatigue and weakness
Fever
Painful, swollen joints
Rashes
Diagnosis
Hx & physical examination, Blood tests can check level of autoantibodies,
RF, urinalysis ,LFT , KFT , ESR, C- XR
Treatment
Some may go into remission spontaneously
Depends on the extent of the disease
Steroids or anti-inflammatory medications to ease discomfort
In advanced cases, immunosuppressant drugs may help

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a. Behçet's disease
Recurrent multisystemic disease presented during third to fourh decade
Major diagnostic criteria :
Recurrent oral aphthous stomatitis
Skin lesions ( erythema nodosum,…)
Recurrent genital ulceration
Uveitis : both anterior and posterior
Minor diagnostic criteria
Arthritis , epididymitis, intestinal ulceration , neurological and vascular complications
Four major criteria simultaneously or at different times is diagnostic
© 2007 Microsoft Corporation. All rights reserved. Microsoft, Windows, Windows Vista and other product names are or may be registered trademarks and/or trademarks in the U.S. and/or other countries.
The information herein is for informational purposes only and represents the current view of Microsoft Corporation as of the date of this presentation. Because Microsoft must respond to changing market conditions, it should not be interpreted to be a commitment on the part of Microsoft, and Microsoft cannot guarantee the accuracy of any information provided after the date of this presentation. MICROSOFT MAKES NO WARRANTIES, EXPRESS, IMPLIED OR STATUTORY, AS TO THE INFORMATION IN THIS PRESENTATION.

6. Hypopyon which can be seen in anterior uveitis in a patient with Behcet's disease.
 Acute anterior uveitis generally responds to corticosteroid eye drops.
Inflammation involving the back of the eye requires treatment that is more aggressive with corticosteroids given by injection and/or pill (systemic therapy).
may lead to blindness in 20% of those affected 

7. b. Sjögren's syndrome Ophthalmic features
Autoimmune inflammation and destruction of lacrimal and salivary glands
Primary or secondary
F>M
Presented in adult life with gritteness( sandiness) of eyes and dryness of the mouth
Signs
Large salivary glands with diminished salivary flow
Dry nasal passages
Raynaud’s phenomenon
Diagnosed by serum autoantibodies and biopsies
Ophthalmic features
Common :Keratoconjunctivitis sicca
Rare : adie pupil
Treatment
If mild tear substitutes would help
Avoiding low humidity environment
Occluding the punctum in severe cases

8. keratoconjunctivitis sicca
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keratoconjunctivitis sicca
Complete cure could never be achieved but fowolloing a strict treatment guidelines may improve the outcome
© 2007 Microsoft Corporation. All rights reserved. Microsoft, Windows, Windows Vista and other product names are or may be registered trademarks and/or trademarks in the U.S. and/or other countries.
The information herein is for informational purposes only and represents the current view of Microsoft Corporation as of the date of this presentation. Because Microsoft must respond to changing market conditions, it should not be interpreted to be a commitment on the part of Microsoft, and Microsoft cannot guarantee the accuracy of any information provided after the date of this presentation. MICROSOFT MAKES NO WARRANTIES, EXPRESS, IMPLIED OR STATUTORY, AS TO THE INFORMATION IN THIS PRESENTATION.

9. c. Systemic Lupus Erythematosus
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c. Systemic Lupus Erythematosus
Autoimmune non-organ specific connective tissue disease
F>M
Presented during third to fifth decade with fatiguabilty without specific organ involvement
Signs
Mucocutaneous, musculoskeletal, renal, cardiovascular, pulmonary, haemopoitic, reticuloendothelial, neurological.
Diagnosis: ESR is raised, C- ractive protein is normal, serology.
Treated by steriods and cytotoxic agents
Ophthalmic complications
Common: Madarosis and keratoconjunctivitis sicca
Uncommon: peripheral ulcerative keratitis
Rare: scleritis, retinal vasculitis and optic neuropathy
© 2007 Microsoft Corporation. All rights reserved. Microsoft, Windows, Windows Vista and other product names are or may be registered trademarks and/or trademarks in the U.S. and/or other countries.
The information herein is for informational purposes only and represents the current view of Microsoft Corporation as of the date of this presentation. Because Microsoft must respond to changing market conditions, it should not be interpreted to be a commitment on the part of Microsoft, and Microsoft cannot guarantee the accuracy of any information provided after the date of this presentation. MICROSOFT MAKES NO WARRANTIES, EXPRESS, IMPLIED OR STATUTORY, AS TO THE INFORMATION IN THIS PRESENTATION.

10. Discoid lesions is the cause in this case
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Madarosis is the absence or loss of the eyelashes (and sometimes the eyebrows)
Discoid lesions is the cause in this case
© 2007 Microsoft Corporation. All rights reserved. Microsoft, Windows, Windows Vista and other product names are or may be registered trademarks and/or trademarks in the U.S. and/or other countries.
The information herein is for informational purposes only and represents the current view of Microsoft Corporation as of the date of this presentation. Because Microsoft must respond to changing market conditions, it should not be interpreted to be a commitment on the part of Microsoft, and Microsoft cannot guarantee the accuracy of any information provided after the date of this presentation. MICROSOFT MAKES NO WARRANTIES, EXPRESS, IMPLIED OR STATUTORY, AS TO THE INFORMATION IN THIS PRESENTATION.

11. d. Rheumatoid arthritis
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d. Rheumatoid arthritis
Autoimmune systemic disease characterized by symmetrical, destructive, deformating, inflammatory, polyarthropathy
F>m
Presented during the fourth decade and occasionally during the childhood
Signs
Arthritis ( symmetrical involvement of small joints of hands and feet, ulnar deviation ,…)
Skin ( raynaud’s phenomenon, vasculitis , …)
Treated by NSAIDs, cytotoxic drugs ,…
Ophthalmic features
Common: keratoconjunctivitis sicca ( secondary )
Uncommon: scleritis and peripheral ulcerative keratitis
Rare: acquired superior oblique tendon sheath syndrome
© 2007 Microsoft Corporation. All rights reserved. Microsoft, Windows, Windows Vista and other product names are or may be registered trademarks and/or trademarks in the U.S. and/or other countries.
The information herein is for informational purposes only and represents the current view of Microsoft Corporation as of the date of this presentation. Because Microsoft must respond to changing market conditions, it should not be interpreted to be a commitment on the part of Microsoft, and Microsoft cannot guarantee the accuracy of any information provided after the date of this presentation. MICROSOFT MAKES NO WARRANTIES, EXPRESS, IMPLIED OR STATUTORY, AS TO THE INFORMATION IN THIS PRESENTATION.

12. Scleromalacia ; potential perforation
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Scleromalacia ; potential perforation
tumour necrosis factor (TNF) blockers (infliximab) are effective therapeutic agents in ocular complications of rheumatoid arthritis
© 2007 Microsoft Corporation. All rights reserved. Microsoft, Windows, Windows Vista and other product names are or may be registered trademarks and/or trademarks in the U.S. and/or other countries.
The information herein is for informational purposes only and represents the current view of Microsoft Corporation as of the date of this presentation. Because Microsoft must respond to changing market conditions, it should not be interpreted to be a commitment on the part of Microsoft, and Microsoft cannot guarantee the accuracy of any information provided after the date of this presentation. MICROSOFT MAKES NO WARRANTIES, EXPRESS, IMPLIED OR STATUTORY, AS TO THE INFORMATION IN THIS PRESENTATION.

13. e. Systemic scleroderma
Chronic connective tissue disease affecting the skin and internal organs
F>M
May be limited , diffuse and overlapping with other autoimmune diseases
Presented in the fourth to sixth decade with raynaud phenomenon
Signs:
Skin: tightening and thickening on hands , feet , face and trunk. Subcutaneous fibrosis, typical facial appearance( fixed expression, restrictive movements of lips and “ beaking of nose)
Organs: esophageal dysmotility, heart , lung and kidney disease, mild arthritis
Diagnosis: serology
Treatment: Immunosuppressive agents, Antifibrotic agents, Anti-infammatory agents,…..
Ophthalmic features
Common: eyelid tightening and telangectasia
Uncommon: keratoconjunctivitis sicca

14. Skin tightening on the face ( including the eyelids)
Telangiectasia

15. 2.Spondyloarthropathies
Group of inflammatory joint diseases including:
Ankylosing spondylitis
Reactive arthritis( reiter’s syndrome )
Psoriatic arthropathy
Arthritis associated with inflammatory bowel disease(enteropathic spondylitis )
Isolated acute anterior uveitis
An association with HLA –B27 occurs in all of them but in particularly strong for ankylosing sponndylitis ( > 95%) and reiter’s disease ( 90 %)
They share common clinical features:
Asymmetrical inflammatory oligoarthritis, Sacroiliitis and inflammatory spondylitis
Diagnosis: Hx , exam ,radiographs, HLA B- 27, serology
Ophthalmic features:
Thay all cause acute anterior uveitis
conjunctivitis, keratitis, keratoconjunctivitis sicca, scleritis

16. Recurrent Iritis caused Synechiae(adhesions between the lens and iris) in ankylosing spondylitis

17. corneal infiltrate is Uncommon feature
Conjunctivitis (figure) is a common ophthalmic feature of Reiter's syndrome along with acute anterior uveitis
corneal infiltrate is Uncommon feature
Conjunctivitis is not very painful, does not affect vision, and is not serious and goes without treatment

18. Scleritis in Crohn disease
If mild, could be treated by NSAIDs but if severe , systemic steriods may be introduced
Also we may have conjunctivitis , peripheral corneal infiltrate & retinal periphlebitis in Crohn disease

19. 3.Sarcoidosis Multi- systemic granulumatous inflammatory disorder
Presentaion:
Acute onset sarcoidosis typically occurs during the third decade
Insidious onset sarcoidosis occurs during the fifth decade
Signs
Pulmonary : in 90 % of patients, ranges from asymptomatic bilateral hilar lymphadenopathy to progressive pulmonary fibrosis and bronchectasis
Skin : erythema nodosum, granuloma
Neurological : cranial nerve palsies ( particularly facial) , intra cranial granuloma
Diagnosis:
Chest radiographs
Biopsy :
Lungs , gives the greatest yield
Conjunctiva , positive in about 70 %
Lacrimal glands, positve in 25 % of unenlarged and 75 % of enlarged
Calcium assay

20. Treatment options include NSAIDs steroids and low dose cytotoxic agents
Ophthalmic features
Common : conjunctival granulomas, anterior uveitis, posterior uveitis and retinal periphlebitis
Uncommon : keratoconjunctivitis sicca and intermediate uveitis
Rare: fundus granulomas, retinal and disc neovascularization, and papilloedema
Mild symptoms are treated by topical steroids and cycloplegics.
Systemic steroids are indicated in cases not responding to topical steroids

21. Anterior segment granulomatous involvement in a case of ocular sarcoidosis

22. Conjunctival granulomas in ocular sarcoidosis

23. A: Scattered peripheral multifocal lesions characteristic of ocular sarcoidosis. 
B: The macula of the same patient demonstrating a choroidal neovascular membrane.

24. 4.Systemic vasculitis
The systemic vasculitides are characterized by inflammation of blood vessel walls
Vessels of any type, in any organ can be affected,
classified based on affected vessel size (large, medium, and small)
Signs and symptoms
Nonspecific complaints
Those should raise a strong suspicion:
Mononeuritis multiplex, palpaple purpura, pulmonary renal syndrome
Diagniosis : a compatible clinical presentation supported by specific laboratory or imaging tests and confirmatory histology
Glucocorticoids are the primary treatment for many forms of vasculitis , Additional immunosuppressive agents are sometimes required

25. a. Giant cell arteritis
A granulumatous necrotizing arteritis with predilection for large and medium size arteries , particularly the superficial temporal , ophthalmic, posterior ciliary and proximal vertebral
Presented during the seventh to eighth decade with the following:
Scalp tenderness, headach, jaw claudication (pthognomonic) , polymyalgia rheumatica , non specifc symptoms, blindness ( sudden with minimal systemic upset), superficial temporal arteritis ( thick , tender, inlammed, and nodular arteries which can’t be flattened against the skull), the pulsation later ceases which strongly suggestive of GCA
Diagnostic tests
High ESR , CRP
Temporal artery biopsy should be performed
Treatment : by administration of systemic steriods

26. Ophthalmic features Common: anterior ischemic optic neuropathy
Uncommon: cotton wool spots, central retinal artery occlusion , ocular motor nerve palsy ( commonly a pupil sparing third nerve palsy )
Rare: ocular ischemic syndrome
Typical findings of a patient with arteritic ischemic optic neuropathy. Note the pallid disk edema, associated hemorrhages, and adjacent cotton wool spot
Treatment for arteritic ischemic optic neuropathy is CORTICOSTEROID MEDICATIONS

27. b. Polyarteritis nodosa
Idoipathic , potentially lethal , collagen vascular disease affecting medium sized and small arteries
Presented during the sixth decade
Signs
Skin: purpura and easy bruising , gangrene
Muscles: weakness and tenderness
Complications
Renal involvement and hypertension
Coronary arteritis
GI bleeding
Stroke or multifocal neuropathy
Diagnosis: skin biopsy
Treatment: systemic steriods and immunosuppressive agents

28. Ophthalmic features
Common: peripheral ulcerative keratitis and scleritis
Rare: orbital pseudo-tumour and occlusive retinal periarteritis
Peripheral ulcerative keratitis presents with a crescent shaped destructive lesion of the juxtalimbal corneal stroma

29. c. Wegener’s granulomatosis
Idiopathic , multisystem, granulomatous disorder characterized by generalized small-vessel vasculitis affecting predominantly the respiratory tract and the kidneys
Presented in the fifth decade , often with pulmonary symptoms
Signs
Respiratory tract
Upper: perforation of the nasal septum
Lower: nodular lesions , infiltrates and cavitaions
Organs
Necrotizing glomerulonephritis with renal failure
Affects heart , spleen and adrenals
Neurological: polyneuritis and meningoencephalitis
Diagnosis: Anti-neutrophil cytoplasm antibodies
Treated with systemic steriods and cyclophosphamide

30. Ophthalmic features
Common : nasolacrimal obstruction and dacrycystitis
Uncommon: scleritis and peripheral ulcerative keratitis
Rare: orbital pseudo- tumour and occlusive retinal periarteritis
Nasolacrimal duct involvement can result in epiphora, dacryocystitis and draining fistulas.
sclerokeratitis 
Dacrocystitis treated by surgey (Dacryocystorhinostomy ) . Good outcome

31. 5.Hematologic disorders
Hematological diseases encompass a wide spectrum of disorders ranging from benign to malignant conditions that can present with ocular involvement
Ocular findings related to anemia, hematological malignancies and platelet disorders are to be disscussed

32. a. Anemia
  Anemia is a common condition that occurs when the level of healthy RBCs or Hb is below normal
Causes include: destruction, increased blood loss and inadequate production of RBCs by the bone marrow
Additional causes related to poor nutrition or absorption defects in the GI tract include iron, vitamin B12or folic acid deficiencies
general symptoms: pallor of the skin or nail beds, weakness, headaches, shortness of breath, GI disturbances, fever, and numbness or coldness of extremities
Diagnosis: CBC is the most important, assess level of B12 and folic acid, bone marrow biopsy

33. Ocular changes conjunctival pallor and hemorrhages
 anemic retinopathy(intraretinal hemorrhages, Roth’s spot hemorrhages, cotton-wool spots, retinal exudates, venous dilation and optic nerve pallor)
These changes generally occur in patients with severe anemia or when thrombocytopenia is present

34. Sickle cell anemia may also cause  sickled conjunctival vessels, central retinal artery occlusion (CRAO), branch retinal artery occlusion (BRAO), sickling maculopathy

35. b.

36. c. Platelets disorders Ocular changes can include :
Conjunctival petechial hemorrhages
Amaurosis fugax
Vascular occlusions
Retinal, vitreal and choroidal hemorrhages