Cornea in Systemic Disease Dr Deepak Khadka MD, Geta Eye Hospital

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Cornea in Systemic Disease:

Cornea in Systemic Disease Deepak Khadka, MD Vitreo-retina Specialist Geta Eye Hospital

Slide 2:

Introduction Brief Review of anatomy and embryology Systemic association of abnormal Corneal size and shape Metabolic disease and cornea Nutritional disease and cornea Hereditary disorders that can affect multi organ system Disease of skin and cornea Cornea in immunological disease Outlines:

Slide 3:

A wide variety of systemic disease can be associated with corneal manifestation. Corneal deposits are the first indication of the systemic disease. Fabry’s disease Some corneal signs are important accessible part of diagnosis. KF ring. Conjunctival and corneal biopsy are relatively accessible means of diagnosing inborn error of metabolism. Some corneal signs are good indicator for effectiveness of the therapy. Introduction:

Cornea:

Cornea Transparent, avascular, watch glass like structure. It forms anterior one sixth of the outer fibrous coat of the eyeball.

Cornea:

Cornea Dimension: Anterior surface elliptical with an average horizontal diameter of 11.5mm and vertical diameter of 11mm. Posterior surface of cornea is circular with average 11.5mm diameter.

Cornea:

Cornea Thickness at centre 0.52mm and periphery 0.67mm. Radius of curvature: Anterior 7.8mm and posterior 6.5mm Refractive power 45D and refractive index 1.37

Cornea histology and embryology::

Cornea histology and embryology: Epithelium: 5 layers derived from surface ectoderm. Bowman’s membrane and stroma: derived from mesenchymal cells. Decemet’s membrane and endothelium derived from mesenchymal cells from neuroectoderm.

Abnormalities of corneal size and curvature::

Abnormalities of corneal size and curvature:

Megalocornea::

Megalocornea: Adult: 13mm or more in horizontal diameter Newborn: 12mm or more not associated with congenital glaucoma and is non progressive. Bilateral, sex linked recessive trait, 90% males. Systemic association: Marfan’s syndrome, Apert syndrome, Mucolipidosis type II

Microcornea:

Microcornea Corneal diameter is 10mm or less. Nanopthalmus Micropthalmus. Systemic association: Ehler Danlos syndrome Weil Marchacheini syndrome, Waardenburg syndrome, Turner syndrome, Rubella etc

Abnormal curvature::

Abnormal curvature: Cornea plana:30-35D Keratoconus/ Keratoglobus: Systemic association: Elher Danlos syndrome Down’s syndrome Turner syndrome Osteogenesis imperfecta Mitral valve prolapse Atopic dermatitis Marfan’s syndrome Apert’s syndrome Crouzen syndrome etc.

Metabolic Disease and Cornea::

Metabolic Disease and Cornea:

Metabolic Disease and Cornea: Endocrine disorders::

Metabolic Disease and Cornea: Endocrine disorders: Hyperthyroidism: ophthalmic involvement 50% cases. Corneal involvement secondary to proptosis and lid retraction. Earliest change: punctuate staining of cornea in interpalpebral zone. More severe forms epithelial defects, ulceration and even perforation can occur.

Multiple endocrine Neoplasia::

Multiple endocrine Neoplasia: Rare hereditary disorder. MEN type I: Visual field defect, rarely prominent corneal nerves. MEN IIa: Rarely prominent corneal nerves. MEN IIb: Thickened corneal nerves are relatively common. “The ophthalmologists may have the opportunity to make the diagnosis”

Differential diagnosis of prominent corneal nerves::

Differential diagnosis of prominent corneal nerves: Fuch’s dystrophy Keratoconus Neurofibromatosis Refsum’s syndrome Ichthyosis Leprosy MEN Medullary carcinoma of thyroid.

Disorders of calcium metabolism::

Disorders of calcium metabolism: Band keratopathy: commonly in localized ocular disease; hyperparathyroidism, chronic renal failure. Exact cause unknown. Equilibrium between Ca and P, increase in PH. Evaporation of tear - increase in local ion concentration. CO 2 loss – locally elevate PH lack of blood vessels – less buffering action

Band keratopathy::

Band keratopathy: Clinically grayish white superficial deposits in the interpalpebral cornea.

Disorders of lipid metabolism::

Disorders of lipid metabolism: Hyperlipoproteinemas Sphingolipidosis

Hyperlipoproteinemas::

Hyperlipoproteinemas: Earlier arcus and xanthelesma. Arcus: extra cellular deposits of cholesterol, cholesterol esters and PL and TG in peripheral cornea. 60 yrs: 60%, 80 yrs > 90%. Why is it important? If younger than 50 yrs significant risk factor for CAD.

Sphingolipidosis: Fabry’s disease:

Sphingolipidosis: Fabry’s disease Deficiency of galactosidase A; X linked recessive trait. Accumulation of alpha galactosyl moieties in different parts of the body. Ocular findings: corneal opacity in all males and 90% of female carriers.

Sphingolipidosis: Fabry’s disease:

Sphingolipidosis: Fabry’s disease Ocular findings: superficial, white, yellow or brown dots, distributed in a vortex pattern. Differential diagnosis of vortex keratopathy: Chloroquine Amiodarone Naproxen Indomethacin

Sphingolipidosis: Gaucher’s disease, GM1 gangliosidosis:

Sphingolipidosis: Gaucher’s disease, GM1 gangliosidosis Whitish deposits in peripheral cornea with sometimes corneal clouding. Important ocular findings include cherry red spot in the macula.

Disorders of carbohydrate metabolism::

Disorders of carbohydrate metabolism: Systemic mucopolysaccharidosis Diabetes mellitus

Disorders of carbohydrate metabolism: MPS:

Disorders of carbohydrate metabolism: MPS The extracellular matrix: vital role in maintenance and regulation of cellular function as well as intracellular support. Keratan and dermatan/ chondroitin sulphate in stroma, heparan sulphate in retina. AR deficiency of enzymes that degrade GAGs.

Disorders of carbohydrate metabolism: MPS:

Disorders of carbohydrate metabolism: MPS Deficiency of enzymes: Corneal clouding Retinopathy and optic atrophy

Disorders of carbohydrate metabolism: Diabetes:

Disorders of carbohydrate metabolism: Diabetes Cornea : decreased corneal sensation though mild neurotrophic ulcers can develop. Decreased epithelial adhesion and impaired wound healing are often noted in diabetics.

Disorders of amino acid metabolism::

Disorders of amino acid metabolism: Alkaptonuria Cystinosis

Disorders of amino acid metabolism: Alkaptonuria:

Disorders of amino acid metabolism: Alkaptonuria AR, deficiency of Homogentisic acid oxidase Characterized by bluish grey or black pigmentation of connective tissue: Ochronosis Cornea: brownish black deposits in Bowman’s layer and deep epithelium immediately inside the limbus.

Disorders of amino acid metabolism: cystinosis:

Disorders of amino acid metabolism: cystinosis AR, impaired transport of cystine across lysosomal membranes. Cornea: glistening, polychromatic, needle like to rectangular crystals In advanced cases corneal erosions can occur and the cornea becomes thickened. Band keratopathy is a frequent complication.

Disorder of cupper metabolism: Wilson’s Disease:

Disorder of cupper metabolism: Wilson’s Disease

Wilson’s Disease::

Wilson’s Disease: AR, associated with progressive neurological and liver dysfunction. Excretion of Cu from liver impaired: spillover of cupper into the blood circulation leading to deposition of cupper in all body tissues.

Wilson’s Disease: ocular manifestation:

Wilson’s Disease: ocular manifestation Cornea: KF ring Lens: sunflower cataract KF ring: 1-3mm wide, peripheral band at the level of Decemet’s membrane and extending to a limbus without a lucid interval. Usually yellow brown but may appear gold, red, blue or green Superior then inferior Gonioscopy

Wilson’s Disease: ocular manifestation:

Wilson’s Disease: ocular manifestation KF ring seen in 95% of Wilson’s disease all patient who have neurological involvement 70-90% of hepatic involvement Sunflower cataract in 15-20% patient

Wilson’s Disease: diagnosis and management:

Wilson’s Disease: diagnosis and management Diagnosis: serum Ceruloplasmin level< 20microgram/dl and either KF ring or increased concentration of cupper in liver biopsy. Treatment: D- penicillamine which prevents and regress clinical manifestation. KF ring is a good indicator of effectiveness of therapy.

Diseases of skin and cornea::

Diseases of skin and cornea: Pemphigoid Stevens Johnson Syndrome Rosacea Xeroderma pigmentosa

Diseases of skin and cornea: Pemphigoid:

Diseases of skin and cornea: Pemphigoid Cicatricial disease of the conjunctiva and to a lesser extent other mucus membranes and skin presumed to be autoimmune origin. Autoantibody to epithelial basement membrane (type II Hypersensitivity reaction) Ocular manifestations- majority of cases and can be the only manifestation of the disease. Usually bilateral presentation.

Diseases of skin and cornea: Pemphigoid:

Diseases of skin and cornea: Pemphigoid Common serious complication result from ocular involvement. Cornea remarkably spared except in the later stage. Cornea may seriously compromised by the secondary effects of cicatrisation , drying and trichiasis which is then subjected to scarring and vascularization, persistent epithelial defects, sterile ulceration, and bacterial infection.

Diseases of skin and cornea: Stevens Johnson Syndrome:

Diseases of skin and cornea: Stevens Johnson Syndrome

Diseases of skin and cornea: Stevens Johnson Syndrome:

Diseases of skin and cornea: Stevens Johnson Syndrome Episodic, self limited, mucocutaneous inflammatory disorder. Commonly precipitated by Herpes simplex infection or drugs, but can be associated with other infectious agents or carcinoma, and often no precipitating factors can be found.

Diseases of skin and cornea: Stevens Johnson Syndrome:

Diseases of skin and cornea: Stevens Johnson Syndrome Immune complexes, cell mediated immunity or both. Children and young adults. Prodrome followed by skin eruptions. Mucus membranes of eyes and mouth are frequently involved by bullous erosive lesions.

Diseases of skin and cornea: Stevens Johnson Syndrome:

Diseases of skin and cornea: Stevens Johnson Syndrome Ocular manifestation: conjunctival inflammation ultimately leading to cicatrisation. Tendency to relative sparing of the cornea until late complication of conjunctival scarring ensue. Corneal ulceration, drying and peripheral vascularization can occur in active disease. Final outcome: no visible sequale to severe scarring, KCS, corneal vascularisation, symblepharon, trichiasis and entropion.

Diseases of skin and cornea: Rosacea:

Diseases of skin and cornea: Rosacea Dermatosis in which significant ocular morbidity can occur with relatively mild skin disease. M:F equal, 25-50 yrs of age Pathogenesis unknown, type IV hypersensitivity reaction. Skin of forehead, nose, cheeks and chin. Vascular component with or without acneiform component. Rhinophymoma.

Diseases of skin and cornea: Rosacea:

Diseases of skin and cornea: Rosacea Ocular manifestation: common blepharitis Dilated blood vessels of eyelid, skin and conjunctiva. Cornea can be severely involved wiyh scarring and vascularisation.

Diseases of skin and cornea: Rosacea:

Diseases of skin and cornea: Rosacea Rosacea keratitis: pain, photophobia and F.B sensation, SPK’s most often due to blepharitis. Vascularisation of the cornea with superficial infiltrates, broad areas of pannus ultimately involving whole of the cornea. Frequent ophthalmic remission and exacerbation. Marginal or less commonly central corneal melt can occur.

Diseases of skin and cornea: Xeroderma pigmentosa:

Diseases of skin and cornea: Xeroderma pigmentosa

Xeroderma pigmentosa:

Xeroderma pigmentosa AR disease characterized by intolerance to sunlight caused by defect in the repair of UV light induced DNA damage. With time the sun exposed skin develops macules and atrophies with hypopigmented plaques, telangiectasias, dryness and scaling.

Xeroderma pigmentosa:

Xeroderma pigmentosa Ocular manifestation: most of the patient are photophobic and may have conjunctivitis. The conjunctiva is dry and hyperemic telangiectasias, pigmented lesions and tumor can occur in the bulbar conjunctiva and limbus.

Xeroderma pigmentosa:

Xeroderma pigmentosa Loss of cilia, ectropion and entropion are common. The cornea becomes vascularised, ulcerated and scarred and limbal tumors particularly squamous cell carcinoma are frequently encountered. Endothelial cell density is less than normal .

Immunological diseases and cornea::

Immunological diseases and cornea: Immune characteristics of cornea SLE Rheumatoid Arthritis Relapsing polychondritis Inflammatory bowel disease Wegener’s granulomatosis

Immune characteristics of cornea::

Immune characteristics of cornea: Although normally devoid of blood vessels, lymphatics and inflammatory cells. Number of immunologically active cells and substances are present in the cornea. Ig and complement – diffusion through limbal vessels. C3, C4 and C5; IgG and IgA– throughout the corneal stroma; larger IgM in periphery. Langerhans’ cells (APC) – corneal epithelium. Central cornea less than periphery.

Immune characteristics of cornea::

Immune characteristics of cornea: Cornea enjoys relative “immune privilege” i.e. tolerance of corneal grafts . Absence of corneal blood vessels and lymphatics – Afferent and efferent limbs of immune system affected. Relative lack of APC or the development of ACAID. In pathologic states, vascularization of cornea is accompanied by development of lymphoid channels and increase in Langerhans’ cells.

Immunological diseases and cornea::

Immunological diseases and cornea: Systemic lupus erythematosus (SLE):

Systemic lupus erythematosus (SLE)::

Systemic lupus erythematosus (SLE): Chronic multisystem disease related to immunologic dysfunction and production of auto antibody. Female > Male 20-30 yrs Dysfunction in immune regulation Impaired suppressor T cell function antinuclear Antibody Production of auto antibody anti cell membrane antibody Immune complex (type III Hypersensitivity reaction) deposition in many tissues .

Systemic lupus erythematosus (SLE)::

Systemic lupus erythematosus (SLE): Certain drugs can produce similar condition. Ds DNA Ab are highly specific for SLE and correlate with disease severity. Ribosomal P and Sm autoAb are fairly specific but do not correlate with disease severity. Production of Phospholipid Ab correlates with thrombotic complication.

Systemic lupus erythematosus (SLE): clinical manifestation:

Systemic lupus erythematosus (SLE): clinical manifestation Most common butterfly rash. Discoid rashes, alopecia, mucosal ulcer. Other findings: arthritis, polyarthralgia, pleuritis, pericarditis, myocarditis, lymphadenopathy, splenomegaly, nephritis and hematological and neurological disease.

Systemic lupus erythematosus (SLE): clinical manifestation:

Systemic lupus erythematosus (SLE): clinical manifestation Most common findings in the eye: CW spots, retinal hemorrhages, edema of the retina and disc, rarely exuduative RD. Epscleritis , scleritis and anterior uveitis can occur. Keratoconjunctivitis sicca is common. Corneal findings: punctuate keratitis, interstitial keratitis and quiet and relatively non infiltered marginal melts.

Immunological diseases and cornea: Rheumatoid arthritis:

Immunological diseases and cornea: Rheumatoid arthritis Multisystem, inflammatory autoimmune disorder that primary affects the joints. women>men, 30-40 yrs Small joints of hands and feet. Morning stiffness, pain, swelling and with time characteristics deformity develops.

Rheumatoid arthritis: pathogenesis:

Rheumatoid arthritis: pathogenesis HLA DR4 prevalence IgG and IgM antiimmunoglobulin antibody are formed (RA factors) which react with immunoglobulin and form immune complexes. 10-20% above 60 yrs have positive RA factor and may be seen in other inflammatory disorder.

Rheumatoid arthritis: ocular manifestation:

Rheumatoid arthritis: ocular manifestation Most common is KCS (15-25%) Episcleritis and scleritis are commonly seen. The scleritis can take any of the typical forms: diffuse anterior nodular anterior necrotizing with inflammation posterior scleritis or scleromalacia perforans.

Rheumatoid arthritis: ocular manifestation:

Rheumatoid arthritis: ocular manifestation Diffuse anterior scleritis is the commonest form. Presence of necrotizing scleritis in a patient with RA indicates systemic disease activity and a poor overall prognosis; without aggressive treatment majority of patient die within 5 yrs.

Rheumatoid arthritis: Corneal changes :

Rheumatoid arthritis: Corneal changes Most common corneal changes are due to KCS. 50-70% of the patient with scleritis have corneal involvement. Corneal involvement usually occurs as a result of perilimbal scleritis onto the peripheral cornea (sclerokeratitis). Episcleritis can also be associated with peripheral corneal lesions, most commonly peripheral stromal infiltration and edema.

Rheumatoid arthritis: Corneal changes :

Rheumatoid arthritis: Corneal changes Watson has described corneal changes in scleritis into Sclerosing keratitis Acute stromal keratitis Marginal furrowing Keratolysis.

Rheumatoid arthritis: Corneal changes :

Rheumatoid arthritis: Corneal changes Corneal melting can occur in the absence of scleritis. Central ulcer most commonly related to dryness and peripheral related to vasculitis

Immunological diseases and cornea: relapsing polychondritis:

Immunological diseases and cornea: relapsing polychondritis Recurrent inflammation of cartilaginous tissue, M: F equal, 40-60 yrs Can occur in most of the connective tissue disease or vasculitis including RA, Wegner’s granulomatosis and SLE Pathogenesis unknown, but humoral and cell mediated immunity are thought to play a role. Anticartilage antibody has been found. Inflammation of the cartilage of pinnae, nose, larynx, trachea and joint.

Relapsing polychondritis: ocular involvement:

Relapsing polychondritis: ocular involvement Ocular involvement in 60% of patient and includes episcleritis (40%), conjunctivitis (25%), scleritis, keratitis and iritis. Corneal involvement usually occurs as a result of adjacent scleritis, but isolated marginal keratitis can occur. Peripheral corneal infiltration and marginal corneal melts can be seen.

Immunological diseases and cornea: Wegener's granulomatosis:

Immunological diseases and cornea: Wegener's granulomatosis Distinct form of vasculitis that mostly affects the upper and lower respiratory tracts and the renal glomeruli. 3 rd to 4 th decade Patient typically presents with URTI, pulmonary, kidney, skin and nervous system involvement.

Wegener's granulomatosis:

Wegener's granulomatosis Pathogenesis unknown. Circulating immune complex and immune complex mediated vasculitis. Antibody to corneal epithelium has been found in some patient with peripheral ulcerative keratitis. Autoantibody against ANCA is positive in nearly all cases with ocular disease and are specific for wegener’s granulomatosis. ESR may be very high.

Wegener's granulomatosis: ocular involvement:

Wegener's granulomatosis: ocular involvement Ocular manifestation in 60% and is the presenting sign in 16%. Proptosis – corneal exposure, restricted motility and disc edema. Retinal vasculitis, uveitis, optic neuropathy, Episcleritis, scleritis and conjunctivitis can occur. Cornea- necrotizing infiltrative marginal ulcers. Marginal corneal infiltrates occur in nearly all patient with anterior scleritis and correlate with disease severity.

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