The Eye in Systemic Disease

Cornwall

Preface to the second edition

The second edition of this book has undergone complete revision. Whilst the number of chapters has been reduced from eighteen to fifteen, the number of illustrations has been increased. In addition the systemic aspects of the diseases have been presented in greater detail and more emphasis has been placed on current methods of investigation such as CT and MR scanning.

Despite these modifications the main purpose of this book remains unchanged, that is to acquaint the general physician with a concise overview of the frequently complex relationships between the eye and medicine. For this reason detailed information of little or no interest to non-ophthalmologists which can be found in standard textbooks on ophthalmology has not been included.

We are greatly indebted to the following colleagues for reviewing the manuscript and for making many helpful suggestions: Dr Peter Mackie, Dr John Easton, Dr Ann Hall, Dr Robin Scott, Dr Michael Smith, Dr Stephen Dawson, Dr Ian White and Dr Teifi James. We would also like to thank Miss Daphne Bannister and Mrs Jane Tyler for taking many of the photographs and to Mr Terry Tarrant for the artwork.

J.J.K. and D.J.T.

1

Introduction

Publisher Summary

This chapter discusses the various eye conditions. Conjunctivitis is a very common—usually microbial—inflammation of the mucous membrane which lines the anterior sclera and the inside of the eyelids. Episcleritis is a common—non-microbial—inflammation of the vascular connective tissue layer, which is located between the conjunctiva and the sclera. It typically affects one eye of a healthy middle-aged female. Keratoconjunctivitis sicca is a common condition in which tear secretion by the lacrimal glands is reduced. Treatment is effective with frequent instillation of artificial tears. Keratitis is an inflammation of the cornea. The most common causes are herpes simplex, herpes zoster, bacteria and hypersensitivity to staphylococcal exotoxins. Corneal haziness and ulceration are the two most common signs. The treatment is with topical antimicrobial agents, and in selected cases, steroids may also be indicated. Anterior uveitis is an inflammation of the anterior part of the uveal tract which consists of the iris and ciliary body. The two main types are acute and chronic. The treatment includes topical steroids to suppress the inflammation and mydriatics to prevent the formation of adhesions between the iris and the lens.

The main aim of this chapter is to familiarize the non-ophthalmologist with the various eye conditions that will be described in this book.

Conjunctivitis

Conjunctivitis is a very common, usually microbial, inflammation of the mucous membrane which lines the anterior sclera and the inside of the eyelids.

Causes

The most common causes are bacteria, chlamydia, and viruses. Occasional systemic associations include:

• Reiter’s disease.

• Psoriatic arthritis.

• Wegener’s granulomatosis.

• Cicatricial pemphigoid.

• Stevens—Johnson syndrome.

• Gonorrhoea.

• Crohn’s disease.

Symptoms

Subacute onset of grittiness, usually bilateral, and a mucoid or serous discharge.

Signs

These include normal visual acuity, redness which is maximal in the lower fornices (Figure 1.1) and a variable amount of discharge.

Figure 1.1 Acute conjunctivitis with maximal injection in the lower fornix

Treatment

This is usually with topical antimicrobial agents.

Prognosis

This is excellent and, even without treatment, the vast majority resolve within 7—10 days.

Episcleritis

Episcleritis is a common non-microbial inflammation of the vascular connective tissue layer, which is located between the conjunctiva and the sclera. It typically affects one eye of a healthy middle-aged female.

Causes

Most cases are idiopathic and unassociated with any systemic disease.

Symptoms

These are acute or subacute onset of mild unilateral discomfort and redness.

Signs

A localized red patch involving either the medial or the lateral aspects of the eye (Figure 1.2).

Figure 1.2 Localized area of injection in episcleritis (courtesy of Mr A. Shun-Shin)

Treatment

Topical steroids may be helpful if started early.

Prognosis

This is excellent and most cases resolve within a few days but recurrences may occur.

Keratoconjunctivitis sicca

Keratoconjunctivitis sicca (KCS) is a common condition in which tear secretion by the lacrimal glands is reduced.

Causes

Pure KCS This is common and is characterized by involvement of the lacrimal gland alone.

Primary Sjögren’s syndrome This is an autoimmune disease characterized by the frequent presence of rheumatoid factor, antinuclear antibodies and hypergammaglobulinaemia. Less common findings include antibodies to DNA, salivary duct tissue, smooth muscle and gastric parietal cells. Involvement of the salivary glands may cause a dry mouth (xerostomia), and the bronchial epithelium and the vagina may also be affected.

Secondary Sjögren’s syndrome This is the presence of KCS in association with a systemic disorder such as:

• Rheumatoid arthritis.

• Psoriatic arthritis.

• Connective tissue disorder.

• Sarcoidosis.

• Crohn’s disease.

Symptoms

These are chronic ocular irritation and a foreign body sensation.

Signs

Slit lamp biomicroscopy shows an absent or diminished lower lid tear meniscus, mucous threads in the tear film and corneal filaments. Rose bengal is a dye which shows up mucous threads and corneal filaments more clearly (Figure 1.3), and also stains the bulbar conjunctiva in the form of two triangles with their bases at the limbus (Figure 1.4). The amount of tear secretion can also be determined by measuring the amount of moistening of a special strip of filter paper folded so that 5 mm of the strip lies within the conjunctival sac (Schirmer’s test). A value of less than 5 mm in 5 minutes is indicative of KCS and values between 5 and 10 mm are borderline.

Figure 1.3 Mucous threads and corneal filaments in keratoconjunctivitis sicca stained with rose bengal

Figure 1.4 Conjunctival staining with rose bengal in keratoconjunctivitis sicca

Treatment

Frequent instillation of artificial tears (e.g. hypromellose) is effective in most patients.

Prognosis

This is excellent although treatment may have to be continued indefinitely.

Keratitis

Keratitis is an inflammation of the cornea.

Causes

The most common causes are herpes simplex, herpes zoster, bacteria and hypersensitivity to staphylococcal exotoxins. Occasional systemic associations include:

• Rheumatoid arthritis.

• Connective tissue disorders

• Wegener’s granulomatosis.

• Crohn’s disease.

• Acne rosacea.

• Atopic eczema.

• Syphilis.

Symptoms

These are variable blurring of vision, watering, pain and photophobia.

Signs

Corneal haziness and ulceration are the two most common signs (Figure 1.5).

Figure 1.5 Severe bacterial keratitis with hypopyon

Treatment

Treatment is with topical antimicrobial agents and, in selected cases, steroids may also be indicated.

Prognosis

This is usually good provided treatment is started early.

Scleritis

Scleritis is a very rare inflammation of the tough white outer collagenous layer of the eye.

Causes

The most common local cause is herpes zoster. The most frequent systemic associations include:

• Rheumatoid arthritis.

• Connective tissue disorders.

• Wegener’s granulomatosis.

Symptoms and signs

For these see Chapter 4.

Treatment

Treatment is with systemic non-steroidal anti-inflammatory agents in mild cases, and steroids and/or cytotoxic agents in severe cases.

Prognosis

Many cases develop complications such as keratitis, cataract and glaucoma.

Anterior uveitis

Anterior uveitis (iridocyclitis) is an inflammation of the anterior part of the uveal tract which consists of the iris and ciliary body. The two main types are acute and chronic.

Acute anterior uveitis

Causes

Many cases are idiopathic. Systemic associations include:

• Ankylosing spondylitis.

• Reiter’s syndrome.

• Psoriatic arthritis.

• Behçet’s disease.

• Sarcoidosis.

• Syphilis.

• Crohn’s disease.

• Ulcerative colitis.

Symptoms

These are subacute onset of unilateral photophobia, pain, redness, decreased vision and watering.

Signs

These are a small pupil, circumcorneal ‘ciliary’ injection, which has a violaceous hue (Figure 1.6), and inflammatory cells in the aqueous humour (Figure 1.7), which can be detected only with a special microscope called a slit lamp. In very severe cases a fibrinous exudate develops in the aqueous (Figure 1.8) and the inflammatory cells may form a white fluid level in the bottom of the anterior chamber called a hypopyon (see Figure 4.25).

Figure 1.6 Circumcorneal (ciliary) injection in acute iritis.

Figure 1.7 Acute iritis with aqueous cells and flare as seen with the slit lamp microscope

Figure 1.8 Fibrinous exudate in the aqueous in very severe acute iritis associated with ankylosing spondylitis

Treatment

This is with topical steroids to suppress the inflammation and mydriatics to prevent the formation of adhesions between the iris and lens called posterior synechiae (Figure 1.9).

Figure 1.9 Irregular pupil due to adhesions between the iris and lens (posterior synechiae) following a severe attack of acute iritis

Prognosis

This is usually good provided treatment is instituted early. Most cases last less than 6 weeks although recurrences are common.

Chronic anterior uveitis

Chronic anterior uveitis is less common than the acute type. It frequently persists for several months and sometimes for years.

Causes

Some cases are idiopathic. Systemic associations include:

• Sarcoidosis.

• Tuberculosis.

• Juvenile chronic arthritis.

• Syphilis.

• Whipple’s disease.

Symptoms

These may be minimal despite severe inflammation. Patients may report slight blurring of vision and mild redness.

Signs

These are similar to the acute type, although the eye is not usually red and the condition is frequently bilateral.

Treatment

Treatment is similar to the acute type although the response may be less satisfactory.

Prognosis

This is less good than in the acute type. The inflammation may persist for many months and even years and give rise to complications such as glaucoma, band keratopthy and cataract (see Figure 4.20)

Cataract

A cataract is an opacity in the crystalline lens of the eye.

Causes

Old age is by far the most common cause. Other causes include:

• Trauma and irradiation.

• Inherited.

• Chronic anterior uveitis.

• Steroids.

• Diabetes.

Symptoms

There is usually a gradual impairment of vision.

Signs

A lens opacity can be detected by observing the red reflex with an ophthalmoscope held about 30 cm from the patient’s eye (Figure 1.10).

Figure 1.10 (a) Normal red reflex; (b) red reflex in an eye with a central lens opacity

Treatment

Treatment is surgical.

Prognosis

This is excellent in the vast majority.

Posterior uveitis

Posterior uveitis is an inflammation of the choroid and/or retina. According to the site of primary involvement it is subdivided into retinitis, choroiditis and retinochoroiditis.

Causes

In the UK the most common cause is reactivation of congenital toxoplasmosis. Systemic associations include:

• Behçet’s disease.

• Sarcoidosis.

• Tuberculosis.

• AIDS.

• Syphilis.

• Crohn’s disease.

• Whipple’s disease.

• Reticulum cell sarcoma.

Signs

These vary according to the type.

• Vitritis is characterized by cells in the vitreous. It is responsible for symptoms of vitreous floaters and, if severe, it impairs visualization of the fundus.

• Active retinochoroiditis is characterized by white cloudy patches with indistinct outlines (Figure 1.11).

Figure 1.11 Acute focal retinochoroiditis due to toxoplasmosis — note old inactive lesions

• Inactive retinochoroiditis appears as white well-defined areas of chorioretinal atrophy with pigmented borders. The retinal blood vessels pass over the lesions undisturbed (Figure 1.12).

Figure 1.12 Old multifocal retinochoroiditis

• Vasculitis may involve the retinal veins (periphlebitis) or, less commonly, the arterioles. Active periphlebitis is characterized by a fluffy white haziness surrounding the blood column (see Figure 7.9).

Treatment

This varies according to the cause and includes systemic antimicrobial agents, steroids and cytotoxic agents.

Prognosis

This varies according to the cause and location of the inflammatory focus. Lesions involving the fovea have a poor prognosis.

Vascular disorders of the fundus

Haemorrhages

• Flame-shaped haemorrhages are located in the superficial nerve fibre layer of the retina. They may occur in hypertension, papilloedema and retinal vein occlusion (see Figures 3.6] and