Systemic sclerosis (scleroderma) is a rare inflammatory rheumatic systemic disease and is one of the collagenoses that can affect multiple organs, including the eye. It is characterised by vasculopathy (damage to blood vessels) and fibrosis (abnormal proliferation of connective tissue in organs containing collagen fibres with subsequent hardening). Depending on the course of the disease, systemic sclerosis is classified in a limited form (approx. 45%) and a diffuse form (approx. 30-35%).
The most common manifestations in the eyes manifest themselves in the eyelids, the ocular surface and the retina.
Eyelids:
The skin of the eyelid may have reduced elasticity, as well as telangiectasia (vascular dilatation), frequent inflammation of the eyelid margins or madarosis (loss of eyelashes/the outer end of the eyebrows). Eyelid abnormalities are more common in the diffuse subtype of systemic sclerosis.
Ocular surface:
A tear film disorder as well as a dry eye syndrome occur more frequently in patients with systemic sclerosis than in unaffected patients. Dryness of the ocular surface is mainly associated with a fibrosis of the lacrimal gland and a dysfunction of the meibomian glands at the edge of the eyelid.
Conjunctiva:
Conjunctival redness, dilation of the conjunctival vessels, loss of fine vessels, shortened arch of the conjunctival sac.
Cornea:
corneal dents, corneal inflammations, corneal scars, keratoconus (progressive thinning and conical deformation of the cornea)
Rain-borrowing skin:
Inflammations (iritis/iridocyclitis/uveitis), visible vascular dilatations, iris atrophy (tissue loss, visible at the slit lamp as a "church window phenomenon")
Lens:
clouding of the lens, early cataract
Vitreous body:
Vitreous body opacities, condensations (floaters), rear vitreous body lift-off
Retina/choroid:
Due to the generalized vasculopathy in systemic sclerosis, anomalies of the small blood vessels of the retina/choroid often occur, with accompanying retinal bleeding or hard exudates (deposits of fatty substances in the retina). Other manifestations include retinal thinning, drusen (deposits of metabolic products), choroidal scars, age-related macular degeneration (AMD), epiretinal membrane (thin membrane over the retina that can impair vision), macular foramen (retinal hole at the site of sharpest vision).
Glaucoma (green star):
Open angle glaucoma, normal pressure glaucoma
The increased risk of glaucoma in systemic sclerosis can be explained by the chronic circulatory disorders of the optic nerve. Another explanation would be the increase in intraocular pressure caused by systemic cortisone therapy.
Other ocular manifestations:
inflammation of the optic nerve, oculomotor paresis (paralysis of the third cranial nerve)
Summary:
The eye is a sensitive marker of the onset or deterioration of the autoimmune response in rheumatic diseases such as collagenosis. Early detection of possible complications increases the quality of life and the visual quality for the patient.
