Wide Range of Eye Complications Associated with Scleroderma, Study Shows

Wide Range of Eye Complications Associated with Scleroderma, Study Shows

People with systemic sclerosis (SSc) show a wide range of eye complications, with dry eye disease, eyelid skin alterations, and retinal abnormalities as the most common, a Hungarian study shows.

The variety of eye complications found in these patients reflect the heterogeneity, or diversity, of SSc — and highlights the need for routine eye examinations in people with the disease, the researchers said.

The study, “A Wide Spectrum of Ocular Manifestations Signify Patients with Systemic Sclerosis,” was published in the journal Ocular Immunology and Inflammation.

SSc is a chronic disease characterized by microvasculopathy — degeneration or inflammatory damage in small blood vessels — and abnormal activation of the immune system. Another hallmark of the disease is progressive, widespread fibrosis, or tissue scarring, in the skin and in multiple organs — including the eyes. Microvasculopathy is a predictor of mortality in SSc patients.

While eye symptoms appear to be common and diverse in SSc patients, most data on these complications come from a few case reports and studies involving a small number of patients.

Eye complications may develop at any stage of the disease, and also may involve the structures around the eye, including the eyelid. Due to the great variability in eye symptoms observed in SSc patients, and given that aging also is associated with eye problems, there is still some uncertainty about whether some eye symptoms are associated or not with the disease itself.

To learn more, researchers at University of Debrecen, in Hungary, set out to evaluate the clinically significant SSc-associated eye symptoms in 51 people with the disease. All were recruited at a tertiary referral center.

Participants — 48 women and three men — were all Caucasian, had a mean age of 65.39 years, and had been living with the disease for a mean of 18.45 years. A total of 44 patients (86%) were diagnosed with limited scleroderma, and seven (14%) with diffuse scleroderma.

The patients’ degree of microvasculopathy was assessed using a standard method called nailfold capillaroscopy (NFC).

The team conducted a comprehensive ophthalmological evaluation on the participants. It included the determination of the best corrected visual acuity (BCVA) using a Snellen eye chart. Close examination was done of the eyelids, lashes, precorneal tear film — film covering the anterior surface of the cornea — cornea, iris, and conjunctiva, or the protective and lubricative layer around the eyeball. Dry eye disease was assessed through the patient-reported ocular surface disease index (OSDI) questionnaire and the tear film breakup time (tBUT) test.

Since eye symptoms between the two scleroderma subtypes were not significantly different, the researchers analyzed them as a whole, without dividing them into two categories.

Results showed that only two participants (3.92%) had no eye complications. The most common SSc-associated eye symptoms detected were dry eye disease (64.71% of patients), eyelid skin alterations (56.86%), and abnormalities in the retina (50.98%).

A high percentage of participants (50.98%) also showed cataracts, or clouding of the eye lens leading to blurry, dim vision. However, the researchers did not consider cataracts to be associated with the underlying disease, as it is expected to occur with age and may be partially linked to systemic corticosteroid administration, which SSc patients often receive.

Less common eye symptoms included altered conjunctiva (15.69% of participants), iris abnormalities (13.73%), and several types of glaucoma (21.57%). Glaucoma refers to damage to the eye due to elevated ocular blood pressure.

These findings were in agreement with those reported in previous studies.

“The diverse ocular findings show that SSc exists along a wide spectrum ranging from its most common manifestations, such as skin abnormalities, DED [dry eye disease], or retinal microvascular abnormalities, to rare iris abnormalities that are attributed to the heterogeneity of SSc,” the researchers said.

Researchers also found that greater damage to the small blood vessels, assessed by NFC, was significantly associated with weaker tear film stability (lower tBUT score), and more frequent dry eye symptoms (higher OSDI scores). They noted that further research is required to determine whether NFC could be used as a diagnostic tool for dry eye disease.

The team also emphasized that “clinicians must be aware of the diverse spectrum of the eye and [surrounding structures’] involvement in SSc, and accordingly include ocular investigations into their routine practice.”

“Early recognition and appropriate treatment can prevent sight-threatening complications or other significant abnormalities in addition to help maintain patients’ quality of life,” the researchers said.

Marta Figueiredo holds a BSc in Biology and a MSc in Evolutionary and Developmental Biology from the University of Lisbon, Portugal. She is currently finishing her PhD in Biomedical Sciences at the University of Lisbon, where she focused her research on the role of several signalling pathways in thymus and parathyroid glands embryonic development.
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Patrícia holds her PhD in Medical Microbiology and Infectious Diseases from the Leiden University Medical Center in Leiden, The Netherlands. She has studied Applied Biology at Universidade do Minho and was a postdoctoral research fellow at Instituto de Medicina Molecular in Lisbon, Portugal. Her work has been focused on molecular genetic traits of infectious agents such as viruses and parasites.
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Marta Figueiredo holds a BSc in Biology and a MSc in Evolutionary and Developmental Biology from the University of Lisbon, Portugal. She is currently finishing her PhD in Biomedical Sciences at the University of Lisbon, where she focused her research on the role of several signalling pathways in thymus and parathyroid glands embryonic development.
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