An autoimmune disorder called GPA (or Granulomatosis with polyangiitis) can affect the eyes. While the most common symptoms are respiratory, the inflamed blood vessels featured in GPA can cause eye pain and blurred vision. Formerly called Wegener’s Granulomatosis, GPA can occur following an infectious disorder and/or toxic environmental exposure (according to the National Organization for Rare Disorders). If you have keratoconus, developing GPA can worsen your keratoconus symptoms.
What is GPA and How Does it Differ From Other Autoimmune Disorders?
Autoimmune disorders are featured by an immune system that mistakenly attacks the body’s own organs instead of resisting invading particles (e.g., germs). In contrast to most other autoimmune disorders – that cause inflammation affecting particular parts of the body (e.g., joints or intestinal tract) – GPA is characterized by inflammation of small and medium-sized blood vessels (termed vasculitis).
An article in Ophthalmology and Therapy in 2019 reported that ocular and orbital eye manifestations are common in almost half of patients with GPA. Moreover, this article also notes that vasculitis in the eye can be the initial symptom of GPA, and that corneal involvement is common in people with GPA who have ocular vasculitis.
Since bacterial and viral infections – such as infection with the Covid-19 coronavirus – can trigger the onset of GPA (which is often featured by rapidly-progressive nasal congestion and coughing), an awareness of the symptoms of this potentially life-threatening disorder is important during this pandemic.
How Vasculitis Can Impact Vision in Someone with Keratoconus
Cornea pain is common in people with moderate-to-severe keratoconus, and many keratoconus-afflicted people experience seeing a “halo” around objects that interferes with driving a motor vehicle. Three of the most common symptoms for anyone with vasculitis affecting the eyes are redness, a sensitivity to light, and eye pain. Therefore, the vasculitis associated with GPA – if existent in the eyes – can both damage the cornea and worsen overall eyesight. Since someone with moderate-to-severe keratoconus is already at heightened risk for legal-blindness, a diagnosis of GPA can increase the risk of progressing to legal-blindness.
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Prevalence of GPA in US Population
The prevalence of GPA in the US prior to the Covid-19 pandemic was considered to be three cases per 100,000 people. GPA also primarily affects people of Caucasian ancestry, and people between the ages of 40-60. Furthermore, people with underlying chronic disorders (e.g., diabetes) appear to be more at risk for developing GPA after a bacterial or viral infection. Overall, this autoimmune disorder has been considered extremely rare. However, unusual inflammatory responses have been identified in people who have survived severe Covid-19 infections (involving placement on a ventilator to rest the lungs during an ICU hospitalization).
The symptoms of GPA can be mild at first, but tend to progress over the course of a year. Meanwhile, the primary diagnostic tools for GPA are the following (per the National Institutes of Health’s National Center for Advancing Translational Research):
- Blood tests (including an ANCA test);
- Biopsy of affected area(s);
- Imaging of the lungs (e.g., MRI of the lungs)
The earlier that a GPA diagnosis is confirmed, the earlier medication treatment can commence to enable its remission.
If you are a keratoconus-afflicted person who is experiencing increased corneal pain and sensitivity to light, please do not delay phoning a professional at the Precision Keratoconus Center to discuss your symptoms.