Eye Involvement of Systemic Sclerosis (Scleroderma)

Eye Involvement

ISN Guide to Eye Involvement: Dr. Liz Irvin

Hello, I am Dr. Liz Irvin, your ISN Guide to Eye Involvement. In addition to helping Dr. C. Stephen Foster develop resources for this section of the ISN website, I am happy to try to respond to questions that you may have or to redirect you to resources that may be helpful. Email liz-irvin@sclero.org.

Normal Tension Glaucoma
Scleroderma Eye Involvement

NTG Symptoms/Diagnosis
NTG Treatments

Autoimmune Iritis or Uveitis
Eye Support Groups

Personal Stories
Related Pages
Media Stories

These symptoms may sometimes occur in systemic sclerosis (scleroderma). Scleroderma (SD) affects everyone differently. Just because something is listed here does not mean it is caused by scleroderma nor that an individual scleroderma patient will ever experience it. See Disclaimer.

What is Normal Tension Glaucoma (NTG)?

What is Glaucoma? Glaucoma is a group of eye diseases that gradually steals sight without warning and often without symptoms. Glaucoma Research Foundation.

A Patient Guide to Ocular Inflammatory Disease. This is an online guide, covering symptoms, diagnosis and treatment for inflammatory eye diseases. In addition, printed copies of this guide can be requested free of charge from the Ocular Immunology and Uveitis Foundation. (Also see: Dr. C. Stephen Foster, ISN Medical Advisory Board)

Glaucomatous Type Abnormalities in Patients with Systemic Sclerosis. Glaucomatous type abnormalities were more frequent in patients with SSc compared to the control group. Such abnormalities may be attributed to the vasculopathy that is observed in patients with SSc, affecting the vessels of the optic disc. G. Kitsos. FRI0347 EULAR 2006.

Eye Involvement in Scleroderma (Systemic Sclerosis)

Eye symptoms and diseases that may be related to scleroderma, including Normal Tension Glaucoma and Autoimmune Iritis or Uveitis. ISN

Sclerokeratitis and facial skin lesions: a case report of pyoderma gangrenosum and its response to dapsone therapy. Pyoderma gangrenosum is a rare autoimmune disorder that can affect the eye. Biopsy of affected tissue is the key to diagnosis. Immunomodulating therapy can be a useful adjunct to the traditional steroid therapy. PubMed. Cornea 2007 Feb;26(2):215-9.

Proliferative vascular retinopathy in polymyositis and dermatomyositis with scleroderma (overlap syndrome). Retinal vascular occlusion with development of proliferative changes can occur in polymyositis/dermatomyositis. PubMed. Ocul Immunol Inflamm. 2007 Jan-Feb;15(1):45-9. (Also see: Overlap Syndrome and Polymyositis/Dermatomyositis)

Therapy insight: The recognition and treatment of retinal manifestations of systemic vasculitis. A variety of retinal signs can occur in patients who have systemic vasculitides, or who experience complications of these diseases or their treatment. Although treatment of these retinal manifestations is usually the treatment of the systemic disease, specific treatment is occasionally indicated to preserve vision. PubMed. Nat Clin Pract Rheumatol. 2006 Aug;2(8):443-51. (Also see: Vasculitis)

Ophthalmological involvement in rheumatic disease. The main conclusion of our study is that rheumatic patients need to be referred to an ophthalmologist for the diagnosis and the optimal treatment of ocular involvement. PubMed. Oftalmologia. 2006;50(2):56-61.

Laser in situ keratomileusis (LASIK) in patients with autoimmune diseases. Laser in situ keratomileusis may be a reasonable option in patients with well-controlled or inactive autoimmune disease. PubMed. J Cataract Refract Surg. 2006 Aug;32(8):1292-5.

A significant increase of normal tension glaucoma (NTG) has been discovered in patients with systemic scleroderma. In a 2003 study of 88 scleroderma patients in France, 10 of the patients were found to have NTG, compared to none of the controls.

Pellucid marginal degeneration and scleroderma. Pellucid marginal degeneration is characterised by non-inflammatory and progressive peripheral corneal thinning inferiorly, often with high against-the-rule astigmatism. We report a case of a 55-year-old woman with systemic scleroderma who presented with rapidly progressing against-the-rule astigmatism. PubMed. Clin Exp Optom. 2004 May;87(3):180-4.

Increased prevalence of ocular glaucomatous abnormalities in systemic sclerosis. Ocular abnormalities suggesting glaucomatous neuropathy without ocular hypertension were dramatically more prevalent in patients with SSc. These abnormalities seem to be mild but justify long term follow up. They are consistent with the vascular pathogenic hypothesis for NTG (normal tension glaucoma).PubMed. Ann Rheum Dis. 2004 Oct;63(10):1276-8.

Investigations for retinopathy in an avian model for systemic sclerosis. Our data raise serious doubts about primary posterior ocular involvement in human SSc. However, fundal examinations in patients with SSc may have their justification for assessment of hypertensive retinopathy. PubMed. Exp Eye Res. 2004 Jul;79(1):85-92.

Increased Incidence of Normal Tension Glaucoma (NTG) in Systemic Sclerosis (SSc). NTG is dramatically increased in SSc compared to patients with osteoarthritis. These results might have important clinical implications in SSc and justify long-term follow-up to assess the possible ophthalmological risk. They are also consistent with the vascular pathogenic hypothesis for NTG. Y. Allanore. FRI0077 EULAR 2003.

Scleroderma, stroke, optic neuropathy: a rare association. A known case of scleroderma presented with right hemiparesis, focal seizures, optic atrophy and gangrene of digits. MRI showed multifocal infarcts in both cerebral hemispheres. The rarity of central nervous system affliction in scleroderma and large vessel vasculitis is discussed along with review of literature. PubMed. Neurol India 2002 Dec;50(4):504-7. (Also see: Cardiac Involvement: Stroke)

Symptoms and Diagnosis of Normal Tension Glaucoma

Symptoms of Normal Tension Glaucoma. Regular eye examinations with an ophthalmologist are important to screen for optic nerve damage and vision loss despite a normal eye pressure. eMedicine.

Antiphospholipid antibodies in patients with sensorineural hearing loss. Sensorineural hearing loss can be associated with autoimmune diseases and the presence of antiphospholipid antibodies. Our data suggest that antibodies against beta(2)-glycoprotein seem to coincide with an acute event, such as sudden sensorineural hearing loss, whereas antibodies against phosphatidylserine IgG are detectable in the prolonged sequel, such as in patients with progressive sensorineural hearing loss and normal tension glaucoma. PubMed. Eur Arch Otorhinolaryngol. 2005 Feb 25. (Also see: Antiphospholipid Syndrome and Ear Involvement)

Treatments of Normal Tension Glaucoma

Therapy-resistant inflammatory glaucoma responds to transscleral red laser cyclophotocoagulation. More than one treatment was needed in 52% of eyes, but no cases of hypotony, phthisis bulbi or other complications occurred. OSN Supersite. 09/04/07.

Effect of sex hormones on experimental autoimmune uveoretinitis (EAU). The data support the hypothesis that sex hormones may affect autoimmune diseases by inducing changes in the cytokine balance. This suggests that sex hormone therapy could be considered as an adjunct to anti-inflammatory agents to treat ocular autoimmune diseases in humans. PubMed. Immunol Invest. 2003 Nov;32(4):259-73.

Retinal findings in systemic sclerosis: a comparison with nailfold capillaroscopic patterns. Retinal abnormalities are often seen in patients with SSc and they may reflect the vascular changes characteristic of SSc. However, retinal changes may differ in quality from the changes of nailfold capillaries. PubMed. Ann Rheum Dis 2003 Mar;62(3):204-7. (Also see: Types of Scleroderma)

Autoimmune Iritis or Uveitis (Eye Inflammation)

Autoimmune optic neuropathy. This review considers optic neuropathy that occurs as part of recognized systemic autoimmune disorders and collagen vascular diseases and isolated autoimmune optic neuropathy. PubMed. Curr Neurol Neurosci Rep. 2006 Sep;6(5):396-402.

Help for inflammatory eye disease. Inflammatory Eye Diseases (the most common being scleritis and uveitis) are the fifth-leading cause of blindness in the United States. Prednisone is the common treatment. Now, CellCept—a drug used to prevent organ transplant rejection —can treat eye inflammation and get patients on lower doses of, or even off, prednisone. Ivanhoe Broadcast News 02-20-06.

Uveitis in the internist's office: are a patient's eye symptoms serious? If the diagnosis of uveitis is made, internists must search for an underlying cause, such as infection or an autoimmune disease. PubMed. Cleve Clin J Med. 2005 Apr;72(4):329-39.

Intraocular inflammation in autoimmune diseases. An underlying autoimmune disease was identified in up to 40% of patients with intraocular inflammation (IOI). Immunosuppressive drugs, biologic agents, and IVIG (intravenous immunoglobulin) are employed for the treatment of IOI in autoimmune diseases. PubMed. Semin Arthritis Rheum. 2004 Dec;34(3):602-9.

Prevalence of the Spondyloarthritides in Patients with Uveitis. Uveitis is associated not only with AS and reactive arthritis, but also with undifferentiated spondyloarthritis and psoriatic arthritis. J Rheumatol. November 2004;31:2226-9.

Type I collagen is the autoantigen in experimental autoimmune anterior uveitis. Although human anterior uveitis has been historically characterized as a collagen disease, this is first time collagen has been directly identified as the target autoantigen in uveitis. PubMed. J Immunol. 2004 Jun 1;172(11):7086-94.

Sulfasalazine Reduces the Number of Flares of Acute Anterior Uveitis Over a One-Year Period. The mean number of flares in the pre-SSZ year was 3.4, which was significantly reduced to 0.9 in the year of treatment. J Rheumatol NO. 6 JUNE 2003;30:1277-9.

Granulomatous uveitis, CREST syndrome, and primary biliary cirrhosis. Our case report illustrates an association between ocular and hepatic diseases, which may be entirely coincidental but could, nevertheless, encourage further investigation for a common immunological pathway. Br J Ophthalmol 2000;84:546. (Also see: CREST Syndrome and Liver Involvement)

Support Groups for Eye Diseases

Uveitis Online Support Group C. Stephen Foster, M.D., Medical Advisor.

Uveitis Glossary. Ocular Immunology and Uveitis Foundation, Cambridge, MA USA.

Harvard University / Ocular Immunology and Uveitis Foundation. Extensive patient support forums, glossary, articles, support community. Ocular Immunology and Uveitis Foundation, Cambridge, MA USA.

Uveitis Kids. This is a resource website for kids only and they have access to an online support group page there as well. Ocular Immunology and Uveitis Foundation, Cambridge, MA USA.

Uveitis Support . International online support community for anyone living with ocular inflammatory disease. Ocular Immunology and Uveitis Foundation, Cambridge, MA USA.

Uveitis Information Group The UIG is a UK based organization devoted to providing easily understood patient information and support. UIG.

Eye Involvement Patient and Caregiver Stories

Tata P: Diffuse Scleroderma I am thirty-two years old, and I have been suffering this illness since I was nine...

(Español/Spanish) Tata P: Esclerodermia Difusa Hola, tengo 32 años, y padezco esta enfermedad desde los 9...

Dr. C. Stephen Foster: ISN Medical Advisory Board. Dr. Foster is Clinical Professor of Ophthalmology at Harvard Medical School and Director of the Ocular Immunology and Uveitis Foundation in Cambridge, MA USA. ISN.

Cataracts Surgery and Scleroderma? Ask Dr. Foster Forum.

Sjogren's Syndrome A systemic autoimmune disease that sometimes occurs in overlap with systemic sclerosis (scleroderma) and causes dryness of the eyes, mouth and skin. ISN.

Media Stories and Research

LASIK in Patients with Rheumatic Diseases A Pilot Study. In this small series, we found good outcomes when correcting refractive errors using LASIK in selected patients with controlled rheumatic diseases. In this series, a favorable postoperative visual outcome was obtained with no operative or postoperative vision-threatening complications. PubMed. Ophthalmology. 2005 Sep 14.

A Protein in the Eye May Prevent Immune Response and Protect Eyes from Disease. A protein known as F4/80 found on immune cells in the eye and other parts of the body may have a function in the regulation of the body's immune response and protect delicate tissues that cannot survive the "inflammation" inherent in full-blown immunity. redorbit News. 06/21/05.