Autoimmune Diseases Affecting Ocular Surface
Mohammed Fawzy Abdel Latif Ata;
Abstract
Autoimmune diseases arise from an abnormal immune response of the body against substances and tissues normally present in the body (autoimmunity).
Ocular surface autoimmune diseases encompass a diverse spectrum of pathologies and manifest as ocular specific (e.g., Dry Eye, Mooren's ulcerative keratitis), systemic (e.g., Sjögren's syndrome, ocular cicatricial pemphigoid (OCP)), or occur secondary to other common autoimmune diseases (e.g., rheumatoid arthritis, systemic lupus erythematosus (SLE)). Aberrant activation of the innate and adaptive immune responses underlies the immunopathogenesis of these disorders. The etiologies are unknown, but the general hypothesis predicts a combination of excessive or atypical stimuli and/or immunoregulatory dysfunction, combined with genetically predisposed factors and/or hormone imbalance provides an environment conducive to activation of autoreactive lymphocytes. These autoreactive T and B cells are the basis of autoimmune-mediated pathology.
The hallmark symptom of Sjögren's syndrome is a generalized dryness, typically including xerostomia and keratoconjunctivitis sicca, part of what are known as sicca symptoms neither a cure for Sjögren's syndrome nor a specific treatment is known to permanently restore gland secretion. Instead, treatment is generally symptomatic and supportive. Moisture replacement therapies such as artificial tears may ease the symptoms of dry eyes. Some patients with more severe problems use goggles to increase local humidity or have punctal plugs inserted to help retain tears on the ocular surface for a longer time. Additionally, cyclosporine (Restasis) is available by prescription to help treat chronic dry eye by suppressing the inflammation that disrupts tear secretion.
Patients with MU typically present with redness, tearing, photophobia, but pain is the most outstanding feature. The pain often is incapacitating and may be out of proportion to the inflammation. There may be a decrease in visual acuity secondary to associated iritis, central corneal involvement, irregular astigmatism due to peripheral corneal thinning. The disease maybegin with several patchy, peripheral stromal infiltrates which coalesce, more often in the medialand lateral quadrants than in the superior and inferior ones. Most experts would agree on a step-wise approach to the management of Mooren’s ulcer, which is outlined as follows: Topical Steroids, conjunctival Resection, Immunosuppressive Chemotherapy and Amniotic membrane transplant.
OCP is a chronic, slowly progressive, bilateral blinding, systemic autoimmune disease. Multiple antigens in the BMZ of squamous epithelia may serve as targets for a spectrum of autoantibodies observed in OCP. Molecular definition of these autoantigens facilitates the classification and characterization of subsets of OCP. Sera from patients with OCP have been shown to recognize beta 4 integrin, which is a 205-kDa protein, also known as CD104. A subset of patients with clinical features similar to OCP also has been shown to have autoantibodies against epiligrin, which is identified as laminin 5, a ligand for alpha 6 beta 4 integrin, and autoantibodies to the alpha 6 integrin subunit. OCP probably is a spectrum of several different diseases associated with different target antigens, different triggers, and different therapeutic responses.
Ocular manifestations include red eye, tearing, dry eye, blepharospasm, itching, grittiness, heavy eyelid, foreign body sensation, decreased vision, burn sensation, photophobia, diplopia no topical agent is effective in stopping OCP activity. In selected patients, subconjunctival steroid injections or subconjunctival injections of mitomycin C may be used temporarily for slowing disease progression, while systemic therapy takes effect. Adjuvant treatment with topical lubricants should be used in patients with dry eye symptoms. The use of topical cyclosporine and tacrolimus ointment has also been described in anecdotal reports to aid in the control of surface inflammation.
Ocular surface autoimmune diseases encompass a diverse spectrum of pathologies and manifest as ocular specific (e.g., Dry Eye, Mooren's ulcerative keratitis), systemic (e.g., Sjögren's syndrome, ocular cicatricial pemphigoid (OCP)), or occur secondary to other common autoimmune diseases (e.g., rheumatoid arthritis, systemic lupus erythematosus (SLE)). Aberrant activation of the innate and adaptive immune responses underlies the immunopathogenesis of these disorders. The etiologies are unknown, but the general hypothesis predicts a combination of excessive or atypical stimuli and/or immunoregulatory dysfunction, combined with genetically predisposed factors and/or hormone imbalance provides an environment conducive to activation of autoreactive lymphocytes. These autoreactive T and B cells are the basis of autoimmune-mediated pathology.
The hallmark symptom of Sjögren's syndrome is a generalized dryness, typically including xerostomia and keratoconjunctivitis sicca, part of what are known as sicca symptoms neither a cure for Sjögren's syndrome nor a specific treatment is known to permanently restore gland secretion. Instead, treatment is generally symptomatic and supportive. Moisture replacement therapies such as artificial tears may ease the symptoms of dry eyes. Some patients with more severe problems use goggles to increase local humidity or have punctal plugs inserted to help retain tears on the ocular surface for a longer time. Additionally, cyclosporine (Restasis) is available by prescription to help treat chronic dry eye by suppressing the inflammation that disrupts tear secretion.
Patients with MU typically present with redness, tearing, photophobia, but pain is the most outstanding feature. The pain often is incapacitating and may be out of proportion to the inflammation. There may be a decrease in visual acuity secondary to associated iritis, central corneal involvement, irregular astigmatism due to peripheral corneal thinning. The disease maybegin with several patchy, peripheral stromal infiltrates which coalesce, more often in the medialand lateral quadrants than in the superior and inferior ones. Most experts would agree on a step-wise approach to the management of Mooren’s ulcer, which is outlined as follows: Topical Steroids, conjunctival Resection, Immunosuppressive Chemotherapy and Amniotic membrane transplant.
OCP is a chronic, slowly progressive, bilateral blinding, systemic autoimmune disease. Multiple antigens in the BMZ of squamous epithelia may serve as targets for a spectrum of autoantibodies observed in OCP. Molecular definition of these autoantigens facilitates the classification and characterization of subsets of OCP. Sera from patients with OCP have been shown to recognize beta 4 integrin, which is a 205-kDa protein, also known as CD104. A subset of patients with clinical features similar to OCP also has been shown to have autoantibodies against epiligrin, which is identified as laminin 5, a ligand for alpha 6 beta 4 integrin, and autoantibodies to the alpha 6 integrin subunit. OCP probably is a spectrum of several different diseases associated with different target antigens, different triggers, and different therapeutic responses.
Ocular manifestations include red eye, tearing, dry eye, blepharospasm, itching, grittiness, heavy eyelid, foreign body sensation, decreased vision, burn sensation, photophobia, diplopia no topical agent is effective in stopping OCP activity. In selected patients, subconjunctival steroid injections or subconjunctival injections of mitomycin C may be used temporarily for slowing disease progression, while systemic therapy takes effect. Adjuvant treatment with topical lubricants should be used in patients with dry eye symptoms. The use of topical cyclosporine and tacrolimus ointment has also been described in anecdotal reports to aid in the control of surface inflammation.
Other data
| Title | Autoimmune Diseases Affecting Ocular Surface | Other Titles | أمــراض المناعـة الذاتيـة وتأثــيرها علـى سطـح العــين | Authors | Mohammed Fawzy Abdel Latif Ata | Issue Date | 2015 |
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