Autoimmune conjunctivitis refers to a group of conditions that can lead to epithelial damage of the ocular surface, reduced tear film stability, and the development of ocular surface tear dysfunction, thereby significantly impairing vision. Major associated conditions include Sjögren's syndrome, cicatricial pemphigoid, and Stevens-Johnson syndrome.
Sjögren's Syndrome
Etiology
Sjögren's syndrome (SS) is a systemic, multi-organ disease characterized by a triad of symptoms: dry eye, dry mouth, and connective tissue damage (e.g., arthritis). A diagnosis can be made if two of the three symptoms are present. This condition is more common among postmenopausal women. The lacrimal glands exhibit infiltration by lymphocytes and plasma cells, resulting in glandular hyperplasia, structural disruption, and loss of function.
Clinical Manifestations
Dry eye symptoms are typically seen in affected individuals. Conjunctival hyperemia, irritation, mild conjunctival inflammation, and mucous stringy discharge are common signs. Corneal epithelial defects, particularly in the inferior corneal region, are frequently observed, along with filamentary keratitis. Pain tends to be more pronounced in the evening compared to the morning. Tear film instability, abnormal results in tear secretion tests, and positive conjunctival and corneal staining with vital dyes such as rose bengal and lissamine green provide diagnostic clues.
Diagnosis
Lymphocytic and plasma cell infiltration observed in salivary gland biopsies combined with clinical symptoms confirms the diagnosis.
Treatment
Symptomatic management is the primary approach aimed at alleviating symptoms. Therapies such as artificial tears, punctal occlusion, or moisture chamber spectacles may be utilized. Additional details can be found in information related to dry eyes.
Stevens-Johnson Syndrome
Etiology
The onset of Stevens-Johnson Syndrome (SJS) is associated with the deposition of immune complexes in the dermis and conjunctival stroma. Certain drugs, including sulfonamides, anticonvulsants, salicylates, penicillin, ampicillin, and isoniazid, or infections caused by herpes simplex virus, Staphylococcus aureus, or adenovirus, have been implicated as potential triggers.
Clinical Manifestations
This condition is characterized by polymorphic erythematous lesions involving mucous membranes and the skin. It is more common in young individuals and rarely occurs after the age of 35.
Patients may present with prodromal symptoms, such as fever, headache, or upper respiratory tract infection, after exposure to certain drugs or compounds. Severe cases may include high fever, myalgia, nausea, vomiting, diarrhea, migratory arthralgia, and pharyngitis. Cutaneous and mucosal manifestations typically appear a few days later, with the disease process lasting approximately 4–6 weeks.
Ocular manifestations fall into acute and chronic categories. During the acute phase, symptoms such as ocular pain, irritation, discharge, and photophobia are commonly reported. Bilateral conjunctival involvement manifests initially as mucopurulent conjunctivitis and superficial scleritis, with corneal ulcers being less common. In some patients, severe anterior uveitis may also develop. Intense inflammation of the ocular surface leads to the loss of conjunctival goblet cells and mucin deficiency, resulting in tear film instability. Goblet cell destruction combined with cicatricial obstruction of the lacrimal gland ducts can cause severe dry eye. Chronic sequelae such as entropion, trichiasis, and eyelid margin keratinization can trigger persistent epithelial damage to the cornea, leading to vascular scarring of the cornea and substantial visual impairment.
Treatment
Systemic corticosteroid therapy can slow disease progression, and topical corticosteroids are beneficial during the acute phase to reduce inflammation and alleviate symptoms. Cleaning conjunctival secretions followed by artificial tear application can relieve discomfort. Surgical correction of entropion and trichiasis is necessary when present.
Cicatricial Pemphigoid
Cicatricial pemphigoid is a chronic, nonspecific conjunctivitis of unknown etiology that is often challenging to treat. It may be accompanied by lesions in the oral cavity, nasal cavity, valvular regions, and skin. The condition tends to be more severe in female patients compared to male patients. Some cases exhibit a tendency for spontaneous remission.
Clinical Manifestations
This condition is commonly characterized by recurrent episodes of moderate, nonspecific conjunctivitis, occasionally accompanied by mucopurulent discharge. A hallmark of this disease is conjunctival scarring, which leads to symblepharon, particularly of the lower eyelid, and sequelae such as entropion and trichiasis. Based on disease severity, it can be classified into four stages: stage I as subconjunctival fibrosis, stage II as fornix shortening, stage III as symblepharon, and stage IV as extensive symblepharon resulting in restricted ocular movements.
Recurrent conjunctival inflammation can damage goblet cells, and conjunctival scarring can obstruct the secretory ducts of the lacrimal glands. The combined deficiency of aqueous and mucin components in the tear film ultimately results in dry eye. In cases with concurrent entropion or trichiasis, corneal injury, neovascularization, scarring, ulcers, or squamous metaplasia of the ocular surface epithelium can occur.
Diagnosis
The diagnosis can be made based on clinical manifestations and conjunctival biopsy findings showing eosinophils or immune-positive substances (IgG, IgM, IgA) within the basement membrane as observed through immunofluorescence studies. Circulating anti-basement membrane antibodies can also be detected in the serum of some patients with pemphigoid.
Treatment
Treatment should be initiated before significant scarring develops to limit tissue damage. Oral dapsone and immunosuppressive agents such as cyclophosphamide have shown efficacy in some patients. In patients with a prolonged disease course, severe complications such as corneal dryness and complete symblepharon often lead to blindness. Ocular surface reconstruction surgery may be considered in select cases based on clinical need.