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Ocular cicatricial pemphigoid (Review)
Daniel Constantin Branisteanu1, Gabriela Stoleriu2, Daciana Elena Branisteanu3
1Department of Ophthalmology, 'Grigore T. Popa' University of Medicine and Pharmacy, 700115 Iasi, Romania.
Experimental and Therapeutic Medicine
|September 9, 2020
Summary
Ocular cicatricial pemphigoid, a chronic autoimmune eye disease, can cause vision loss without treatment. Prompt diagnosis and long-term systemic therapy effectively control 90% of cases, preventing severe ocular complications.
Area of Science:
- Ophthalmology
- Autoimmune Diseases
- Immunology
Background:
- Ocular cicatricial pemphigoid (OCP) is a chronic autoimmune subepithelial blistering disease affecting the conjunctiva.
- It is a severe form of mucous membrane pemphigoid, leading to significant ocular complications and potential vision loss if untreated.
- The pathogenesis involves a type II hypersensitivity response, likely triggered by genetic predisposition and environmental factors.
Purpose of the Study:
- To provide a comprehensive overview of ocular cicatricial pemphigoid, including its pathogenesis, diagnosis, and management.
- To highlight the importance of early diagnosis and effective treatment strategies to prevent vision loss.
- To discuss current therapeutic approaches, from first-line treatments to options for recalcitrant cases.
Main Methods:
- Review of existing literature on ocular cicatricial pemphigoid.
- Analysis of diagnostic criteria, including biopsy and immunofluorescence.
- Evaluation of treatment protocols and their efficacy.
Main Results:
- Without therapy, 75% of OCP cases develop visual loss due to complications like severe dry-eye syndrome and corneal damage.
- Conjunctival biopsy with direct immunofluorescence is the gold standard for diagnosis, though up to 40% of results may be negative.
- Long-term systemic therapy achieves 90% disease control, with Dapsone as first-line for mild-moderate cases and immunosuppressants for severe disease.
Conclusions:
- Ocular cicatricial pemphigoid requires prompt diagnosis and aggressive, long-term management to prevent irreversible vision loss.
- Treatment strategies vary based on disease severity, ranging from Dapsone to potent immunosuppressants and biologics for refractory cases.
- Multidisciplinary care is essential, addressing ocular surface disease and considering surgical interventions only during quiescent phases.
