(P-BT-9) Epidemiology of and treatments for ligneous conjunctivitis: A literature review amid a surging demand for plasminogen eye drops in Québec, Canada

Background/Case Studies: Ligneous conjunctivitis (LC) is the most common manifestation of congenital type 1 plasminogen deficiency (cPD). LC can be treated with plasminogen eye drops, which (in the absence of a commercial preparation) may be prepared by a blood service. Until recently, only one patient with LC had been initiated on plasminogen eye drops in Québec (Canada). However, three more patients with LC have recently been initiated on this treatment. Whether this surging demand may continue to grow is unclear, in part because the characteristics of patients with LC and their treatment patterns are not well characterized. This review aimed to synthesize information on documented cases of LC, focusing on epidemiological data and treatment patterns.

Study

Design/Methods: On May 5, 2021, MEDLINE records were reviewed to identify published cases of LC. Only articles published in 1997 or later were selected owing to concerns related to diagnostic accuracy before the identification of cPD as the predominant cause of LC.

Results/Findings: Seventy-eight articles were published in 1997 or later and reported data on 210 patients with LC. Among the 206 patients with available data on sex, 130 (63.1%) were females, corresponding to a female:male ratio of 1.71:1. Among the 118 patients with available data on the timing of symptom onset, median age of symptom onset was 1.4 years, with 99 (83.9%) patients experiencing their first symptoms < 10 years of age. Included articles reported information on ≥1 medical (ie, non-surgical) intervention for 117 patients (55.7%). At the patient level, local or systemic corticosteroids (N=56 [47.9%]), antibiotics (N=46 [39.3%]), cyclosporine A (N=46 [39.3%]), heparin (N=30 [25.6%]), and FFP eye drops (N=27 [23.1%]) were the most common medical interventions. Only nine patients (7.7%) received plasminogen eye drops, and seven articles explicitly mentioned the lack of a commercial preparation as a reason for not initiating this treatment.

Conclusions: To the best of our knowledge, no prior reviews have synthesized data on so many documented cases of LC. The first symptoms of LC typically manifest during early childhood, so that patients may need lifelong treatment. The treatment patterns for LC were highly heterogeneous, and few patients in our review received plasminogen eye drops, at least in part due to the lack of a commercial preparation. Therefore, the demand for plasminogen eye drops is poised to grow further as healthcare providers become aware of the apparent effectiveness of plasminogen eye drops. Nonetheless, more studies are needed to assess the efficacy of plasminogen eye drops.

Importance of research: This review synthesized data on an unprecedented number of LC cases. The above data will help providers of plasminogen eye drops (e.g., blood services) anticipate the demand for this product.