Guidelines for the management of open‐angle glaucoma

Abstract

These guidelines provide a brief guide on how primary open-angle glaucoma, exfoliation glaucoma and pigment glaucoma can be diagnosed, treated and monitored. The aim is to nationally spread knowledge-based high-quality care, to stimulate the use of scientifically evaluated and effective measures, to even out differences in care and to provide support in setting priorities. Angle-closure glaucoma is not dealt with in this publication but is mentioned where relevant. The National Working Group Glaucoma has made an inventory of applicable current international, Nordic and Swedish guidelines, care programmes and guidelines for glaucoma. Through the regional representatives, we have reviewed a majority of regional and local documents. Answers to specific questions have been sought in PubMed and via Cochrane reports. In compiling these guidelines, we have primarily based our work on the European glaucoma guidelines (European Glaucoma Society, 2021) (which are evidence-graded according to the AGREE system regarding so-called key questions 2020). For evidence grading (A–D), the Finnish guidelines from 2014 were also used (Tuulonen et al., 2014). Furthermore, the current document is based on the previous Swedish guidelines, Guidelines for Glaucoma Care from 2010 (Heijl et al., 2012). On the issues where there is insufficient scientific evidence, the group has discussed its way to a consensus on best clinical practice. The goal of all glaucoma treatment is to preserve the patient's visual function, well-being and related quality of life with a long-term sustainable use of resources. Treatment costs in the form of discomfort and side effects, as well as economic costs for the individual patient and society, should be taken into account. Quality of life is strongly linked to visual function and is affected differently depending on life situation. In general, patients with mild glaucoma damage have good visual function and a largely preserved quality of life, while quality of life is severely affected if both eyes have advanced vision loss that, e.g., affects driving. Glaucoma treatment must be individualized and adapted to the patient, situation and available resources. The increasing availability of randomized controlled clinical trials (RCTs) makes it possible to base clinical recommendations to a greater extent on scientific evidence. There are a number of RCTs that clearly show that intraocular pressure lowering treatment of glaucoma is effective. Glaucoma damage can be slowed down and, in the best case, stopped if patients are properly diagnosed, treated and followed up. The degree of visual field damage and how it develops over time determines the individual treatment strategy (Figure 1). The goal is to maintain good quality of life without advanced loss of visual function throughout life. Glaucoma is a progressive disease that causes typical damage to the optic nerve head, the retinal nerve fibre layer and the visual field. Intraocular pressure is not included in the definition. In primary open-angle glaucoma, the chamber angles should be open and specific causes of glaucoma should not be identifiable. Subjects with primary open-angle glaucoma may have high (high tension glaucoma) or normal (normal tension glaucoma) intraocular pressure. In exfoliation glaucoma, there are also protein precipitates and exfoliations. This type of glaucoma is also known as pseudoexfoliation glaucoma (PEX glaucoma) or capsular glaucoma. The exfoliation material is most easily visible on the front surface of the lens. In the Nordic countries, we often include exfoliation glaucoma in the group of primary glaucomas, while in other parts of the world, it is considered a secondary glaucoma. Pigmentary glaucoma is a secondary glaucoma characterized by the Krukenberg spindle (pigment on the corneal endothelium), slits in the mid-periphery of the iris (transillumination defects) and a smooth, dark pigmented trabecular meshwork in the chamber angle. It is a rare form of glaucoma that typically affects younger people (20–50 years), often myopic and more common in men (5:1). Glaucoma is one of the most common age-related eye diseases and among the leading causes of blindness in the world. In a Swedish study, 42% of patients with open-angle glaucoma became blind in one eye and 16% in both eyes during their lifetime. The disease is uncommon before the age of 40 and increases with age. It is estimated that the global prevalence of open-angle glaucoma is 3.5% in the 40–80-year-old group and 0.5% for angle-closure glaucoma. In 2014, the number of subjects with glaucoma was estimated to reach 76 million by 2020 and is expected to increase to 112 million by 2040. In the context of glaucoma, the terms exfoliation, pseudo-exfoliation (PEX) and exfoliation syndrome are often used interchangeably. A Swedish study published in 2008 showed that about 25% of all visits to Swedish ophthalmic care providers were glaucoma-related. The number of patients in Sweden with diagnosed glaucoma is not known but has previously been estimated at around 100 000. However, this figure is very uncertain. Based on prescriptions in Sweden in 2008 and 2017, the number of unique individuals treated with intraocular pressure-lowering drops was found to be 144 000 and 172 000, respectively, an increase of almost 20% in 9 years. This increase is expected to continue. In addition to patients with diagnosed glaucoma and treated ocular hypertension, many patients with risk factors for developing glaucoma are also checked at Swedish eye clinics, such as untreated ocular hypertension, exfoliations, pigment syndrome and more. This means that significantly more patients than the 172 000 are covered by the guidelines and undergo examination at Swedish eye clinics. Life expectancy in Sweden has increased significantly in recent decades and with it the proportion of older people in the population. According to Statistics Sweden's population projection for 2019, the number of inhabitants over the age of 80 will increase by over 50% between 2019 and 2030. Several studies predict a similar percentage increase in the number of patients monitored and/or treated in glaucoma care by 2040. In order to maintain a decent level of glaucoma care, significant resources must therefore be added. Open-angle glaucoma usually does not cause symptoms until at late stages. The elevated eye pressure is usually painless. The loss of visual field usually affects the paracentral visual field first and is difficult to detect for the patient. In connection with pigment release in people with pigmentary glaucoma, acute pressure increase may occur. At the same time, corneal oedema can cause blurred vision and halo phenomena. Traditionally, the upper limit of normal intraocular pressure has been considered to be ≤21 mm Hg. It is based on several population studies where the average pressure in the adult population is about 16 mm Hg and has a standard deviation of about 2.5 mm Hg. However, this pressure level does not say anything about what eye pressure is harmful at the individual level, since some people suffer injuries at significantly lower levels, and some can tolerate higher levels. Thus, from a functional point of view, all pressure levels that do not give rise to glaucoma damage are ‘normal’ in the individual. There are a number of factors that increase the risk of developing and/or worsening glaucoma. Knowledge of risk factors is of great importance in order to identify persons who may require (more frequent) monitoring or (more vigorous) treatment, at least until the individual rate of progression is known. Several risk factors in the same person further increase the risk. Individual Eyes General diseases Do not correct intraocular pressure with CCT algorithms. Rule of thumb: thin CCT < 500 μm, thick CCT > 600 μm. Individual Eyes It is unclear whether cardiovascular diseases also affect the risk of glaucoma progression. The following examination and patient history data should be included in the primary assessment of patients with manifest as well as suspected glaucoma, or with risk factors for developing glaucoma. These parameters then form the basis for decisions on how any continued follow-up and treatment should be designed. OCT abnormalities alone are not sufficient to make a diagnosis of glaucoma. The above initial examination package is extensive, but of value in order to be able to perform an adequate assessment and decide on further checks and treatment. Patients with suspected or manifest glaucoma are checked regularly over a long period of time and a proper investigation helps to avoid both over- and under-diagnosis. All examinations do not necessarily have to be carried out at the same time. Rather, it may be valuable to divide these, for example into a visit to the doctor and one to a nurse, optician or optometrist. The time interval between these initial assessments is determined by the level of eye pressure and other risk factors. To get a good idea of the untreated pressure before starting treatment, at least two, but preferably three, separate pressure measurements are recommended. These are often done at different times of the day. At very high-pressure levels, treatment can be started at the first visit, but even in these cases, it should be preceded by at least two separate pressure measurements, preferably performed by different examiners and with different Goldmann tonometers. Follow each patient using the same pressure measurement method – GAT is standard. 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