Primary immunodeficiencies in Central and Eastern Europe—the power of networking Report on the activity of the Jeffrey Modell Foundation Centers Network in Central and Eastern Europe

Primary immunodeficiencies (PIDs) are genetically inherited disorders of the immune system that encompass a range of inborn errors of immunity [1], from specific disorders affecting selective immune defense mechanisms to serious, even life-threatening diseases characterized by a profound lack of immune functions. The clinical history of these disorders dates back to the 1950s, when the first clinical entity, Bruton’s agammaglobulinemia, was identified and gave rise to the field of PIDs [2]. Since then, the field has grown enormously in unprecedented leaps. To date, up to 400 well-defined PIDs have been described, and our knowledge of their etiology continues to expand. Similar progress is ongoing in the diagnostic and therapeutic algorithms of these disorders, thus significantly improving the clinical outcome [3, 4].

While historically, some important discoveries in the field of immunology have been made in Eastern Europe, such as the discovery of phagocytes and phagocytosis by Elie Metchnikov, Ukrainian and Russian scientist; the beginning and early development in the field of PIDs in the second half of the twentieth century found countries of Eastern Europe in information isolation with restrained access to scientific knowledge. However, at the turn of the millennium, new connections with colleagues and centers in Western Europe and the USA have been driving forces behind the progress in Eastern Europe. While such contacts are important for individual centers, they were not sufficient for the systematic development of the whole field. Individual internal efforts arose in Eastern European countries that strengthened the cooperation within centers in these countries. The first pioneering activity was the initial organization and cooperation of some countries in Central and Eastern Europe under the J Project, a successful educational project that still exists that created the foundation for future activities [5‐7]. Progressive cooperation within the J Project and the establishment of relations with the Jeffrey Modell Foundation (JMF) led to the formation of the JMF Central and Eastern European network (JMF CEE network). This unique situation gave the countries in the Central and Eastern European regions the opportunity to join the global community of JMF Centers that aimed to better identify, diagnose, and treat patients with PIDs at the global scale. The beginning of this program in 2011 marked an era of new possibilities for the involved centers and countries.

Here, we describe the principles of the structure and function of the JMF Centers Network in Central and Eastern Europe and give a report on the progress of the field in involved countries as it was achieved in multiple relevant areas characterizing the complex principles of care in PIDs.

The JMF Centers Network is represented by a global network of JMF Centers that specialize in advancing the diagnosis and treatment of PIDs. The network currently includes nearly 400 centers in 86 countries, spanning 6 continents (for a recent review, see 7).

In the following section, data effective until October 2018 are presented.

The field of PIDs has recently experienced unprecedented development, mainly due to the rapid introduction of novel diagnostic and treatment options. New trends, collaborations, and recommendations around the world are orchestrated by societies for PIDs, such as the ESID in Europe, the Clinical Immunology Society (CIS) in the USA, the Latin American Society for Immunodeficiencies (LASID), the African Society for Immunodeficiencies (ASID), and the Asia-Pacific Society for Immunodeficiencies (ASPID). Similar professional international coordinating activities have been put into place for nursing staff in association with the International Nursing Group for Immunodeficiencies (INGID) and patients collaborating through the International Patient Organization for Primary Immunodeficiencies (IPOPI). In addition to these professional societies, the field is fortunate to benefit from the activities of several great foundations for PIDs that are active in many countries. Prominently, the international dimension of the PID field is encompassed by the worldwide network of the JMF foundation and its centers located in all continents.

The region of Central and Eastern Europe was historically set aside from all these activities and only started to slowly join at the beginning of the twenty-first century. Here, we demonstrate that the JMF Network for Central and Eastern Europe is complementing the J Project which was already active in the geographical area and that recently celebrated its 15th anniversary [7]. The JMCN provided further impulse for Eastern European countries to develop and improve the care of patients with PIDs and to connect with the worldwide field of PIDs. The existence of the JMF Network in the region brings an additional value on top of the natural progress in the field. While in the region of Central and Eastern Europe, JMF Centers working on individual basis had existed previously and encompassed larger regional area including Russia; it was the creation and internal organization of JMF CEE network that added further value for comprehensive development of participating centers. JMF CEE network thus represents more tightly cooperating subset of Eastern European JMF Centers reported in JMF global report on primary immunodeficiencies [8]. The abovementioned internal organization of the network facilitated the internal reorganization of individual centers and inspired continuous progress and development. Regular reporting and participation in network activities also contributed to the centers’ internal structural organization and further translated to better care for patients in the respective countries, which was shown by an improvement in nearly all of the measured parameters. Currently, after ongoing continuous efforts targeted at education in the field, the clinical care of patients with PIDs in countries involved in the network is at the level of the latest knowledge and in line with international guidelines. It is, however, also clear that the achieved state and level of care may not be permanent as much still depends on varying circumstances, such as the economic and political situation in individual countries. A reminder of this instability recently occurred in Romania, where the power of international networking averted the government’s decision to withdraw funding for immunoglobulin treatment and restore it to its original extent.

Despite the progress especially in the clinical care of PID patients, the PID Centers of Central and Eastern Europe are still somewhat falling behind the traditional Western centers, particularly in research, which is one of the most important aspects in the rapidly developing field of immunodeficiencies. Experience has shown how difficult obstacles may arise from the interruption of research continuity, especially in the already economically disadvantaged countries of Eastern Europe. Overcoming these obstacles requires long and systematic work, mainly involving young colleagues, which is particularly facilitated by engagement in international networks. Indeed, we showed a gradual improvement in research activities documented by publications from individual centers as well as in those involving joint publications arising from the network. Nevertheless, there is still an additional scope for improvement, and overcoming the gap between Eastern and Western Europe will require continuous future efforts, for example, the engagement of Eastern European Centers in international research activities organized by the EU.

We showed a major overall improvement in the complex area of PIDs in Central and Eastern Europe to which the JMF Network has considerably contributed. The principles of the organization and activities of the entire network described here have proven to be very effective and beneficial to the participating countries, PID centers, and their patients. The scheme has initiated a long-term project heading towards self-sustainability and has laid the foundation for knowledge transfer within the field and across other areas.