THU0298 INITIAL MANIFESTATIONS AND OUTCOME OF PATIENTS WITH INCOMPLETE BEHÇET’S SYNDROME
Authors: Sinem Nihal Esatoglu, Seyda Bilgin, Cem Sulu, Vedat Hamuryudan, Zekayi Kutlubay, Serdal Ugurlu, Emire Seyahi, Melike Melikoglu, Izzet Fresko, Sebahattin Yurdakul, Hasan Yazici, Gulen Hatemi
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background:</h3> Behçet's syndrome (BS) has a heterogeneous expression involving many organ systems and is diagnosed by recognizing the coexisting manifestations. Several patients initially have few of these manifestations and do not fulfill the International Study Group (ISG) criteria. When these are major organ manifestations, failure to recognize BS and treat promptly may lead to permanent damage in these organs. <h3>Objectives:</h3> The aim of this study is to highlight the magnitude of this problem by surveying the frequency, presentation patterns and outcome of patients who did not fulfill ISG criteria when they presented to our clinic, but were followed and treated for manifestations strongly suggesting BS. <h3>Methods:</h3> We conducted a retrospective chart review of all BS patients who were registered between 2003 and 2008. Among these 2385 patients, 199 (8%) BS patients who did not fulfill ISG criteria at their initial visit were included in this study. Patients were called and a standard form was used for collecting demographic characteristics, BS manifestations at initial visit and during follow-up and treatment. <h3>Results:</h3> Among the 199 patients (M/W: 90/109, mean age: 34 ± 11 years) who did not fulfill ISG criteria when they presented to our clinic, 70 (35%) had major organ involvement. The types of major organ involvement that led to a diagnosis of BS at initial visit despite not fulfilling ISG criteria were eye involvement in 37, vascular involvement in 29 (venous thrombosis in 22, arterial aneurysms in 7), nervous system involvement in 3 and gastrointestinal (GI) involvement in 1 patient. Thirty-five patients (18%) had a family history of BS. Of 199 patients, 167 had at least one more visit with a median follow-up of 11 years (IQR: 7-12). We were able to contact 116 of these patients and saw that 53 had fulfilled ISG criteria in the meantime. Among the 51 patients that we were not able to contact, 17 had fulfilled criteria while they were being followed in our clinic. Thus, a total of 70 (42%) patients fulfilled ISG criteria after a median follow-up of 1.5 years (IQR: 1-4.25). All but 2 patients who developed eye involvement during the follow-up had fulfilled ISG criteria with a new mucocutaneous manifestation. After a median follow-up of 4 years (IQR: 1-7), 23 (14%) patients had developed at least one non-criteria BS manifestation, including vascular involvement in 10, arthritis in 13, neurologic involvement in 2 patients and GI involvement in 1 patient. Among the 81 patients who developed at least one new manifestation, 16 (20%) were under immunosuppressive or interferon-alpha treatment at the time they developed their new manifestation. The remaining 65 patients had only received colchicine. Among the 29 patients who had vascular involvement at initial visit, 7 (24%) had a vascular relapse at different vascular site. Three patients had another rheumatologic diagnosis (ankylosing spondylitis, seropositive rheumatoid arthritis and sarcoidosis) at the end of the follow-up. <h3>Conclusion:</h3> In our 10-year follow-up cohort study, 42% of the 167 incomplete BS patients had fulfilled ISG criteria within a median duration of 1.5 years. Sixty-two (37%) of these had major organ involvement that could have caused severe morbidity and mortality if the diagnosis of BS was missed and patients were untreated. <h3><b>Disclosure of Interests:</b>&nbsp;:</h3> Sinem Nihal Esatoglu: None declared, Seyda Bilgin: None declared, Cem Sulu: None declared, Vedat Hamuryudan Consultant for: Abbvie, Amgen, BMS, Jansen, MSD, Pfizer, UCB, Speakers bureau: Abbvie, Amgen, BMS, Jansen, MSD, Pfizer, UCB, Zekayi Kutlubay: None declared, Serdal Ugurlu: None declared, Emire Seyahi: None declared, Melike Melikoglu: None declared, Izzet Fresko: None declared, Sebahattin Yurdakul: None declared, Hasan Yazici: None declared, Gulen Hatemi Consultant for: Abbvie, Amgen, BMS, Janssen, MSD, Pfizer, UCB, Speakers bureau: Abbvie, Amgen, BMS, Jansen, MSD, Pfizer, UCB,&nbsp;