Background/Purpose: Behçet’s syndrome (BS) shows a heterogeneous phenotype involving many organ systems and is diagnosed by recognizing the coexisting manifestations. Patients may have major organ manifestations before they fulfill the International Study Group (ISG) criteria and failure to recognize BS and treat promptly may lead to permanent damage in such patients. We aimed to determine the magnitude of this problem by identifying the presentation patterns and the 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.

Methods: We conducted a retrospective chart review of all BS patients who were registered between 2003 and 2008. Among the 2385 patients, there were 199 (8%) patients who did not fulfill ISG criteria at their initial visit but had manifestations strongly suggesting BS. A standard form was used for recording data on demographic features, BS manifestations, and treatment at the initial visit and during follow-up. An attempt was also made to invite them to our dedicated clinic to assess the outcome.

Of these 199 patients, 6 died (massive hemoptysis due to pulmonary artery aneurysm in 1, pancreatic cancer in 1, lung adenocarcinoma in 1 and unknown reasons in 3), 32 including 3/6 patients who died due to unknown reasons had only 1 visit, 54 including 3/6 who died could not be reached but there were enough data in the charts for the purposes of this study, 71 were contacted by telephone and 42 were evaluated in the clinic. Overall, 167 patients who had at least one more visit with a median follow-up of 11 years (IQR: 7-12) were analyzed.

Results: Among the 167 patients (M/W: 77/90, mean age: 33.4 ± 11.4 years), 62 (37%) had major organ involvement (eye disease in 32, venous thrombosis in 22, arterial aneurysms in 8, nervous system disease and gastrointestinal disease in 1 patient each) at the initial visit. Eighteen (11%) did not have recurrent oral ulcers but had major organ involvement at the initial visit. Thirty-two patients (19%) had a family history of BS.

49/113 patients we could contact and 13/54 patients we could not reach but were being followed in our clinic had fulfilled ISG criteria. Thus, a total of 62 (37%) patients fulfilled ISG criteria after a median follow-up of 1.5 years (IQR: 1-4.5). Sixty of these 62 patients fulfilled ISG criteria with added skin-mucosa findings and 2 with added eye involvement.

Among the remaining 102 patients who did not fulfill ISG criteria during the follow-up, 19 (19%) patients had developed at least one non-criteria BS manifestation (arthritis in 14 and vascular involvement in 5) after a median follow-up of 4 years (IQR: 1-6). 12 (12%) had developed at least one mucocutaneous manifestations. Three patients had another rheumatologic diagnosis (Figure).

Conclusion: In this 10-year follow-up study among at least 167 BS patients not fulfilling ISG criteria at presentation, 62 of 167 (37%) patients had fulfilled ISG criteria after a median duration of 1.5 years. In 60/62 (97%) of these patients, this was additional skin-mucosa disease.  

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/disease-course-of-behcets-syndrome-patients-not-fulfilling-international-study-group-criteria-at-presentation/