Fever, uveitis, and myocarditis as the initial presentation of Behcet's Disease: A case report and review of the literature

Behcet's disease (BD) is an inflammatory disease characterized by recurrent aphthous ulcers, genital ulcers, and eye involvement. The involvement of the vascular system, heart, lungs, gastrointestinal tract, and the brain is less common, and BD myocarditis is very rare, with only a few cases reported in the literature. The prevalence of BD is variable globally, with Turkey having the highest prevalence, with 370 cases/100,000 population. Out of the 17 criteria proposed for the diagnosis of BD, the most commonly used criteria are the International Study Group (ISG) on BD criteria and the “International Criteria of BD” (ICBD) criteria. We present a case of a 40-year-old female who presented with fever of unknown origin, oral ulcers, odynophagia, and a rash over the face, extremities, and trunk. She developed acute-onset bilateral painful red eyes in the hospital with marked diminution of vision and persistent tachycardia. On evaluation, she was diagnosed to have bilateral intermediate uveitis based on the clinical finding of conjunctival congestion, and 1+ cells in the anterior chamber of both eyes, dispersion of iris pigments on the anterior surface of lens bilaterally, and Grade 1 vitreous haze on slit-lamp examination. The patient had persistent tachycardia for which an electrocardiogram was done, which revealed nonspecific ST-changes. Echocardiography was done, which showed a left ventricular ejection fraction of 40%, with global hypokinesia, suggestive of myocarditis. Our patient fulfilled the ICBD criteria, and she tested positive for human leukocyte antigen B51. She also had persistent transaminitis, and on evaluation, she was found to have occult hepatitis B infection with possible reactivation. The final diagnosis of BD with myocarditis and occult hepatitis B infection was established. She was managed with oral glucocorticoids, colchicine, azathioprine, and entecavir with a favorable outcome. BD causing myocarditis is an infrequent presentation, and we could find only 15 cases reported in the literature since 1947 when Dr. Hulusi Behcet first described the disease.