Complete atrioventricular block and aseptic meningitis in a patient with adult-onset Still's disease and concurrent hemophagocytic syndrome

infection or reactivation, including blood cultures and serological tests or PCR examinations for hepatitis B, C, human immunodefi ciency virus, cytomegalovirus, Epstein – Barr virus, varicella virus, rubella virus and measles virus were negative. Urinalysis showed no abnormalities. Findings of a cerebral computed tomography (CT) scan were within normal limits. Cerebrospinal fl uid (CSF) analysis showed pleocytosis, 193/mm (predominantly lymphocytes), a high protein level of 60 mg/dl and a normal glucose level of 56 mg/dL. Gram staining, bacterial, fungal, and mycobacterial cultures and PCR testing for herpes simplex virus of the CSF were negative. Whole body CT scan, skin biopsies of the erythematous skin and cervical lymph node biopsy were negative for malignant lymphoma, and non-specifi c but severe infl ammatory cell infi ltration was observed. Bone marrow aspiration revealed macrophages actively phagocytizing blood cells. Electrocardiogram (Figure 1a) and chest radiography revealed no abnormalities. Therefore, aseptic meningitis accompanied by AOSD and concurrent HPS was diagnosed according to the Yamaguchi criteria [1]. Steroid pulse therapy (Dexamethasone 40 mg/day) and intravenous cyclosporine (200 mg/day) were initiated. On day 2, after admission, severe bradycardia developed and complete atrioventricular block was detected on an electrocardiogram (Figure 1b). Echocardiography revealed neither pericardial eff usion nor any wall motion abnormality and laboratory blood tests for myocarditis (i.e. creatinine kinase, troponin I) had not been elevated since admission. As there was no other cause of cardiac conduction disturbance (i.e. electrolyte abnormalities, myocardial ischemia), complete atrioventricular block due to AOSD and/or HPS was suspected. An external temporary pacemaker was implanted immediately and initial immunosuppressive therapy was continued. Subsequently, there was marked improvement in the patient ’ s symptoms and laboratory markers over the next 7 days, and on day 10 the electrocardiogram had normalized (Figure 1c) and so the pacemaker was removed. Thereafter, the steroid dose was slowly tapered and all clinical symptoms disappeared. Three months after admission, the patient was discharged on cyclosporine and 27.5 mg/day of prednisone. At a 2-year follow-up, the patient remained asymptomatic. Mod Rheumatol, 2014; Early Online: 1–2 © 2014 Japan College of Rheumatology DOI: 10.3109/14397595.2014.882220