Adult-onset Still’s disease in pregnancy

We report a case of adult-onset Still’s disease (AOSD) in a pregnant patient that was successfully treated using intravenous methylprednisolone, cyclosporine, and plasma exchange, allowing safe delivery. Pregnancy appears to be one risk factor for AOSD, and we summarize the relationship between AOSD and pregnancy. A 28-year-old Japanese woman, in the 21st week of her first pregnancy, had suffered from low-grade fever and sore throat for 2 weeks. She was referred to us for a high spiking fever, cervical lymph node enlargement, non-pruritic diffuse rash with typical Kobner’s sign, and painful polyarthritis with palpable synovitis affecting the wrists, knees, and ankle joints bilaterally. She was referred to our department and admitted in September 2010. Chest radiography revealed bilateral pleural effusion, which was suggestive of pleuritis. Laboratory studies showed high levels of C-reactive protein (115 mg/L) and ferritin (24,883 ng/mL, normal \120 ng/mL), anemia (hemoglobin 10.5 g/dL), and an elevated white blood cell count (25,100/lL with 96.5% peripheral neutrocytes). Liver dysfunction was identified (aspartate transaminase 123 IU/ L, normal \39 IU/L; alanine aminotransferase 94 IU/L, normal \40 IU/L). Negative results were obtained for serum rheumatoid factor and antinuclear antibody. Blood cultures and exhaustive serological investigation for bacterial and viral etiologies of AOSD-like syndromes repeatedly yielded negative results. Her serum interleukin (IL)-18 level was significantly elevated (128,000 pg/mL), but IL-12 was not detectable. A diagnosis of AOSD was therefore made, according to the criteria described by Yamaguchi et al. [1]. Her symptoms worsened despite the initiation of prednisolone at 60 mg/day (treatment conducted in consultation with the obstetrician). As hemophagocytic syndrome developed as a complication after a few days, she received two courses of intravenous methylprednisolone pulse therapy, and plasma exchange, performed 15 times. Because the short-term effect of the pulse therapy was insufficient, we consulted her obstetrician again, and together with the obstetrician, we judged that maternal survival was the priority. We obtained informed consent for the use of cyclosporine in pregnancy from the patient and her family. Cyclosporine and liposomal dexamethasone palmitate were added after the pulse therapy, leading to improvement in the patient’s condition. During the clinical course, the patient suffered from severe oral ulcers induced by cytomegalovirus. Ganciclovir was prescribed because of concerns that congenital cytomegalovirus infection could develop in the fetus. At gestational week 33, she underwent urgent Cesarean section because the fetus showed intrauterine growth restriction, and she gave birth to a 1.3-kg healthy girl with no apparent abnormalities. After 1 month, with no clinical or biological signs of AOSD recurrence, she was discharged with her baby. M. Yamamoto (&) T. Tabeya C. Suzuki Y. Naishiro H. Yajima Y. Shimizu M. Obara H. Yamamoto T. Sugaya H. Takahashi Y. Shinomura First Department of Internal Medicine, Sapporo Medical University School of Medicine, South 1, West 16, Chuo-ku, Sapporo, Hokkaido 060-8543, Japan e-mail: mocha@cocoa.plala.or.jp