Adult Onset Still's Disease as a Cause of Acute Severe Mitral and Aortic Regurgitation
In October 2003, a 55-year-old woman with chronic polyarthritis and myalgia was referred by the local tertiary hospital to our hospital. Four months prior, she developed general myalgia and polyarthralgia, mainly in the ankles and shoulders, and recorded body temperatures up to 39.8℃. She was admitted to the local tertiary hospital and intravenous ampicillin/sulbactam was empirically administered. After 3 days, the body temperature of the patient normalized, while polyarthralgia and myalgia remained. Two weeks later, the patient's body temperature again rose to 39.4℃, and an erythematous skin rash developed on both legs and the upper arms. She was empirically administered intravenous vancomycin and ciprofloxacin, and the body temperature normalized and the erythematous skin rash disappeared after 5 days. Polyarthralgia and myalgia persisted.\nThe patient underwent a series of blood tests which revealed the following: leukocytes 18,400/mL (4,000-10,000/mL, neutrophil 90.2%), hemoglobin 10.5 g/dL (12-16 g/dL), platelets 467,000/mL (150,000-350,000/mL), AST/ALT 110/111 IU/L (0-40 IU/L), ALP 730 IU/L (40-120 IU/L), GTP 473 IU/L (8-35 IU/L), LDH 567 U/L (120-250 U/L), ESR 64 mm/hr (0-20 mm/hr), CRP 23 mg/dL (0-0.6 mg/dL), and ferritin 998 ng/mL (10-290 ng/mL). Testing for rheumatoid factor (RF) and antinuclear antibodies (ANA) returned negative results. Repeated blood cultures returned negative results. Bone marrow examination showed marked granulocytic hyperplasia without evidence of hematologic malignancy, and no growth was observed on bacterial, fungal, or mycobacterial culture plates. Echocardiogram showed minimal mitral regurgitation without evidence of infectious endocarditis. Abdominopelvic computed tomography (CT) showed mild hepatomegaly with four hepatic hemangiomas. Skin biopsy showed diffuse neutrophilic infiltration in the entire dermis, consistent with Sweet's syndrome.\nOn the basis of the clinical and laboratory findings, the patient was administered 40 mg prednisolone per day, after which the symptoms of polyarthralgia and myalgia were relieved. The patient was discharged two weeks later (August 13, 2003) with a treatment regime of 15 mg prednisolone per day. Two weeks later, she revisited the local hospital outpatient clinic with signs that the polyarthralgia and myalgia had resurfaced, and that ESR and CRP were elevated. The prednisolone dose was increased to 60 mg per day and the symptoms were alleviated for 2 months. However, on October 30, 2003, the patient was transferred to our hospital and, due to complaints of polyarthralgia involving both first metatarsophalangeal joints, both ankles, both hips, both shoulders, and general myalgia, was admitted. Upon physical examination, tenderness and swelling were observed at both metatarsophalangeal joints, the left ankle, and the left sternoclavicular joint. Laboratory testing revealed that ESR, CRP, and ferritin levels were elevated (ESR 70 mm/hr, CRP 11 mg/dL, ferritin 1792 ng/mL).\nAccording to her clinical history and local tertiary hospital laboratory data, the patient was diagnosed with AOSD using Yamaguchi's classification criteria: fever lasting 1 week or longer, arthralgia lasting 2 weeks or longer, leukocytosis, liver dysfunction, and negative RF and ANA tests.\nFollowing admission, we administered 30 mg prednisolone twice per day and 10 mg methotrexate per week. The polyarthralgia and myalgia were subsequently alleviated. On follow up laboratory tests, ESR, CRP, and ferritin levels had decreased (ESR 52 mm/hr, CRP 2.01 mg/dL, ferritin 789 ng/mL). The patient was discharged five days later. She was readmitted one week later for dyspnea and general edema. Systolic murmurs and diastolic murmurs at the apex were observed upon cardiac auscultation. Chest X-rays showed cardiomegaly and bilateral pulmonary edema. Transthoracic echocardiogram showed severe mitral regurgitation (MR) due to prolapse of the posterior mitral valve leaflet, and moderate to severe aortic regurgitation (AR) due to right coronary cusp prolapse. Transesophageal echocardiogram also showed severe MR and AR due to valve leaflet prolapse. The valve prolapse appearance was notable because the prolapsing posterior mitral valve was focally fixed in the left atrium during systolic and diastolic cycles (). The patient underwent a mitral valve replacement (MVR) and Bentall's operation on November 22, 2003.\nHistological examination of heart tissue showed fibromyxoid degeneration with chronic active inflammation in the aortic wall, and chronic active inflammation with fibrinoid necrosis of the aortic and mitral valves (). Ziehl-Neelson, Periodic acid shiff, and Gram staining failed to identify any organisms. The presence of extensive fibrinoid necrosis, which is unusual in the case of ordinary valvulopathy, suggested valvulopathy associated with connective tissue disease. After MVR and Bentall's operation, the patient acquired normal mitral and aortic valvular function. She was, thereafter, administered oral medication through the outpatient clinic. Upon outpatient follow-up laboratory testing, ESR, CRP, and ferritin levels were 28 mg/hr, 1.30 mg/dL, and 720 ng/mL, respectively. The patient is currently taking 10 mg prednisolone per day and 17.5 mg methotrexate per week without relapse.