Severe hyperferritinemia--a clue for severe hepatitis in a patient with adult-onset Still's disease.

A 19-year old previously healthy man developed the adult-onset Still's disease (AOSD) with high ferritin levels. Corticosteroids induced clinical remission with resolution of fever, arthritis, and rash. While tapering corticosteroids, the patient developed severe liver enzymes elevation, very high ferritin levels and, subsequently, acute liver failure. After other causes of liver disease (infections, metabolic, autoimmune hepatitis, lymphoma, and hemophagocytosis) were excluded, severe hepatitis was attributed to AOSD itself. Cyclosporine induced rapid normalization of liver enzymes and reduction in ferritin levels. Severe hepatitis and very high ferritin levels could be the only manifestation of disease activity in AOSD; therefore, monitoring of liver enzymes and ferritin levels is recommended even after resolution of the clinical symptoms of AOSD. Prompt initiation of cyclosporine can improve liver function and prevent progression to liver failure.

Long-term follow-up of patients with scleroderma interstitial lung disease treated with intravenous cyclophosphamide pulse therapy: a single-center experience.

BACKGROUND: Scleroderma lung disease (ILD-SSc) is treated mainly with cyclophosphamide (CYC). The effectiveness of CYC was judged after 12-24 months in most reports. OBJECTIVES: To analyze the effect of monthly intravenous CYC on pulmonary function tests including forced vital capacity (FVC) and diffusing lung capacity (DLCO), as well as Rodnan skin score (mRSS), during long-term follow-up. METHODS: We retrospectively collected the data on 26 ILD-SSc patients who began CYC treatments before 2007. Changes in FVC, DLCO and mRSS before treatment, and at 1,4 and 7 years after completion of at least six monthly intravenous CYC treatments for ILD-SSc were analyzed. RESULTS: Mean cumulative CYC dose was 8.91 ± 3.25 G. More than 30% reduction in FVC (0%, 8%, and 31% of patients), DLCO (15%, 23%, 31%), and mRSS (31%, 54%, 62%) at years 1, 4 and 7 was registered. During the years 0-4 and 4-7, annual changes in FVC, DLCO and mRSS were 3.2 vs. 0.42% (P < 0.040), 4.6 vs. 0.89% (P < 0.001), and 1.8 vs. 0.2 (P = 0.002). The greatest annual FVC and DLCO reduction over the first 4 years correlated with mortality (P = 0.022). There were no differences in the main variables regarding doses of CYC (< 6 G and > 6 G). CONCLUSIONS: In patients with ILD-SSc, CYC stabilized the reduction of FVC during treatment, but this effect was not persistent. The vascular characteristic of ILD-SSc (DLCO) was not affected by CYC treatment. CYC rapidly improved the mRSS. This effect could be achieved with at least 6 G of CYC. Higher rates of annual reduction in FVC and DLCO in the first 4 years indicate the narrow window of opportunity and raise the question regarding ongoing immunosuppression following CYC infusions.

Unusual sequelae of adult-onset dermatomyositis.

A 44-year-old woman diagnosed with dermatomyositis 5 years ago based on progressive proximal muscle weakness, elevated creatine kinase, typical findings on electromyography and muscle biopsy. Despite the treatment, in contrast to improvement in her muscle symptoms, the heliotrope rash of her eyelids persisted. After several years, the patient developed multiple limited skin retraction lesions with hyperpigmentation on both lower limbs. Palpation of these lesions revealed dry, cold and very firm skin on both thighs and calves, particularly in the distal areas. X-ray and ultrasound imaging of the calves showed multiple subcutaneous calcifications in the distal muscles.

Restrictive left ventricular filling pattern and risk of new-onset atrial fibrillation after acute myocardial infarction.

Mechanisms for atrial arrhythmias that occur in the context of acute myocardial infarction (AMI) have not been well characterized. AMI often leads to alterations in left ventricular (LV) filling dynamics, which may result in advanced diastolic dysfunction. Diastolic dysfunction may produce increased left atrial (LA) pressure and initiate LA remodeling, promoting the progression to atrial fibrillation (AF). We studied 1,169 patients admitted with AMI. Advanced diastolic dysfunction was defined as a restrictive filling pattern (RFP), defined as ratio of early to late transmitral velocity of mitral inflow >1.5 or deceleration time <130 ms. The relation between RFP and the primary end point of new-onset AF occurring within 6 months was analyzed using multivariable Cox models. Of 1,169 patients (70% men, mean ± SD 64 ± 10 years of age), 110 (9.4%) developed new-onset AF (19.6% and 7.5% in patients with and without RFP, respectively, p <0.0001). RFP was associated with a hazard ratio of 2.72 for AF (95% confidence interval 1.83 to 4.05, p <0.0001). After multivariable adjustments for clinical variables, LV ejection fraction (EF) and LA size, RFP remained an independent predictor of AF (hazard ratio 2.17, 95% confidence interval 1.42 to 3.32, p <0.0001). Risk of AF was higher in patients with RFP for preserved (≥45%, hazard ratio 2.14, 95% confidence interval 1.09 to 4.20, p = 0.03) or decreased (hazard ratio 2.80, 95% confidence interval 1.63 to 4.82, p <0.0001) LVEF. In contrast, decreased LVEF in the absence of RFP was similar to that of patients with preserved LVEF and without RFP. In conclusion, in patients with AMI, presence of advanced diastolic dysfunction was independently associated with new-onset AF, suggesting that increased filling pressures may contribute to the development of AF after AMI.