Antisynthetase syndrome with refractory lung involvement and myositis successfully treated with double filtration plasmapheresis

Antisynthetase syndrome (ASS) is characterized by inflammatory muscle disease, pulmonary and joint involvement, and antisynthetase autoantibodies, with anti‐Jo‐1 antibody being the most common. Despite the use of immunosuppressive drugs, the prognosis of lung involvement seems poor. Herein, we report a case of refractory ASS, which maintained long‐term remission by double filtration plasmapheresis (DFPP) combined with immunosuppressive therapy. For a 65‐year‐old woman, who was diagnosed with ASS, immunosuppressive therapy was initiated and plasmapheresis (PP) was performed five times due to acute interstitial pulmonary disease and inflammatory myopathy. She remained in remission for eight months following PP. Increase in interstitial involvement was identified by lung tomography when the patient presented again with complaint of progressive increase in dyspnea and muscle pain. Although the immunosuppressive therapy was increased for the patient with elevated creatine phosphokinase (CPK) (2776 IU/mL), a rapid decrease in diffusion capacity of the lung for carbon monoxide (DLCO) was observed and the patient underwent PP. After four sessions of therapy, insufficient clinical and laboratory response was obtained (control CPK 1797 IU/mL) and because of that issue DFPP using a 2A filter was performed to the patient. There was a marked improvement in complaints of the patient, DLCO, and laboratory findings (control CPK 508 IU/mL) after three sessions of DFPP. The patient, who continued the immunosuppressive therapy after DFPP procedure, is being followed for 12 months in remission. Although our experience is limited with only one patient, DFPP seems promising as a treatment option for ASS with severe lung involvement. J. Clin. Apheresis, 28:422–425, 2013. © 2013 Wiley Periodicals, Inc.     

Evaluation of prostate volume in mpMRI: comparison of the recommendations of PI-RADS v2 and PI-RADS v2.1

PURPOSEWe aimed to evaluate the prostate volumes calculated as recommended in the PI-RADS v2 and PI-RADS v2.1 guidelines, intraobserver and interobserver variability, and the agreement between the two measurement methods.METHODSProstate mpMRI examinations of 114 patients were evaluated retrospectively. T2-weighted sequences in the axial and sagittal planes were used for the measurement of the prostate volume. The measurements were performed by two independent observers as recommended in the PI-RADS v2 and PI-RADS v2.1 guidelines. Both observers conducted the measurements twice and the average values were obtained. In order to prevent bias, the observers carried out measurements at one-week intervals. In order to assess intraobserver variability, observers repeated the measurements again at one-week intervals. The prostate volume was calculated using the ellipsoid formula (W×H×L×0.52).RESULTSIntraclass correlation coefficient (ICC) revealed almost perfect agreement between the first and second observers for the measurements according to both PI-RADS v2 (0.93) and PI-RADS v2.1 (0.96) guidelines. The measurements were repeated by both observers. According to the ICC values, there was excellent agreement between the first and second measurements with respect to both PI-RADS v2 and PI-RADS v2.1 for first (0.94 and 0.96, respectively) and second observer (0.94 and 0.97, respectively). For both observers, the differences had a random, homogeneous distribution, and there was no clear relationship between the differences and mean values.CONCLUSIONThe ellipsoid formula is a reliable method for rapid assessment of prostate volume, with excellent intra- and interobserver agreement and no need for expert training. For the height measurement, the recommendations of the PIRADS v2.1 guideline seem to provide more consistently reproducible results.

The prostate gland is one of the organs for which the disease incidence and prevalence in men increases with age. Prostate volume (PV) has an important role in the evaluation and management of both malignant and benign prostate diseases (1–3). In benign prostatic hyperplasia (BPH), prostate volume is used to decide upon treatment and evaluate response to medical therapy (3–5). In the diagnosis of prostate cancer, one of the important markers is prostate-specific antigen (PSA), but it has low specificity, and therefore PSA derivatives are used to increase its specificity. One example is PSA density, which is obtained by dividing the PSA value by PV. In the treatment of prostate cancer, PV is important, and the effectiveness of brachytherapy decreases in prostates with a volume greater than 50 mL (6). Furthermore, PV is used to identify appropriate patients for brachytherapy and select the number of radioactive seeds, and also determine fractionation for external beam radiation, radical prostatectomy operating planning and continence rate counseling, and focal therapy candidacy preparation (7, 8). For these reasons, it is vital to accurately calculate PV.There are many methods that can be used to calculate PV, with the ellipsoid formula being one of the most preferred since it is easy to apply and highly time-efficient (1–4, 9). Many studies have shown that this method has high accuracy due to the elliptic shape of the prostate (1, 2, 10–13). The ellipsoid formula is obtained by multiplying the height (anterior-posterior), width (medio-lateral) and length (cranio-caudal) values of the prostate by 0.52. These measurements can be performed by transrectal ultrasonography (TRUS) or magnetic resonance imaging (MRI). TRUS has certain disadvantages, such as being operator-dependent and susceptible to sonographic artifacts (14). MRI, which has become increasingly popular in recent years, allows for an accurate definition of the prostate boundaries and multiplanar measurements through its high contrast resolution of soft tissues (1, 5). It also provides more accurate measurements than TRUS (4, 15, 16).In order to ensure global standardization in the reporting of prostate MRI findings, PI-RADS v2 published in 2015, which is the revised version of PI-RADS 1.0, and the last updated version PI-RADS v2.1 made available in 2019, propose different calculation methods for the measurement of height in obtaining PV (17, 18). The midaxial plane is recommended for this measurement in PI-RADS v2, while the midsagittal plane is recommended in PI-RADS v2.1. This study aimed to evaluate the interobserver and intraobserver variability of PV calculated by both measurement methods and the agreement between the two measurement methods.     

High-thoracic ultrasound-guided erector spinae plane block for acute herpes zoster pain management in emergency department

Herpes zoster is a painful, eruptive, viral condition occurring with reactivation in immunosuppressed individuals. The selection of an effective analgesic method in the acute phase of herpes zoster can decrease the incidence of postherpetic neuralgia by reducing neural sensitization. The erector spinae plane block has been reported to provide diffuse and effective analgesia in the cervical, thoracic, and lumbar regions. We report an effective decrease in pain with the application of the high-thoracic erector spinae plane block in the emergency department in a patient with herpes zoster pain in the cervicothoracic and shoulder region.     

Analysis of autonomic outcomes in APOLLO,a phase III trial of the RNAi therapeutic patisiran in patients with hereditary transthyretin-mediated amyloidosis

Hereditary transthyretin-mediated (hATTR) amyloidosis is a progressive, debilitating disease often resulting in early-onset, life-impacting autonomic dysfunction. The effect of the RNAi therapeutic, patisiran, on autonomic neuropathy manifestations in patients with hATTR amyloidosis with polyneuropathy in the phase III APOLLO study is reported. Patients received patisiran 0.3 mg/kg intravenously (n = 148) or placebo (n = 77) once every 3 weeks for 18 months. Patisiran halted or reversed polyneuropathy and improved quality of life from baseline in the majority of patients. At baseline, patients in APOLLO had notable autonomic impairment, as demonstrated by the Composite Autonomic Symptom Score-31 (COMPASS-31) questionnaire and Norfolk Quality of Life-Diabetic Neuropathy (Norfolk QOL-DN) questionnaire autonomic neuropathy domain. At 18 months, patisiran improved autonomic neuropathy symptoms compared with placebo [COMPASS-31, least squares (LS) mean difference, − 7.5; 95% CI: − 11.9, − 3.2; Norfolk QOL-DN autonomic neuropathy domain, LS mean difference, − 1.1; − 1.8, − 0.5], nutritional status (modified body mass index, LS mean difference, 115.7; − 82.4, 149.0), and vasomotor function (postural blood pressure, LS mean difference, − 0.3; − 0.5, − 0.1). Patisiran treatment also led to improvement from baseline at 18 months for COMPASS-31 (LS mean change from baseline, − 5.3; 95% CI: − 7.9, − 2.7) and individual domains, orthostatic intolerance (− 4.6; − 6.3, − 2.9) and gastrointestinal symptoms (− 0.8; − 1.5, − 0.2). Rapid worsening of all study measures was observed with placebo, while patisiran treatment resulted in stable or improved scores compared with baseline. Patisiran demonstrates benefit across a range of burdensome autonomic neuropathy manifestations that deteriorate rapidly without early and continued treatment.

     

Early port-site metastasis during neoadjuvant chemotherapy in advanced stage ovarian cancer: report of two cases

Port-site metastases in gynecological malignancies subsequent to laparoscopy have been reported with an incidence of 1.1-16%. These metastases tend to be disappearing after primary debulking surgery and subsequent primary chemotherapy. Local resection, chemotherapy and/or radiotherapy have been defined in the management of these metastases with enhanced clinical success. However, in extremely rare cases these metastases were also defined very early during neoadjuvant chemotherapy. Herein, we present two ovarian cancer cases which are clinically diagnosed with port site metastasis during neoadjuvant chemotherapy following diagnostic laparoscopy. Although neoadjuvant chemotherapy is sometimes needed in cases of fully advanced ovarian cancers, port-site metastasis may be encountered during neoadjuvant chemotherapy. The possible poor prognosis of these patients, especially those who have ascites, should make us careful in performing diagnostic laparoscopy with preventive measures for port-site metastasis and to start the chemotherapy immediately.