The management of women of childbearing age with rheumatoid arthritis: an expert report.

INTRODUCTION: The introduction of biologic therapies and a treat-to-target approach has transformed the management of rheumatoid arthritis (RA), which has led to improved outcomes for women with RA who wish to become pregnant. However, guidelines for the management of reproductive health in female patients with RA are still lacking. AREAS COVERED: A task force (Women of Childbearing Age [WoCBA]-Rheumatoid Arthritis in Japan) comprising 10 experts in the fields of rheumatology, obstetrics and orthopedic surgery developed 10 clinical questions (CQ) related to the management of WoCBA with RA. For each CQ, a systematic literature review was conducted to identify relevant evidence. Based on this evidence, a set of recommendations for each CQ were drafted and evaluated using the modified Delphi method. This article describes the agreed recommendations along with the supporting evidence. EXPERT OPINION: There are many ongoing challenges associated with the provision of reproductive healthcare in WoCBA with RA. It is hoped that the consensus-based recommendations provided here can be implemented in clinical practice in order to increase collaboration between rheumatologists and obstetricians/gynecologists and to improve reproductive health outcomes for WoCBA with RA.

Six-month survival of a monochorionic monoamniotic twin with sirenomelia.

BACKGROUND: Sirenomelia is a congenital malformation of the lower body characterized by a single midline lower limb and severe urogenital and gastrointestinal malformations. Sirenomelia is rare (estimated incidence of approximately 1/100,000) and usually lethal in the perinatal period. CASE: A 2,042 g Japanese male infant, one of monochorionic monoamniotic twins, was born at 34 weeks of gestation by elective caesarean section. Sirenomelia was prenatally diagnosed. Single midline lower limb, bilateral dysplastic kidneys, an omphalomesenteric fistula, colon atresia, imperforate anus, indiscernible genital structures, and myelomeningocele were detected at birth. The amniotic fluid volume was normal throughout the pregnancy course, which led to appropriate lung maturation of the twin with sirenomelia. Although renal replacement therapy was initiated soon after birth, stable peritoneal dialysis was difficult because of the limited intraperitoneal space, and the infant frequently developed peritonitis. He died of sudden cardiorespiratory arrest at 6 months of age. Postmortem examination showed bilateral dysplastic kidneys, agenesis of the ureters and urinary bladder, abnormal branching and agenesis of the distal colon, bilateral inguinal hernias, and small testes. CONCLUSION: Infants with sirenomelia, even those with end-stage kidney disease at birth, may survive if they have a stable cardiorespiratory status at birth and renal replacement therapy is appropriately initiated.

Fiducial marker position affects target volume in stereotactic lung irradiation.

PURPOSE: Real-time tracking systems of moving respiratory targets such as CyberKnife, Radixact, or Vero4DRT are an advanced robotic radiotherapy device used to deliver stereotactic body radiotherapy (SBRT). The internal target volume (ITV) of lung tumors is assessed through a fiducial marker fusion using four-dimensional computed tomography (CT). It is important to minimize the ITV to protect normal lung tissue from exposure to radiation and the associated side effects post SBRT. However, the ITV may alter if there is a change in the position of the fiducial marker with respect to the tumor. This study investigated the relationship between fiducial marker position and the ITV in order to prevent radiation exposure of normal lung tissue, and correct target coverage. MATERIALS AND METHODS: This study retrospectively reviewed 230 lung cancer patients who received a fiducial marker for SBRT between April 2015 and September 2021. The distance of the fiducial marker to the gross tumor volume (GTV) in the expiratory (dex ) and inspiratory (din ) CT, and the ratio of the ITV/V(GTVex ), were investigated. RESULTS: Upon comparing each lobe, although there was no significant difference in the ddiff and the ITV/V(GTVex ) between all lobes for dex  < 10 mm, there was significant difference in the ddiff and the ITV/V(GTVex ) between the lower and upper lobes for dex ≥ 10 mm (p < 0.05). Moreover, there was significant difference in the ddiff and the ITV/V(GTVex ) between dex ≥10 mm and dex  < 10 mm in all lung regions (p < 0.05). CONCLUSION: The ITV that had no margin from GTVs increased when dex was ≥10 mm for all lung regions (p < 0.05). Furthermore, the increase in ITV tended to be greater in the lower lung lobe. These findings can help decrease the possibility of adverse events post SBRT, and correct target coverage.

Re-evaluation of the predictive value of Quintero staging of twin-twin transfusion syndrome for fetal death after fetoscopic laser photocoagulation.

OBJECTIVE: To elucidate the significance of sonographic indices, including Doppler waveforms, that constitute the Quintero classification for predicting death of the recipient or donor after fetoscopic laser photocoagulation (FLP) for twin-twin transfusion syndrome (TTTS). METHODS: Prospectively collected data of twins who underwent FLP for TTTS were reviewed. Among the abnormal indices of ultrasound performed just before FLP, factors that were significantly associated with fetal and neonatal deaths in the log-rank test, including fetal demise of co-twins and preterm birth before 28 weeks of gestation, were introduced into the Cox proportional-hazards model to calculate risk ratio (RR). RESULTS: We included 235 cases with a prevalence of recipient and donor deaths of 7% and 14%, respectively. In the proportional-hazards model, absent or reversed umbilical artery end-diastolic velocity (UA AREDV) of recipients (n = 7) was independently associated with recipient death (RR = 6.97). In recipients without UA AREDV, reversed ductus venosus (DV) a-wave of recipients (RR = 3.55) was independently associated with recipient death. In donors, UA AREDV with a visible bladder (stage III atypical donor) was independently associated with donor death (RR = 4.24). CONCLUSION: Some individual components of the Quintero stage are associated with death of either recipient or donor twins following FLP.

Placental histopathological features of fetoscopic laser photocoagulation for monoaminotic diamniotic twin pregnancies.

INTRODUCTION: This study aimed to compare the histopathological placental features of monochorionic diamniotic (MCDA) twins who did and did not undergo fetoscopic laser photocoagulation (FLP). METHODS: This was a retrospective single-institution cohort study on MCDA twins who underwent FLP between October 2010 and December 2018. The control group included MCDA twins who did not undergo FLP and were delivered during the same period in the institute. The incidence of chorioamnionitis (CAM), funisitis, and other pathological findings was compared between the FLP and control groups after matching by gestational age at delivery. RESULTS: In total, 292 MCDA pregnant women who underwent FLP and 356 controls gave birth during the study period. After matching the two groups in the ratio 1:1 by gestational age at delivery, each group comprised 194 subjects. The incidence of histological CAM with Blanc association (stage I, 6.2% vs. 3.1%, crude odds ratio (cOR) = 3.1, P = 0.052; stage II, 7.2% vs. 5.7%, cOR = 1.6, P = 0.30; stage III, 2.1% vs. 2.6%, cOR = 0.66, P = 0.52) and funisitis (artery, 5.2% vs. 3.6%, cOR = 1.3, P = 0.63; vein 7.2% vs. 4.1%, cOR = 1.6, P = 0.29) was not statistically significant difference between the FLP and control groups. The FLP group demonstrated a higher incidence of partial placental infarction than the control group (10.3% vs. 3.1%, cOR = 4.3, P = 0.004, adjusted OR = 2.8, P = 0.031). DISCUSSION: FLP did not appear to increase the incidence of histological CAM or funisitis in subjects matched by gestational age at delivery. The FLP group demonstrated a higher incidence of partial placental infarction than the control group.

Perinatal outcomes of intrauterine transfusion for the surviving twin in monochorionic twin gestation involving a single fetal demise.

AIM: This study aimed to evaluate the feasibility and outcome of intrauterine transfusion (IUT) for the surviving co-twin in monochorionic diamniotic (MCDA) twin gestations involving single intrauterine fetal death (sIUFD). METHODS: Cases of MCDA twin gestations involving emergent IUT for co-twins experiencing acute feto-fetal hemorrhage (AFFH) subsequent to sIUFD during the second trimester were reviewed. Fetal anemia was confirmed via fetal blood sampling, and perinatal data were retrieved from medical charts to determine the outcomes of surviving co-twins. A poor outcome at 28 days of age was defined as fetal death, neonatal death or neurological impairment such as severe intraventricular hemorrhage or cystic periventricular leukomalacia (PVL). RESULTS: This study included 16 cases of sIUFD diagnosed at a median of 22.5 weeks of gestation (range: 18-25 weeks). The median interval in hours between diagnosis of fetal demise and IUT was approximately 4.2 (0-22) hours. All cases achieved IUT without significant intraoperative complications. Four cases experienced a fetal loss of the co-twin, whereas 12 co-twins were born alive (including 9 preterm births), with only 1 poor outcome in a preterm infant with PVL. CONCLUSION: IUT may be a feasible prenatal intervention for surviving co-twins with AFFH. However, more extensive or pooled studies are needed to determine the efficacy of this intervention.

Ovarian insufficiency following allogeneic hematopoietic stem cell transplantation.

Ovarian insufficiency is a serious complication for young women who undergo hematopoietic stem cell transplantation (HSCT). Reduced-intensity conditioning (RIC) has been utilized more widely due to its reduced toxicity; however, there is a lack of data concerning ovarian function after HSCT with RIC. We investigated the ovarian function in patients who received HSCT with RIC, compared to those who received myeloablative conditioning (MAC). The records of 69 female patients who received allogeneic HSCT at the institution under 40 years of age at transplantation from 1991 to 2012 were retrospectively analyzed. Prevalence of ovarian insufficiency was significantly lower in patients conditioned with RIC than in those conditioned with MAC (4/27 = 14.8% for RIC and 36/42 = 85.7% for MAC, p < 0.0001). A younger age at HSCT was associated with a lower risk of ovarian insufficiency. Among the 40 patients with ovarian insufficiency, four patients recovered ovarian function, and two conceived following hormone-replacement therapy (HRT). A higher serum E2 level prior to HRT was a significant predictor for the restoration of ovarian function (p = 0.0028). In conclusion, RIC was significantly less toxic to ovarian function compared with MAC. HSCT-associated ovarian insufficiency is not irreversible, and a higher E2 level may predict the restoration of ovarian function.

Perinatal outcome of monochorionic diamniotic twin pregnancies managed from early gestation at a single center.

AIM: The aim of this study was to evaluate the perinatal outcome of monochorionic diamniotic (MD) twin gestations managed from early gestation onward at a single center. MATERIAL AND METHODS: This was a retrospective single-cohort study, spanning 6 years, of 219 MD twin pregnancies who received prenatal care before 14 weeks of gestation and delivered at our center. The subjects were all under the same perinatal management protocol. The incidence of fetal or neonatal death, major neurological complications at 28 days of age, twin-twin transfusion syndrome (TTTS), twin anemia-polycythemia sequence, and discordant birth was evaluated. Laser surgery was offered for TTTS at less than 26 weeks; however, selective feticide was not performed. RESULTS: Pregnancy termination was selected in two cases. Miscarriage occurred in two (1%) of the cases and preterm delivery occurred in 91 (42%). In regard to perinatal outcome at 28 days of age, 195 (89%) women possessed two live infants and 205 (94%) possessed at least one live infant. The overall survival rate was 92% (403/438). The prevalence of TTTS was 17 cases (8%), seven of whom underwent laser surgery. Four cases of twin anemia-polycythemia sequence were diagnosed postnatally (2%); discordant birth was diagnosed in 24 (12%). Major neonatal neurological abnormalities were noted in six cases (2%). CONCLUSIONS: The incidence of perinatal complications in 219 sets of MD twins managed from early gestational age to the neonatal period in one perinatal center was demonstrated. The incidence of TTTS was 8%; the survival rate was 89% at 28 days of age.

Coexistent intramedullary spinal cord and choroidal metastases in ovarian cancer.

Involvement of intramedullary spinal cord and the choroid by ovarian cancer is rare, and coexistence of metastases at these sites is extremely rare and has never been reported. This condition rapidly progresses to a neurological emergency; however, an efficient standard treatment method is not available for this rare condition. The case presented herein is of a female patient with stage II, poorly differentiated serous cystadenocarcinoma of the ovary. She presented with blindness and other neurologic complaints during the course of treatment for a recurrence at 50 months after the primary surgical treatment for the tumor. Magnetic resonance imaging (MRI) revealed intramedullary spinal cord metastasis and choroidal metastasis, coexisting with multiple brain metastases and intra-abdominal lesions. Neurological emergency was prevented by administering whole-brain irradiation therapy followed by systemic chemotherapy. Early diagnosis and multidisciplinary treatment, including radiotherapy and chemotherapy, may offer good palliation for such unusual metastases of ovarian cancer.