Rheumatoid arthritis disease activity significantly impacts on the severity of interstitial lung disease.

OBJECTIVES: Rheumatoid arthritis (RA) related interstitial lung disease (ILD) impacts on the treatment strategy and its prognosis in patients with RA. However, the relationship between RA disease activity and the severity of comorbid ILD has not been fully investigated. This study aimed to investigate the impact of RA disease activity on the severity of comorbid ILD in detail based on currently established visual scoring method along with physiological severity. METHODS: Consecutive patients with RA visiting to our Rheumatology Centre between December 2020 and December 2023 were analysed. The radiological severity of ILD was evaluated by averaging the extent of the combined lesion of ground glass opacity, reticulation and honeycombing in 5% increments in six representative high-resolution computed tomography slices ranging from 0% (no involvement) to 100% (all lung fields affected) according to Goh and Walsh's method. Associations between the radiological and physiological severity of ILD and patients' features were investigated using linear regression analysis. RESULTS: Among 124 patients (32 men, 92 women), the median age was 70 years, and the median disease duration was 2.92 years. Radiological severity of ILD was 0% (without ILD) in 107 (86.2%), ILD with extent < 10% in nine (7.2%), ILD with extent ≥10% and < 20% in three (2.4%), ILD with extent ≥20% in five (4.0%). Both disease activity score (DAS)28-erythrocyte sedimentation rate (ESR) (standardized coefficient = 0.199, P = 0.03) and rheumatoid factor titre (standardized coefficient = 0.247, P = 0.01) were significantly associated with the radiological quantitative severity of ILD in multivariate analysis adjusted for age, sex, disease duration, smoking status and anti-citrullinated peptide antibody titre. DAS28-ESR was significantly associated with forced vital capacity% predicted (standardized coefficient = -0.230, P = 0.047). CONCLUSIONS: Disease activity of RA was significantly associated with the severity of RA-ILD both radiologically and physiologically.

A technique for reconstruction of a giant extracranial internal carotid artery aneurysm: A technical note.

Background: Surgery is effective for extracranial internal carotid artery (EICA) aneurysms. However, the risk of cranial nerve injury associated with surgical repair, such as graft-assisted resection and extracranial-intracranial bypass techniques, is relatively high. Here, we report two cases of surgical treatment for EICA aneurysms and describe the surgical techniques and strategies to avoid cranial nerve injury. Methods: Two patients presented to our facility with an increasing cervical pulsatile mass and no neurological symptoms. Angiography showed a large aneurysm in the cervical internal carotid artery. Surgical treatment was performed to prevent rupture of the aneurysm. In both patients, the aneurysm was strongly attached to the vagus nerve. The aneurysm and vagus nerve were carefully dissected using a low-power bipolar (20 Malis; 3 watts), leaving connective tissue on the vagus nerve side. Results: The aneurysm was detached from the vagus nerve without injury. Based on intraoperative findings, one patient underwent clipping, and the other underwent aneurysmectomy and primary closure for aneurysm obliteration and angioplasty. Both patients were discharged without any cranial nerve dysfunction. Conclusion: The selection of a strategy based on intraoperative findings and low-power bipolar cutting is important for the treatment of extracranial carotid artery aneurysms to preserve cranial nerves.

Interhemispheric Acute Subdural Hematoma Secondary to Falx Meningioma: A Case Report.

This report describes an unusual case of falx meningioma associated with acute subdural hematoma, which is a rare presentation. A 76-year-old woman presented with right-sided hemiparesis and a known falx meningioma that had rapidly increased in volume over the previous year. Computed tomography revealed interhemispheric and left-hemispheric acute subdural hematomas. Preoperative embolization and surgical tumor removal were performed to improve the symptoms, and pathological examination of the tumor revealed transitional meningioma (WHO Grade I). The patient's paresis symptoms improved postoperatively. This report provides valuable insights into the management and outcomes of falx meningioma with acute subdural hematoma, suggesting aggressive surgery to improve postoperative recovery.

Iatrogenic Arteriovenous Fistula between the Middle Meningeal Artery and Pterygoid Venous Plexus Secondary to a Le Fort I Osteotomy: A Case Report.

Le Fort I osteotomy is a common surgical technique for correcting jaw deformities. Although it is generally a safe procedure, there have been cases of postoperative vascular complications, which include arteriovenous fistulas. Published reports describe the development of arteriovenous fistulas that originate from the maxillary artery after this procedure; nevertheless, no cases of arteriovenous fistulas arising from the middle meningeal artery (MMA) have been reported. We present the case of a 19-year-old woman who developed pulsatile tinnitus immediately following a Le Fort I osteotomy. Angiography with proximal flow control of the external carotid artery showed an arteriovenous fistula between the extracranial portion of the MMA and the pterygoid venous plexus. The fistula was successfully treated using coil embolization, preserving the maxillary artery, which results in tinnitus resolution. In this report, the effectiveness of endovascular treatment for arteriovenous fistulas of the MMA that occur following a Le Fort I osteotomy was demonstrated. Angiography with proximal flow interruption was effective in shunt location detection.

Mechanical Thrombectomy for Patients with Occlusions in Both the Anterior Cerebral Artery and Middle Cerebral Artery: Case Series and Review of the Literature.

Objective: Most large-vessel occlusions (LVOs) amenable to acute recanalization occur in the internal carotid or middle cerebral artery. However, few LVOs with a multivessel disease can be difficult to treat. This study aimed to determine the outcomes of mechanical thrombectomy in patients with both anterior and middle cerebral artery occlusions. Methods: We retrospectively collected data for patients who had undergone mechanical thrombectomy since January 2016 at Fukushima Medical University and its affiliated institutions (10 institutions). Patients with occluded vessels in the anterior and middle cerebral arteries were selected, and patient background, treatment course, and outcomes were reviewed. Results: A total of 341 mechanical thrombectomies were performed during the study period. Seven patients had occlusions involving both anterior and middle cerebral arteries. In these seven patients, the median time from onset to imaging, imaging to puncture, and puncture to recanalization was 106, 60, and 74 min, respectively. Only one patient (14%) had a modified Rankin Scale of 0-2 at 90 days. Conclusion: Comorbid anterior cerebral artery occlusion may worsen the outcome of patients with middle cerebral artery occlusion.

A Woman with Rheumatoid Arthritis Who Successfully Delivered a Healthy Child with Continuous Administration of Sarilumab Throughout Pregnancy.

We herein report a patient with rheumatoid arthritis (RA) who successfully delivered a healthy child with continuous administration of sarilumab throughout pregnancy. She delivered her first child, a healthy boy, following in vitro fertilization-embryo transfer (IVF-ET) while using etanercept and low-dose prednisolone. Disease activity persisted after delivery, so etanercept was switched to sarilumab. She became pregnant by IVF-ET again. Because RA was still active, sarilumab was continued during pregnancy. She delivered a healthy girl at the 38th week of gestation by Caesarean section. No abnormalities were detected at or within 6 months after birth. Sarilumab was safe and effective in this pregnant woman with RA.

High Blood Pressure and Changes in the Body Mass Index Category Among Japanese Children: A Follow-Up Study Using the Updated American Academy of Pediatrics Guidelines.

Background High blood pressure (HBP) has become a public health issue worldwide. The relationship between high BP and changes in the body mass index (BMI) category in Japanese pubertal children has not yet been examined. To resolve this issue, we examined existing data with a focus on the primordial prevention of high BP signs, including elevated BP, among pubertal children aged 12 and 15 years. Methods Height, body weight, and BP data were examined from health checkups of 18,247 children conducted between 1993 and 2000 in the Karatsu Study, which was a cohort of pediatric lifestyle-related disease prevention medical health checkups in Japan. BP and BMI were assessed using the updated American Academy of Pediatrics (AAP) guidelines and Endocrine Society's clinical practice guidelines definitions, respectively. Results Follow-up data were obtained from 7,090 subjects (50.5% boys). Stage 2 hypertension (HTN) was detected in 3% and 2.7% of boys and girls aged 12 years, respectively, and in 2.7% and 1% of boys and girls aged 15 years, respectively. Among children aged 15 years, 1.4% were newly classified with stage 2 hypertension, and 15.6% exhibited improvements to a normal BP. A binomial logistic regression analysis of high BP and BMI category changes revealed odds ratios (OR) in the group with a deteriorated BMI category of 1.51 (95% confidence interval (CI), 1.17-1.94), 2.30 (95%CI, 1.66-3.17), and 6.83 (95%CI, 4.14-11.29) for elevated BP, stage 1 hypertension, and stage 2 hypertension, respectively. Conclusion High BP in puberty positively correlated with BMI category changes. Considering the presence of the tracking phenomenon in hypertension, BP monitoring is an essential part of the early strategy for the prevention of lifestyle-related diseases in childhood, and improvements in BP control are crucial in early life.

Organizing pneumonia as a possible pulmonary manifestation of systemic lupus erythematosus: Three cases and a review of literature.

Background: Organizing pneumonia (OP) is a rare manifestation of systemic lupus erythematosus (SLE). It has been described in very few case reports.Purpose and Methods: We encountered OP in three patients with SLE in 1 year; two manifested OP as an initial presentation of SLE, while the third manifested OP when SLE relapsed. To elucidate the clinical features and outcomes of OP in patients with SLE, we screened the PubMed database for cases diagnosed with OP either at or after the diagnosis of SLE; the search was restricted to articles that were published after 1990, when OP became widely recognized.Results: We identified 15 cases of OP in patients with SLE. Thus, we analyzed a total of 18 cases (including our three). OP developed at the initial diagnosis of SLE in 15 cases and at relapse of SLE in three cases. In most cases, the disease activity of SLE was moderate or high. In all cases, OP was accompanied by other extrapulmonary symptoms of SLE, namely, fever (77.8%), cutaneous manifestations (61.1%), arthralgia/arthritis (50%), and lupus nephritis (33.3%). Steroid monotherapy or increasing the dose of the steroids was effective in seven cases (38.8%); however, steroid monotherapy was ineffective and led to death due to respiratory failure in two cases (11.1%). Combination therapies of steroids with hydroxychloroquine, azathioprine, tacrolimus, mycophenolate mofetil, intravenous pulse cyclophosphamide therapy, and belimumab were effective in seven cases (38.8%).Conclusions: Based on the review of previously reported and our cases, we concluded that OP is an important pulmonary manifestation of SLE. Clinicians should be aware of it as it may require intensive immunosuppressive therapy either at or after the diagnosis of SLE.

Acute Endovascular Therapy for Iatrogenic Vertebral Artery Injury: A Case Report.

Objective: Accidental puncture of the vertebral artery (VA) by central venous catheters and other devices has been reported as a rare complication. We performed endovascular therapy in the acute phase in a patient in whom a large-caliber sheath was misinserted into the VA. Case Presentation: A 68-year-old woman scheduled for open heart surgery had an 8-Fr. sheath inserted through the right internal jugular vein (IJV). This sheath penetrated the IJV and was misplaced in the V1 segment of the right VA. Endovascular therapy was performed. First, a 9-Fr. balloon-guiding catheter (BGC) was inserted and a 0.035-inch guidewire was pulled through it and the 8-Fr. sheath misinserted into the right VA. A 6-Fr. guiding catheter was inserted into the left VA and the microcatheter reached distal of where the sheath was inserted via the basilarunion. Then, the same area was embolized with coils to block retrograde blood flow. The BGC was then guided to the right VA origin using a pull-through wire while the 8-Fr. sheath was carefully withdrawn. The sheath was pulled back until just before exiting the VA and additional coils were placed via a microcatheter inserted into the BGC to occlude the right VA. Postoperatively, the patient had no neurological findings. Conclusion: We reported a rare case of iatrogenic VA injury. Attention to hemorrhage and intracranial blood flow resulted in a favorable outcome.

Clinical and Serological Features and Pregnancy Outcomes in Women with Polymyositis/Dermatomyositis: A Case-based Review.

We encountered a 30-year-old woman who developed dermatomyositis during pregnancy and was positive for anti-Mi-2 antibodies. She was successfully treated with prednisolone and tacrolimus and delivered a healthy child. We reviewed the cases of idiopathic inflammatory myositis (IIM) that developed during pregnancy that were published after the year 2000 to elucidate the profile of myositis-specific antibodies (MSAs) in them and to evaluate their obstetric outcomes. In cases with IIM that developed during pregnancy, anti-Mi-2, anti-TIF1-g, anti-Jo-1, and anti-EJ antibodies was detected in one case each. The obstetric outcomes of the IIM-complicated pregnancies were poor, especially when complicated with active maternal myositis. Further studies focusing on the possible causal relationships between MSAs and cases with IIM that developed during pregnancy are needed. For better obstetric outcomes, appropriate suppression of the maternal disease activity using immunosuppressants and vigilance regarding the patient's requirement of Caesarean section is important.

Pneumatosis Intestinalis Developed in a Patient with Giant Cell Arteritis While in a Clinically Sustained Remission Phase.

We herein report a patient with giant cell arteritis (GCA) who developed pneumatosis intestinalis (PI) while she was in a clinically sustained remission phase. A 79-year-old woman with GCA involving the thoracic aorta and its first branches to the posterior tibial arteries had been treated with high-dose prednisolone. Nine weeks after initiating treatment and while in clinically sustained remission with a normal CRP level, PI and pneumoperitoneum were incidentally found during scheduled positron emission tomography-computed tomography, which also revealed slight residual inflammation of GCA. This is a very rare case of PI complicated by GCA, and we discuss the possible relationships.

Results of Mechanical Thrombectomy 6 Hours after Stroke Onset: Analysis of Multiple Stroke Centers in Fukushima Prefecture.

Objective: The purpose of this study was to examine the efficacy and safety of mechanical thrombectomy in patients with acute occlusion of a large cerebral artery in the anterior circulation beyond 6 hours of the time last known to be well using the real-world clinical data collected from non-urban areas of Japan. Methods: We analyzed a retrospective multicenter database collected at 10 thrombectomy capable primary stroke centers in Fukushima Prefecture. In all, 188 patients were presenting a large cerebral artery occlusion in the anterior circulation, that is, internal carotid and middle cerebral artery (M1 and M2 segment). In all, 158 patients received mechanical thrombectomy within 6 hours from symptom onset (early time window), and 30 patients exceeded 6 hours (late time window). We compared the patient background, outcomes, and safety variables between the two groups. The modified Rankin Scale (mRS) score of 0-2 at 90 days after treatment and the incidence of symptomatic intracranial hemorrhage were compared between groups to evaluate treatment efficacy and safety. Results: There was no significant difference in the proportion of mRS score 0-2 at 90 days after treatment (51.3 vs. 46.7%: P = 0.644). However, symptomatic intracranial hemorrhage was more frequent in the late time window group (7.0 vs. 16.7%: P = 0.081). Symptomatic intracranial hemorrhage was a significant factor of a poor functional outcome in the late time window group (P = 0.022). Conclusion: This study reflects the real-world results of mechanical thrombectomy in the non-urban areas of Japan. The treatment efficacy in the late time window patients was equivalent to that in the early time window patients. On the other hand, the incidence of symptomatic intracranial hemorrhage showed a trend to high in patients beyond 6 hours, which was a significant factor related to a poor functional outcome.

[Coexisting Symptomatic Adrenocorticotropic Hormone- and Growth Hormone-producing Double Pituitary Adenomas with Difficulty in Tumor Localization:A Case Report].

Double functional pituitary adenomas are rare, and only a few cases of excessive clinical symptoms of both adrenocorticotropic hormone(ACTH)and growth hormone(GH)have been reported. We herein report a case of symptomatic ACTH-and GH-producing double pituitary adenomas, which were discretely located within the same pituitary gland. A 38-year-old woman presented with general malaise, facial and lower limb edema, unexplained weight gain, facial redness, acne, and nasal enlargement. Endocrinological findings matched with the diagnostic criteria for both acromegaly and Cushing's disease. Preoperative magnetic resonance imaging showed a 15-mm cyst-like lesion on the right side of the sellae surrounded by what was thought to be the normal contrast-enhancing pituitary gland. We assumed that the cyst-like lesion was an adenoma and performed endoscopic endonasal transsphenoidal surgery. However, the cyst-like lesion was a parenchymal tumor. Furthermore, the region we considered to be a normal pituitary gland was also found to be an adenoma. Both adenomas were completely resected. The postoperative blood analysis showed ACTH<1.0pg/dL, cortisol 1.8µg/dL, and insulin-like growth factor-1 60ng/mL, all of which were below reference levels. The histopathological examination confirmed the coexistence of two adenomas, a GH-producing adenoma and an ACTH-producing adenoma. We concluded that these adenomas were endocrinologically active within the pituitary gland. Thus, a diagnosis of double pituitary adenomas was made. When treating a patient with symptoms caused by hypersecretion of multiple anterior pituitary hormones, the possibility of coexisting multiple pituitary adenomas should be considered.

Pulmonary nocardiosis caused by Nocardia blacklokiae in an immunocompetent patient.

A subset of Nocardia isolates, previously belonging to N. transvalensis, has recently been given the new species designation N. blacklokiae. Here we report a case of pulmonary nocardiosis caused by N. blacklokiae in a 52-year-old immunocompetent woman presenting with low-grade fever and fatigue. The isolated Nocardia strain was resistant to sulfamethoxazole-trimethoprim and amikacin, but susceptible to amoxicillin-clavunate, ceftriaxone, clarithromycin and linezolid. With amoxicillin-clavunate treatment, the patient recovered and her condition remained stable, although recurrence occurred after cessation of the initial treatment. While infection by Nocardia is rare, clinicians should be aware of its resistance to antimicrobials including amikacin and sulfamethoxazole-trimethoprim.

[Repair of Spontaneous Cerebrospinal Fluid Leakage due to Multiple Defects in the Skull Base:A Case Report].

Spontaneous cerebrospinal fluid(CSF)rhinorrhea may have multiple leakage sites, and occasionally, may become difficult to manage. A 46-year-old man was admitted to our hospital presenting with bacterial meningitis that occurred after CSF rhinorrhea. The CSF leak had stopped following conservative medical treatment;however, it recurred immediately. The leakage was assumed to be in the left lateral recess of the sphenoid sinus based on the location of fluid accumulation on the CT and MR images and the nasal endoscopic findings. First, we performed an endoscopic endonasal repair of the CSF leakage sites. There were multiple defects in the roof of the sphenoid sinus, including the left lateral sphenoid recess. These bone defects were repaired with abdominal fat;however, this did not stop the CSF leak. Therefore, he underwent a craniotomy and nasal endoscopy, which were performed simultaneously using a galeal flap. The galeal flap was inserted from the middle of the skull base to the sphenoid sinus to cover the bone defects, and the position was adjusted under the guidance of nasal endoscopy. Moreover, intraoperatively, another meningocele with CSF leak was discovered in the cribriform plate and was repaired using abdominal fat. The CSF leak stopped after the second surgery. It is important to be mindful of possible multiple leakage sites when treating cases of idiopathic CSF leakage;moreover, the determination of the leakage sites can be difficult. A repair surgery using a galeal flap is preferable in cases of multiple CSF leakage sites, such as in this case, because it allows for all defects to be covered and repaired simultaneously.

Interstitial pneumonitis associated with dasatinib: two case reports and literature review.

Dasatinib has increasingly been used to treat chronic myeloid leukemia (CML), although interstitial pneumonitis has been found as a complication in large clinical trials. In the present study, 23 patients received dasatinib for CML between 2012 and 2017 at our institution, of whom 2 developed symptomatic interstitial pneumonitis. Notably, the first patient developed interstitial pneumonitis five years after initiating dasatinib. Interstitial pneumonitis should be considered as a complication in patients receiving dasatinib for CML, which may even occur after a long period of uncomplicated administration.

Chronic Nodular Pulmonary Aspergillosis in a Patient with Rheumatoid Arthritis.

Chronic pulmonary aspergillosis, which features nodular lesions known as Aspergillus nodules, is a relatively uncommon disorder. We herein report a case of slowly progressing chronic multiple nodular pulmonary aspergillosis in a 59-year-old man with rheumatoid arthritis, dyspnea, and fatigue. One nodule was surgically resected. The surgical specimen featured central necrosis and was located adjacent to a respiratory bronchiole and pulmonary artery, without parenchymal invasion. Branching septate hyphae, compatible with Aspergillus, were seen inside this necrotic nodule. Chronic pulmonary aspergillosis should therefore be considered in the differential diagnosis of patients who present with slowly progressing pulmonary multiple nodules.

Radiological fibrosis score is strongly associated with worse survival in rheumatoid arthritis-related interstitial lung disease.

OBJECTIVES: High-resolution computed tomography (HRCT) parenchymal patterns have been used to predict prognosis in patients with interstitial lung disease (ILD). In idiopathic pulmonary fibrosis, the fibrosis score (i.e. the combined extent of reticulation and honeycombing) has been associated with worse survival. This study aimed to identify HRCT patterns and patient characteristics that can predict poor prognosis in rheumatoid arthritis-related ILD (RA-ILD). METHODS: We retrospectively analysed 65 patients with newly diagnosed RA-ILD from 2007 to 2016 at Kurashiki Central hospital. Using univariate and bivariate Cox regression analysis, associations with mortality, were identified. RESULTS: During a median follow-up of 56.5 months, 16/65 (24.6%) patients died. Univariate analysis identified six significant poor prognostic factors: lower baseline % predicted forced vital capacity, total interstitial disease score, reticulation score, traction bronchiectasis score, fibrosis score, and definite UIP pattern. Fibrosis score remained to be an independently significant poor prognostic factor of survival on bivariate analysis. Patients with a fibrosis score >20% had higher mortality (HR, 9.019; 95% CI, 2.87-28.35; p < .05). CONCLUSION: This study showed that fibrosis score is strongly associated with worse survival in RA-ILD, and patients with fibrosis score >20% had a 9.019-fold increased risk of mortality.

Intraoperative Fluorescence Cerebral Angiography by Laser Surgical Microscopy: Comparison With Xenon Microscopy and Simultaneous Observation of Cerebral Blood Flow and Surrounding Structures.

BACKGROUND: Laser surgical microscopes should enable uniform illumination of the operative field, and require less luminous energy compared with existing xenon surgical microscopes. OBJECTIVE: To examine the utility of laser illumination in fluorescence cerebral angiography. METHODS: Fluorescein sodium (fluorescein) was used as a fluorescent dye. We first compared the clarity of cerebral blood flow images collected by fluorescence angiography between the laser illumination and xenon illumination methods. We then assessed use of the laser illuminator for simultaneous observation of blood flow and surrounding structures during fluorescence angiography. Furthermore, the study was designed to evaluate usefulness of the thus determined excitation light in clinical cases. RESULTS: Fluorescence angiography using blue light laser for excitation provided higher clarity and contrast blood flow images compared with using blue light generated from a xenon lamp. Further, illumination with excitation light consisting of a combination of 3 types of laser (higher level of blue light, no green light, and lower level of red light) enabled both blood flow and surrounding structures to be observed through the microscope directly by the surgeon. CONCLUSION: Laser-illuminated fluorescence angiography provides high clarity and contrast images of cerebral blood flow. Further, a laser providing strong blue light and weak red light for excitation light enables simultaneous visual observation of fluorescent blood flow and surrounding structures by the surgeon using a surgical microscope. Overall, these data suggest that laser surgical microscopes are useful for both ordinary operative manipulations and fluorescence angiography.