Novel deep-learning analysis for connective tissue disease -related interstitial lung disease extent assessment on CT: a preliminary cross-sectional study.

OBJECTIVES: Physician's evaluation of interstitial lung disease (ILD) extension with high-resolution computed tomography (HRCT) has limitations such as lack of objectivity and reproducibility. This study aimed to investigate the utility of computer-based deep-learning analysis using QZIP-ILD® software (DL-QZIP) compared with conventional approaches in connective tissue disease (CTD) -related ILD. METHODS: Patients with CTD-ILD visiting our Rheumatology Centre between December 2020 and April 2024 were recruited. Quantitative scores, including the percentage of lung involvement in ground-glass opacity (QGG), total fibrotic lesion (QFIB), and overall ILD extension encompassing both QGG and QFIB (QILD), calculated by DL-QZIP, were compared with semiquantitative visual method, employing intraclass correlation coefficients (ICC). We compared the capability of QILD scores to distinguish patients with forced vital capacity (FVC) % <70 in both methods determined by the area under the curve (AUC) by the receiver-operating characteristic curve analysis and DeLong's test. RESULTS: Eighty patients (median age, 66 years; 14 men) were included. Median QGG, QFIB, and QILD scores were 3.45%, 2.19%, and 5.35% using DL-QZIP, and 3.25%, 4.06%, and 8.48% using visual method, respectively. Correlations between DL-QZIP and visual method were 0.75 for QGG, 0.61 for QFIB, and 0.75 for QILD. The AUC of QILD scores for FVC% <70 was significantly higher with DL-QZIP (0.833) compared with visual method (0.660) (p < 0.01). CONCLUSION: QZIP-ILD® demonstrates superior capability in distinguishing patients with a radiological scenario correlated to severe physiological impairment, while showing relatively good correlations in quantifying the extent on HRCT compared with conventional method in CTD-ILD.

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.

[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.

[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.

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.

Radiographic fibrosis score predicts survival in systemic sclerosis-associated interstitial lung disease.

BACKGROUND AND OBJECTIVE: Interstitial lung disease (ILD) is a common pulmonary manifestation of systemic sclerosis (SSc). It is unknown whether radiographic fibrosis score predicts mortality in SSc-associated ILD (SSc-ILD). We retrospectively analysed patients with SSc-ILD to evaluate whether radiographic fibrosis score was a useful predictor of mortality. METHODS: We identified SSc-ILD patients evaluated at Kurashiki Central Hospital (Japan) from 2006 to 2016, and radiographic fibrosis scores based on the extent of reticulation and honeycombing on high-resolution computed tomography (HRCT) scanning were calculated by manually tracing around each fibrotic area. Independent predictors of overall survival were determined using the Cox proportional hazards model. RESULTS: The study included 48 patients, of whom 19 had usual interstitial pneumonia on HRCT. The median follow-up period was 56.6 months, and over the follow-up period 15 patients died. The 5-year survival was 72.4%. In the multivariate analysis, radiographic fibrosis score, age, being male and forced vital capacity were independently associated with an increased risk of death, while HRCT pattern was not. CONCLUSION: A high radiographic fibrosis score was a poor prognostic factor in SSc-ILD. More widespread fibrosis was associated with an increased risk of death, independent of HRCT pattern.

Impact of procalcitonin-guided therapy for hospitalized community-acquired pneumonia on reducing antibiotic consumption and costs in Japan.

BACKGROUND AND OBJECTIVE: This study aimed to investigate the usefulness of procalcitonin-guided therapy in hospitalized community-acquired pneumonia patients to reduce antibiotic duration and costs without worsening prognosis. METHODS: 352 hospitalized community-acquired pneumonia patients in an observational cohort study in which procalcitonin was measured three times serially, on admission (Day 1) and 2-3 days (Day 3) and 6-8 days (Day 7) after admission, between October 2010 and February 2016 were reviewed retrospectively. Antibiotics could be stopped if Day 7 procalcitonin was <0.25 ng mL-1 or ≤10% of the higher value of procalcitonin on Day 1 or 3. Antibiotic duration and costs and recurrence and mortality rates were evaluated in mild to moderate or severe pneumonia by theoretical procalcitonin guidance for community-acquired pneumonia treatment. RESULTS: Using theoretical procalcitonin guidance, antibiotic duration could be reduced from 12.6 to 8.6 days (P < 0.001), while costs could be reduced from 45,833 to 38,952 yen (P = 0.005). Among the patients in whom theoretical procalcitonin guidance could be adopted, recurrence rates (5.6% vs. 8.1%, P = 0.15) and mortality rates (0% vs. 5.1%, P = 0.07) did not worsen between the group having the same antibiotic durations as with theoretical procalcitonin guidance in actual practice (N = 71) and the group having durations more than 2 days longer in actual practice than in theoretical procalcitonin guidance (N = 198). There was no significant difference in pneumonia severity using A-DROP, CURB-65, and PSI between two groups. CONCLUSIONS: Procalcitonin-guided therapy may be useful in hospitalized community-acquired pneumonia patients to reduce antibiotic duration and costs without worsening the prognosis.

Prognostic factors in hospitalized community-acquired pneumonia: a retrospective study of a prospective observational cohort.

BACKGROUND: To date, only few studies have examined the prognostic factors of community-acquired pneumonia (CAP) defined according to the latest criteria, which excludes healthcare-associated pneumonia (HCAP). Therefore, we aimed to investigate the factors that affect prognosis, and evaluate the usefulness of existing pneumonia severity scores for predicting the prognosis of CAP. METHODS: We retrospectively analyzed patients with CAP, excluding HCAP, who were enrolled prospectively between April 2007 and February 2016. Four patients who used macrolides other than azithromycin (AZM) were excluded. We used age, sex, comorbidities, laboratory findings and antimicrobial therapy as prognostic variables. The primary outcome was 30-day mortality and secondary outcome was ICU admission. We also performed receiver operating characteristic curve analysis of Pneumonia Severity Index (PSI), Infectious Diseases Society of America (IDSA)/American Thoracic Society (ATS) severe criteria, CURB-65 and A-DROP pneumonia severity scores. RESULTS: Among 1834 CAP patients, mean age was 73.5 ± 14.3 years; 1281 (69.8%) were men; and 30-day mortality was 6.7% (122/1834). In total, 1830 patients were analyzed. Multivariate analysis identified age [Odds Ratio (OR): 1.04, 95% Confidence Interval (CI): 1.02-1.07], chronic obstructive pulmonary disease (COPD) [OR: 1.77, 95% CI: 1.13-2.76], malignancy (OR: 2.25, 95% CI: 1.25-4.06), body temperature (OR: 0.81, 95% CI: 0.67-0.99), respiratory rate (OR: 1.04, 95% CI: 1.01-1.07), PaO2/FiO2 ≤ 250 (OR: 3.15, 95% CI: 1.93-5.14), Alb (OR: 0.27, 95% CI: 0.19-0.39), BUN (OR: 1.01, 95% CI: 1.00-1.02), and mechanical ventilation (OR: 2.99, 95% CI: 1.75-5.12) as prognostic factors. AZM and ß-lactam combination therapy significantly reduced 30-day mortality (OR: 0.50, 95% CI: 0.26-0.97). Areas under the curve of PSI, IDSA/ATS severe criteria, CURB-65 and A-DROP were 0.759, 0.746, 0.754 and 0.764, respectively. CONCLUSIONS: Increasing age, presence of COPD and malignancy as comorbidities, hypothermia, tachypnea, PaO2/FiO2 ratio ≤250 mmHg, low Alb level, high BUN level and mechanical ventilatory support predict a worse prognosis; AZM combination therapy should be considered for CAP, excluding HCAP. All four pneumonia severity scores are useful for assessing the severity of CAP defined by the latest criteria. TRIAL REGISTRATION: UMIN-CTR UMIN000004353 . Registered 7 October 2010. Retrospectively registered.

Impact of lymphocyte differential count > 15% in BALF on the mortality of patients with acute exacerbation of chronic fibrosing idiopathic interstitial pneumonia.

BACKGROUND: Chronic fibrosing idiopathic interstitial pneumonia (CFIIP) has a potential risk of acute exacerbation (AE). However, the usefulness of cellular analysis of bronchoalveolar lavage fluid (BALF) has never been evaluated. This study aimed to evaluate the impact of the lymphocyte differential count > 15% in BALF on the mortality of patients with AE of CFIIP. METHODS: We retrospectively analysed 37 patients with AE of CFIIP who underwent BAL on admission. Patients were divided into two groups: one group consisting of those with a lymphocyte differential count > 15% and the other consisting of those with a lymphocyte differential count ≤ 15%. We compared the 90-day mortality between the two groups as the primary outcome, using the two-tailed log-rank test. RESULTS: The median follow-up duration was 6.9 months. Twenty-four patients had a lymphocyte differential count > 15%. The 90-day mortality was significantly higher in the group with a lymphocyte differential count ≤ 15% than in the group with a lymphocyte differential count > 15% (long rank test, p = 0.003). In the multivariate analysis a lymphocyte differential count > 15% was shown to be an independent favourable prognostic factor for 90-day mortality (HR: 0.125; 95% CI: 0.0247-0.589; p = 0.009). CONCLUSIONS: A lymphocyte differential count > 15% in BALF may be associated with favourable outcomes in patients with AE of CFIIP.

Serological and morphological prognostic factors in patients with interstitial pneumonia with autoimmune features.

BACKGROUND: To identify the prognostic factors for survival in patients with interstitial pneumonia with autoimmune features (IPAF) who meet the serological domain of the IPAF criteria. METHODS: We retrospectively analysed 99 IPAF patients who met the serological domain and were hospitalised at the Respiratory Medicine Unit of Kurashiki Central Hospital from 1999 to 2015. The high-resolution computed tomography findings were usual interstitial pneumonia (UIP; n = 1), non-specific interstitial pneumonia (NSIP; n = 63), NSIP with organizing pneumonia (OP) overlap (n = 15), and OP (n = 20). One patient who had radiological UIP pattern, and met the serological and clinical domains was excluded. The clinical characteristics, radiological findings, administered therapy, and prognosis of the remaining 98 IPAF patients who met the serological and morphological domains were analysed. RESULTS: The median age of the 98 IPAF patients was 68 years, and 41 (41.8%) of them were men. Twelve (12.2%) of the 98 IPAF patients developed other characteristics and were diagnosed with connective tissue disease (CTD) later during the median follow-up of 4.5 years. Univariate Cox analysis revealed systemic sclerosis (SSc)-specific and SSc-associated antibodies (ANA nucleolar pattern, ANA centromere pattern, anti-ribonucleoprotein and anti-Scl-70) positive IPAF, radiological NSIP pattern, bronchoalveolar lavage fluid lymphocytes >15%, and age as significant prognostic factors for survival. Multivariate Cox analysis revealed radiological NSIP pattern (hazard ratio [HR], 4.48; 95% confidence interval [CI], 1.28-15.77, p = 0.02) and age (HR, 1.07; 95% CI, 1.02-1.11, p = 0.01) were significantly associated with worse survival. CONCLUSIONS: We confirmed that radiological NSIP pattern and age are poor prognostic factors for the survival of IPAF patients. This study suggested that the autoantibodies that are highly specific for certain connective tissue diseases might be less important for the prognosis of IPAF compared with the radiological-pathological patterns. The relatively high proportion of IPAF patients who developed CTD later suggests the importance of careful observation for evolution to CTD in IPAF.

Serial procalcitonin levels for predicting prognosis in community-acquired pneumonia.

BACKGROUND AND OBJECTIVE: This study aimed to investigate the usefulness of addition of serial measurements of procalcitonin (PCT) to C-reactive protein (CRP) values and pneumonia severity scores, such as CURB-65 (confusion, urea > 7 mmol/L, respiratory rate ≥ 30 breaths/min, low blood pressure (systolic < 90 mm Hg or diastolic ≤ 60 mm Hg) and age ≥ 65 years) and the Pneumonia Severity Index, and attempted to create and evaluate a new scoring system for predicting mortality risk using the biomarkers and pneumonia severity scores. METHODS: A total of 365 hospitalized community-acquired pneumonia (CAP) patients in an observational cohort study in which PCT was measured serially from admission to 2-3 days after admission between December 2010 and December 2014 were reviewed retrospectively. PCT and CRP were measured on admission (PCT D1 and CRP D1) and within 48-72 h after admission (PCT D3 and CRP D3). RESULTS: Twenty-one patients died (5.8%), and 52 patients (14.2%) did not respond to initial therapy. On multivariate analysis, CRP D1 ≥ 100 mg/L (P = 0.002), CURB-65 ≥ 3 (P < 0.001) and PCT D3/D1 ≥ 1 (P < 0.001) were significant predictors of 30-day mortality. Peak CRP (P = 0.02) and PCT D3/D1 ≥ 1 (P = 0.03) were significant predictors of initial treatment failure. Using the new scoring system that defines CRP D1 ≥ 100 mg/L as 2 points, CURB-65 ≥ 3 as 1 point and PCT D3/D1 ≥ 1 as 1 point, in CAP patients with both CRP D1 ≥ 100 mg/L and CURB-65 ≥ 3 on admission, the 30-day mortality rate was 21.8%, and with PCT D3/D1 ≥ 1, it increased to 50.0%. CONCLUSION: It is useful to add serial measurements of PCT to CRP measurement and assessment of CURB-65 on admission of CAP patients to predict prognosis and initial treatment failure.

18 Cases of pulmonary Mycobacterium abscessus: Clinical difference depending on the presence or absence of Mycobacterium avium complex.

BACKGROUND AND OBJECTIVES: It can be difficult to treat respiratory tract infections caused by Mycobacterium abscessus (M. abscessus) as there is no established treatment strategy. Complications involving other nontuberculous mycobacterial infections such as Mycobacterium avium complex (MAC) are also commonly observed. METHODS: We investigated the clinical background and course of 18 cases of pulmonary M. abscessus infection treated over 8 years at Kurashiki Central Hospital. Radiological evaluation was performed using NICE scoring system, a method of semi-quantitative evaluation of imaging findings of pulmonary MAC infection. RESULTS: The mean age of the 18 patients (males, 6; females, 12) was 74.7 years. The median follow-up period was 1316 days (95% confidence interval; 720-1675 days), and 11 patients were concomitantly infected with pulmonary MAC. Among the patients that underwent antibacterial treatment for M. abscessus, there was one MAC-complication case and one non-MAC-complication case. All MAC-complication cases underwent antibacterial treatment including clarithromycin. Chest X-ray NICE scores for all cases were 8.50 ± 5.45 and 10.94 ± 6.03 at baseline and follow-up, respectively (p = 0.0063). For MAC-complication cases, scores were 8.36 ± 4.74 and 12.00 ± 6.02 at baseline and follow-up, respectively (p = 0.00818), and for non-MAC-complication cases, scores were 8.71 ± 6.82 and 9.29 ± 6.13 at baseline and follow-up, respectively (p = 0.356). MAC-complication cases were significantly further exacerbated than non-MAC-complication cases (p = 0.027). CONCLUSIONS: Some cases of pulmonary M. abscessus infection progressed well without undergoing antibacterial treatment. In particular, results suggested that the clinical course of MAC-complication and non-MAC-complication cases differs.

Comparative investigation of respiratory tract involvement in granulomatosis with polyangiitis between PR3-ANCA positive and MPO-ANCA positive cases: a retrospective cohort study.

BACKGROUND: The clinical characteristics of myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA) positive granulomatosis with polyangiitis (GPA) remain unclear, as does the difference between MPO-ANCA positive GPA and proteinase 3 (PR3)-ANCA positive GPA, especially with regard to the details of respiratory tract involvement. We investigated the differences in clinical, radiological, and histopathological features between PR3-ANCA positive GPA and MPO-ANCA positive GPA. METHODS: We retrospectively reviewed 16 patients who were newly diagnosed with GPA between December 2000 and July 2014. One patient, who was positive for both PR3-ANCA and MPO-ANCA, was excluded. Our review was based on the European Medicine Agency (EMA) algorithm. RESULTS: Fifty-six percent of GPA patients were positive for PR3-ANCA, 38 % for MPO-ANCA, and the remaining 6 % for both. The MPO-ANCA positive group included a greater number of females (67 %). There were no statistically significant differences in laboratory data, symptoms and signs, Birmingham Vasculitis Activity Score, or CT findings between the two groups. As for upper respiratory tract involvement, the most common manifestation was paranasal sinusitis, whereas lung nodules were most common as the lower respiratory tract involvement in both groups. Although the combination therapy with prednisone and cyclophosphamide was the most common initial treatment in both groups, the relapse rate in MPO-ANCA positive cases was lower than that of PR3-ANCA positive cases (17 % and 56 %, respectively). CONCLUSION: A high prevalence of MPO-ANCA positive GPA was noted. No significant differences in clinico-radiological findings were observed except for the prevalence of relapse between the PR3-ANCA positive cases and MPO-ANCA positive cases, suggesting that the type of ANCA may be of little help in the diagnosis of GPA. Examination for granulomatous findings in the respiratory tract is important, even in MPO-ANCA positive cases. There is a need to accumulate more cases and conduct a further investigation in the future.

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.

Ruptured Occipital Artery Aneurysm Following Craniotomy in a Patient with Neurofibromatosis Type 1: Case Report.

Objective: Neurofibromatosis type 1 (NF1) is associated with vascular fragility, which results in aneurysms, arteriovenous fistulas, and dissections. Here, we describe a case of endovascular treatment of a ruptured occipital artery aneurysm that occurred after a craniotomy in a patient with NF1. Case Presentation: A 46-year-old man with a history of NF1 underwent a right lateral suboccipital craniotomy to remove a cavernous hemangioma in the right middle cerebellar peduncle. Severe bleeding occurred in the occipital artery during the craniotomy. Due to vessel fragility, coagulation and ligation were not possible, and pressure hemostasis was achieved using cellulose oxide and fibrin glue. On postoperative day 12, the patient developed a sudden swelling on the right side of the neck as well as tracheal compression. Contrast-enhanced CT revealed a ruptured aneurysm in the right occipital artery. Transarterial embolization was performed under general anesthesia the same day. Right external carotid angiography showed an 18-mm-diameter fusiform aneurysm in the occipital artery. The aneurysm ruptured inferiorly to form a large pseudoaneurysm with significant jet flow. An arteriovenous fistula was also observed in a nearby vein. A microcatheter was inserted into the fusiform aneurysm under proximal blood flow control, and embolization was performed using coils and N-butyl-2-cyanoacrylate. Conclusion: Compared to surgical repair of ruptured occipital artery aneurysms, endovascular treatment appears to be safe, effective, minimally invasive, and rapid. Ruptured occipital artery aneurysms in NF1 patients can cause neck swelling and airway compression and should be recognized as a potentially lethal condition.

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.

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.