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Background: Solitary fibrous tumors (SFTs) of the pleura are usually benign. We present a case of SFT of the pleura which grew rapidly after slow long-term progression. Case presentation: A 78-year-old man was referred to our hospital for left-sided back pain and shortness of breath. He was found to have a left mediastinal mass at 15 years of age. He remained asymptomatic for 60 years, and chest computed tomography (CT) during treatment of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis revealed that the mediastinal tumor was 8.0 cm in diameter. The size remained unchanged for 3 years but increased to 15.0 cm over the next 5 years. As the left main pulmonary artery was compressed by the mass, there were concerns regarding the worsening of haemodynamics and exacerbation of symptoms of respiratory distress. A sixth-rib intercostal thoracotomy with a posterolateral incision was performed to remove the large tumor. Perioperative steroid administration (methylprednisolone 125 mg/day) and positive pressure ventilation were administered to prevent re-expansion of the pulmonary oedema. The patient was discharged following an uneventful course. The tumor was pathologically diagnosed as an SFT with no malignant findings. Conclusion: SFTs require surgical intervention because of their potential for rapid growth. [ABSTRACT FROM AUTHOR]

Background: Infectious episodes contribute to morbidity and mortality in patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Renal involvement, also known as ANCA-associated glomerulonephritis (AGN), is frequently observed in AAV. Little is known about whether co-infection at initial diagnosis is associated with renal outcome and prognosis in children with AGN. Methods: Clinical and prognostic data for children admitted to our center with AAV from January 2001 to August 2023 were analyzed retrospectively. We compared the incidence of end-stage renal disease (ESRD) and mortality according to infection status at initial diagnosis. Results: A total of 33 children with AGN were included in this study, 22 had an infection at the time of AGN diagnosis. A trend toward higher levels of proteinuria in the infected group than in the non-infected group was observed (p = 0.42). Patients in the infected group had higher creatinine and lower eGFR values than those in the non-infected group (p = 0.09). A significant decrease in HGB was observed in the infected group (p < 0.05). There were no significant differences in the baseline values of ALB and complement c3 between the two groups. A similar proportion of patients in both groups required dialysis at the time of diagnosis (27.3% vs. 31.8%). Patients with infection presented with significantly greater ESR and CRP levels (p < 0.05), and the most commonly infected site was the lung. After 6 months of treatment, compared with those in the non-infected group, the median levels of creatinine and proteinuria were higher in the infected group. Besides, lower levels of eGFR and ALB were also observed in the infected group. 5 (45.5%) and 13 (59.1%) patients died or progressed to ESRD, respectively, in the non-infected group and infected group at the last follow-up. Conclusions: Infection at initial diagnosis does not affect the outcomes of children with AGN, although it could lead to a reduction in kidney function. [ABSTRACT FROM AUTHOR]

Purpose: To investigate the diversity of ocular manifestations in patients with positive perinuclear antineutrophil cytoplasmic antibody (p-ANCA). Methods: The clinical records of five patients exhibiting ocular manifestations and testing positive for serum MPO-ANCA were retrospectively reviewed. Results: Three out of five patients were female. The youngest patient was aged 26, whereas the eldest was 83 years old. 80% (n = 4) of them had purely anterior segment involvement, with the commonest manifestation being peripheral ulcerative keratitis and scleritis. Only one patient had posterior segment involvement, specifically posterior ischemic optic neuropathy. Two patients demonstrated unilateral involvement. There was only one patient diagnosed with systemic involvement, presenting as mixed nephrotic and nephritic syndrome, who required a course of intravenous methylprednisolone during the active stage. Three patients required second-line immunosuppressants throughout the course due to frequent relapse. Conclusion: Anterior segment ocular manifestations are not uncommon and can be the initial presentation of p-ANCA-associated vasculitis (AAV). Therefore, its evaluation should be considered in establishing the diagnosis of AAV, a life-threatening disease. [ABSTRACT FROM AUTHOR]

Objective: Pulmonary involvement is commonly observed in anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), presenting with manifestations such as diffuse alveolar hemorrhage, inflammatory infiltrates, pulmonary nodules, and tracheobronchial disease. We aimed to identify distinct subgroups of tracheobronchial disease patterns in patients with anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) using latent class analysis (LCA), and to evaluate their clinical characteristics and outcomes. Methods: We conducted a retrospective cohort study using electronic medical records of patients aged >18 years diagnosed with AAV and tracheobronchial disease between 1 January 2002 and 6 September 2022. Patients with follow-up <6 months were excluded. LCA was employed to identify disease subtypes based on 10 pre-defined indicators. Maximum likelihood estimation with 10 repetitions per model ensured robustness in model selection, guided by the Akaike information criterion (AIC). Patient and disease characteristics were summarized and compared across predicted classes. Statistical analyses included Kruskal–Wallis and Fisher's exact tests for continuous and categorical variables, respectively. The primary outcome was time to relapse of the tracheobronchial inflammation after starting immunosuppressive medication, analyzed using the Kaplan–Meier method and log-rank tests. Secondary outcomes included severity of pulmonary disease on pulmonary function tests, endoscopic interventions, tracheostomy, or mortality during follow-up. Results: Among 136 identified AAV patients assessed for tracheobronchial involvement, 111 (81.6%) were included after excluding 25 without tracheal or bronchial disease. Predominant findings included subglottic stenosis (91.0%), lower tracheal stenosis (16.2%), and bronchial stenosis (17.1%). LCA identified a three-class model as optimal: tracheal predominant (n = 94), tracheobronchial (n = 12), and bronchial predominant (n = 5). Tracheal predominant patients showed reduced risk of ear, eye, and lower respiratory manifestations, with milder obstruction on pulmonary function testing (PFT). Tracheobronchial-class patients were prone to saddle nose deformity (50%), extensive lower respiratory involvement (91.7%), and renal disease (66.7%). Bronchial predominant patients exhibited severe obstructive disease (median forced expiratory volume in 1 s (FEV1)% predicted: 58, IQR 34–66; FEV1/forced vital capacity (FVC) ratio: 56.9, interquartile range (IQR) 43–63.3) but lacked systemic AAV manifestations. LCA classes did not predict outcomes such as endoscopic intervention, tracheostomy, recurrent tracheobronchial narrowing, or mortality. Conclusion: LCA shows promise in subtype stratification of AAV patients, yet its utility in predicting outcomes and guiding treatment remains limited based on our analysis. Future studies with enhanced phenotypic data and larger cohorts are warranted to improve predictive accuracy. [ABSTRACT FROM AUTHOR]

The article discusses autoimmune hepatitis (AIH), a condition characterized by immune-mediated inflammation affecting patients of all ages. Diagnosis of AIH involves a combination of clinical, laboratory, and histologic criteria. Treatment typically involves corticosteroids as the first-line therapy, with options for steroid-sparing agents like azathioprine or mycophenolate mofetil. In refractory cases, calcineurin inhibitors or other targeted therapies may be considered. Long-term management includes monitoring for relapse and potential withdrawal of immunosuppression in patients with complete biochemical response. Future directions in AIH treatment involve novel therapies targeting inflammatory pathways. [Extracted from the article]

Several studies have examined the utility of quadriceps femoris muscle biopsies for diagnosing antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). We aimed to investigate the effects of glucocorticoid therapy on the muscle biopsy findings of patients with AAV. Data from patients with AAV who underwent a biopsy of the bilateral vastus lateralis of the quadriceps femoris at Tokyo Metropolitan Tama Medical Center between January 2015 and December 2023 were retrospectively analyzed. In total, 126 patients with suspected small vessel vasculitis (SVV) or medium vessel vasculitis (MVV) underwent a muscle biopsy at the study center. Of these, the present study included 71 patients with a final diagnosis of AAV who received a muscle biopsy of the bilateral vastus lateralis at disease onset and began glucocorticoid therapy at the study center. Of the 71 patients, 41 (57.7%) had a positive muscle biopsy finding as defined by the presence of inflammatory infiltrates with fibrinoid necrosis in the vessels. Fifty-six and 15 patients underwent a biopsy before and after starting glucocorticoid therapy, respectively. Of the former, 33 (58.9%) had a positive finding compared to eight (53.3%) of the latter (Pearson's chi-square test: p = 0.697). Among the patients with a positive biopsy result, 22 (53.7%) and 19 (46.3%) had a unilaterally and bilaterally positive finding, respectively. Glucocorticoid therapy did not significantly affect the findings of quadriceps femoris muscle biopsies. Unilateral biopsies may have a lower diagnostic sensitivity for AAV than bilateral biopsies. Key Points • Glucocorticoid therapy does not significantly impact the results of quadriceps femoris muscle biopsies in patients with antineutrophil cytoplas¬mic antibody (ANCA)-associated vasculitis (AAV). • Unilateral biopsies may have a lower diagnostic sensitivity for AAV than bilateral biopsies. [ABSTRACT FROM AUTHOR]

There are very few reports of extranodal nasal-type NKTL with septal perforation, as seen in our patient. In addition, extranodal nasal-type NKTL is often diagnosed as chronic inflammation on histopathological examination. Therefore, if nasal septal perforation does not improve with appropriate conservative treatment, clinicians should consider a malignant disease such as extranodal nasal-type NKTL. [ABSTRACT FROM AUTHOR]

A 31-year-old man presented with painful nodules on the sole of his right foot, diagnosed as cutaneous polyarteritis nodosa (CPAN). CPAN is a rare form of vasculitis affecting small and medium-sized vessels in the skin. The patient was treated with clobetasol propionate ointment and oral prednisolone, with the rash recurring intermittently. This case highlights the unique presentation of CPAN and the need for appropriate investigation and therapy. [Extracted from the article]