サルコイドーシス

Objective Although endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) is the standard procedure for diagnosing mediastinal and hilar lymphadenopathy, its diagnostic yield for sarcoidosis remains suboptimal due to nodal fibrosis. Endobronchial ultrasound-guided intranodal forceps biopsy (EBUS-IFB) has emerged as a promising adjunctive technique; however, concerns remain regarding the potential for crush artifacts when using mini-forceps. This study aimed to evaluate the histological specimen quality, diagnostic utility, and safety of EBUS-IFB in patients with suspected sarcoidosis in a single institution.Methods We retrospectively analyzed 11 consecutive patients with suspected sarcoidosis who underwent EBUS-IFB after EBUS-TBNA at a single center. We assessed the specimen quality, diagnostic yield, and safety. Two pathologists independently evaluated the specimens using a validated scoring system focusing on the amount of cellular material, background blood, degree of cellular degeneration and trauma, and retention of normal architecture.Results EBUS-IFB yielded significantly higher-quality specimens than EBUS-TBNA (median total score, 7.0 vs. 4.0; p = 0.01). Notably, EBUS-IFB specimens demonstrated well-preserved tissue architecture without significant crush artifacts and significantly less blood contamination (p = 0.006). The overall diagnostic yield was 81.8% (9 of 11), and definitive diagnoses of sarcoidosis were obtained by EBUS-IFB alone in six cases. No procedure-related complications were noted.Conclusions Despite concerns regarding crush artifacts, EBUS-IFB provides high-quality, structurally intact histological specimens with minimal blood contamination compared to EBUS-TB. EBUS-IFB is a safe and effective adjunctive modality that successfully compensates for the limitations of fine-needle aspiration in sampling fibrotic lymph nodes.

OBJECTIVES: Cardiac sarcoidosis (CS) is a potentially life-threatening manifestation of sarcoidosis that is frequently underdiagnosed. Identifying accessible biomarkers that could identify cardiac involvement would improve early detection. Coxsackievirus B (CVB) is proposed as a potential infectious trigger in sarcoidosis, but its relationship to CS remains poorly understood. METHODS: We conducted a retrospective observational cohort study of patients with CS and available CVB antibody testing at the University of Kansas Health System between 2011 and 2025. Seropositivity was compared with published external comparator cohort populations, including participants from the MAVERIC registry and previously reported population-based studies. Patients ≥18 years who met Heart Rhythm Society or Japanese Circulation Society criteria for probable or definite CS were included. Demographic data, imaging findings, clinical characteristics and CVB antibody titres were extracted from the electronic medical record. RESULTS: A total of 56 patients with confirmed CS were included. Overall, 51 of 56 patients (91%) demonstrated CVB antibody positivity, with 80% exhibiting moderate or high titres. In an external comparator cohort population CVB positivity was 14.5%. Advanced imaging findings were consistent with cardiac involvement, including delayed gadolinium enhancement on cardiac MRI in 78% of patients and myocardial 18-fluorodeoxyglucose uptake on positron emission tomography in 87%. Conventional screening tests demonstrated limited sensitivity with abnormalities on ECG, echocardiography and ambulatory monitoring observed in a minority of patients. CONCLUSIONS: This study demonstrates a high prevalence of CVB antibody positivity among patients with CS. These findings suggest that CVB serology may represent a low-cost screening biomarker to help identify patients who may benefit from further evaluation with advanced cardiac imaging.

BACKGROUND: Sarcoidosis is a systemic granulomatous disease of unknown etiology. The efficacy of antifibrotic drugs for progressive pulmonary fibrosis (PPF) associated with sarcoidosis has not yet been sufficiently clarified. Therefore, we retrospectively examined the effectiveness and safety of nintedanib in patients with pulmonary sarcoidosis who met PPF criteria. METHODS: Participants comprised six patients with sarcoidosis complicated by pulmonary fibrosis who were treated with nintedanib for PPF between 2020 and 2023. The forced vital capacity (FVC) change rate (mL/year) before and after nintedanib initiation was compared using the Wilcoxon signed-rank test. RESULTS: Among the six patients (5 male; median age at nintedanib initiation, 59 years), two had autoimmune pulmonary alveolar proteinosis. Oral prednisolone (2-7.5 mg daily) was simultaneously administered in five patients. The initial nintedanib doses were 200 mg (n = 3) and 300 mg daily (n = 3). The median annual declines in FVC before and after nintedanib initiation were -254.0 mL/year and -65.9 mL/year, respectively. Although this difference did not reach significance, the FVC increased in two patients and the annual FVC decline rate decreased in another patient after nintedanib initiation. Consolidation with traction bronchiectasis and honeycombing on chest computed tomography (CT) suggested improved FVC change and no improvement, respectively. Nintedanib was discontinued in one patient because of death. Diarrhea was observed in two patients. CONCLUSIONS: Our exploratory study yielded two hypotheses; a potential benefit of nintedanib on the FVC annual decline and a possible role for chest CT findings predicting nintedanib efficacy. Unexpected adverse events were not observed.

BACKGROUND: Sarcoidosis may represent a broad umbrella term for various diseases that induce non-necrotising granulomas in affected organs. A recent multinational consensus proposed distinct computed tomography (CT) phenotypes for pulmonary sarcoidosis; however, the clinical implications remain unclear. What is the prevalence of individual phenotypes in a tertiary referral cohort, and are these phenotypes associated with disease progression? MATERIALS AND METHODS: This retrospective study comprised 232 biopsy-proven pulmonary sarcoidosis patients treated between 2010 and 2020. Baseline CT scans were grouped into 1) non-fibrotic/fibrotic/unclear for fibrosis, and 2) normal/nodular/non-nodular. Disease progression was assessed over 36 months using predefined clinical, functional and therapeutic criteria. RESULTS: Most patients had non-fibrotic phenotypes (91.0%, n=211), while fibrotic (5.6%, n=13) and unclear (3.4%, n=8) patterns were less frequent. Inter-reader agreement was moderate for individual phenotypes (κ=0.55) and good for fibrotic versus non-fibrotic classification (κ=0.79). At 36 months, 69% of patients had progressed; all fibrotic cases progressed, compared with 67% of non-fibrotic. Nodular phenotypes progressed in a similar way to normal CT (64% versus 68%) and at a lower rate than non-nodular disease (88%). Fibrotic disease was associated with an increased risk of disease progression after adjustment for age, sex, body mass index and baseline lung function (hazard ratio 2.31, 95% CI 1.21-4.38; p=0.038). CONCLUSIONS: CT-based phenotype assessment of sarcoidosis seems feasible but may require refinement. Fibrotic patterns predict progression, while nodular phenotypes carry a risk similar to normal CT. Further radiological splitting may not reflect distinct disease behaviour.