Dynamic changes in the blood-based biomarkers could be used as a prognostic biomarker in patients treated with immune checkpoint inhibitors (ICIs), although the data are limited. We evaluated the association between the neutrophil–lymphocyte ratio (NLR) and early NLR changes with survival in ICI-treated patients. We retrospectively evaluated the data of 231 patients with advanced-stage cancer. We recorded baseline clinical characteristics, baseline NLR and fourth-week NLR changes, and survival data. A compound prognostic score, the NLR2-CEL score, was developed with the following parameters: baseline NLR (<5 vs. ≥5), ECOG status (0 vs. ≥1), Charlson Comorbidity Index (CCI, <9 vs. ≥9), LDH (N vs. ≥ULN), and fourth-week NLR change (10% or over NLR increase). In the multivariable analyses, higher NLR (HR: 1.743, p = 0.002), 10% or over NLR increase in the fourth week of treatment (HR: 1.807, p = 0.001), higher ECOG performance score (HR: 1.552, p = 0.006), higher LDH levels (HR: 1.454, p = 0.017), and higher CCI (HR: 1.400, p = 0.041) were associated with decreased OS. Compared to patients with the lowest scores, patients in the highest score group had significantly lower OS (HR: 7.967, 95% CI: 3.531–17.979, p < 0.001) and PFS. The composite score had moderate success for survival prediction, with an AUC of 0.702 (95% CI: 0.626–0.779, p < 0.001). We observed significantly lower survival in patients with higher baseline NLR values and increased NLR values under treatment.

Objectives: Cardiopulmonary involvement is the leading cause of mortality in SSc, and early detection of interstitial lung disease (ILD) is critical to improving outcomes. Conventional spirometry has limitations, whereas impulse oscillometry (IOS) could provide a non-invasive, effort-independent method to detect and monitor pulmonary involvement in these patients. We aimed to evaluate IOS parameters in SSc-ILD, comparing their performance with conventional spirometry. Methods: This study assessed the detection of the presence and severity of ILD in SSc patients using conventional spirometry, diffusing lung capacity for carbon monoxide (DLCO), IOS, and high-resolution CT (HRCT). Results: Among 66 SSc patients, ILD was identified in 62.1%, with 26 patients having extensive and 15 limited ILD on HRCT. IOS demonstrated higher mean resonant frequency (Fres) in ILD-positive patients than in ILD-negative patients (22.95 ± 5.47 vs 15.01 ± 6.01, P = 0.001). Fres was also significantly higher in extensive ILD than in limited ILD and ILD-negative groups (P = 0.001 and P = 0.011, respectively). ROC analysis demonstrated that Fres achieved comparatively better diagnostic accuracy for detecting extensive ILD [area under the curve (AUC): 0.841, cut-off: 22.35, sensitivity: 73.1%, specificity: 85%], outperforming forced vital capacity (FVC) (% predicted) and DLCO (% predicted). For limited ILD, Fres showed moderate diagnostic performance (AUC: 0.752, cut-off: 17.42, sensitivity: 80%, specificity: 64%). Conclusion: Our findings indicate that impulse oscillometry, particularly Fres, may provide a non-invasive and effort-independent approach to assessing interstitial lung disease in SSc. Fres appeared to be related to radiological ILD extent and showed some diagnostic value, including in cases less evident by conventional spirometry.

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Summary: Background: Systemic lupus erythematosus is a multisystem autoimmune disease primarily manifesting as lupus nephritis. While lupus nephritis is the most common renal pathology in lupus, non-lupus nephropathies such as IgA nephropathy occasionally occur. This study aims to evaluate the clinical features and outcomes of lupus patients with IgA nephropathy, comparing them with those of primary IgA nephropathy and lupus nephritis. Methods: A comprehensive literature review was conducted using the PubMed and Google Scholar databases to identify cases of systemic lupus erythematosus with IgA nephropathy reported between 1995 and December 2023. A total of 16 cases were identified and 2 additional cases from our clinic were included. These cases were compared with 47 lupus nephritis patients from our clinic and 215 primary IgA nephropathy patients from the literature. Data were collected on demographics, serology, renal biopsy findings, treatment, progression to renal failure and mortality. Results: We identified 18 cases of lupus with IgA nephropathy with a median age of 41.6 years and a female predominance (72.2%). In comparison to the primary IgA nephropathy cohort, lupus with IgA nephropathy group exhibited a lower rate of renal failure (11.1% vs. 34%) and mortality (5.6% vs. 20%). Additionally, the lupus-IgA nephropathy group showed a slightly lower mortality rate compared to the lupus nephritis cohort (5.6% vs. 10.6%). Conclusion: Lupus with predominantly IgA deposits often follows a more indolent course than primary IgA nephropathy but severe cases with crescentic glomerulonephritis can still progress to renal failure.

Background: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory disorder that may arise secondary to rheumatic diseases such as Still’s disease, where macrophage activation syndrome (MAS) represents its clinical counterpart. The pathogenic significance of the PRF1 A91V variant remains uncertain, although functional data suggest partial perforin dysfunction and a possible contribution to late-onset or atypical HLH. This study aimed to clarify the clinical implications of the PRF1 A91V variant through a systematic review of published HLH and MAS cases and a comparative analysis with a single-center Still’s disease cohort. Results: A total of 20 studies, including 38 individual HLH or MAS cases carrying the PRF1 A91V variant, were identified. The median age at diagnosis was 22 years, and 18.4% of patients were homozygous. Fever (82.6%), splenomegaly (57.9%), and hepatomegaly (36.8%) were the most frequent clinical findings. Anemia (57.1%) and thrombocytopenia (85.7%) were the predominant laboratory abnormalities, accompanied by marked hyperferritinemia (median 9319 ng/mL). Compared with 43 active Still’s disease cases, PRF1-mutated HLH patients showed significantly higher rates of cytopenias, hepatomegaly, and central nervous system involvement, together with substantially elevated ferritin levels (9,193 vs 800 ng/mL, p = 0.0023), whereas C-reactive protein levels were comparable. Receiver-operating characteristic analysis identified a ferritin cutoff of 7000 ng/mL (sensitivity 63.2%, specificity 84.6%) as the optimal discriminator for PRF1 A91V positivity. In multivariate regression, ferritin ≥ 7,000 ng/mL remained the only independent predictor (OR 17.3, 95% CI 2.0–146.3, p = 0.009). Conclusions: Patients carrying the PRF1 A91V variant represent a distinct subgroup within the spectrum of hyperinflammatory syndromes. Extreme hyperferritinemia combined with cytopenias should raise suspicion for perforin-related HLH rather than cytokine-driven MAS or classic Still’s disease. Recognition of this variant as a risk-modifying allele may guide early genetic testing and therapeutic decisions, including consideration of advanced interventions in selected cases.