Ultrasonic Evaluation of Diaphragm in Patients with Systemic Sclerosis.

The diaphragm is the most important muscle in respiration. Nevertheless, its function is rarely evaluated. Patients with systemic sclerosis (SSc) could be at risk of diaphragmatic dysfunction because of multiple factors. These patients often develop interstitial lung disease (SSc-ILD) and earlier studies have indicated that patients with different ILDs have decreased diaphragmatic mobility on ultrasound (US). This study aimed to evaluate diaphragmatic function in SSc patients using US with regard to the ILD, evaluated with the Warrick score on high-resolution computed tomography (HRCT), and to investigate associations between ultrasonic parameters and dyspnea, lung function, and other important clinical parameters. In this cross-sectional study, we analyzed diaphragm mobility, thickness, lung function, HRCT findings, Modified Medical Research Council (mMRC) dyspnea scale, modified Rodnan skin score (mRSS), autoantibodies, and esophageal diameters on HRCT in patients with SSc. Fifty patients were enrolled in the study. Patients with SSc-ILD had lower diaphragmatic mobility in deep breathing than patients without ILD. The results demonstrated negative correlations between diaphragmatic mobility and mMRC, mRSS, anti-Scl-70 antibodies, esophageal diameters on HRCT, and a positive correlation with lung function. Patients with SSc who experience dyspnea should be evaluated for diaphragmatic dysfunction for accurate symptom phenotyping and personalized pulmonary rehabilitation treatment.

The Importance of a Multidisciplinary Approach in a Patient with Long-term Multisystemic Manifestations of Unrecognized Hereditary Hemorrhagic Telangiectasia.

Hereditary hemorrhagic telangiectasia (HHT), also called Rendu-Osler-Weber syndrome, is a rare autosomal dominant multisystemic vascular disorder, characterized by widespread mucocutaneous teleangiectasias, frequent visceral arteriovenous malformations (AVM) and a tendency for bleeding. This diagnosis should be suspected in all dermatological patients with generalized mucocutaneous vascular lesions at sites of predilection, associated frequent epistaxis and a positive family history. The aim of this paper is to emphasize the importance of a multidisciplinary approach, the role and timely cooperation of dermatologists and otorhinolaryngologists in the early clinical recognition and diagnosis of the disease. We present a family case of a 63-year - old patient with typical clinical features of HHT and long-standing multisystemic complications of unrecognized disease.

Baseline and progressive adipopenia in newly diagnosed patients with diffuse large B-cell lymphoma with unfavorable features are associated with worse clinical outcomes.

We retrospectively analyzed perirenal and subcutaneous fat thickness and their dynamics from baseline to end-of-treatment computerized-tomography scans in a cohort of 118 newly diagnosed diffuse large B-cell lymphoma (DLBCL) patients with unfavorable features treated with R-DA-EPOCH regimen. Higher revised-international-prognostic-index (R-IPI) score was significantly associated with higher baseline perirenal and lower subcutaneous fat thickness. Up to 51% patients experienced perirenal and 40% subcutaneous fat-tissue loss during immunochemotherapy period. R-DA-EPOCH feasibility, toxicity and obtained response to therapy did not significantly differ regarding baseline perirenal and subcutaneous fat measurements whereas higher number of febrile-neutropenia cycles was associated with more pronounced subcutaneous fat loss. In multivariate-analyses subcutaneous fat loss of ≥6% (hazard-ratio (HR) =4.58, p < 0.001) and achieving response to therapy (HR = 0.03, p < 0.001) predicted overall-survival, and baseline subcutaneous fat thickness ≤24 mm (HR = 3.14, p = 0.023), baseline minimal perirenal fat thickness ≤8 mm (HR = 2.44, p = 0.042) and achieving response to therapy (HR = 0.04, p < 0.001) predicted progression-free-survival independently of each other.

Psoas muscle index at the time of diagnosis might reflect the prognosis of classical Hodgkin's lymphoma patients.

We retrospectively investigated clinical and prognostic significance of psoas muscle index (PMI) calculated as total psoas muscle area at L3 vertebra level obtained from baseline computed tomography (CT) scans in 49 newly diagnosed classical Hodgkin's lymphoma (cHL) patients prior to specific treatment. Median PMI was 572.5 mm2/m2 and was significantly higher in males (P < 0.001), patients with higher body mass index (BMI, P < 0.001), absence of extranodal disease (P = 0.037), higher absolute lymphocyte count (P = 0.037), higher hemoglobin (P = 0.010) and lower lactate dehydrogenase (LDH, P = 0.050). There were no significant associations with age, disease subtype, presence of constitutional symptoms, Ann Arbor disease stage, presence of advanced disease or international prognostic score. Patients with lower PMI had significantly worse PFS (hazard ratio [HR] 4.91; P = 0.009). This phenomenon persisted in the multivariate model (HR = 5.09; P = 0.042) adjusted for International Prognostic Score (IPS) and chemotherapy type.

More Pronounced Muscle Loss During Immunochemotherapy is Associated with Worse Clinical Outcomes in Newly Diagnosed Patients with Diffuse Large B-Cell Lymphoma with Unfavorable Features.

INTRODUCTION: Cancer-induced cachexia is associated with poor prognosis in patients with non-Hodgkin lymphoma, but it is unknown how and to what extent curable lymphoma treatments affect the musculoskeletal system. PATIENTS AND METHODS: We retrospectively analyzed 104 newly diagnosed diffuse large B-cell lymphoma (DLBCL) patients with unfavorable disease features treated with the R-DA-EPOCH regimen. Psoas muscle area (PMA) measured at L3 vertebra level was compared between staging (pre-therapy) and revision (end of treatment) computerized tomography (CT) scans. RESULTS: Small but significant decline in PMA was observed during the immunochemotherapy period (average loss 5%; P=0.016) with 57.7% of patients experiencing muscle loss. Higher body surface area (OR=17.98 for each m2; P=0.034), number of cycles with dose reduction (OR=2.86 for each cycle; P=0.039) and worse response to therapy (OR=3.09 for each response category; P=0.052) were recognized as independent contributors to the PMA loss in multivariate analysis. One quarter of patients had more pronounced PMA loss (≥21%), which was associated with significantly worse overall and progression-free survival. Both ≥21% PMA loss and non-achieving response to therapy remained independently associated with inferior OS (PMA loss HR=2.98; P=0.016 and achieving response HR=0.04; P<0.001) and PFS (PMA loss HR=3.16; P=0.005 and achieving response HR=0.08; P=0.001) in multivariate analyses. DISCUSSION: Muscle loss occurs in approximately half of newly diagnosed DLBCL patients with unfavorable disease features during R-DA-EPOCH immunochemotherapy. If pronounced, this is associated with worse clinical outcomes irrespectively of achieved response to therapy. Muscle loss seems to be mostly affected by the efficacy and tolerability of the regimen.