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2026-01-29
Alojzija Hočevar, Anja Srpčič, Katja Lakota, Miša Fister
Dear editor, The interleukin-6 receptor inhibitor tocilizumab has been successfully used to treat various autoimmune diseases and has an acceptable, well-established safety profile...
2025-12-22
Filippo Gozzi, Chiara Nani, Caterina Vacchi, Luca Caffarri, Dario Andrisani, Marco Sebastiani, Cecilia Burattini
Anti-melanoma differentiation-associated protein 5 (MDA5) antibody-positive syndrome is a severe condition classified among the idiopathic inflammatory myopathies associated with rapidly progressive interstitial lung disease (RP-ILD). The present case report describes a complex patient with RP-ILD in the context of anti-MDA5-positive dermatomyositis (DM). The patient showed severe lung and skin manifestations involving hands, arms, and face, and the disease was first complicated by acute renal failure and then by macrophage activation syndrome (MAS). Finally, infectious risk delayed an effective treatment, probably increasing the rapid progression of lung involvement. A pathogenetic link between DM and MAS has been recently suggested; anti-MDA5 positive patients present higher serum soluble CD163 levels, which is a scavenger receptor for the hemoglobin/haptoglobin complex expressed on macrophages and a marker of macrophage activation in various conditions, including MAS. The patient has been treated according to the American College of Rheumatology/American College of Chest Physicians guidelines, adapting the strategy to his infectious complications. Beyond the complex clinical picture and treatment strategy, this case highlights the challenging decision-making process involved in the management of acute respiratory failure in patients with acute exacerbation of interstitial lung disease. The patient underwent helmet non-invasive ventilation (NIV), with a favorable clinical response. Helmet NIV has demonstrated advantages over mask interfaces, including better patient comfort, reduced air leaks, and more consistent delivery of positive end-expiratory pressure. These features contribute to lower inspiratory effort and improved clinical outcomes, including reduced intubation and mortality rates. In conclusion, patients with anti-MDA5 antibody disease need a multidisciplinary approach, including expert rheumatologists, pulmonologists, and radiologists for both monitoring and disease management.
2025-11-19
Ernesto Damiani, Piero Marson, Francesco M. Galassi
Objective . To comprehensively trace the historical journey of adrenal gland research from the first anatomical descriptions in the 16 th century to the development and clinical application of cortisone in the mid-20 th century. Methods . This review examines key phases in adrenal gland research, including anatomical discoveries, microscopic studies, experimental physiology, biochemical advancements, and clinical applications. We analyzed primary historical sources, scientific papers, and medical records to construct a chronological narrative of adrenal gland understanding and cortisone development. Results . The review highlights significant milestones, beginning with Bartolomeo Eustachio’s 1564 discovery of the adrenal glands. It details the crucial microscopic phase, initiated by Moritz Nagel’s 1836 study, which revealed the cortex-medulla distinction. Julius Arnold’s 1866 description of the three-zone cortical structure and the identification of chromaffin cells are also discussed. The experimental physiology phase, featuring groundbreaking work by Thomas Addison and Charles-Édouard Brown-Séquard, established the vital role of the adrenal cortex. The biochemical phase, marked by the isolation and synthesis of cortisone, is examined in depth, with particular focus on the contributions of Edward Calvin Kendall, Tadeus Reichstein, and others. Finally, the clinical phase is detailed, emphasizing Philip Showalter Hench’s revolutionary application of cortisone in rheumatoid arthritis treatment in 1948. Conclusions . This historical journey demonstrates how advancements in anatomy, histology, physiology, and biochemistry synergistically contributed to our understanding of the adrenal glands. The development of cortisone, culminating with this collective knowledge, revolutionized the treatment of inflammatory diseases, marking a significant milestone in medical history and setting the stage for modern endocrinology and rheumatology.