This article presents a case of fulminant interstitial lung disease (ILD) with macrophage activation syndrome (MAS) as a rare complication of adult-onset Still's disease in a 43-year-old male patient. Initial presentation showed severe ILD. Early treatment focused on high-dose corticosteroids; however, disease remission could not be achieved. After an additional inflammatory focus and underlying malignancy were excluded, the triad of pancytopenia, fever, and high ferritin levels indicated MAS; bone marrow biopsy confirmed secondary hemophagocytic histiocytosis. Treatment with tocilizumab induced fast and effective therapeutic success.
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http://dx.doi.org/10.1007/s00393-021-01063-7 | DOI Listing |
Cureus
December 2024
Department of Neurosciences, Philippine General Hospital, Manila, PHL.
The combination of severe myalgia, progressive weakness, and blood in the urine often leads a neurologist to consider myositis. Accordingly, reddish urine may be linked to urine myoglobinuria brought about by muscle destruction. Nevertheless, in a young patient with normal creatine kinase complaining of immobility, adult-onset Still's disease (AOSD) should be one of the top differentials.
View Article and Find Full Text PDFReumatologia
December 2024
Department of Internal Medicine, Trofa Saúde Hospital Privado em Gaia, Vila Nova de Gaia, Portugal.
Autoinflammatory bone disorders (ABDs) are characterized by sterile bone inflammation stemming from dysregulated innate immune responses. This review focuses on the occurrence of sterile osteomyelitis in ABDs and related diseases, notably chronic nonbacterial osteomyelitis (CNO) and its sporadic and monogenic forms, such as deficiency of the interleukin-1 (IL-1) receptor antagonist, Majeed syndrome, CNO related to mutation, and pyogenic arthritis, pyoderma gangrenosum, and acne (PAPA syndrome). Additionally, other autoinflammatory disorders (AIDs) are discussed, including classical periodic fever syndromes (e.
View Article and Find Full Text PDFRheumatology (Oxford)
January 2025
Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, University of Siena, Siena, Italy.
Objectives: To assess the lung involvement in patients with Still's disease, an inflammatory disease assessing both children and adults. To exploit possible associated factors for parenchymal lung involvement in these patients.
Methods: A multicentre observational study was arranged assessing consecutive patients with Still's disease characterized by the lung involvement among those included in the AIDA (AutoInflammatory Disease Alliance) Network Still's Disease Registry.
J Coll Physicians Surg Pak
January 2025
Department of Rheumatology and Immunology, The Second Affiliated Hospital of Anhui Medical University, Anhui, China.
Objective: To investigate the characteristics of Adult-onset Still's disease (AOSD) patients with macrophage activation syndrome (MAS) and explore the risk factors for the development of MAS.
Study Design: A case-control study. Place and Duration of the Study: Department of Rheumatology and Immunology, the Second Hospital of Anhui Medical University, Anhui, China, from January 2008 to June 2024.
Clin Case Rep
January 2025
Department of Rheumatology, Institute of Medicine Tribhuvan University Maharajgunj Kathmandu Nepal.
Hemophagocytic lymphohistiocytosis (HLH), is a fatal systemic hyperinflammatory syndrome. HLH may be due to immunosuppression, infections, cancer, or autoimmune diseases with fever and cytopenia. HLH which occurs in adult-onset Stills disease (AOSD) is called secondary HLH, also known as macrophage activation syndrome (MAS).
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