A 79-year-old Armenian-born woman with stable, long-term familial Mediterranean fever had progression of chronic renal failure concurrently with two types of skin lesions. One lesion resembled erysipelas, which is quite common in familial Mediterranean fever, whereas the other was panniculitis, only occasionally described in familial Mediterranean fever. The unique histopathologic features of the latter are presented. The onset of acute cutaneous disease in this patient coincided with worsening renal disease and preceded a flare of disease activity in other sites after a 14-year period of quiescence. The severe and unremitting pain from the skin lesions and their tendency to form nonhealing ulcers were substantial causes of morbidity in this patient with familial Mediterranean fever.
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http://dx.doi.org/10.1016/0002-9343(87)90023-4 | DOI Listing |
Objective: The aim of this study was to evaluate and validate the accuracy and performance characteristics of administrative codes in diagnosing autoinflammatory syndromes (AISs).
Methods: We identified potential AIS patients from the electronic medical records at the University of Iowa Hospital and Clinics and the Stead Family Children's Hospital using a screening filter based on the 10th edition of the International Classification of Diseases (ICD-10) codes and interleukin-1 antagonists. Diagnostic criteria for adult-onset Still disease, systemic juvenile idiopathic arthritis, Behçet disease (BD), familial Mediterranean fever (FMF), cryopyrin-associated periodic syndrome (CAPS), and SAPHO (synovitis, acne, pustulosis, hyperostosis, and osteitis) syndrome and chronic nonbacterial osteomyelitis (SAPHO-CNO) were reviewed for each patient.
Stem Cells
January 2025
Department of Physiology, Anatomy and Genetics, University of Oxford, Oxford OX13QX, UK.
The large majority of Alzheimer's disease (AD) cases are sporadic with unknown genetic causes. In contrast, only a small percentage of AD cases are familial, with known genetic causes. Paradoxically, there are only few validated mouse models of sporadic AD but many of familial AD.
View Article and Find Full Text PDFNeuropediatrics
January 2025
Department of Pediatric Neurology, Izmir Katip Celebi University, Tepecik Training and Research Hospital, Izmir, Turkey.
Purpose: Familial Mediterranean fever (FMF) is an autosomal recessive disorder characterized by recurrent episodes of fever and serositis, caused by mutations in the gene. Inflammatory pathways associated with FMF are linked to increased proinflammatory cytokines, which may be related to primary headaches, including migraine. The aim of this study was to evaluate the frequency of migraine and other primary headaches in FMF patients.
View Article and Find Full Text PDFClin Rheumatol
January 2025
Division of Pediatric Rheumatology and Nephrology, Department of Pediatrics, Ankara University Faculty of Medicine, Ankara, Turkey.
Objective: Chronic non-bacterial osteomyelitis (CNO) is a rare autoinflammatory bone disease associated with other chronic inflammatory diseases such as familial Mediterranean fever (FMF), juvenile idiopathic arthritis (JIA), spondylarthropathies, inflammatory bowel disease (IBD), and pyoderma gangrenosum. We aimed to describe the clinical and follow-up characteristics of patients with CNO and to compare findings between patients with and without comorbidities.
Methods: The clinical records of patients with CNO who were followed up in our pediatric rheumatology clinic between 2018 and 2023 were reviewed.
Cureus
December 2024
Department of Pediatric Rheumatology, Kocaeli University, Kocaeli, TUR.
Background Health literacy (HL) refers to the ability of individuals to find, understand, and use information and resources to make informed health-related decisions and actions for themselves and others. Managing chronic diseases in children and adolescents requires active family involvement. The primary objective of the study is to evaluate the HL levels of parents of children diagnosed with familial Mediterranean fever (FMF).
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