Pathogenetic concept Consequences Autoimmunity Mucosal initiation Self-protein modifications Ag presentation by the SE Ab production Citrullination of self-proteins in the lung generates neoepitopes, which are presented by the shared epitope…
American College of Rheumatology revised SLE classification criteria Systemic Lupus International Collaborating Clinics (SLICC) criteria Classification as SLE requires 4 out of 11 criteria Classification as SLE requires either 4…
Monogenic periodic fever syndromes FMF (familial Mediterranean fever) MKD (mevalonate kinase deficiency) NLRP3-AID (NLRP3-associated autoinflammatory disease) TRAPS (TNF receptor-associated periodic fever syndrome) NLRP12-AID (NLRP12-associated autoinflammatory disease) NOD2-associated diseases Blau syndrome…
Endocrine abnormalities Cushing’s syndrome, hyperthyroidism, hypogonadism hyperparathyroidism, hypercalciuria, hyperprolactinemia, Panhypopituitarism, diabetes, androgen insensitivity Hematological disorders Multiple myeloma, leukemia, lymphoma, systemic mastocytosis, hemophilia, thalassemia, monoclonal gammopathies Rheumatologic Rheumatoid arthritis, systemic lupus…
Axial SpA Peripheral SpA Back pain ≥3 months Age of onset <45 years Peripheral arthritis and/or enthesitis and/or dactylitis and either column below Sacroiliitis on imaging (Acute inflammation on MRI OR definite…
Classification Clinical features Gene/chromosome/OMIM Protein Inheritance Monogenic periodic fevers Familial Mediterranean fever (FMF) Recurrent febrile episodes with serositis and arthritis. Long-term development of AA amyloidosis in untreated patients. Good clinical…
!DOCTYPE html> © Springer Nature Switzerland AG 2020P. Efthimiou (ed.)Absolute Rheumatology Reviewhttps://doi.org/10.1007/978-3-030-23022-7_17 17. Pediatric Rheumatology for Adult Rheumatologists Natalie Rosenwasser1 and Karen Onel2 (1) University of Washington, Seattle, SA, USA (2) Hospital for Special Surgery,…
Fig. 12.1 Giant cell arteritis. The wall of this muscular artery is diffusely involved by granulomatous inflammation, including scattered multinucleated giant cells, with significant loss of the muscular media and…
Cell Pathogenic effects Fibroblasts Produce collagen and other extracellular matrix molecules Adipocytes, endothelial cells, epithelial cells Undergo transition to myofibroblasts expressing a smooth actin and producing collagen T cells Are…
