Manifestations of Systemic Diseases
Fig. 18.1 Avascular necrosis of the left hip in a patient with sickle cell disease (Reprinted with permission from Springer [2]) Acromegaly is characterized by hypersecretion of growth hormone (GH) and…
Fig. 18.1 Avascular necrosis of the left hip in a patient with sickle cell disease (Reprinted with permission from Springer [2]) Acromegaly is characterized by hypersecretion of growth hormone (GH) and…
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…