Other Systemic Inflammatory Diseases Flashcards
HLA which imparts fivefold increased risk of Behçet disease
HLA-B51
Cytokines elevated in adult-onset Still disease (4)
IL-1, IL-6, IL-18, and TNF-alpha
Periodicity of fever in adult-onset Still disease
Quotidian
Most commonly involved joints in adult-onset Still disease (3)
Wrists, knees, ankles
This potentially deadly complication should be suspected in patients with adult-onset Still disease and pancytopenia
Hemophagocytic syndrome
Familial autoinflammatory diseases inherited in an autosomal recessive manner (2)
Familial Mediterranean fever and hyperimmunoglobulinemia D with periodic fever syndrome
Familial autoinflammatory diseases which may be seen in all ethnicities (2)
Tumor necrosis factor receptor-associated periodic syndrome and neonatal-onset multisystem inflammatory disease
Treatment for familial Mediterranean fever
Colchicine
Most common familial autoinflammatory disease
Familial Mediterranean fever
Familal autoinflammatory disease associated with conjunctivitis, amyloidosis, and sensorineural deafness
Muckle-Wells syndrome
Familial autoinflammatory diseases treated with IL-1 receptor antagonists (3)
Familial cold autoinflammatory syndrome, Muckle-Wells syndrome, neonatal-onset multisystem inflammatory disease
Osteogenesis imperfecta is most commonly caused by mutations in the gene for this protein
Type I collagen
Most common and mildest form of osteogenesis imperfecta
Type I OI
Classic Ehlers-Danlos syndrome is associated with mutations in the gene for this protein
Type V collagen
Vascular Ehlers-Danlos syndrome is associated with mutations in the gene for this protein
Type III collagen
The skin and aorta of patients with Marfan syndrome contain abnormally low levels of this protein
Elastin
Marfan syndrome is associated with mutations in the gene for this extracellular matrix protein important in the regulation of sequestered transforming growth factor beta
Fibrillin
Leading cause of Marfan syndrome-associated mortality
Aortic root aneurysms
An acute, self-limited form of sarcoidosis characterized by hilar lymphadenopathy, erythema nodosum, and acute arthritis or tenosynovitis, usually of the ankles
Löfgren syndrome
Indurated violaceous plaques on the face and ears of patients with sarcoidosis
Lupus pernio
Typical cranial nerve involved in sarcoidosis
Facial nerve
Most common musculoskeletal manifestation of sarcoidosis
Symmetric oligoarthritis of the large joints of the lower extremities (strong predilection for the ankles)
Treatment for Löfgren syndrome
NSAIDs or corticosteroids
