Other Systemic Inflammatory Diseases

Question 1

HLA which imparts fivefold increased risk of Behçet disease

Answer 1

HLA-B51

Question 2

Cytokines elevated in adult-onset Still disease (4)

Answer 2

IL-1, IL-6, IL-18, and TNF-alpha

Question 3

Periodicity of fever in adult-onset Still disease

Answer 3

Quotidian

Question 4

Most commonly involved joints in adult-onset Still disease (3)

Answer 4

Wrists, knees, ankles

Question 5

This potentially deadly complication should be suspected in patients with adult-onset Still disease and pancytopenia

Answer 5

Hemophagocytic syndrome

Question 6

Familial autoinflammatory diseases inherited in an autosomal recessive manner (2)

Answer 6

Familial Mediterranean fever and hyperimmunoglobulinemia D with periodic fever syndrome

Question 7

Familial autoinflammatory diseases which may be seen in all ethnicities (2)

Answer 7

Tumor necrosis factor receptor-associated periodic syndrome and neonatal-onset multisystem inflammatory disease

Question 8

Treatment for familial Mediterranean fever

Answer 8

Colchicine

Question 9

Most common familial autoinflammatory disease

Answer 9

Familial Mediterranean fever

Question 10

Familal autoinflammatory disease associated with conjunctivitis, amyloidosis, and sensorineural deafness

Answer 10

Muckle-Wells syndrome

Question 11

Familial autoinflammatory diseases treated with IL-1 receptor antagonists (3)

Answer 11

Familial cold autoinflammatory syndrome, Muckle-Wells syndrome, neonatal-onset multisystem inflammatory disease

Question 12

Osteogenesis imperfecta is most commonly caused by mutations in the gene for this protein

Answer 12

Type I collagen

Question 13

Most common and mildest form of osteogenesis imperfecta

Answer 13

Type I OI

Question 14

Classic Ehlers-Danlos syndrome is associated with mutations in the gene for this protein

Answer 14

Type V collagen

Question 15

Vascular Ehlers-Danlos syndrome is associated with mutations in the gene for this protein

Answer 15

Type III collagen

Question 16

The skin and aorta of patients with Marfan syndrome contain abnormally low levels of this protein

Answer 16

Elastin

Question 17

Marfan syndrome is associated with mutations in the gene for this extracellular matrix protein important in the regulation of sequestered transforming growth factor beta

Answer 17

Fibrillin

Question 18

Leading cause of Marfan syndrome-associated mortality

Answer 18

Aortic root aneurysms

Question 19

An acute, self-limited form of sarcoidosis characterized by hilar lymphadenopathy, erythema nodosum, and acute arthritis or tenosynovitis, usually of the ankles

Answer 19

Löfgren syndrome

Question 20

Indurated violaceous plaques on the face and ears of patients with sarcoidosis

Answer 20

Lupus pernio

Question 21

Typical cranial nerve involved in sarcoidosis

Answer 21

Facial nerve

Question 22

Most common musculoskeletal manifestation of sarcoidosis

Answer 22

Symmetric oligoarthritis of the large joints of the lower extremities (strong predilection for the ankles)

Question 23

Treatment for Löfgren syndrome

Answer 23

NSAIDs or corticosteroids