40 Autoinflammatory Bone Disease

Question 1

Autinflamm bone dse where fever is common

Answer 1

Majeed syndrome

Question 2

Sites of osseous involvement of CRMO

Answer 2

Metaphyses of long bones > vertebrae, clavicle, sternum, pelvis

Question 3

Autinflamm bone dse: Extraosseous manif include pustulosis palmoplantaris, psoriasis, and IBD

Answer 3

CRMO

Question 4

Autinflamm bone dse: Extraosseous manif include Dyerythropoetic anemia

Answer 4

Majeed syndrome

Question 5

Autinflamm bone dse: Extraosseous manif include pyoderma gangrenosum and severe cystic acne

Answer 5

PAPA

Question 6

Autinflamm bone dse: Extraosseous manif include generalized pustulosis

Answer 6

DIRA

Question 7

Autoinflamm bone dse: Site/s of osseous involvement include anterior rib ends

Answer 7

DIRA

Question 8

Autoinflamm bone dse: Site/s of osseous involvement similar to CRMO

Answer 8

Majeed syndrome

Question 9

Autoinflamm bone dse: Site/s of osseous involvement include pauci-articular sterile arthritis

Answer 9

PAPA

Question 10

Autoinflamm bone dse: Site/s of osseous involvement include mandible > maxilla

Answer 10

Cherubism

Question 11

Autoinflamm bone dse: Site/s of osseous involvement include vertebrae, hind>forefeet

Answer 11

CMO and lupo mice

Question 12

Typical clinical presentation of CNO/CRMO

Answer 12

Local bone pain which may peak at night, with or without fever

Question 13

Most important tools for dx of CNO and exclusion of ddx

Answer 13

Imaging

Question 14

Clavicular lesions in CNO are typically located where

Answer 14

Medial clavicle

Question 15

Typical lesion of CNO/CRMO on radiograph

Answer 15

Osteolytic lesion with surrounding sclerosis

Question 16

T/F arthritis of CNO/CRMO occurs most frequently in joints distant to the osteitis

Answer 16

F, 60% adjacent to bone lesions

Question 17

Usually used as first line for CRMO

Answer 17

NSAIDs

Question 18

T/F Most patients with CNO/CRMO have diseases that wax and wane but eventually resolves after several years with NO long-term sequela

Answer 18

T

Question 19

Classic clinical triad of congenital dyserythropoietic anemia, early-onset CRMO, and neutrophilic dermatosis consistent with Sweet syndrome

Answer 19

Majeed syndrome