Osteotic Lesions / Periosteal Elevations

 

 

DIRA

 

DIRA - Patients develop bony abnormalities including multi-focal osteolytic lesions, periosteal elevation, periosteal cloaking, rib widening. Other clinical features include markedly elevated inflammatory markers and cutaneous pustulosis. Despite the marked inflammatory response, patients typically do not develop recurrent fevers. The treatment of choice is with recombinant IL-1 receptor antagonist (Anakinra).