Emily M Rabjohns1, Katlyn Hurst2, Arin Ghosh2, Maria C Cuellar3, Rishi R Rampersad1, Teresa K Tarrant4. 1. Division of Rheumatology and Immunology, Department of Medicine, Duke University, DUMC #3874, 200 Trent Dr, Durham, NC, 27710, USA. 2. Duke University, Durham, NC, USA. 3. Division of Rheumatology, Department of Medicine, Hospital del Salvador, Santiago, Chile. 4. Division of Rheumatology and Immunology, Department of Medicine, Duke University, DUMC #3874, 200 Trent Dr, Durham, NC, 27710, USA. teresa.tarrant@duke.edu.
Abstract
PURPOSE OF REVIEW: The purpose of this review is to recognize clinical features of Paget's disease of bone and to describe how the osteoclast, a myeloid-derived cell responsible for bone resorption, contributes to the disease. RECENT FINDINGS: Recent studies have identified several variants in SQSTM1, OPTN, and other genes that may predispose individuals to Paget's disease of bone; studies of these genes and their protein products have elucidated new roles for these proteins in bone physiology. Understanding the pathologic mechanisms in the Pagetic osteoclast may lead to the identification of future treatment targets for other inflammatory and autoimmune diseases characterized by abnormal bone erosion and/or osteoclast activation.
PURPOSE OF REVIEW: The purpose of this review is to recognize clinical features of Paget's disease of bone and to describe how the osteoclast, a myeloid-derived cell responsible for bone resorption, contributes to the disease. RECENT FINDINGS: Recent studies have identified several variants in SQSTM1, OPTN, and other genes that may predispose individuals to Paget's disease of bone; studies of these genes and their protein products have elucidated new roles for these proteins in bone physiology. Understanding the pathologic mechanisms in the Pagetic osteoclast may lead to the identification of future treatment targets for other inflammatory and autoimmune diseases characterized by abnormal bone erosion and/or osteoclast activation.
Entities:
Keywords:
Bisphosphonate; Chemokine receptor; Chemokines; Optineurin; Osteoclast; Osteoimmunology; Paget’s disease of bone
Authors: Anna Daroszewska; Lorraine Rose; Nadine Sarsam; Gemma Charlesworth; Amanda Prior; Kenneth Rose; Stuart H Ralston; Robert J van 't Hof Journal: Dis Model Mech Date: 2018-08-23 Impact factor: 5.758