No hereditary multicentric osteolysis with nephropathy: report of a case and review of the literature

Abstract

Idiopathic Multicentric Osteolysis (IMO) is a rare disease characterized by osteolysis of carpus and tarsus, with inflammation and pain with onset in childhood and arrest y the second or third decade. It is classified by Hardegger in 5 Types, 1: Hereditary IMO with dominant transmission. 2: Hereditary IMO with recessive transmission. 3: Non Hereditary IMO, associated with nephropathy and hypertension. 4: Gorham´s Syndrome: massive osteolysis and replacement of bone by lymphatic or blood vessel tissue. 5: Winchester Syndrome: IMO with short stature, contractures, thick skin, corneal opacities and osteoporosis. A 50 year old male with a history of pain, swelling and deformity in both wrists and tarsi evolving from age 4, attends the consultation. He has functional involvement of hands and feet. At age 34 he had referred the presence of proteinuria, edema and hypertension, and developed chronic renal falilure (CRF). Dialysis and renal transplantation were indicated. No family history of IMO. Findings: Reduced function of boths hands, limited range of wrist and finger deformity, muscular forearms and legs dystrophy. He walked with difficulty. It is observed on radiographs, absence of carpal and tarsal bones, phalanges commitment. Physiotherapy rehabilitation and treatment with bisphosphonates and vitamin D was indicated. A patient with IMO associated with CRF and hypertension, was presented. He has absence of family history. It is considered Type III IMO. The IMO is a rare diseases, early diagnosis prevents unnecessary treatment and can treat kidney disease and hypertension at an earlier stage.