A hereditary type of idiopathic articular chondrocalcinosis: Survey of a pedigree

Abstract

Idiopathic articular Chondrocalcinosis was found in 22 of 108 adults belonging to two generations of one pedigree. An equal number of males and females were affected. In only 14 of the affected persons did acute joint attacks occur; six of these patients have not sought medical help to date. Degenerative arthropathy with conspicuous preservation of joint space, involving the spine and the larger peripheral joints, was encountered in affected members older than 60 years of age. Osteoarthritic joint complaints did not have any relationship to the occurrence of previous acute joint symptoms. Periarticular calcifications, especially of the hip and knee joints, and paravertebral calcifications were predominant features in affected members who had reached senescence. Amorphous deposits only were identified in articular material, except in one synovial fluid sample that contained an unidentified crystalline deposit. In this benign type of articular Chondrocalcinosis, a single autosomal dominant hereditary factor seems to be involved, because the disease occurred only in children of affected members, including sons of affected fathers.