Camptodactyly-Arthropathy-Coxa Vara-Pericarditis Syndrome with Shoulder Joint Involvement: A Case Report with Literature Review

Introduction

Camptodactyly-Arthropathy-Coxa vara-Pericarditis (CACP) syndrome is a genetic disorder characterized by congenital or early onset camptodactyly, which is defined as flexion deformity of proximal interphalangeal joints, non-inflammatory arthopathy with synovial hyperplasia, progressive coxa vara deformity and/ or pericardial or pleural effusion (Choi BR et al. Korean Med Sci 2004; 19: 907–910; Kakkar RM et al. J Clin Imaging Sci 2013; 3: 24). This syndrome share a common age of presentation with Juvenile idiopathic arthritis (JIA), which considered as the main differential diagnosis and the umbrella-term for all arthritis lasting longer than 6 weeks in children under the age of 16 years (Offiah AC et al. Am J Roentgenol 2005; 185: 522–529).

Radiological signs are of paramount importance to make correct diagnosis, and bilateral involvement is an important feature. Appropriate diagnosis will prevent patient exposure to the side effect from the medications used to treat JIA, as well as, making other associated clinical features understandable and expectable by the clinician and the patient.

Symmetrical arthropathy has been described to wrists, knees, ankles, elbows and hips, however, shoulder joint involvement has been never radiologically described before.