Diagnosis and Clinical Manifestations of Calcium Pyrophosphate and Basic Calcium Phosphate Crystal Deposition Diseases

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Key points

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    Calcium pyrophosphate (CPP) and basic calcium phosphate (BCP) crystals are the 2 main families of calcium-containing crystals that can form simultaneously in all joint structures, ligament, tendon, muscle, and soft tissue.

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    BCP and CPP crystal deposition are 2 common multifaceted diseases that can mimic alarming clinical manifestations.

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    Ultrasonography seems to be an excellent imaging technique for CPP crystal detection but lacks efficacy for deep locations.

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    Computed tomography remains the gold

Calcification formation

Mechanisms of ectopic calcifications remain unresolved and have been reviewed recently.3, 4, 5, 6, 7, 8 Multiple factors contribute to this cellular driven process, including genetics, aging, imbalance between inhibitors and stimulators of calcification, alteration of calcium, phosphate and pyrophosphate metabolisms, extracellular matrix lesions, cellular differentiation state, and cell death.

Two types of CPP crystals have been identified in synovial fluid, hyaline cartilage, and

Clinical manifestations related to BCP and CPPD

CPPD and BCP crystal deposition are often asymptomatic and identified as an incidental radiographic finding. However, both calcium crystals may be associated with several manifestations.25 Intra-articular CPP crystals may cause acute and relapsing CPP crystal arthritis attacks, chronic CPP crystal inflammatory arthritis, and OA with CPP crystal.1 Extra-articular CPP and BCP crystals can cause tenosynovitis, peripheral nerve and spinal cord compression, and pseudotumoral deposition.25 Both CPPD

Diagnosis

Diagnosis of BCP and CPP crystal–related diseases relies on clinical history, radiography, and synovial fluid analysis whenever possible. Although some radiographic and US characteristics (location of calcification, radiography patterns) may differentiate BCP crystal deposition from CPPD, only microscopic examination of synovial fluid permits correct identification of CPP crystals. Detection of BCP crystals by light microscopy is unusual, because these crystals are too small. Alizarin red

Summary

BCP and CPPD are 2 common multifaceted diseases that can mimic alarming clinical manifestations. Awareness of less common presentations is helpful and prevents misdiagnosis. Diagnosis is based on direct and indirect identification of calcium-containing crystals and deposits. US seems to be an excellent imaging technique for CPP crystal detection but lacks efficacy for deep locations. CT remains the gold standard imaging modality for detection of calcification in the axial skeleton, especially

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    This work is supported by ANR grant 2013-2017 (CAPYROSIS), “Association Rhumatisme et Travail,” “Association ARPS.”

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