- •Including calcifications on radiographic reports is essential, as they may become symptomatic.
- •The 3 manifestations of calcium pyrophosphate dihydrate crystal deposition disease: chondrocalcinosis, acute calcium pyrophosphate crystal arthritis, and pyrophosphate arthropathy may overlap or be seen in isolation.
- •In crystal deposition diseases a cascade of reactions leading to joint inflammation can be age related, triggered by a trauma or disease, and result in sometimes rapid joint destruction.
- •Ultrasound-guided barbotage procedure is straightforward and accelerates the natural course of hydroxyapatite crystal deposition disease.
- •Dual-energy computed tomography is rapid, noninvasive, and enables diagnosis and follow-up of gout, in multiple joints on a single scan without the use of contrast agents.
Calcium pyrophosphate dihydrate crystal deposition disease
- •Chondrocalcinosis: the asymptomatic form of this crystal arthropathy is probably the most common with absence of symptoms in at least 10% to 20%1(Fig. 2).
- •Acute CPP crystal arthritis (pseudogout syndrome): CPPD was first recognized as a source of acute crystal-induced peripheral-joint inflammation. Most cases develop spontaneously, but there are situations that trigger arthritis, for instance joint trauma, medical illness, surgery, or a blood transfusion.2,8The European League Against Rheumatism has made recommendations for the diagnosis of CPPD based on rapid development of inflammatory symptoms, the location of arthritis (knee, shoulders, wrist), age of the patient (>65 years), imaging findings, and absence of another disease (eg, rheumatoid arthritis and septic arthritis).8The theory of pathogenesis of acute arthritis and synovitis in CPPD is shown in the drawing of Fig. 3A, B . The shedding of CPP crystals into the joint is key in this process. This acute onset of CPP arthritis is self-limiting.8
- •Pyrophosphate arthropathy: the clinical presentation often simulates osteoarthritis. CPPD flares may occur, and a chronic and progressive form of arthritis develops. Its presence is often bilateral and symmetric. Preferential locations include the knee, hip, metacarpophalangeal joints, elbow, ankle, wrist, and glenohumeral joints.1
Imaging Findings and Pathology
- ○Cystic degeneration and sclerosis (no erosions)
- ○Fragmentation of bone and cartilage
- ○Debris in synovial membrane
- ○Ultrasonography (US)
- US can show synovitis and can sensitively show CPP deposits in cartilage, with a typical appearance of hypoechoic (cartilage) and echoic (CPPD) layers, being part of the EULAR criteria.8
- •Computed tomography (CT)
- CT technique is able to accurately show calcifications but is rarely used additional for diagnosis or evaluation of a painful joint.1
- •MR Imaging
Calcium hydroxyapatite crystal deposition disease
Imaging Findings and Pathology
- •Type I calcifications have a sharp border and a dense structure
- •Type II calcifications either have a sharp border and inhomogeneous structure or a vague border and a homogenous structure
- •Type III calcifications have a vague border, and are more or less transparent, with a cloudy appearance.
- •CT and MR imaging are less frequently used in HADD
- ○Early findings are nonspecific soft-tissue swelling in the affected joint secondary to synovitis, capsular distension, and periarticular soft tissue edema. Fine lacy periosteal reaction may be observed secondary to urate crystals in adjacent soft tissue.
- ○Intermediate-stage features include faint calcification of affected soft tissues, intracortical erosions and irregularity, and osteochondral compression or cupping. The joint space is preserved until late in the disease.
- ○Late stage is characterized by well-defined juxta-articular erosions with sclerotic rims and overhanging margins. Progressive erosions and intraarticular deposits may result in tapering deformities of the shafts, subluxation, and mutilating arthritis. Progressive joint space narrowing with secondary degenerative changes is a late manifestation. Rarely, ankylosis may occur. Appositional bone deposition may cause apparent expansion of bone ends with a bulbous appearance.50,51
- •Snow-storm sign and tophi: hyperechoic microtophi appearing as white spots within the fluid collection generate the typical snow storm appearance. Larger, dense aggregates develop into hypoechoic to hyperechoic (mainly chronic tophi) inhomogeneous tophi, with a cloudy appearance and a hypoechoic or anechoic rim54,55(Fig. 13).
- •MR Imaging
- •Dual-energy Computed Tomography
Clinics care points
- •Aging is the main risk factor for CPPD crystal deposition disease.
- •CPP crystals almost exclusively deposit into articular fibrocartilage and hyaline cartilage, the most common cause of chondrocalcinosis.
- •Calcifications need to be part of the radiological report, as they may become symptomatic.
- •Absence of crystal deposit detection on radiographs does not exclude the diagnosis of CPPD, gout, or HADD.
- •Rapid joint destruction without visible calcifications should raise the diagnosis of CPPD.
- •Barbotage is an effective method to accelerate the natural course of HADD.
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