10 In an unknown percentage of patients, chondrocalcinosis is present without clinical arthritis. 9 Rarely, tumoral deposits of CPP crystals occur in soft tissues, where they can cause considerable tissue damage and may be mistaken for cancers. CPP crystals have also been associated with a severely destructive arthritis that is similar to neurotrophic (Char-cot’s) arthropathy. 8 This syndrome is often confused with meningitis or sepsis. 6, 7 The crowned dens syndrome is caused by the deposition of CPP crystals around the C2 vertebra and manifests as acute severe neck pain, fever, and high levels of inflammatory markers. CPP crystals are commonly seen in spinal tissues, including inter-vertebral disks and spinal ligaments. Other less common clinical presentations of CPPD disease have been described. McCarty estimated that the chronic degenerative polyarticular form of CPPD disease accounts for roughly 50% of the cases of CPPD disease, whereas acute CPP crystal arthritis represents approximately 25% of the cases. Flares in this phenotypic variant of CPPD disease often involve joints sequentially, and involvement is less symmetric than that seen with rheumatoid arthritis. Patients with this condition have persistent inflammatory arthritis that affects large and small joints. A rarer form of polyarticular CPPD disease resembles rheumatoid arthritis. The involvement of joints such as the glenohumeral joint, the wrist, and the metacarpophalangeal joints, which are not often affected by typical osteoarthritis, should lead one to suspect the presence of CPPD disease ( Fig. This osteoarthritis-like arthritis is usually distinguishable from typical osteoarthritis by flares of inflammatory signs and symptoms and by unusually severe articular damage. Most affected patients have a polyarticular form of arthritis that resembles osteoarthritis. 4Ĭhronic CPP crystal arthritis comprises several clinical phenotypes. In contrast to the brief attacks of acute gouty arthritis that typically last for several days to 1 week, acute attacks of CPPD disease may last for weeks to months. Systemic symptoms including fevers, chills, and constitutional symptoms often occur with acute CPP crystal arthritis. The knee is the most commonly involved joint, followed by the wrist acute podagra in the first metatarsophalangeal joint is rare. Along with other findings, the distribution of joint involvement may provide a helpful clue with regard to the presence of acute CPP crystal arthritis. The vigorous inflammatory response to CPP crystals manifests as warmth, erythema, and swelling in and around the affected joint, and the clinical picture is often indistinguishable from acute gouty arthritis or septic arthritis. Patients typically present with the acute onset of monoarticular or oligoarticular arthritis. We use the term “CPP deposition” (CPPD) to refer to the presence of CPP crystals and the term “CPPD disease” to include all the related clinical presentations.Īcute CPP crystal arthritis (or pseudogout) is the most widely recognized form of CPPD disease. 3 The term “chondrocalcinosis” refers to the common radiographic correlate of CPPD disease and does not imply clinical arthritis. In 2011, a group from the European League against Rheumatism recommended that calcium pyrophosphate crystals be referred to as CPP crystals, that the term “acute CPP crystal arthritis” refer to the acute inflammatory arthritis that was formerly known as pseudogout, and that the term “chronic CPP crystal arthritis” be used to denote other types of arthritis associated with CPP crystals. Various cumbersome terms such as “calcium pyrophosphate dihydrate deposition disease” achieved common use. Nomenclature issues have plagued CPPD disease since its original description. Chondrocalcinosis is seen in the substance of the cartilage the arrow indicates the direction of the probe. The probe was pointed at the femoral cartilage on the “V” of the patellar groove. Panel D shows an ultrasonographic image of a right knee, which was obtained with the transducer in the anatomical axial plane, with the knee flexed 90 degrees. Panel C shows a radiograph of a hand with hooklike osteophytes (arrows). Panel B shows a radiograph of a wrist with chondrocalcinosis of the triangular cartilage (arrow). Panel A shows a radiograph of a knee with meniscal chondrocalcinosis (arrow). Imaging of Chondrocalcinosis in Patients with CPPD Disease.
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