Treatment of calcium pyrophosphate crystal deposition (CPPD) disease
- Michael A Becker, MD
Michael A Becker, MD
- Section Editor — Crystal Diseases
- Professor Emeritus of Medicine
- University of Chicago Pritzker School of Medicine
- Lawrence M Ryan, MD
Lawrence M Ryan, MD
- Will and Cava Ross Professor of Medicine Emeritus
- Medical College of Wisconsin
Precipitation of crystals of calcium pyrophosphate dihydrate (CPP) in connective tissues may be asymptomatic or may be associated with several clinical syndromes. These disorders, including an acute and chronic inflammatory arthropathy, as well as radiographic calcification, comprise the spectrum of calcium pyrophosphate crystal deposition (CPPD) disease [1-3].
Treatment of CPPD disease is discussed here. The pathogenesis, etiology, clinical manifestations, and diagnosis of this disorder are discussed separately. (See "Pathogenesis and etiology of calcium pyrophosphate crystal deposition (CPPD) disease" and "Clinical manifestations and diagnosis of calcium pyrophosphate crystal deposition (CPPD) disease".)
The names traditionally used for the varying clinical manifestations of calcium pyrophosphate dihydrate (CPP) crystal deposition (CPPD) disease include pseudogout, chondrocalcinosis, and pyrophosphate arthropathy. A European League Against Rheumatism (EULAR) consensus panel has suggested alternative terminology and has reviewed diagnostic approaches to these conditions . The clinical syndromes, findings, and limitations of these terms, together with the nomenclature proposed by EULAR, include:
●Pseudogout – Pseudogout is the term that has been used to describe a disease with episodic acute attacks of CPP-induced synovitis, which clinically resemble acute attacks of urate gout. However, the majority of individuals with CPP crystal deposition never experience such episodes, which comprise only one form of CPPD disease. For this reason, the EULAR consensus panel prefers the term “acute CPP crystal arthritis” rather than pseudogout to describe acute attacks. We will use the term “acute pseudogout” here when referring to acute attacks as a compromise term to provide clarity for the many clinicians who are not yet familiar with the new nomenclature.
●Chondrocalcinosis – Chondrocalcinosis refers to radiographic calcification in hyaline and/or fibrocartilage. Chondrocalcinosis is commonly present in patients with CPPD disease but is neither absolutely specific for CPP crystals nor universal among affected patients. The EULAR group designates this finding as “cartilage calcification (CC).”
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- GENERAL APPROACH
- ACUTE PSEUDOGOUT ("ACUTE CPP CRYSTAL ARTHRITIS")
- Initial treatment/one or two joints
- Initial treatment/more than two joints
- - NSAIDs
- - Colchicine
- - Unable to use oral NSAIDs and colchicine
- Resistant disease
- Prophylaxis for acute pseudogout
- PSEUDO-RHEUMATOID ARTHRITIS ("CHRONIC CPP CRYSTAL INFLAMMATORY ARTHRITIS")
- OTHER CHRONIC PRESENTATIONS
- Chronic and progressive calcium pyrophosphate crystal deposition
- Joint degeneration ("OA with CPPD")
- Severe joint degeneration/Pseudo-neuropathic joint disease
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS