• Special Issue Topic

    Calcium Pyrophosphate Deposition Disease

    Submission Deadline: April 30, 2024

    Guest Editor

    Prof. Jürgen Braun E-Mail

    Rheumazentrum Ruhrgebiet, Ruhr Universität Bochum, Herne, Germany

    Research Keywords: Rheumatoid arthritis, gout, osteoporosis

    About the Special Issue

    Calcium pyrophosphate deposition (CPPD) disease is a rather prevalent form of crystal-induced arthritis worldwide. Recently, the first classification criteria for this rheumatic musculoskeletal disease (RMD) which may present in rather heterogeneous clinical forms have been developed. These classification criteria for CPPD diseases have been validated by the European Alliance of Associations for Rheumatology (EULAR) and the American College of Rheumatology (ACR). For the classification as CPPD, the presence of a crowned dens syndrome or calcium pyrophosphate crystals in the synovial fluid in patients with joint pain, swelling or tenderness (entry criterion) whose symptoms cannot be fully explained by another rheumatic disease (exclusion criterion) is sufficient to classify an individual as a CPPD patient. In the absence of these findings, a score of more than 56 points based on weighted criteria consisting of clinical features, concomitant metabolic disorders such as and results of laboratory and imaging tests may be used to classify a person as having CPPD. CPPD is often found in the context of osteoarthritis. There is some overlap in the clinical presentations of CPPD disease and osteoarthritis, both relatively common with advanced age, diagnostic mimicry and co-occurrence by chance may occur. Some metabolic conditions such as hyperparathyroidism, hypomagnesemia, hypophosphatasia and hemochromatosis are known as risk factors for CPPD. A few patients with gout have CPP crystals in their synovial fluid which supports the hypothesis that these diseases share common local and systemic risk factors. Although most CPPD disease is sporadic, multiple kindreds with premature or extensive CPPD have been described worldwide. CPPD that occurs in patients younger than 60 years of age should lead to questions about similarly affected family members. At least two genetic loci are associated with familial CPPD.

    CPPD is underdiagnosed and there are only a few therapeutic trials. Very recently colchicine was found to be as good as prednisolone with differences in the safety profile. This special issue was planned with the intention to spread decent scientific knowledge about the disease and the very promising recent achievements in the epidemiology and methodology of CPPD.

    Keywords: CPPD, gout, classification criteria, synovial fluid analysis, crowned dens syndrome, acute arthritis, crystals, osteoarthritis

    Call for Papers

    Published Articles

    Open Access
    Calcium pyrophosphate deposition disease: points to be considered for quality assurance in clinical practice
    Calcium pyrophosphate deposition disease is known as crowned dens syndrome or peripheral arthritis, especially of knees, hips and shoulders. The disease course is asymptomatic, with acute or chronic [...] Read more.
    Michael Schirmer, Johannes Dominikus Pallua
    Published: July 18, 2024 Explor Musculoskeletal Dis. 2024;2:270–278
    DOI: https://doi.org/10.37349/emd.2024.00055
    Times Cited: 0
    Open Access
    Treatment strategies for calcium pyrophosphate deposition disease
    Calcium pyrophosphate deposition disease (CPPD) is a cause of inflammatory arthropathy that increases in prevalence with increasing age, presents in acute and chronic forms, and is characterized by  [...] Read more.
    Anna J. Turlej, Angelo L. Gaffo
    Published: July 19, 2024 Explor Musculoskeletal Dis. 2024;2:279–292
    DOI: https://doi.org/10.37349/emd.2024.00056
    Times Cited: 0
    Open Access
    Original Article
    Progression of arthritis after four-corner fusion in patients with calcium pyrophosphate deposition disease: a case series of eleven patients
    Aim: The purpose of this study is to evaluate outcomes and radiographic progression of wrist arthritis after four-corner fusion (4CF) in patients with evidence of calcium pyrophosphate deposition [...] Read more.
    John F. Hoy ... Xavier C. Simcock
    Published: July 10, 2024 Explor Musculoskeletal Dis. 2024;2:256–263
    DOI: https://doi.org/10.37349/emd.2024.00053
    Times Cited: 0
    Open Access
    Calcium pyrophosphate crystal deposition disease—what’s new?
    Jürgen Braun
    Published: December 06, 2023 Explor Musculoskeletal Dis. 2023;1:257–263
    DOI: https://doi.org/10.37349/emd.2023.00027
    Times Cited: 0