Calcium pyrophosphate deposition disease M11.29

Deposition disease with mono- or oligoarticular arthritis and synovitis (especially of the knee joint) due to the deposition of calcium pyrophosphate dihydrate (CPPD) crystals in joints(crystal arthropathy). In addition to acute events, recurrent or chronic arthritis with arthrosis can occur. Asymptomatic courses with incidental detection of CPP crystals in X-rays have also been described.

CPPD includes, for example, crowned dens syndrome (CPPD deposits around the atlantodental joint) and Milwaukee shoulder syndrome.

Risk factors include advanced age, previous joint injuries and, rarely, metabolic diseases such as hyperparathyroidism, haemochromatosis, hypomagnesia and hypophosphatemia. There are also genetic variants (ANKH, osteoprotein gene, TNFRSF11B).

Patients typically suffer from acute onset mono- or oligoarticular arthritis. The severe inflammatory reaction to CPP crystals manifests as warmth, redness and swelling in and around the affected joint, and the clinical picture is often indistinguishable from acute gouty arthritis or septic arthritis. Among other findings, the distribution of joint involvement can provide a helpful clue to the presence of acute CPP crystal arthritis. The knee is most commonly affected, followed by the wrist; acute podagra in the first metatarsophalangeal joint is rare. Acute CPP crystal arthritis often presents with systemic symptoms such as fever, chills and constitutional symptoms. In contrast to the short attacks of acute gouty arthritis, which usually last several days to a week, acute attacks of CPPD disease can last weeks to months.

Polarization-optical detection of CPP crystals in the synovial fluid, conventional radiography, sonography, CT, especially of the axial joints (dens syndrome).

No treatment to dissolve CPP crystals. Treatment of inflammation with non-steroidal anti-inflammatory drugs, prednisone in acute attacks, low-dose colchicine.

In recurrent relapses or chronic arthritis, low-dose methrotrexate, hydroxychloroquine for prophylaxis and reduction of relapses. IL-1 and IL-6 inhibiting biologics in cases of resistance to therapy.

Animal studies have shown anti-inflammatory effects through inhibition of the mitochondrial enzyme monoamine oxidase B: monoamine oxidase inhibitors - MAO inhibitors, rasagiline and safinamide - were used (Venegas FC et al. 2024). Preventively and therapeutically, the release of interleukin -6 and IL1-β, as well as chemokines (CXCL10, CXCL1, CCL2 and CCL5) was attenuated.

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6. https://pmc.ncbi.nlm.nih.gov/articles/PMC10529191/
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