Guideline sources The following summarized guidelines for the evaluation and management of calcium pyrophosphate deposition disease are prepared by our editorial team based on guidelines from the American College of Rheumatology (ACR 2023), the European League Against Rheumatism (EULAR 2022; 2011), and the American College of Radiology (ACR 2017). 1 2 3 4 5 Guidelines 1. Diagnostic investigations Diagnostic imaging: As per ACR 2017 guidelines: Obtain radiography (often showing characteristic disease distribution and calcification) as initial imaging in patients with suspected calcium pyrophosphate dihydrate disease. E Obtain ultrasound or gradient-recalled echo MRI for the detection of chondrocalcinosis and to complement radiography by showing synovitis and additional osseous findings. E Screening for chronic infections: screen for HBV infection in all patients eligible for treatment with conventional synthetic, biological, or targeted synthetic DMARDs, immunosuppressants, or corticosteroids (according to dose and duration). B Show 3 more https://web.pathway.md/diseases/recMVeSNumsojJLjr 1/3 6/29/23, 3:24 AM Calcium pyrophosphate deposition disease Pathway 2. Medical management General principles: Offer both nonpharmacological and pharmacological modalities for optimal treatment of patients with CPPD, tailored according to: clinical features (isolated chondrocalcinosis, acute, chronic calcium pyrophosphate crystal inflammatory arthritis, OA with CPPD) general risk factors (age, comorbidities) the presence of a predisposing metabolic disorder. B Show 3 more Management of acute CPP crystal arthritis: advise applying ice or cool packs and having temporary rest in patients with acute calcium pyrophosphate crystal arthritis. B Show 4 more Management of chronic CPP crystal arthritis: Offer the following pharmacological options (in order of preference) in patients with chronic calcium pyrophosphate crystal inflammatory arthritis: oral NSAIDs (with gastroprotective treatment if indicated) colchicine (0.5-1.0 mg daily) low-dose corticosteroid methotrexate hydroxychloroquine. B Management of OA with CCPD: manage patients with OA and CPPD similar to patients with OA without CPPD. A 3. Preventative measures Routine immunizations: consider offering high-dose or adjuvanted influenza vaccination, rather than regular-dose influenza vaccination, in 65 years old patients with rheumatic or musculoskeletal diseases and in 18-65 years old patients with rheumatic or musculoskeletal diseases on immunosuppressive medications. C Show 17 more Prophylaxis for Pneumocystis jirovecii pneumonia: consider administering prophylaxis against P. jirovecii pneumonia in patients initiating high-dose corticosteroids, especially in combination with immunosuppressants and depending on the risk-benefit ratio. C Clinical findings Patient demographics Past medical history Age > 50 OA Musculoskeletal exam Lab findings https://web.pathway.md/diseases/recMVeSNumsojJLjr 2/3 6/29/23, 3:24 AM Calcium pyrophosphate deposition disease Pathway Arthritis ESR Hand arthritis serum CRP Knee arthritis Histological findings Polyarticular arthritis Polyarticular synovitis Synovial fluid calcium pyrophosphate crystals Wrist arthritis Imaging findings Chondrocalcinosis References 1. W Zhang, M Doherty, T Bardin et al. European League Against Rheumatism recommendations for calcium pyrophosphate deposition. Part I terminology and diagnosis. Ann Rheum Dis. 2011 Apr;70 4 563 70. Open 2. W Zhang, M Doherty, E Pascual et al. EULAR recommendations for calcium pyrophosphate deposition. Part II management. Ann Rheum Dis. 2011 Apr;70 4 571 5. Open 3. Expert Panel on Musculoskeletal Imaging:, Jon A Jacobson, Catherine C Roberts et al. ACR Appropriateness Criteria Chronic Extremity Joint Pain-Suspected Inflammatory Arthritis. J Am Coll Radiol. 2017 May;14 5S S81 S89. Open 4. George E Fragoulis, Elena Nikiphorou, Mrinalini Dey et al. 2022 EULAR recommendations for screening and prophylaxis of chronic and opportunistic infections in adults with autoimmune inflammatory rheumatic diseases. Ann Rheum Dis. 2022 Nov 3;ard-2022 223335. Open 5. Anne R Bass, Eliza Chakravarty, Elie A Akl et al. 2022 American College of Rheumatology Guideline for Vaccinations in Patients With Rheumatic and Musculoskeletal Diseases. Arthritis Rheumatol. 2023 Jan 4. Online ahead of print. Open 6. Jackson JL, O'Malley PG, Kroenke K. Evaluation of acute knee pain in primary care. Ann Intern Med. 2003 Oct 7;139 7 575 88. Open https://web.pathway.md/diseases/recMVeSNumsojJLjr 3/3