BackgroundCalcium pyrophosphate deposition (CPPD) disease is typically a disease of older age and is male-predominant.[1] It can present as various phenotypes, including acute monoarthritis, polyarthritis resembling osteoarthritis with intermittent flares, and polyarticular disease resembling rheumatoid arthritis.[2] In younger patients affected by CPPD, a metabolic workup is warranted to assess for secondary causes.CaseA 40-year-old woman with a past history of Grave’s disease presents to the emergency room with left wrist pain and swelling, alongside a five-year history of intermittent monoarthritis affecting various joints, including ankles, wrists, knees and shoulders. Each episode resolved spontaneously within 5 days, raising a suspicion of crystal-induced arthritis. Initial treatments with NSAIDs, followed by a combination of colchicine, hydroxychloroquine, and methotrexate provided minimal relief, and flare-ups were occurring twice monthly. Steroids were effective but caused significant side effects even with short-term use. Physical examination revealed a left wrist effusion and tenderness over the right sternoclavicular joint. Imaging of the left wrist demonstrated significant chondrocalcinosis with dense mineralization of the ulnocarpal space and intercarpal hyaline cartilage. Ultrasound identified a small radiocarpal joint effusion with synovial thickening, nodularity, and hyperemia that were compatible with synovitis. The imaging findings were compatible with CPPD arthropathy with active inflammation at the wrist. Initial synovial fluid analysis was negative (left wrist) but subsequently positive in the knee, confirming CPPD crystals. Laboratory evaluation revealed significant hypomagnesemia of 0.45 mmol/L (normal range 0.66-1.07 mmol/L). Despite ongoing oral magnesium supplementation, her hypomagnesemia persisted, and renal wasting was confirmed by the fractional extraction of magnesium (FeMg) in a 24-hour urine collection. Genetic testing revealed a heterozygous pathogenic variant in HNF1B, which is associated with renal magnesium wasting and polycystic kidney disease. Due to the inadequate response to the conventional disease-modifying agents and persistent recurrent monoarthritis, the patient was given on-demand anakinra, which shortened the duration of her flare-ups and reduced her need for corticosteroids. Since recurrent episodes continued, anakinra was changed to daily dosing, which essentially abolished the episodes, contributing to an improved quality of life.ConclusionThis case highlights the importance of assessing for secondary causes of CPP disease, particularly in younger individuals. Hypomagnesemia is a crucial risk factor as it plays a key role in regulating pyrophosphate crystal formation. This case illustrates how ongoing hypomagnesemia can lead to resistant CPP disease that responds poorly to traditional agents and the benefits of an IL-1 inhibitor in disease management. [1.] Pascart T. Lancet Rheumatol 2024;6(11):e791-804. [2.] Abhishek A. Curr Opin Rheumatol 2016;28(2):133-9.
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